Cardiology

Anatomy
 Right ventricle lies anterior to the left ventricle
 Tricuspid valve  most anterior valve (most common to be injured – stabbing)
 Left atrium  most posterior chamber, the right atrium is just anterior and to
the right of the left atrium.
 The left atrial appendage is not readily seen on transthoracic
echocardiography and requires transoesophageal echocardiography.
 The aortic valve is tricuspid

Arterial anatomy
 Internal thoracic artery arises from the subclavian artery.
 The subscapular artery arises from the axillary and is its largest branch,
eventually anastomosing with the lateral thoracic and intercostal arteries.

Anatomical relations
 Descending aorta lies behind (posterior to) the left main bronchus.
 Ascending aorta is anterior to the pulmonary trunk.
 Left pulmonary artery is anterior to the left main bronchus.
 Right main bronchus should be beside the left following bifurcation of
the trachea.
 Superior vena cava can be found next to the ascending aorta.
 Oesophagus is also a posterior structure to the left main bronchus.
 Left atrial enlargement can result in MR by affecting which leaflet?
o Posterior leaflet
o Anterior leaflet is not affected, because of its attachment to the root of the aorta.

Coronary arteries
 RCA supplies the AV node, so heart
block following inferior MI is common.
However, heart block following anterior
MI is a grave prognostic marker as this
indicates a large anterior wall infarct.
 The right coronary system also supplies
the right ventricle, hence problems
relating to a RV infarct are commonly
associated with an inferior MI.
 RCA supplies the inferior myocardium
and occlusion causes ST elevation in II,
III and aVF.

Posterior descending artery (PDA)

 Coronary dominance: A branch of RCA
in 85%, circumflex in in 15%
 Supplies the posterior left ventricular myocardium
 Occlusion  posterior MI (ST depression in V1-V4 with
a dominant R wave in V1).

Circumflex: lateral
 Occlusion produces ST elevation in V5, V6, I and aVL.

Left anterior descending: anterior and

septum
 Occlusion  ST segment elevation in leads V1-V4
 RBBB in acute anterior MI suggests obstruction prior to the first septal branch of the LAD

Left main stem:

 Branches into the LAD and circumflex and supplies most of the LV
 Complete left main stem occlusion is invariably fatal.
 Occlusion  extensive ST elevation across all the chest leads, I and aVL and possibly aVR, too.

Obtuse marginal
 One of the branches of the circumflex and supplies the 'high lateral' region of the left ventricle (ECG leads I and aVL).
Coronary circulation
Arterial supply of the heart
 Left aortic sinus  left coronary artery (LCA)
 Right aortic sinus  right coronary artery (RCA)
 LCA  LAD + circumflex
 RCA  posterior descending
 RCA supplies SA node in 60%, AV node in 90%
o The sinus node artery is a branch of the right coronary artery in 60% of cases.

Venous drainage of the heart

 Coronary sinus drains into the right atrium

Other notes
 Adenosine is an important mediator of metabolic vasodilatation
o Adenosine has a particularly short half-life,
o Acts on specific adenosine cell surface receptors (A1 and A2)
o Inactivated by adenosine deaminase.
o It results in coronary vasodilatation and depression of sinus node automaticity and AVN conduction.
 Coronary blood flow is dependent on myocardial oxygen consumption and is pretty independently maintained throughout
the ranges of blood pressure.
 Increasing O2 demands are met by increased blood supply facilitated by vasodilatation brought about by adenosine
production.

Pulmonary circulation
The normal pulmonary circulation is characterised by:
 Low pressures
 Low flow rates
 High compliance vessels

Internal jugular vein

 Originates at the jugular foramen.
 It initially lies posterior to the carotid artery, as it descends in the carotid sheath it lies lateral first to the internal then the
common carotid artery within the carotid sheath.
o Lies lateral to the common carotid artery
 It passes anterior to the subclavian artery to join the subclavian vein and then form the brachiocephalic vein; the left and
right brachiocephalic veins unite to form the superior vena cava.
 The internal jugular vein receives a lymphatic trunk at its union with the subclavian vein.

Jugular venous pressure

 Essential clinical sign that reflects patient filling
status and is essential to detect for correct fluid
management.
 Reflects right atrial pressure, and in healthy
individuals at 45° is 3 cm in vertical height above
the sternal angle (the angle of Louis, the
manubriosternal junction).
 Inspiration generates negative intrathoracic
pressure and a suction of venous blood towards
the heart, causing the JVP to fall


Raised JVP with normal waveform:

• • Heart failure – biventricular or isolated right heart failure
• • Fluid overload of any cause
• • Severe bradycardia.

Raised JVP upon inspiration and drops with expiration

• • Kussmaul’s sign is the opposite of what occurs in health and implies that the right heart chambers cannot
increase in size to accommodate increased venous return.
• • This can be due to pericardial disease (constriction) or fluid in the pericardial space (pericardial effusion and
cardiac tamponade).
Raised JVP with loss of normal pulsations:
• • SVC syndrome is obstruction caused by - mediastinal malignancy, such as bronchogenic malignancy, which
causes head, neck and/or arm swelling.

Arterial pulse associations

The radial arterial pulse is suitable for assessing the rate and rhythm, and whether it is collapsing. The central arterial
pulses, preferably the carotid, are used to assess the character.

Absent radial pulse

• • Iatrogenic: post-catheterisation or arterial line
• • Blalock–Taussig shunt for congenital heart disease, eg tetralogy of Fallot
• • Aortic dissection with subclavian involvement
• • Trauma
• • Takayasu’s arteritis
• • Peripheral arterial embolus.

Pathological pulse characters

• • Collapsing: aortic regurgitation, arteriovenous fistula, patent ductus arteriosus (PDA), other large
extracardiac shunt, hyperkinetic (anaemia, thyrotoxic, fever, exercise, pregnancy)
• • Slow rising: aortic stenosis (delayed percussion wave)
• • Bisferiens: a double shudder/ ‘double pulse’ - 2 systolic peaks
◦ ◦ Mixed aortic valve disease with significant regurgitation (tidal wave second impulse)
• • Jerky: hypertrophic obstructive cardiomyopathy (+/- bisferiens pulse)
• • Alternans: Severe left ventricular dysfunction.
◦ ◦ The ejection fraction is reduced meaning the end-diastolic volume is elevated. This may sufficiently
stretch the myocytes (Frank–Starling physiology) to improve the the ejection fraction of the next heart
beat.
◦ ◦ This leads to pulses that alternate from weak to strong
◦ ◦ Associated with a third heart sound
• • Paradoxical (pulsus paradoxus): excessive drop in SBP >10 mmHg on inspiration
◦ ◦ LV compression, tamponade, constrictive pericarditis or severe asthma as venous return is
compromised.

Cardiac apex
The cardiac apex pulsation reflects the ventricle striking the chest wall during isovolumetric contractions, and gives an
indication of the position of the left ventricle and it size. It is typically palpable in the fifth intercostal space in the
midclavicular line.

Absent apical impulse

• • Obesity/emphysema
• • Right pneumonectomy with displacement
• • Pericardial effusion or constriction
• • Dextrocardia (palpable on right side of chest)

Pathological apical impulse

• • Heaving: left ventricular hypertrophy (LVH) (and all its causes), sometimes associated with palpable fourth
heart sound
• • Thrusting/hyperdynamic: high left ventricular volume (eg in mitral regurgitation, aortic regurgitation, PDA,
ventricular septal defect)
• • Tapping: palpable first heart sound in mitral stenosis
• • Displaced and diffuse/dyskinetic: left ventricular impairment and dilatation (eg dilated cardiomyopathy,
myocardial infarction [MI])
• • Double impulse: with dyskinesia is due to left ventricular aneurysm; without dyskinesia in hypertrophic
cardiomyopathy (HCM)
• • Pericardial knock: constrictive pericarditis
• • Parasternal heave: due to right ventricular hypertrophy (eg atrial septal defect [ASD], pulmonary
hypertension, chronic obstructive pulmonary disease [COPD], pulmonary stenosis)
• • Palpable third heart sound: due to heart failure and severe mitral regurgitation.

Subclavian vein
• • Each subclavian vein is a continuation of the axillary vein and runs from the outer border of the first rib to
the medial border of anterior scalene muscle. From here it joins with the internal jugular vein to form the
brachiocephalic vein (also known as "innominate vein"). The angle of union is termed the venous angle.
• • The subclavian vein follows the subclavian artery and is separated from the subclavian artery by the
insertion of anterior scalene. Thus, the subclavian vein lies anterior to the anterior scalene while the
subclavian artery lies posterior to the anterior scalene (and anterior to the middle scalene).
• • The subclavian and internal jugular vein unite to form the brachiocephalic vein, subsequently the left and
right brachiocephalic veins unite to form the superior vena cava.
• • The thoracic duct enters the left subclavian.
• • Right or left subclavian is regarded as the cleanest site for central venous access. It also the most tolerated
by patients.
• • However the incidence of subclinical pneumothorax even in the hands of experienced clinicians has led to it
falling out of favour.
• • Compared with femoral site access, internal jugular or subclavian access was associated with a lower risk
of catheter-related bloodstream infections (CRBSIs) in earlier studies, but subsequent studies (since 2008)
found no significant differences in the rate of CRBSIs between these three sites.
• • The subclavian approach remains the most commonly used blind approach for subclavian vein cannulation.
Its advantages include consistent landmarks, increased patient comfort, and lower potential for infection or
arterial injury compared with other sites of access.

Questions:
• • Optimal point of needle insertion of subclavian venom catheter: 1 cm inferior of the junction of the. Middle
and medial third of the clavicle
• • Which vessel would become important for transporting blood from the lower to upper parts of the body in the
event of complete occlusion of the inferior vena cava? Ascending lumbar vein
• • A left sided internal jugular central venous catheter has been inserted and you are reviewing the chest
radiograph to check the position of the tip of the catheter. What is the safest position to leave the catheter
tip?
◦ ◦ In the lower superior vena cava
◦ ◦ If the catheter tip is above the carina on a post-procedure radiograph then it is generally accepted that
the catheter lies outside the right atrium in the lower superior vena cava (SVC).
◦ ◦ It is also recommended that the catheter tip should lie in the long axis of the SVC without acute
abutment to the vein wall.
◦ ◦ Left sided catheters are more likely to erode the vessel wall if they lie in the innominate veins or the
upper SVC due to the abutment of the catheter tip to the vessel wall.
◦ ◦ They are also more likely to cause pain on injection, thrombosis and infection if the tip lies in the
innominate veins.
◦ ◦ It is considered negligent to site the catheter tip in the right atrium as this may lead to arrhythmias,
tricuspid valve disfunction and placement in the coronary sinus.

Subclavian steal syndrome

• • Retrograde flow into the vertebral or internal thoracic arteries, due to stenosis and/ or occlusion of the
subclavian artery.

Symptoms may be precipitated by extreme exercise of the affected side

• • Many are asymptomatic.
• • Fatigue, aching, coolness of the affected arm and numbness.
• • Upper limb ischemia: arm pain and numbness, especially during exertion.
• • Neurological symptoms (25%): vertigo, diplopia, decreased vision, nystagmus and gait unsteadiness
• • BP is different between the upper limbs by at least 15 mmHg.

Pathophysiology:
• • Flow-related phenomena rather than embolic.
• • Atherosclerotic lesion in the proximal subclavian artery —> significant stenosis —> collateral vessels
enlargement from subclavian artery distal to obstruction
• • The upper extremity becomes dependent on these collaterals
• • Exercise —> BVs dilate to enhance perfusion to the ischaemic muscle —> lowering the resistance in the
outflow vessels.
• • Blood is siphoned from the head, neck and shoulder through collateral vessels to supply this low-resistance
vascular bed, satisfying increased oxygen demand by the exercising muscles of the upper extremity —>
posterior cerebral circulation neurological symptoms.

What is the most likely mechanism that maintains blood flow to the affected extremity?
Blood from the contralateral vertebral artery is shunted away from the basilar artery (away from the brainstem) and
retrograde into the ipsilateral vertebral artery to supply the affected arm .

Diagnosis
• • Non-invasive arterial flow studies, Doppler and arteriography.
• • Duplex ultrasound is the best initial radiological test
• • The most accurate test is angiography of the subclavian vessels.

Management:
• • Most patients require no intervention,
• • Surgical reconstruction may be required where symptoms are severe.
________________________________________________________________________________
Physiological notes
• • The sinoatrial node has the fastest firing rate of all potential pacemakers in the heart.
• • Sinoatrial node impulses must occur at a rate slower than 200 impulses per minute to be considered in
normal sinus rhythm.
• • Endothelin
◦ ◦ Preferentially constricts renal afferent arterioles.
◆ ▸ Efferent arteriole vasoconstriction is particularly mediated by angiotensin-II, to defend GFR in
states of generalised vasoconstriction and reduced blood flow.
• • Efferent arteriole vasodilation will occur when angiotensin-II levels fall.
◦ ◦ Stimulates the renin-angiotensin-aldosterone system
◦ ◦ Leads to release of atrial natriuretic peptide
◦ ◦ Inhibits the action of vasopressin
◦ ◦ Two types of endothelin receptor have been characterised, A and B.
◆ ▸ Binding of endothelin to the A receptor induces vasoconstriction,
◆ ▸ Binding to the B receptor leads to nitric oxide release and hence vasodilatation.

Atrial natriuretic peptide

• • Secreted mainly from myocytes of right atrium and ventricle in response to increased blood volume
◦ ◦ secreted by both the right and left atria (right >> left)
• • Synthesis increased by thyroid hormones, glucocorticoids, endothelin-1, angiotensin-II, and tachycardia,
independent of the haemodynamic effects of these factors.
• • 28 amino acid peptide hormone, which acts via cGMP
• • Degraded by endopeptidases
• • Actions
◦ ◦ Natriuretic, i.e. promotes excretion of sodium
◦ ◦ Lowers BP
◦ ◦ Reduced pre-load due to relaxing effects on vascular smooth muscle.
◦ ◦ Antagonises actions of angiotensin II, aldosterone

B-type natriuretic peptide

• • Hormone produced mainly by the left ventricular myocardium in response to strain.
• • Causes of raised BNP levels
◦ ◦ Heart failure is the most obvious cause
◦ ◦ The stimulus for BNP release is myocyte stretch, rather than transmural pressure load.
◦ ◦ Any cause of left ventricular dysfunction such as myocardial ischaemia or valvular disease
◦ ◦ Chronic kidney disease (due to reduced excretion).
◦ ◦ BNP synthesis is increased by thyroid hormones as well as glucocorticoids, endothelin-1, angiotensin-
II, and tachycardia, independent of the haemodynamic effects of these factors.
• • Factors which reduce BNP levels
◦ ◦ Treatment with ACEi, ARBs and diuretics. Effects of BNP

Normal waves in the JVP

The a wave
Due to atrial contraction – actively push up superior vena cava (SVC) and into the right ventricle (may cause an audible S4).
The c wave
An invisible flicker in the x descent due to closure of the tricuspid valve, before the start of ventricular systole.
The x descent
Downward movement of the heart causes atrial stretch and a drop in pressure.
The v wave
Due to passive filling of blood into the atrium against a closed tricuspid valve.
The y descent
Opening of the tricuspid valve with passive movement of blood from the right atrium to the right ventricle (causing an S3 when
audible).
Causes of a raised JVP
1. Raised JVP with normal waveform:
Heart failure – biventricular or isolated right heart failure
Fluid overload of any cause
Severe bradycardia.
Raised JVP upon inspiration and drops with expiration: Kussmaul’s sign is the opposite of what occurs in health and implies that
the right heart chambers cannot increase in size to
accommodate increased venous return. This can be due to pericardial disease (constriction) or fluid in the pericardial space
(pericardial effusion and cardiac tamponade).
Raised JVP with loss of normal pulsations: SVC syndrome is obstruction caused by
mediastinal malignancy, such as bronchogenic malignancy, which causes head, neck and/or arm
swelling.
Pathological waves in the JVP
This is a common source of MRCP Part 1 questions. See Table 1.1 and Figure 1.2 for these waves. Table 1.1 Pathological waves
in the jugular venous pressure (JVP)

a waves
Absent Large Cannon
Giant Steep
Steep
Atrialfibrillation–noco-ordinatedcontraction
Tricuspid stenosis, right heart failure, pulmonary hypertension
Caused by atrioventricular dissociation – allowing the atria and ventricles to contract at same time:
Atrial flutter and atrial tachycardias Third-degree (‘complete’) heart block Ventricular tachycardia and ventricular ectopics
Tricuspid regurgitation – technically a giant ‘c-V’ wave Tamponade and cardiac constriction
If steep x descent only, then tamponade
Cardiac constriction
v waves
x y

descent descent
Slow
Figure 1.2 Different JVP morphologies can reflect different disease states
Tricuspid stenosis
1.2.2 Arterial pulse associations
The radial arterial pulse is suitable for assessing the rate and rhythm, and whether it is collapsing. The central arterial pulses,
preferably the carotid, are used to assess the character.
Absent radial pulse
Iatrogenic: post-catheterisation or arterial line
Blalock–Taussig shunt for congenital heart disease, eg tetralogy of Fallot
Aortic dissection with subclavian involvement
Trauma
Takayasu’s arteritis
Peripheral arterial embolus.
Pathological pulse characters
Collapsing: aortic regurgitation, arteriovenous fistula, patent ductus arteriosus (PDA) or other large extracardiac shunt
•
• Slow rising: aortic stenosis (delayed percussion wave)
Bisferiens: a double shudder due to mixed aortic valve disease with significant regurgitation (tidal wave second impulse)
•
• Jerky: hypertrophic obstructive cardiomyopathy
Alternans: occurs in severe left ventricular dysfunction. The ejection fraction is reduced meaning the end-diastolic volume is
elevated. This may sufficiently stretch the myocytes (Frank–Starling physiology) to improve the the ejection fraction of the next
heart beat. This leads to pulses that alternate from weak to strong
•
Paradoxical (pulsus paradoxus): an excessive reduction in the pulse with inspiration (drop in
• systolic BP >10 mmHg) occurs with left ventricular compression, tamponade, constrictive pericarditis or severe asthma as venous
return is compromised.
1.2.3 Cardiac apex
The cardiac apex pulsation reflects the ventricle striking the chest wall during isovolumetric contractions, and gives an indication of
the position of the left ventricle and it size. It is typically palpable in the fifth intercostal space in the midclavicular line.
Absent apical impulse
Obesity/emphysema
Right pneumonectomy with displacement
Pericardial effusion or constriction
Dextrocardia (palpable on right side of chest)
Pathological apical impulse
Heaving: left ventricular hypertrophy (LVH) (and all its causes), sometimes associated with palpable fourth heart sound
Thrusting/hyperdynamic: high left ventricular volume (eg in mitral regurgitation, aortic regurgitation, PDA, ventricular septal
defect)
Tapping: palpable first heart sound in mitral stenosis
Displaced and diffuse/dyskinetic: left ventricular impairment and dilatation (eg dilated cardiomyopathy, myocardial infarction [MI])
Double impulse: with dyskinesia is due to left ventricular aneurysm; without dyskinesia in hypertrophic cardiomyopathy (HCM)
Pericardial knock: constrictive pericarditis
••
••
Parasternal heave: due to right ventricular hypertrophy (eg atrial septal defect [ASD], pulmonary hypertension, chronic obstructive
pulmonary disease [COPD], pulmonary stenosis)
•
• Palpable third heart sound: due to heart failure and severe mitral regurgitation.
1.2.4 Heart sounds
Abnormalities of first heart sounds are given in Table 1.2 and of second heart sounds in Table 1.3. Third heart sound (S3)
Due to the passive filling of the ventricles on opening of the atrioventricular (AV) valves, audible in normal children and young
adults. Pathological in cases of rapid left ventricular filling (eg mitral regurgitation, ventricular septal defect [VSD], congestive
cardiac failure and constrictive pericarditis).

Table 1.2 Abnormalities of the first heart sound (S1): closure of mitral and tricuspid valves

Loud
Soft
Split
Vari able

Mobile mitral stenosis

Hyperdynamic states
Tachycardic states
Immobile mitral stenosis
Hypodynamic states
Mitral regurgitation
Poorventricular function
Atrial RBBB fibrilla
tion
Compl LBBB ete heart
Left-to-rightshunts Short PR interval
Inspiration Ebstein’s anomaly
VT
Long PR interval
LBBB, left bundle-branch block; RBBB, right bundle-branch block; VT, ventricular tachycardia.
block

Table 1.3 Abnormalities of the second heart sound (S2): closure of aortic then pulmonary valves (<0.05 s apart)

Inte nsity
Loud:
Systemic hypertension (loud A2)
Pulmonary hypertension (loud P2)
Tachycardic states
ASD (loud P2)
Soft or absent:
Severe aortic stenosis
Splitting
Fixed: ASD
Widely split:
RBBB
Pulmonary stenosis Deep inspiration Mitral regurgitation
Single S2:
Severe pulmonary stenosis/aortic stenosis
Hypertension
Large VSD
Tetralogy of Fallot Eisenmenger’s syndrome Pulmonary atresia Elderly
Reversed split S2:
LBBB
Right ventricular pacing PDA
Aortic stenosis
A2, aortic second sound; ASD, atrial septal defect; LBBB, left bundle-branch block; P2, pulmonary second sound; PDA, patent
ductus arteriosus; RBBB, right bundle-branch block; VSD, ventricular septal defect.
Fourth heart sound (S4)
Due to the atrial contraction that fills a stiff left ventricle, such as in LVH, amyloid, HCM and left
ventricular ischaemia. It is absent in atrial fibrillation.
Causes of valvular clicks
Aortic ejection: aortic stenosis, bicuspid aortic valve
Pulmonary ejection: pulmonary stenosis
Mid-systolic: mitral valve prolapse.
Opening snap
In mitral stenosis an opening snap (OS) can be present and occurs after S2 in early diastole. The closer it is to S2 the greater the
severity of mitral stenosis. It is absent when the mitral cusps become immobile due to calcification, as in very severe mitral
stenosis.
1.3 CARDIAC INVESTIGATIONS 1.3.1 Electrocardiography
Both the axis and sizes of QRS vectors give important information. Axes are defined as:
−30° to +90°: normal
−30° to −90°: left axis
+90° to +180°: right axis
−90° to −180°: indeterminate.
Tip: if the QRS is positive in leads 1 and aVF the axis is normal.
The causes of common abnormalities are given in the box below. Electrocardiography (ECG) strips illustrating typical changes in
common disease states are shown in Figure 1.3.
Causes of common abnormalities in the
ECG
Causes of left axis deviation
Left bundle-branch block (LBBB)
Left anterior hemi-block (LAHB)
LVH
Primum ASD
Cardiomyopathies
Tricuspid atresia
Low-voltage ECG
Pulmonary emphysema
Pericardial effusion
Myxoedema
Severe obesity
Incorrect calibration
Cardiomyopathies
Global ischaemia
Amyloid
Causes of right axis deviation
Infancy
Right bundle-branch block (RBBB)
Right ventricular hypertrophy (eg lung disease, pulmonary embolism, large secundum ASD, severe pulmonary stenosis, tetralogy
of Fallot)
Abnormalities of ECGs in athletes
Sinus arrhythmia
Sinus bradycardia
First-degree heart block
Wenckebach’s phenomenon
Junctional rhythm
•
Clinical diagnoses that can be made from the ECG of an asymptomatic patient
Atrial fibrillation
Complete heart block
HCM
ASDs (with RBBB)
Long QT and Brugada’s syndromes
Wolff–Parkinson–White (WPW) syndrome (δ waves)
Arrhythmogenic right ventricular dysplasia (cardiomyopathy).
Short PR interval
This is rarely <0.12 s; the most common causes are those of pre-excitation involving accessory pathways or of tracts bypassing the
slow region of the AV node; there are other causes.
Pre-excitation
WPW syndrome
Lown–Ganong–Levine syndrome (short PR syndrome)
Other
Ventricular extrasystole falling after P wave
AV junctional rhythm (but P wave will usually be negative)
Low atrial rhythm
Coronary sinus escape rhythm
• Normal variant (especially in young people)
Causes of tall R waves inV1
It is easy to spot tall R waves in V1. This lead largely faces the posterior wall of the left ventricle and the mass of the right ventricle.
As the overall vector is predominantly towards the bulkier left ventricle in normal situations, the QRS is usually negative in V1. This
balance can be reversed in the following situations:
Figure 1.3 ECG strips demonstrating typical changes in common disease states
Right ventricular hypertrophy (myriad causes)
RBBB
Posterior infarction
Dextrocardia
WPW syndrome with left ventricular pathway insertion (often referred to as type A)
HCM (septal mass greater than posterior wall).
Posterior infarctions are easily missed because the rest of the ECG can be normal. Recognition of positive tall R waves in V1 can
be the only sign with a typical history; there may be subtle ST depression in V1–V3. Performing a posterior ECG with leads placed
over the back will reveal ST elevation.

Bundle-branch block and ST-segment abnormalities

Complete bundle-branch block is a failure or delay of impulse conduction to one ventricle from the AV node, requiring conduction
via the other bundle, and then transmission within the ventricular myocardium; this results in abnormal prolongation of QRS
duration (>120 ms) and abnormalities of the normally isoelectric ST segment. In contrast to RBBB, LBBB is always pathological.
During the hyperacute and acute phases of cerebral events, including ischaemic stroke and haemorrhage, marked ST changes
may be observed on the ECG and there may even be an associated rise in cardiac troponin. These changes result from abnormal
autonomic discharges due to intense sympathetic nervous activation, with consequent myocytolysis, which accounts for troponin
leak.
Causes of LBBB
Ischaemic heart disease (recent or old MI)
Hypertension
LVH
Aortic valve disease
Cardiomyopathy
Myocarditis
Post-valve replacement
Right ventricular pacemaker
Tachycardia with aberrancy or concealed conduction
Ventricular ectopy
Causes of RBBB
Normal in young people
Right ventricular strain (eg pulmonary embolus)
ASD
Ischaemic heart disease
Myocarditis
Idiopathic
Tachycardia with aberrancy or concealed conduction
Ventricular ectopy
Causes of ST elevation
Early repolarisation
Acute MI
Pericarditis (saddle shaped)
Ventricular aneurysm
Coronary artery spasm
During angioplasty
Non-standard ECG acquisition settings (eg on monitor)
• Other ST–T wave changes (not elevation)
Ischaemia:
Digoxin therapy:
Hypertrophy:
Post-tachycardia:
Hyperventilation:

Oesophageal/upper abdominal irritation:

Cardiac contusion:
Mitral valve prolapse:

Acute cerebral event (eg subarachnoid haemorrhage):

Electrolyte abnormalities
ST depression, T inversion and peaking downsloping ST depression
ST depression, T inversion
ST depression, T inversion
ST depression, T inversion and peaking ST depression, T inversion
ST depression, T inversion T-wave inversion
ST depression, T inversion
Q waves can be permanent (reflecting myocardial necrosis) or transient (suggesting failure of myocardial function, but not
necrosis).
Permanent Q waves
Transmural infarction
LBBB
WPW syndrome
HCM
Idiopathic cardiomyopathy
Amyloid heart disease
Neoplastic infiltration
Friedreich’s ataxia
Dextrocardia
Sarcoidosis
Progressive muscular dystrophy
Myocarditis (may resolve)
Transient Q waves
Coronary spasm
Hypoxia
Hyperkalaemia
Cardiac contusion
Hypothermia
Potassium and ECG changes
There is a reasonable correlation between plasma potassium and ECG changes.
Hype rkalae mia
Tall T waves
Prolonged PR interval
Flattened/absent P waves
Very severe hyperkalaemia
Wide QRS
Sine wave pattern
Ventricular tachycardia/ventricular fibrillation/asystole
Hypokalae mia
Flat T waves, occasionally inverted
Prolonged PR interval
ST depression
TallUwaves
ECG changes after coronary artery bypass surgery
U waves (hypothermia)
Saddle-shaped ST elevation (pericarditis)
PR-segment depression (pericarditis)
Low-voltage ECG in chest leads (pericardial effusion)
Changing electrical alternans (alternating ECG axis – cardiac tamponade)
S1Q3T3 (pulmonary embolus)
Atrial fibrillation
Q waves
ST-segment and T-wave changes.
ECG techniques for prolonged monitoring
Holter monitoring: the ECG is monitored in one or more leads for 24–72 h. The patient is encouraged to keep a diary in order to
correlate symptoms with ECG changes
External recorders: the patient keeps a monitor with them for a period of days or weeks. At the onset of symptoms the monitor is
placed to the chest and this records the ECG
Wearable loop recorders: the patient wears a monitor for several days or weeks. The device records the ECG constantly on a
self-erasing loop. At the time of symptoms, the patient activates the recorder and a trace spanning some several seconds before a
period of symptoms to several minutes afterwards is stored
Implantable loop recorders: a loop recorder is implanted subcutaneously in the pre-pectoral
region. The recorder is activated by the patient or according to pre-programmed parameters.
• Again the ECG data from several seconds before symptoms to several minutes after are stored;
data are uploaded by telemetry. The battery life of the implantable loop recorder varies between 18–36 months.
••
•
1.3.2 Echocardiography
Principles of the technique
Sound waves emitted by a transducer are reflected back differentially by tissues of variable acoustic properties. Moving structures
(including fluid structures) reflect sound back as a function of their own velocity. The signal-to-noise ratio is improved by minimising
the distance and number of acoustic structures between the transducer and the object being recorded.
M-mode: named after appearance of the mitral valve on this modality, this is a longitudinal beam that achieves high temporal
resolution for a given location. It allows calculations of left atrial (LA) size, aortic root, left ventricular (LV) outflow tract (LVOT), and
ventricular end-systolic and end-diastolic dimensions. These are typically made in the parasternal long-axis view.
Doppler measurements: the Doppler phenomenon is used to measure the velocity of blood flow for estimation of pressure
gradients across valve abnormalities. Velocities can be measured along the length of the Doppler beam (continuous-wave Doppler,
useful for aortic stenosis assessment), or at a specific location (pulsed-wave Doppler, useful for LV filling patterns or for measuring
the velocity of myocardial tissue movement).
Two-dimensional echocardiography: the piezoelectric crystals in the probe head are activated in sequence to reconstruct a two-
dimensional image of the heart. This allows identification of anatomy and structural abnormalities, eg enlarged structures, abnormal
valves or abnormal communications between chambers. Newer probes can produce three-dimensional images to better visualise
defects.
Colour Doppler: this applies Doppler to assess the average velocities of blood within a region of interest. Movement of blood can
be coded red (moving towards transducer) or blue (moving away from transducer) known as the BART convention (blue = away
red = toward). This helps identify and quantify valvular regurgitation.
Diagnostic uses of echocardiography
Conventional echocardiography is used in the diagnosis of:
Pericardial effusion and tamponade
Valvular disease (including large vegetations)
HCM, dilated cardiomyopathy, LV mass and function
Cardiac tumours and intracardiac thrombus
Congenital heart disease (eg PDA, coarctation of the aorta)
Right ventricular function and pressure.
Stress echo is used in the diagnosis of myocardial viability and ischaemia, to help risk stratify patients and consider them for
further investigations such as coronary angiography. Resting images are acquired and then stress is induced, by either intravenous
dobutamine infusion or exercise performed on a bike or treadmill. Stress images are then acquired in the long-axis and short-axis
views at moderate and peak heart rates and compared with resting images, typically arranged in a grid for ease of comparison.
Contrast may be required for optimal myocardial definition – it appears bright. Regional wall motion abnormalities such as
hypokinesia, dyskinesia and akinesia are defined
in a 16- or 17-segment model of the left ventricle. Patients should avoid β blockers before stress echocardiography, because these
will attenuate peak heart rate response.
Standard contrast echo is used in the diagnosis of right-to-left shunts, particularly for patent foramen ovale (PFO) but also ASD
and ventricular septal defect (VSD). Agitated saline or Gelofusine is injected into the venous system and the patient is asked to
undergo Valsalva’s manoeuvre to encourage increased right-sided pressure. Bubbles may then be observed crossing from the right
atrium to the left ventricle through a PFO or ASD.
Transpulmonary contrast echo is used to improve discrimination between the blood pool and the endocardium to help definition
in those individuals whose characteristics lead to poor image quality. It is also used to diagnose LV thrombus and other specific
conditions (eg the congenital failure of muscle fibre alignment [known as non-compaction] and apical hypertrophy).
Tissue Doppler imaging is a new technique that applies Doppler principles to analyse the velocity of myocardial motion.
Specifically, the movement of a given cardiac wall can be interrogated by manually selecting the region of interest on the echo
machine. Each wall will have a unique pattern of velocities, from which many different calculations can be made, most commonly to
estimate cardiac function and pressures. Values will vary according to the site, and the age and function of the heart. Tissue
Doppler differs from conventional Doppler, which focuses on the velocity of blood, and is used to assess blood flow across valves
and the rate of ventricular filling.
Transoesophageal echocardiography (TOE) is performed under general anaesthesia or in sedated patients with local
anaesthetic applied to the oropharynx. The probe is passed into the oesophagus, meaning that it is closer to the cardiac structures,
improving image quality. It is indicated in the diagnosis of aortic dissection (when a CT aortogram is delayed), suspected atrial
thrombus (before cardioversion of atrial arrhythmia), assessment of vegetations or abscesses in endocarditis, prosthetic valve
dysfunction or leakage, and the intraoperative assessment of LV function or success of valvular repair. It may also be indicated
when transthoracic images are suboptimal.
Three-dimensional echocardiography has increasing clinical application in better understanding structural anatomy. This is
particularly useful for planning therapy to valvular heart disease, whether it is surgical or percutaneous replacement. Often imaging
is performed during the procedure to guide valve placement. It has particular usefulness in congenital heart disease. It can be
performed using either transthoracic or transoeophageal approaches.
Intravascular ultrasonography (IVUS) is performed by placing a small probe mounted on a catheter on an intracoronary wire
during coronary angioplasty. It provides high-resolution imaging of coronary arteries for measurement of stenosis severity and
plaque characteristics, and assessment of the success of stent deployment.
Intracardiac ultrasonography images the heart chambers from within; it is used mainly in those with congenital heart disease and
in electrophysiological procedures.
Classic M-mode patterns
Due to improvements in real-time image quality. M-mode imaging is now used less in clinical practice; it does, however, allow
interpretable traces to be printed as still images, and these still occasionally feature in exams. Particular M-mode patterns that have
been used in past MRCP exams include:
• Aortic regurgitation: fluttering of the anterior mitral leaflet is seen
HCM: systolic anterior motion (SAM) of the mitral valve leaflets and asymmetrical septal hypertrophy (Figure 1.4)
•
• Mitral valve prolapse: one or both leaflets prolapse during systole

Mitral stenosis: the opening profile of the cusps is flat and multiple echoes are seen when there is calcification of the cusps.
•
Figure 1.4 Classic valvular disease patterns seen with M-mode echocardiography

1.3.3 Nuclear cardiology: myocardial perfusion imaging (Figure 1.5)

Perfusion tracers such as thallium or technetium can be used to gauge myocardial blood flow, both at rest and during exercise- or
drug-induced stress. Tracer uptake is detected using tomograms and displayed in a colour scale in standard views.
Lack of uptake may be:
• Physiological: due to lung or breast tissue absorption
Pathological: reflecting ischaemia, infarction or other conditions in which perfusion abnormalities also occur (eg HCM or
amyloidosis).
Pathological perfusion defects are categorised as fixed (scar) and reversible (viable but ischaemic tissue).
MPI can be used to:
Detect infarction
Investigate atypical chest pains
Assess ventricular function
Determine prognosis and detect myocardium that may be ‘re-awakened’ from hibernation with an improved blood supply (eg after
coronary artery bypass grafting [CABG]).

•
•
1.3.4 Cardiac catheterisation
Coronary and ventricular angiography
Direct injection of radio-opaque contrast into the coronary arteries allows high-resolution assessment of restrictive lesions and
demonstrates any anomalies. Left ventriculography provides a measure of ventricular systolic function.
Angiography is typically performed via the femoral artery or radial artery through a sheath that allows insertion of specifically
designed catheters that intubate the coronary vessels. Contrast can be hand injected or injected by automated pumps. Imaging is
acquired by fluoroscopy. The contrast provides an image of the vessel lumen but the other parts of the vessel are poorly visualised.
The lumen is carefully assessed for narrowings or stenoses, which represent coronary atherosclerotic lesions that limit blood flow.
Multiple different radiographic views are required to view each vessel, because stenoses can be hidden in different projections.
Figure 1.5 Radionuclide myocardial perfusion imaging. Left panel shows the gamma camera. Right panel shows a reversible
inferolateral perfusion defect: left column stress, right column rest.
The severity of a stenosis can be gauged visually, reported as a percentage of the vessel, or measured objectively using
quantitative coronary angiography (QCA), which uses computer-aided edge detection of coronary vessels. IVUS and optical
coherence tomography (OCT) are intracoronary tools used to visualise the stenosis severity and estimate the lumen area occupied
by atherosclerotic plaque. Functional, or physiological, lesion severity can be detected using a wire with a tiny pressure sensor on it
placed distal to a stenosis to estimate the degree of pressure drop in comparison to the aortic pressure.
Percutaneous coronary intervention (PCI) can be performed immediately after coronary angiography or at a later occasion. It
involves the treatment of stenoses using balloon inflation and stent deployment. Intervention is most commonly performed for the
treatment of coronary obstruction in acute coronary syndrome. Other indications include primary PCI (PPCI) for acute treatment of
an MI or in symptomatic stable angina where there is evidence of cardiac ischaemia.

Complications of cardiac catheterisation

Complications are uncommon (approximately 1%, including minor complications); these include contrast allergy, local haemorrhage
from puncture sites with subsequent occurrence of thrombosis, retroperitoneal haemorrhage, false aneurysm formation (which can
be compressed or injected) or arteriovenous malformation. Vasovagal reactions are common. Other complications are:
Coronary dissection: (particularly the right coronary artery in women) and aortic dissection or ventricular perforation
Air or atheroma embolism: in the coronary or other arterial circulations, with consequent ischaemia or strokes
• Ventricular dysrhythmias: can even cause death in the setting of left main stem disease
••
Mistaken cannulation and contrast injection into the conus branch of the right coronary artery can cause ventricular fibrillation
•
• Overall mortality rates are quoted at <1/1000 cases.
1.3.5 Exercise stress testing
This is used in the investigation of coronary artery disease, exertion-induced arrhythmias, and the assessment of cardiac workload
and conduction abnormalities. Exercise tests also give diagnostic and prognostic information post-infarction, and generate patient
confidence in rehabilitation after an MI. Diagnostic sensitivity is improved if the test is conducted with the patient having
discontinued antianginal (especially rate-limiting) medication.
The main contraindications to exercise testing include those conditions where fatal ischaemia or arrhythmias may be provoked, or
where exertion may severely and acutely impair cardiac function. These include the following:
Severe aortic stenosis or HCM with marked outflow obstruction
Acute myocarditis or pericarditis
Pyrexial or coryzal illness
Severe left main stem disease
Untreated congestive cardiac failure
Unstable angina
Dissecting aneurysm
Ongoing tachy- or bradyarrhythmias
Untreated severe hypertension.
Indicators of a positive exercise test result
The presence of each factor is additive in the overall positive prediction of coronary artery disease:
Development of anginal symptoms
A fall in BP of >15 mmHg or failure to increase BP with exercise
Arrhythmia development (particularly ventricular)
Poor workload capacity (may indicate poor left ventricular function)
Failure to achieve target heart rate (allowing for β blockers)
>1-mm down-sloping or planar ST-segment depression, 80 ms after the J point
ST-segment elevation
Failure to achieve 9 min of the Bruce protocol due to any of the points listed.
Exercise tests have low specificity in the following situations (often as a result of resting ST-segment abnormalities):
Ischaemia in young women with atypical chest pains
Atrial fibrillation
LBBB
WPW syndrome
LVH
Digoxin or β-blocker therapy
Anaemia
Hyperventilation
Biochemical abnormalities such as hypokalaemia.
1.3.6 24-hour ambulatory blood pressure monitoring
The limited availability and relative expense of ambulatory BP monitoring prevent its use in all hypertensive patients. Specific areas
of usefulness include the following situations:
Assessing for ‘white coat’ hypertension
Borderline hypertensive cases who may not need treatment
Evaluation of hypotensive symptoms
Identifying episodic hypertension (eg in phaeochromocytoma)
Assessing drug compliance and effects (particularly in resistant cases)
Nocturnal BP dipper status (non-dippers are at higher risk).
1.3.7 Computed tomography
Computed tomography (CT) has applications in anatomical (coronary arteries, chamber dimension, pericardium) and functional
(contractility, ischaemia, viability) assessments of the heart.
CT coronary angiography has gained considerable attraction in the identification of coronary artery disease, particularly in chest
pain clinics and in some emergency departments following guidance from the National Institute for Health and Care Excellence
(NICE). Calcium scores can be calculated quickly, with higher scores being more predictive of coronary obstruction. More detailed
scanning allows coronary arteries to be identified and followed in two-dimensional cross-sections, as well as in three-dimensional
reconstructions. This enables identification of lesions that appear obstructive. Increased speed, better ECG gating and higher-
resolution scanners have meant that coronary arteries can be assessed with relatively low levels of radiation.
The negative predictive value is higher than the positive predictive value; entirely normal scans are typically normal on invasive
coronary angiography, whereas those with apparently more important
disease may or may not have findings requiring action on invasive assessment. As such, CT coronary angiography is typically used
when patients have a low pre-test likelihood of coronary disease, a negative effectively excluding the condition.
CT pulmonary angiography (CTPA) is the gold standard investigation for:
Pulmonary thromboembolic disease
Anatomical assessment of the pericardium (eg in suspected constriction)
Anomalous coronary artery origins (reliable imaging of the proximal third of major coronary arteries)
Extramyocardial mediastinal masses
Chamber dimensions
Myocardial function, perfusion and ischaemia.
1.3.8 Magnetic resonance imaging
Cardiac magnetic resonance imaging (MRI) is the gold standard technique for assessment of myocardial function, ischaemia,
perfusion and viability, cardiac chamber anatomy and imaging of the great vessels. It has a useful adjunctive role in
pericardial/mediastinal imaging. Limitations include its contraindication in patients with certain implanted devices (eg pacemakers)
and time (consequently also cost), as a full functional study can take about 45 min. The contrast used (gadolinium), although not
directly nephrotoxic, is subject to increased risk of metabolic toxicity in renally impaired individuals.
Chief indications for cardiac MRI:
Myocardial ischaemia and viability assessment
Differential diagnosis of structural heart disease (congenital and acquired)
Chamber anatomy definition
Initial diagnosis and serial follow-up of great vessel pathology (especially aortopathy)
Pericardial and mediastinal structural assessment.
1.4 VALVULAR DISEASE AND ENDOCARDITIS 1.4.1 Murmurs
Benign flow murmurs: soft, short systolic murmurs heard along the left sternal edge to the pulmonary area, without any other
cardiac auscultatory, ECG or chest radiograph abnormalities. Thirty per cent of children may have an innocent flow murmur.
Cervical venous hum: continuous when upright and is reduced by lying; occurs with a hyperdynamic circulation or with jugular
vein compression.
Large arteriovenous fistula of the arm: may cause a harsh flow murmur across the upper mediastinum.
Effect of posture on murmurs: standing significantly increases the murmurs of mitral valve prolapse
•
and HCM only. Squatting and passive leg raising increase cardiac afterload and therefore decrease the murmur of HCM and mitra
valve prolapse, while increasing most other murmurs such as VSD, aortic, mitral and pulmonary regurgitation, and aortic stenosis.
Effect of respiration on murmurs: inspiration accentuates right-sided murmurs by increasing venous return, whereas held
expiration accentuates left-sided murmurs. The strain phase of Valsalva’s manoeuvre reduces venous return, stroke volume and
arterial pressure, decreasing all valvular murmurs but increasing the murmur of HCM and mitral valve prolapse.
Classification of murmurs
Mid-/late systolic murmurs
Innocent murmur
Aortic stenosis or sclerosis
Coarctation of the aorta
Pulmonary stenosis
HCM
Papillary muscle dysfunction
ASD (due to high pulmonary flow)
Mitral valve prolapse
Mid-diastolic murmurs
Mitral stenosis or ‘Austin Flint’ murmur due to aortic regurgitant jet
Carey Coombs murmur (rheumatic fever)
High AV flow states (ASD, VSD, PDA, anaemia, mitral regurgitation, tricuspid
regurgitation)
Atrial tumours (particularly if causing AV flow disturbance)
Continuous murmurs
PDA
Ruptured sinus of Valsalva’s aneurysm
ASD
Large arteriovenous fistula
Anomalous left coronary artery
Intercostal arteriovenous fistula
ASD with mitral stenosis
Bronchial collaterals
•
1.4.2 Mitral stenosis
Mitral stenosis (MS) is the thickening of the mitral leaflets that may occur at the cusps, commissures
or chordal level, to cause an obstruction of blood flow from the left atrium to the left ventricle. Two- thirds of patients presenting with
this are women. The most common cause remains chronic rheumatic heart disease, which involves a sustained inflammatory
reaction against the valve and valvular apparatus, due to antibody cross-reactivity to a streptococcal illness. Rarer causes include
congenital disease, carcinoid, systemic lupus erythematosus (SLE) and mucopolysaccharidoses (glycoprotein deposits on cusps).
Rheumatic heart disease originating in the UK is now exceptionally rare.
2 2
A normal mitral valve has a valve area of 4–6 cm : MS is diagnosed when the valve area is ≤2cm : It
2
is considered severe when ≤1cm ; symptoms are invariable and increased pulmonary pressures lead to pulmonary oedema, when
heart rates increase, and pulmonary hypertension. Atrial fibrillation is invariable and increases thromboembolic stroke risk by 17×;
anticoagulation is essential.
Treatment can be percutaneous (balloon valvuloplasty) or surgical (limited mitral valvotomy – now rarely performed in developed
nations – or open valve replacement).
Features of severe MS
Symptoms
Dyspnoea with minimal activity
Haemoptysis
Dysphagia (due to left atrium enlargement)
Palpitations due to atrial fibrillation
Chest radiograph
Left atrial or right ventricular enlargement
Splaying of subcarinal angle (>90°)
Pulmonary congestion or hypertension
Pulmonary haemosiderosis
• Echocardiogram
Doming of leaflets
Heavily calcified cusps •
2
Direct orifice area >1.0 cm • Signs
Low pulse pressure
Soft first heart sound
Long diastolic murmur and apical thrill (rare)
Very early opening snap, ie closer to S2 (lost if valves immobile)
Right ventricular heave or loud P2
Pulmonary regurgitation (Graham Steell murmur)
Tricuspid regurgitation
• Cardiac catheterisation
Pulmonary capillary wedge end diastole to left ventricular end-diastolic pressure
(LVEDP)
• gradient >15 mmHg
LA pressures >25 mmHg
Elevated right ventricular (RV) and pulmonary artery (P)A pressures
High pulmonary vascular resistance •
2
Cardiac output <2.5 L/min per m with exercise
Mitral balloon valvuloplasty
Valvuloplasty using an Inoue balloon requires either a trans-septal or a retrograde approach, and is used only in suitable cases
where echocardiography shows the following:
The mitral leaflet tips and valvular chordae are not heavily thickened, distorted or calcified
The mitral cusps are mobile at the base
There is minimal or no mitral regurgitation
There is no left atrial thrombus seen on TOE.
1.4.3 Mitral regurgitation
The full structure of the mitral valve includes the annulus, cusps, chordae and papillary musculature, and abnormalities of any of
these can cause regurgitation. The presence of symptoms and increasing left ventricular dilatation are indicators for surgery in the
chronic setting. Surgical mortality rates are 2–7% for valvular replacements in patients with New York Heart Association (NYHA)
grade II–III symptoms. Various techniques have revolutionised mitral valve surgery, transforming outcomes from being no better
than medical therapy with replacement to almost normal with repair. In skilled surgical hands the repair is tailored to the precise
anatomical abnormality.
Functional mitral regurgitation (MR) is a term used to describe MR that is caused by stretching of the annulus secondary to
ventricular dilatation.
Main causes of MR
Myxomatous degeneration
Functional, secondary to ventricular
dilatation
Mitral valve prolapse
Ischaemic papillary muscle rupture
Congenital heart diseases
Collagen disorders
Rheumatic heart disease
Endocarditis
Indicators of the severity of MR
Small-volume pulse
Left ventricular enlargement due to overload
Presence of S3
Atrial fibrillation
Mid-diastolic flow murmur
Precordial thrill, signs of pulmonary hypertension or congestion (cardiac failure).
Signs of predominant MR in mixed mitral valve disease
Soft S1; S3 present
Displaced and hyperdynamic apex (LV enlargement)
ECG showing LVH and left axis deviation.
Mitral valve prolapse
This condition occurs in 5% of the population and is commonly over-diagnosed (depending on the echocardiography criteria
applied). The patients are usually female and may present with chest pains, palpitations or fatigue, although it is often detected
incidentally in asymptomatic patients. Squatting increases the click and standing increases the murmur, but the condition may be
diagnosed in the absence of the murmur by echocardiography. Often there is myxomatous degeneration and redundant valve
tissue due to deposition of acid mucopolysaccharide material. Mitral valve prolapse is usually eminently suitable for mitral valve
repair, although this should be undertaken only if the severity of the regurgitation associated with the condition justifies it (see
above). Several conditions are associated with mitral valve prolapse (see below), and patients with the condition are prone to
certain sequelae.
Sequelae of mitral valve prolapse:
Embolic phenomena
Rupture of mitral valve chordae
Dysrhythmias with QT prolongation
Sudden death
Cardiac neurosis.
Conditions associated with mitral valve
prolapse
Coronary artery disease
Polycystic kidney disease
Cardiomyopathy – dilated cardiomyopathy/HCM
Secundum ASD
WPW syndrome
PDA
Marfan’s syndrome
Pseudoxanthoma elasticum
Osteogenesis imperfecta
Myocarditis
SLE; polyarteritis nodosa
Muscular dystrophy
Left atrial myxoma
1.4.4 Aortic regurgitation
Aortic regurgitation (AR) can occur due to disruption of the aortic valve or the aortic root. Either can occur acutely or chronically.
Acute causes, including aortic dissection or valve rupture from endocarditis, present with acute decompensation and profound
heart failure. Chronic causes allow time for the left ventricle to accommodate, with gradual enlargement of end-diastolic volumes.
There are many echocardiographic criteria used to assess the severity of AR and none is ideal; they are usually used in
combination with symptoms and LV dimensions.
Causes of AR
Valve inflammation
Chronic rheumatic
Infective endocarditis
Rheumatoid arthritis; SLE
Hurler’s syndrome
Aortitis
Syphilis
Ankylosing spondylitis
Reiter’s syndrome
Psoriatic arthropathy
Aortic dissection/trauma
Hype rte nsion
Bicuspid aortic valve
Ruptured sinus of Valsalva’s aneurysm
VSD with prolapse of (right) coronary cusp
Disorders of collagen
Marfan’s syndrome (aortic aneurysm)
Hurler’s syndrome
Pseudoxanthoma elasticum
Eponymous signs associated with AR
Quincke’s sign – nail-bed fluctuation of capillary flow
Corrigan’s pulse – (waterhammer); collapsing radial pulse
Corrigan’s sign – visible carotid pulsation
De Musset’s sign – head nodding with each systole
Duroziez’s sign – audible femoral bruits with diastolic flow (indicating moderate
severity)
Traube’s sign – ‘pistol shots’ (systolic auscultatory finding of the femoral arteries)
Austin Flint murmur – functional mitral diastolic flow murmur
Argyll Robertson pupils – aetiological connection with syphilitic aortitis
Müller’s sign – pulsation of the uvula
Indications for surgery
Acute severe AR will not be tolerated for long by a normal ventricle and therefore requires prompt surgery, except in the case of
infection, where delay for antibiotic therapy is preferable (if haemodynamic stability allows). At 10 years, 50% of patients with
moderate chronic AR are alive, but once symptoms occur deterioration is rapid.
Features of AR indicative of the need for surgery
• Symptoms of dyspnoea/LV failure • Reducing exercise
tolerance
Rupture of sinus of Valsalva’s aneurysm
Infective endocarditis not responsive to medical treatment
Enlarging aortic root diameter in Marfan’s syndrome with AR
Enlarging he art
End-systolic diameter >55 mm at echo
Pulse pressure >100 mmHg
Diastolic pressure <40 mmHg
Lengthening diastolic murmur
ECG: lateral lead T-wave inversion
Bicuspid valves
The aortic valve is typically trileaflet, but can be congenitally bicuspid in 0.5–1% of the population. This abnormal valve has early
onset degeneration with aortic stenosis presenting many years sooner than typical (40–50s versus 60–70s). In cases of biscuspid
disease aortic regurgitation is more common. Bicuspid valves are also associated with an aortopathy and aortic root dilatation,
which itself can lead to aortic regurgitation.
1.4.5 Aortic stenosis
Aortic stenosis (AS) is generally caused by senile degeneration and thickening of normally thin
2 2
pliable leaflets. A normal valve area is >2cm and severe AS typically has a valve area ≤1 cm , with a mean pressure gradient of
>40 mmHg on transthoracic echocardiography. The pressure gradient is dependent not only on the severity of the stenosis, but
also on the speed that blood is pushed across the valve. This means that the pressure gradient can be reduced when LV function
is impaired, or in MS or significant AR. In these cases, the dimensionless index, a ratio of aortic and LVOT velocities, can be useful
(1 is normal, 0.25 severe).
Causes of AS: may be congenital bicuspid valve, degenerative calcification (common in elderly people) and post-rheumatic
disease
Subvalvular: causes of aortic gradients include HCM and subaortic membranous stenosis,
whereas supravalvular stenosis is due to aortic coarctation, or Williams’ syndrome (with elfin
facies, learning disability, hypercalcaemia)
Sudden death: may occur in AS or subvalvular stenosis due to ventricular tachycardia. The vulnerability to ventricular tachycardia
is due to LVH
Complete heart block: may be due to calcification involving the upper ventricular septal
tissue housing the conducting tissue. This can also occur post-operatively (after valve
replacement) due to trauma
Calcified emboli: can arise in severe calcific AS
All symptomatic patients should be considered for surgery: surgical mortality rate for AS is predominantly related to the
absence (2–8%) or presence (10–25%) of LV failure
There is a strong association with ischaemic heart disease: 50% of AS patients have
important coronary disease. Concomitant CABG should be considered at the time of valve
replacement.
•
•
•
Indicators of severe AS
Symptoms of syncope or LV failure
Signs of LV failure
Absent A2
Paradoxically split A2
Presence of precordial thrill
S4
Slow-rising pulse with narrow pulse
pressure
Late peaking of long murmur •
2
Valve area >0.5 cm on echocardiography
1.4.6 Tricuspid regurgitation
Tricuspid regurgitation (TR) is typically an inaudible murmur due to the low pressure in the right heart, but may have low frequency
pansystolic murmur if right ventricular pressures are elevated. Other signs may be more prominent with an elevated JVP and giant
c-V waves; a pulsatile liver edge may be palpable and peripheral oedema is invariable.
Causes of severe TR include the following:
Functional, due to right ventricular dilatation (commonly coexists with significant MR)
Infection: the tricuspid valve is vulnerable to infection introduced by venous cannulation (iatrogenic or through intravenous drug
abuse)
Carcinoid (nodular hepatomegaly and telangiectasia)
Post-rheumatic
Ebstein’s anomaly: tricuspid valve dysplasia with a more apical position to the valve. Patients
have cyanosis and there is an association with pulmonary atresia or ASD and, less commonly,
congenitally corrected transposition.
1.4.7 Prosthetic valves
Valve prostheses may be metal or tissue. Mechanical valves are more durable but tissue valves do not require full lifelong
anticoagulation. All valve replacements have a residual transvalvular gradient across them; for mechanical valves this can cause
loud murmurs, eg an aortic valve replacement may still have a loud AS ejection systolic murmur.
Mechanical valves
There are many different types of mechanical valves. Common ones are:
Ball & Cage valve: e.g Starr–Edwards: ball and cage – ejection systolic murmur (ESM) in the
aortic area and an opening sound in the mitral position are normal. These are the original
mechanical valves, first implanted in 1961, and modified versions are still available
Single tilting disc: Bjork–Shiley was the first, involving a single graphite disc coated in pyrolite carbon which tilts between struts of
metal housing. A variation designed in the 1980s was prone to strut fracture with catastrophic embolisation and is now no longer
manufactured. A modern variant includes the Medtronic Hall valve
Bileaflet valves: two semicircular leaflets that open, creating a central and two peripheral
orifices. Now the most commonly used valve type with many different manufacturers, including
St Jude and the Sorin Carbomedics valve.
Tissue valves
Allografts: porcine or bovine three-cusp valve – 3 months’ anticoagulation sometimes
recommended until tissue endothelialisation. No need for long-term anticoagulation if patient is
in sinus rhythm
Homografts: usually cadaveric and, again, need no long-term anticoagulation.
•
•
Infection of prosthetic valves
Mortality rate is still as high as 60% depending on the organism
Within 6 months of implantation, it is usually due to colonisation by Staphylococcus epidermidis
Septal abscesses may cause PR-interval lengthening
Valvular sounds may be muffled by vegetations; new murmurs may occur
Mild haemolysis can occur, and is detected by the presence of urobilinogen in the urine
Dehiscence is an ominous feature requiring urgent intervention.
Anticoagulation in pregnancy
Warfarin may cause fetal haemorrhage and has a teratogenicity risk of 5–30%. This risk is dose dependent and abnormalities
include chondrodysplasia, mental impairment, optic atrophy and nasal hypoplasia. The risk of spontaneous abortion may be
increased. There is no agreed consensus on the ideal strategy: warfarin, unfractionated heparin and low-molecular-weight heparin
all have advocates and detractors.
1.4.8 Infective endocarditis
Clinical presentation
Commonly presents with non-specific symptoms of malaise, tiredness and infective-type symptoms. Heart failure secondary to
valvular regurgitation or heart block may also occur, as may an incidental presentation in the context of another primary infection.
Signs of infective endocarditis
As well as cardiac murmurs detected at auscultation, there are several other characteristic features of infective endocarditis:
Systemic signs of fever and arthropathy
Hands and feet: splinter haemorrhages, Osler’s nodes (painful), Janeway’s lesions (painless) and clubbing (late); needle-track
signs may occur in arm or groin
Retinopathy: Roth’s spots
Hepatosplenomegaly
Signs of arterial embolisation (eg stroke or digital ischaemia)
Vasculitic rash
Streptococcus viridans (α-haemolytic group) are still the most common organisms, occurring in 50% of cases
Marantic (metastatic-related) and SLE-related (Libman–Sacks) endocarditis are causes of non- infective endocarditis
Almost any pathogenic organism may be implicated, particularly in immunocompromised patients.
•
•
•••
See also Section 1.4.7 on ‘Prosthetic valves’ and Table 1.4. Table 1.4 Infective endocarditis
Groups affected by endocarditis
Chronic rheumatic disease No previous valve disease Intravenous drug abuse Congenital defects Prosthetic
Management of infective endocarditis
Percentage of all cases of endocarditis
30 40 10 10 10
The aim of treatment is to sterilise the valve medically (usually 4–6 weeks of intravenous antibiotics), then assess whether the
valvular damage sustained (eg degree of incompetence) or the risk of recurrence (eg if prosthetic valves) mandates surgical
replacement. Earlier operations are undertaken only if clinically necessary as outcomes are poorer.
Poor prognostic factors in endocarditis
Prosthetic valve
Staphylococcus aureus infection
Culture-negative endocarditis
Depletion of complement levels
Indications for surgery
Cardiac failure or haemodynamic compromise
Extensive valve incompetence
Large vegetations
Septic emboli
Septal abscess
Fungal infection
Antibiotic-resistant endocarditis
Failure to respond to medical therapy
Antibiotic prophylaxis
The conditions listed in the next box are associated with an increased risk of endocarditis.
Acquired valvular heart disease with stenosis or regurgitation
Valve replacement
Structural congenital heart disease, including surgically corrected or palliated structural conditions, but excluding isolated ASD, fully
repaired VSD or fully repaired PDA, and
•
closure devices that are judged to be endothelialised
Previous infective endocarditis
HCM
Antibiotic and chlorhexidine mouthwash prophylaxis is no longer recommended for dental procedures, endoscopies or obstetric
procedures.
Patients should be made aware of non-medical riskprone activities (eg intravenous drug use, piercings) and the symptoms of
possible endocarditis.
1.5 CONGENITAL HEART DISEASE
Causes of congenital acyanotic heart
a
disease
With shunts
Aortic coarctation (with VSD or PDA)
VSD
ASD
PDA
Partial anomalous venous drainage (with ASD)
Without shunts
Congenital AS
Aortic coarctation
a
Associated shunts
Causes of cyanotic heart disease
With shunts
Tetralogy of Fallot (VSD)
Severe Ebstein’s anomaly (ASD)
Complete transposition of great vessels (ASD, VSD/PDA)
Without shunts
Tricuspid atresia
Severe pulmonary stenosis
Pulmonary atresia
Hypoplastic left heart
1.5.1 Atrial septal defect
In ASDs, the interatrial septum may be defective or absent, allowing mixing of oxygenated and deoxygenated blood and shunting at
the atrial level. The size of shunting and reduction in oxygenation will depend upon the size of the defect.
ASDs are the most common congenital defects found in adulthood. Rarely, they may present as stroke in young people, due to
paradoxical embolus that originated in the venous system and reached the cerebral circulation via right-to-left shunting. Fixed
splitting of the second heart sound is the hallmark of an uncorrected ASD. There may be a left parasternal heave and a pulmonary
ESM due to increased blood flow. There are three main subtypes:
Secundum (70%): central fossa ovalis defects often associated with mitral valve prolapse (10–20% of cases). ECG shows
incomplete or complete RBBB with right axis deviation. Note that a PFO (slit-like deficiency in the fossa ovalis) occurs in up to 25%
of the population, but this does not allow equalisation of atrial pressures, unlike ASD
Primum (15%): sited above the AV valves, often associated with varying degrees of MR and TR and occasionally a VSD, and thus
usually picked up earlier in childhood. ECG shows RBBB, left axis deviation and first-degree heart block. Associated with Down’s,
Klinefelter’s and Noonan’s syndromes
Sinus venosus (15%): defect in the upper septum, often associated with anomalous pulmonary venous drainage directly into the
right atrium.
Surgical closure is recommended with pulmonary: systolic flow ratios >1.5 : 1. Closure of secundum defects may be performed via
cardiac catheterisation.
Holt–Oram syndrome (triphalangeal thumb with ASD): a rare syndrome (autosomal dominant with incomplete penetration). It is
associated with absence (or reduction anomalies) of the upper arm.
Lutembacher’s syndrome: a rare combination of an ASD with mitral stenosis (the latter is probably rheumatic in origin).
Investigations forASDs
Right atrial and right ventricular dilatation may be seen on any imaging technique, as may pulmonary artery conus enlargement.
Other characteristic features are:
• Chest radiograph: pulmonary plethora
Echocardiogram: paradoxical septal motion, septal defect and right-to-left flow of contrast during venous injection with Valsalva’s
manoeuvre
Catheterisation: pulmonary hypertension – raised right ventricular pressures and step-up in oxygen saturation between various
parts of the right circulation (eg SVC to high right atrium).
Treatment of ASD
There is no specific medical therapy for ASDs; they are managed by either closure (percutaneous or surgical) or clinical and
echocardiographic follow-up.
•
•
•
••
Indications for closure:
Symptoms (dyspnoea)
Systemic embolism (typically stroke)
Chamber dilatation
Elevated right heart pressures
Significant left-to-right shunt
Systemic embolism (typically stroke).
Patent foramen ovale
A PFO is a channel within the interatrial septum, which is typically covered by a flap that opens to allow right-to-left communication
when right-sided pressures elevate, such as when coughing or sneezing, or during Valsalva’s manoeuvre. During fetal
development and while the placenta provides oxygenation, the interatrial septum allows communication through the foramen ovale.
Upon birth, the reduction in resistance in lungs lowers right-sided pressure, and the septum primum shuts, sealing the foraman
ovale. In a quarter of adults, this closure is incomplete allowing venous embolic material to cross into the arterial circulation. There
may be a history of headaches, migraine or paradoxical embolism. Stroke may occur in young patients. PFOs are visualised on
echocardiography using bubble contrast, by injecting agitated saline into a peripheral vein, which is then seen under
echocardiography travelling from the right to the left atrium during Valsalva’s manoeuvre. Debate continues about the value of
closing PFOs, which can be performed either percutaneously or surgically, but it may be considered according to the clinical
scenario, and particularly after stroke in a young patient.
1.5.2 Ventricular septal defect
The ventricular septum is made of two parts with a superior membranous component, which contains the AV node (AVN), and an
inferior muscular component. Defects can occur in either. Most defects are small, requiring only conservative observation. VSDs
are the most common isolated congenital heart defect (2/1000 births; 30% of all congenital defects). Membranous VSDs can be
more complicated due to the AVN and proximity of the aortic apparatus. Spontaneous closure is more common in muscular defects
but either can be closed by a percutaneous or surgical approach.
Indications for closure
Significant left-to-right shunt
Associated with other defect requiring cardiotomy
Elevated right heart pressure causing pulmonary
hypertension
Endocarditis
Membranous VSD causing AR
• Large defects allow significant left-to-right shunt, causing elevated right heart pressures and consequent pulmonary hypertension
Parasternal thrill and pansystolic murmur are present. The murmur may be ejection systolic in very small or very large defects. With
large defects the aortic component of the second sound is obscured, or even a single/palpable S2 is heard; a mitral diastolic
murmur may occur. The apex beat is typically hyperdynamic.
Once Eisenmenger’s complex develops, the thrill and left sternal edge (LSE) murmur abate and signs are of pulmonary
hypertension regurgitation and right ventricular failure. Surgery should occur earlier to avoid this situation, otherwise a combined
heart/lung transplantation would be required.
•
Other cardiac associations of VSD
PDA (10%)
AR (5%)
Pulmonary stenosis
ASD
Tetralogy of Fallot
Coarctation of the aorta
Types of VSD
Muscular
Membranous
AVdefect
Infundibular
Into the right atrium (Gerbode’s defect)
1.5.3 Patent ductus arteriosus
PDA is common in premature babies, particularly female infants born at high altitude, and also if maternal rubella occurs in the first
trimester. The connection occurs between the pulmonary trunk and the descending aorta, usually just distal to the origin of the left
subclavian artery. PDA often occurs with other abnormalities.
Key features of PDA
A characteristic left subclavicular thrill
Enlarged left heart and apical heave
Continuous ‘machinery’ murmur
Wide pulse pressure and bounding
pulse
Signs of pulmonary hypertension and Eisenmenger’s syndrome develop in about 5% of cases. Indometacin closes the duct in
about 90% of babies whereas intravenous prostaglandin E1 (PGE1)
may reverse the natural closure (useful when PDA is associated with coarctation or hypoplastic left heart syndrome, and in
complete transposition of the great vessels, because it will help to maintain flow between the systemic and pulmonary circulations).
The PDA may also be closed thoracoscopically or percutaneously.
1.5.4 Coarctation of the aorta
Coarctation is an aortopathy, a disease of the aorta, in which there is severe narrowing at the site of the regressed ductus
arteriosus, which connects the aorta to the pulmonary artery in utero. If severe, aortic blood flow is impaired, causing heart failure
and metabolic acidosis; it is life-threatening in early life. PGE1 can keep the ductus arteriosus patent to allow right-to-left shunting
to the descending
aorta while awaiting surgery.
Milder coarctation may present beyond infancy with hypertension, leg cramps, muscle weakness and neurological changes. Pulses
distal to the obstruction are diminished and delayed with lower blood pressure in the legs. Collateral development can be significant
and audible posteriorly, and may cause ‘notching’ of ribs on chest radiographs. Barium swallows may demonstrate oesophageal
compression from the post-stenotic dilatation of the aorta. Echocardiography may be sufficient but MRI is definitive.
Treatment can be surgical or percutaneous. End-to-end anastomosis is the preferred surgical technique but re-stenosis can occur.
Balloon dilatation of the coarctation is typically focused on recurrent coarctation after surgery.
Complications may occur despite repair with 30% of patients with surgically corrected coarctation remaining significantly
hypertensive with end-organ harm. Heart failure, re-coarctation at a new site in the aorta and aneurysm formation at the site of
repair are other complications. Berry aneurysms and bicuspid aortic valves should be sought as there are strong associations with
coarctation.
Associations of coarctation
Cardiac
Bicuspid aortic valve (and thus AS ± AR) in 10–
20%
PDA
VSD
Mitral valve disease
Non-cardiac
Berry aneurysms (circle of Willis)
Turner’s syndrome
Renal abnormalities
Signs of coarctation
• Hypertension
Radiofemoral delay of arterial pulse
Absent femoral pulses
Mid-systolic or continuous murmur (infraclavicular)
Subscapular bruits
Rib notching on chest radiograph
Post-stenotic aortic dilatation on chest radiograph
1.5.5 Eisenmenger syndrome
Eisenmenger syndrome refers to any untreated congenital cardiac defect with intracardiac communication that leads to severe
irreversible pulmonary hypertension, reversal of left-to-right shunts, and cyanosis. Long-standing left-to-right shunts (eg in large
VSDs, ASDs or PDAs) cause remodelling of the pulmonary microvasculature, with obstruction and pulmonary blood flow and raised
pressures. This causes shunts to reverse, sending deoxygenated blood into the systemic circulation, which is evident as cyanosis.
Signs of development include:
Clubbing and central cyanosis
Decrease of original pansystolic (left-to-right) murmur
Decreasing intensity of tricuspid/pulmonary flow murmurs
Single S2 with louder intensity, palpable P2; right ventricular heave
Appearance of Graham Steell murmur due to pulmonary regurgitation
Pansystolic murmur and v waves due to TR
Eisenmenger syndrome
Cause s
VSD (Eisenmenger’s complex)
ASD
PDA
Complications of Eisenmenger
syndrome
Right ventricular failure
Massive haemoptysis
Cerebral embolism/abscess
Infective endocarditis (rare)
1.5.6 Tetralogy of Fallot
The most common cause of cyanotic congenital heart disease (10%), usually presenting after age 6
months (as the condition may worsen after birth).
Key features
Pulmonary stenosis (causes the systolic
murmur)
Right ventricular hypertrophy
VSD
Overriding of the aorta
Right-sided aortic knuckle (25%)
Clinical features
Cyanotic attacks (pulmonary infundibular spasm)
Clubbing
Parasternal heave
Systolic thrill
Palpable A2
Soft ejection systolic murmur (inversely related to pulmonary
gradient)
Single S2 (inaudible pulmonary closure)
ECG features of right ventricular hypertrophy
Possible complications of Fallot’s
tetralogy
Endocarditis
Polycythaemia
Coagulopathy
Paradoxical embolism
Cerebral abscess
Ventricular arrhythmias
Fallot’s tetralogy is a spectrum disorder with clinical manifestations depending on the severity • of the cardiac lesions. Typical
presentation is with small for dates, difficulty feeding, failure to
thrive and episodes of cyanosis when crying or feeding; clubbing is evident from 3–6 months
Although classically considered to be only pulmonary stenosis, the principal lesion is right ventricular outflow tract obstruction,
which can be valvular and/or infundibular. High-grade obstruction increases right-sided pressures, which may exceed LV pressure,
promoting right- to-left shunting through the VSD, while simultaneously reducing pulmonary flow. Catecholamines and hypoxaemia
trigger spasm of the infundibulum
•
Cyanotic episodes occur in infants, triggered by exercise, anxiety, dehydration, fever, anaemia, sepsis or spontaneously. The infant
becomes inconsolable and cyanosis and tachypnoea ensues
Cyanotic attacks worsen with catecholamines, hypoxia and acidosis. The murmur lessens or disappears as the right ventricular
outflow gradient increases
During cyanotic episodes, emergency treatment is necessary to avoid death. Parents should
hold the infant against their shoulder, tucking the infant’s knees up; this increases systemic
• vascular resistance and reduces venous return of acidotic blood from the lower extremities,
which reduces right ventricular infundibular spasm and right ventricular pressure, so breaking the cycle. In older children, squatting
achieves this.
Emergency treatment is necessary.
The presence of a systolic thrill and an intense pulmonary murmur differentiates the condition from Eisenmenger’s syndrome
Traditionally, tetralogy of Fallot underwent a palliative procedure, such as the Blalock–
Taussig shunt (the modern variant connects a graft between the subclavian artery and the
• pulmonary artery) with later complete resection. Modern practice favours total correction
before 12 months. Infants may be stabilised on prostaglandins to maintain the patency of the ductus arteriosus and enable more
elective surgery
A Blalock-Taussigh shunt operation results in weaker pulses in the arm from which the subclavian artery is diverted to the
pulmonary artery.
1.5.7 Important post-surgical circulations
Systemic right ventricle
Transposition of the great vessels and similar conditions in which the right ventricle supplies the aorta and the left ventricle supplies
the pulmonary artery are now treated by arterial switch. Effectively this is a complete correction.
Before the development of the arterial switch procedure, treatment was by ‘venous redirection’ – the vena cavae redirected via the
atria to the left ventricle and the pulmonary veins to the right ventricle via the atria, with the morphological right ventricle then
pumping oxygenated blood into the aorta. However, there was a high risk of ventricular dysfunction, valve regurgitation and
ventricular arrhythmias in these patients, and decompensation would be provoked by development of atrial arrhythmias.
Single ventricular circulation
Individuals born with only one functional ventricle are treated by redirecting the vena cavae directly into the pulmonary arteries
(total cavopulmonary correction) and now do very well. Early versions of this operation (the classic Fontan) used the right atrium
between the vena cavae, but this often led to atrial dilatation and then fibrillation with a risk of decompensation.
Common congenital circulations
Common congenital circulations are summarised in Table 1.5.
••
•
•

1.6 ARRHYTHMIAS AND PACING

Atrial fibrillation (AF) remains the most common cardiac arrhythmia, with incidence increasing with age (Framingham data indicate
a prevalence of 76/1000 men and 63/1000 women aged 85–94 years). Atrial flutter frequently coexists with AF and, although it has
a different immediate causal mechanism, it is a reflection of the same underlying disease. These arrhythmias assume particular
significance because of the stroke risk associated with them.
‘SVT’ (supraventricular tachycardia) is the term usually used to indicate a presumed re-entry tachycardia involving the AV node or
an accessory pathway.
Ventricular tachycardia and ventricular fibrillation are life-threatening conditions, but there is a clear evidence base for the use of
implantable cardioverter defibrillators in both primary and secondary prevention (see Appendix II). Antiarrhythmic drugs or catheter
ablation may be useful adjuncts to treatment or, in some cases, they can be used as alternatives to defibrillators.
1.6.1 Bradyarrhythmias
Any heart rate <60 beats/min is a bradycardia. A bradyarrhythmia is a pathological bradycardia. Bradyarrhythmias are considered
according to their prognostic significance and symptomatic impact. High-grade AV block (Mobitz 2 or complete) is associated with
sudden death and patients should be paced urgently even if asymptomatic. Permanent pacing is very effective in reducing
symptoms in most bradyarrhythmias; the exception is neurocardiogenic syncope where the results are disappointing.
Table 1.5 Common congenital circulations

Common bradyarrhythmias and associated conditions

Neurocardiogenic symptoms
An exaggerated vasodepressor (hypotension), cardioinhibitory (bradycardic) or mixed reflex may cause syncope or presyncope.
Various drugs have been tried as treatment, with limited success. In patients with a predominant cardioinhibitory component, dual-
chamber pacing may reduce the severity and frequency of syncopal episodes but results are often disappointing.
Sinus node disease
Sinus bradycardia and sinus pauses can cause syncope, presyncope or non-specific symptoms. Thyroid function and electrolytes
should be checked on presentation and corrected before considering pacemaker therapy. Pacing is indicated only in significantly
symptomatic cases (as there is no prognostic benefit of pacing in sinus node disease).
First-degree AV block
A PR interval >200 ms is abnormal but usually requires no treatment. The combination of first-degree AV block with (1) LBBB, (2)
RBBB with axis deviation, or (3) alternating LBBB and RBBB is interpreted as trifascicular block (more accurately, block in two
fascicles and delay in the third). If associated with syncope, trifascicular block represents an indication for pacing on both
prognostic and symptomatic grounds.
Second-degree Mobitz I (Wenckebach’s) AV block
Progressive prolongation and then block of the PR interval is categorised as Mobitz I. It may be
normal during sleep and in young, physically fit individuals (who have high vagal tone). If it occurs when the patient is awake and is
associated with symptoms in older people, pacing may be indicated on symptomatic grounds.
High-grade AV block (second-degree Mobitz II block and third-degree complete heart block)
Bradycardias with more than one P wave per QRS complex (second-degree Mobitz II) or with AV dissociation are grouped together
as high-grade AV block. Untreated, they are associated with a mortality rate that may exceed 50% at 1 year, particularly in patients
aged >80 years and in those with non-rheumatic structural heart disease. Pacing is indicated on prognostic grounds even in
asymptomatic individuals.
Complete heart block is the most common reason for permanent pacing
When related to an infarction, high-grade AV block occurs mostly with right coronary artery
occlusion, because the AV nodal branch is usually one of the distal branches of the right coronary
artery
In patients with an anterior infarct, high-grade AV block is a poor prognostic feature, indicating extensive ischaemia
Congenital cases may be related to connective tissue diseases; however, in patients with normal exercise capacities, recent
studies show that the prognosis is not as benign as was previously thought and pacing is therefore recommended in a wide range
of circumstances (see European Society of Cardiology guidelines by Vardas et al Eur Heart J 2007;28:2256–95).
Tachyarrhythmias
Tachyarrhythmias are caused by re-entry, automaticity or triggered activity:
Re-entry: the arrhythmia is anatomically dependent and usually the primary problem as opposed to sequelae of another reversible
state
Automaticity: arrhythmia is often secondary to a systemic cause (eg electrolyte imbalance, sepsis, adrenergic drive) and is
multifocal
Triggered activity: shares features of both mechanisms and is seen in both primary arrhythmias and drug toxicity.
1.6.2 Supraventricular tachycardias
There are two major groups of re-entrant tachycardias often described as SVT:
AV nodal re-entry tachycardia (AVNRT; see Figure 1.6): involves a re-entry circuit in and around the AV node
AV re-entry tachycardia (AVRT; see Figure 1.7): this involves an accessory pathway between • the atria and ventricles some
distance from the AV node
(eg WPW syndrome and related conditions).
AV nodal re-entry tachycardia
Differential conduction in tissue around the AVN allows a micro re-entry circuit to be maintained (see
•
•
•••
•

Figure 1.6), resulting in a regular tachycardia. Accessory pathways

An accessory pathway that connects the atrium and ventricle mediates the tachycardia by enabling retrograde conduction from
ventricle to atrium. More seriously, the accessory pathway may predispose to unrestricted conduction of AF from atria to ventricles
as a result of anterograde conduction through the pathway. This may lead to ventricular fibrillation.
WPW is said to be present when a δ wave (partial pathway-mediated pre-excitation) is present on the resting ECG. Associations
with WPW include: Ebstein’s anomaly (may have multiple pathways), HCM, mitral valve prolapse and thyrotoxicosis; it is more
common in men.
Some accessory pathways are not manifest by a δ wave on the resting ECG but are still able to participate in a tachycardia circuit.
Atrial tachycardias, including flutter, AF, sinus tachycardia and fascicular ventricular tachycardia, may all be mistaken for SVT.
Figure 1.6 Mechanism for atrioventricular nodal re-entry tachycardia
Figure 1.7 Mechanism for atrioventricular re-entry tachycardia
1.6.3 Atrial arrhythmias
Atrial flutter
Atrial flutter involves a macro re-entrant circuit where the electrical activation circles the right atrium. This generates characteristic
sawtoothed flutter waves, which typically have a rate between 250 and 350 beats/min, with a ventricular response of 150 beats/min
(2 : 1 block).
Amiodarone and sotalol may chemically cardiovert, slow the ventricular response or act as prophylactic agents
Radiofrequency ablation is curative in up to 95% of cases
Adenosine cannot terminate atrial flutter but can be useful in revealing it.
Atrial flutter is described as typical when associated with a sawtooth atrial pattern in the inferior leads and positive flutter waves in
V1. Atypical flutters tend to occur in congenital heart disease or after surgery or prior ablation.
The ventricular response may be slowed by increasing the vagal block of the AVN (eg carotid sinus massage) or by adenosine,
which ‘uncovers’ the flutter waves on ECG
•
• This is the most likely arrhythmia to respond to DC cardioversion with low energies (eg 25 V)
•
Atrial fibrillation
This arrhythmia is due to multiple wavelet propagation in different directions. The source of the arrhythmia may be myocardial
tissue in the openings of the four pulmonary veins, which enter into the posterior aspect of the left atrium, and this is particularly the
case in younger patients with paroxysmal AF. AF may be paroxysmal, persistent (but ‘cardiovertable’) or permanent, and in all
three states it is a risk factor for strokes. Treatment is aimed at ventricular rate control, cardioversion, prevention of recurrence and
anticoagulation. Catheter ablation is indicated in symptomatic individuals who are resistant to, or intolerant of, medical therapy.
With AF, a major decision is whether to control rate or alter the rhythm:
Surprisingly, rhythm control does not reduce the risk of stroke (indeed paroxysmal AF carries the same stroke risk as chronic AF)
and therefore does not affect the indications for anticoagulation
Cardioversions, multiple drugs and ablations are all used to alter rhythm
In asymptomatic individuals, rate control is recommended.
•
Associations with atrial fibrillation
Ischaemic heart disease
Pericarditis
Mitral valve disease
Pulmonary embolus
Hypertension
Atrial myxomas
Thyroid disease
LVH
Acute alcohol excess/chronic alcoholic
cardiomyopathy
ASD
Post-CABG
Caffeine excess
Dilated left atrium (>4.5 cm)
Pneumonia
WPW syndrome
Bronchial malignancy
The overall risk of systemic emboli is 5–7% annually (higher with rheumatic valve disease); this falls to 1.6% with anticoagulation.
TOE may exclude atrial appendage thrombus but cannot predict the development of a thrombus in the early stages post-
cardioversion; anticoagulation is therefore always recommended post-cardioversion.
• Risk factors for stroke with non-valvular AF
Previous history of cerebrovascular accident or transient ischaemic attack (risk × 22.5)
Diabetes (×1.7)
Hypertension (×1.6)
Heart failure
• Risk factors for recurrence of AF after cardioversion
Long duration (>1–3 years)
Rheumatic mitral valve disease
Left atrium size >5.5 cm
Older age (>75 years)
Left ventricular impairment
The CHADS2 risk score has been modified to CHA2DS2-VASc and includes further risk factors
(Tables 1.6 & 1.7). Previously, aspirin was considered a suitable alternative to warfarin in low-risk groups, but new studies suggest
that the protective effect is minimal with only deleterious side-effects encountered. Therefore, warfarin is the agent of choice in
most cases.
Table 1.6 The CHA2DS2-VASc risk score

CHA2DS2- VASc score

0123456789
Annual stroke risk (%/year) Suggested medication
0 Nil
Table 1.7 The CHA2DS2-VASc risk factors CHA2DS2-VASc risk factors
Congestive heart failure Hypertension
Age ≥75
Age 65–74
Diabetes mellitus
Score
11211
1.3 2.2 3.2 4.0 6.7 9.8 9.6 6.7 15.2
Aspirin or warfarin Warfarin

Stroke/TIA/thromboembolism 2 Vascular disease 1 Female gender 1

The HAS-BLED score (based on a mnemonic) helps consider a patient’s bleeding risks on anticoagulation (Table 1.8). Online
calculators can estimate the bleeding risk.
Table 1.8 The HAS-BLED score

Le tte r
H
A
SB
LE
D
Characte ristics
Hypertension
Abnormal renal and liver function
Stroke Bleeding
Labile INRs Elderly >65 years
Drugs or alcohol
De finition
Systolic BP >160 mmHg
Dialysis, renal transplantation or Cr >200 βmol/L; cirrhosis or ALT/AST more than three times upper normal limit
Previous bleeding or predisposition to bleeding
INRs out of range >40% time
Concomitant use of NSAIDs, antiplatelet agents or alcohol abuse
Score
1
1 point each (1 or 2)
11
11
1 point each (1 or 2)
ALT, alanine transaminase; AST, aspartate transaminase; Cr, creatinine; INRs, international normalised ratios; NSAIDs, non-
steroidal anti-inflammatory drugs.
1.6.4 Ventricular arrhythmias and channelopathies
Ventricular tachycardia (monomorphic) (Figure 1.8)
VT has a poor prognosis when left ventricular function is impaired. After the exclusion of reversible
causes such patients may need implantable defibrillators and antiarrhythmic therapy. • Ventricular rate is usually 120–260
beats/min
Patients should be DC cardioverted when there is haemodynamic compromise; overdrive pacing may also terminate VT
Amiodarone, sotalol, flecainide and lidocaine may be therapeutic adjuncts or prophylactic agents; magnesium may also be useful.

••
Associations of VT
Myocardial ischaemia
Hypokalaemia or severe
hyperkalaemia
Long QT syndrome (see below)
Digoxin toxicity (VT may arise from either ventricle, especially with associated hypokalaemia)
Cardiomyopathies
Congenital abnormalities of the right ventricular outflow tract (VT with LBBB and right axis deviation pattern)
•
Features favouringVT in broad-complex tachycardia
It is often difficult to distinguish VT from SVT with aberration (disordered ventricular propagation of a supraventricular impulse); VT
remains the most common cause of a broad-complex tachycardia, especially with a previous history of MI. The following ECG
observations favour VT:
Capture beats: intermittent sino-atrial (SA) node complexes transmitted to ventricle
Fusion beats: combination QRS from SA node and VT focus meeting and fusing (causes cannon waves)
RBBB with left axis deviation
Very wide QRS >140 ms
Altered QRS compared with sinus rhythm
Figure 1.8 Ventricular tachycardia can feature fusion and capture beats. Mechanisms and example ECG patterns are shown
V lead concordance with all QRS vectors, positive or negative
Dissociated P waves: marching through the VT
History of ischaemic heart disease: very good predictor
Variable S1
Heart rate <170 beats/min with no effect of carotid sinus massage.
Note: none of the above has as high a positive predictive value for VT diagnosis as a history of structural heart disease (especially
MI).
•

Ventricular tachycardia (polymorphic) – torsades de pointes

This is a particular type of VT in which the QRS complexes are of different amplitudes, appearing to ‘twist’ around the isoelectric
line, with QT prolongation when the patient is in sinus rhythm. QT prolongation can be genetic (see below) or acquired – and can
be of any cause to trigger torsades de pointes:
Antiarrhythmic agents (particularly class III, such as sotalol) may predispose to torsades de
• pointes by induced bradycardia and QT prolongation. Premature ventricular contraction (‘R-on-
T’ phenomenon) is more likely if the QT interval is very long, and can trigger torsades de pointes
+
Intravenous magnesium and K channel openers may control the arrhythmia, whereas isoprenaline and temporary pacing may
prevent bradycardia and hence the predisposition to VT.
Pro-arrhythmic channelopathies
Abnormally prolonged QT intervals may be familial or acquired, and are associated with syncope and sudden death, due to VT
(especially torsades de pointes). Mortality in the untreated symptomatic patient with a congenital abnormality is high but some
patients may reach the age of 50–60 years despite repeated attacks. Causes and associations are shown below.
•
Pro-arrhythmic causes of abnormal repolarisation (ST –
changes)
Familial
Long QT syndromes 1–5
Brugada’s syndrome
Short QT syndrome
Arrhythmogenic right ventricular dysplasia
Drugs
Quinidine
Erythromycin
Amiodarone
Tricyclic antidepressants
Phenothiazines
Probucol
Non-sedating antihistamines (eg terfenadine)
Ischaemic heart disease
Me tabolic
Hypocalcaemia
Hypothyroidism
Hypothermia
Hypokalaemia
• Rheumatic carditis

Long QT syndromes: the corrected QT is >540 ms (normal = 380–460 ms). Ninety per cent are familial, with chromosome 11
defects being common (Romano–Ward syndrome has autosomal dominant inheritance; Jervell–Lange–Nielsen syndrome is
autosomal recessive and associated with congenital deafness). Arrhythmias may be reduced by a combination of β blockers and
pacing.
Cardiac causes of electromechanical dissociation
When faced with a cardiac arrest situation it is important to appreciate the list of causes of electromechanical dissociation (EMD):
Hypoxia
Hypovolaemia
Hypokalaemia/hyperkalaemia
Hypothermia
Tension pneumothorax
Tamponade
Toxic/therapeutic disturbance
Thromboembolic/mechanical obstruction.
1.6.5 Pacing and ablation procedures
Temporary pacing
The pacing electrode is placed in the right ventricular apex causing activation from the right ventricle; as such the ECG will show
LBBB morphology. If the pacing lead has perforated the septum, and entered the left ventricle, the morphology will show RBBB.
Temporary pacing for emergencies such as heart block is typically ventricular only. Pacing post-cardiac surgery employs epicardial
pacing wires, placed at the time of surgery, and this may be atrial or ventricular (atrial appendage and right ventricular apex) to
optimise cardiac output.
Complications include:
Pneumothorax: internal jugular route is preferable to the subclavian one, because it minimises this risk and also allows control
after inadvertent arterial punctures.
Permanent pacing
Permanent pacing can be ventricular only, or atrial and ventricular to preserve AV synchrony. The naming convention reflects the
chamber paced, the chamber sensed and the pacemaker response to sensing, eg VVI means that the ventricle is paced and, when
ventricular activity is sensed, the pacemaker inhibits itself. DDD is now increasingly used, with the device capable of ‘dual’ pacing,
‘dual’ sensing and ‘dual’ response to native cardiac beats (hence the term DDD). These capabilities
Crossing the tricuspid valve during insertion, which causes ventricular ectopics, as does irritating the outflow tract
•
• Atrial or right ventricular perforation and pericardial effusion
•
mean much more sophisticated pacing behaviour can be programmed, such that different actions are taken in response to cardiac
rhythms. For example, if the pacemaker detects activity in both atria and ventricles, it is inhibited and does nothing; alternatively if
atrial activity is present but ventricular activity is absent, it will pace only the ventricle in time with the natural atrial activity. This
allows sophisticated programming to optimise the device for the patient. Most pacemakers are now also rate- responsive (denoted
by ‘R’); these use movement or physiological triggers (respiratory rate or QT interval) to increase heart rates. They reduce rates of
pacemaker syndrome and act more physiologically for active patients. Pacemaker syndrome is a constellation of symptoms related
to even subtle impairment of cardiac output or change in peripheral resistance caused by suboptimal pacemaker settings. Typically,
it can be caused by subtle differences in atrioventricular synchrony which can cause loss of the atrial ‘kick’ (atrial contribution to
cardiac output). Patients may be dizzy, hypotensive or develop signs of heart failure. Optimisation of pacemaker settings for the
individual patient is now a routine clinical activity during pacemaker checks.
Pacing in heart failure
There are several synonymous terms for pacing in patients with cardiac failure. These include ‘cardiac resynchronisation therapy’,
‘biventricular pacing’ and ‘multisite pacing’. In heart failure pacing is indicated when all of the following are present:
NYHA III–IV heart failure
QRS duration >130 ms
Left ventricular ejection fraction >35% with dilated ventricle and patient on optimal medical therapy (diuretics, angiotensin-
converting enzyme [ACE] inhibitors and β blockers).
The atria and right ventricle are paced in the usual fashion and in addition to this a pacing electrode is placed in a tributary of the
coronary sinus on the lateral aspect of the left ventricle. The two ventricles are paced simultaneously or near simultaneously with a
short AV delay. The aim is to optimise AV delay and reduce inter- and intraventricular asynchrony. This therapy is known to reduce
mortality, to improve exercise capacity, to improve quality of life and to reduce hospital admissions.
Studies have not shown benefit in heart failure patients with narrow QRS duration, and echocardiographic markers of dyssynchrony
are unreliable. A recent randomised study in which dyssynchrony markers were used to select patients for CRT instead of the
traditional parameters showed no benefit and significant harm.
Implantable cardioverter defibrillators
Implantable cardioverter defibrillators (ICDs) are devices that are able to detect life-threatening tachyarrhythmias and terminate
them by overdrive pacing or a counter-shock. They are implanted in a similar manner to permanent pacemakers. Current evidence
supports their use in both secondary prevention of cardiac arrest and targeted primary prevention (eg for individuals with LV
impairment and those with familial syndromes such as arrhythmogenic right ventricular dysplasia, Brugada’s syndrome, long QT
variants).
Radiofrequency ablation
Radiofrequency ablation is resistive, heat-mediated (65°C) protein membrane disruption causing cell
•
lysis. Using cardiac catheterisation (with electrodes in right- or left-sided chambers) it interrupts electrical pathways in cardiac
structures. Excellent results are obtained in the treatment of accessory pathways and atrial flutter, and with complete AV nodal
ablation or AV node modification. Ventricular tachycardia is technically more difficult to treat (ventricular myocardium is much
thicker than atrial myocardium).
Isolation of the pulmonary veins by ablation therapy is now an established technique to treat AF. Current cure rates are around
85%, but more than one procedure is required in half the cases. Complete heart block and pericardial effusions are rare
complications of radiofrequency ablation.
Indications to refer to an electrophysiologist
Indications for referral to an electrophysiologist are given in Table 1.9. Table 1.9 Indications for referral to an electrophysiologist

Condition
SVT
Atrial flutter
Atrial fibrillation
Ventricular fibrillation
Ventricular tachycardia Ischaemic cardiomyopathy
NYHA class III–IV heart failure, QRS >130 ms, ejection fraction <35%
When to refer
More than one episode More than one episode
Highly symptomatic, refractory to or intolerant of drug therapy
Unless there is an obvious reversible cause, eg ST-segment elevation MI (STEMI)
Unless obvious reversible cause
Ejection fraction >30% on optimal medical therapy
On optimal medical therapy
Potential treatment
Radiofrequency ablation Radiofrequency ablation
Radiofrequency ablation
ICD
Radiofrequency ablation or ICD Primary prevention ICD
Heart failure pacing
1.7 ISCHAEMIC HEART DISEASE
Ischaemic heart disease has been the leading worldwide cause of death since 1990, claiming 7 million lives a year.
Risk factors for coronary artery
disease
• Primary
Hypercholesterolaemia (LDL)
Hypertension
• Smoking
Uncle ar
Low fibre intake
Hard water
High plasma fibrinogen levels
Raised Lp(a) levels
Raised factor VII levels
Protective factors
Exercise
Moderate amounts of alcohol
Low cholesterol diet
Increased HDL:LDL
Se condary
Reduced HDL-cholesterol
Obesity
Type 1 diabetes mellitus
Type 2 diabetes
Family history of coronary artery disease
Physical inactivity
Stress and personality type
Gout and hyperuricaemia
Race (Asians)
Low weight at 1 year of age
Male sex
Chronic renal failure
Increasing age
Low social class
Increased homocystine levels and homocystinuria
HDL, high-density lipoprotein; LDL, low-density lipoprotein.
Smoking and its relationship to cardiovascular disease
Smokers have an increased incidence of the following cardiovascular complications:
Coronary artery disease
Malignant hypertension
Ischaemic stroke
Morbidity from peripheral vascular disease
Sudden death
Subarachnoid haemorrhage
Mortality due to aortic aneurysm
Thromboembolism in patients taking oral contraceptives
Both active and passive smoking increase the risk of coronary atherosclerosis by a number of mechanisms, including:
Increased platelet adhesion/aggregation and whole-blood viscosity
Increased heart rate; increased catecholamine sensitivity/release
Increased carboxyhaemoglobin level and, as a result, increased haematocrit
Decreased HDL-cholesterol and vascular compliance
Decreased threshold for ventricular fibrillation.
1.7.1 Angina
Other than the usual forms of stable and unstable angina, those worthy of specific mention include:
Decubitus: usually on lying down – due to an increase in LVEDP or associated with dreaming, cold sheets, or coronary spasm
during rapid eye movement (REM) sleep
Variant (Prinzmetal’s): unpredictable, at rest, with transient ST elevation on ECG. Due to coronary spasm, with or without
underlying arteriosclerotic lesions
••
Syndrome X: this refers to a heterogeneous group of patients who have ST-segment depression on exercise testing but
angiographically normal coronary arteries. The patients may have very- small-vessel disease and/or abnormal ventricular function.
It is commonly described in middle- aged women and oestrogen deficiency has been suggested to be an aetiological factor
•
• Vincent’s angina: nothing to do with cardiology; infection of the pharyngeal and tonsillar space!
Causes of non-anginal chest pains
Pericardial pain
Aortic dissection
Me diastinitis
• Associated with trauma, pneumothorax or diving
Ple ural
Usually with breathlessness in pleurisy, pneumonia, pneumothorax or a large peripheral pulmonary
embolus
Musculoske le tal
Gastrointe stinal
•
• Including oesophageal, gastric, gallbladder, pancreatic
• Hype rve ntilation/anxie ty
• Reproduction of sharp inframammary pains on forced hyperventilation is a reliable test
• Mitral valve prolapse
• May be spontaneous, sharp, superficial, short-lived pain
Symptomatic assessment of angina
The Canadian cardiovascular assessment of chest pain is useful for grading the severity of angina:
Grade I: angina only on strenuous or prolonged exertion
Grade II: angina climbing two flights of stairs
Grade III: angina walking one block on the level (indication for intervention)
Grade IV: angina at rest (indication for urgent intervention).
1.7.2 Myocardial infarction
Conservative estimates suggest there are 103 000 MIs per year in the UK with significant prehospital mortality, and a 5–6%
inhospital mortality rate and a 6–7% 30-day mortality rate for those surviving to hospital admission. Overall, 19% of UK deaths are
directly attributable to coronary disease.
Acute coronary syndromes
MI is part of the diagnostic entity now commonly referred to as ‘acute coronary syndrome’ (ACS). This overlapping constellation of
conditions helps identify patients at risk and needing further treatment. The chest pain is typically retrosternal or heaviness, with
radiation to the arms, neck or jaw. It can be intermittent or persistent. It may be associated with sweating, nausea, dyspnoea or
syncope. These accessory symptoms are rare in stable angina, and that pain typically settles upon resting or with nitrates and has
a predictable onset. The chest pain characteristic of an ACS is:
• Prolonged (>20 min at rest)
New-onset severe angina (Canadian Cardiovascular Society angina classification: Class III, angina symptoms with everyday
activities with moderate limitation)
•
• Destabilisation
ACS includes three distinct conditions: ST-elevation myocardial infarctions (STEMI), non-ST- elevation MIs (NSTEMIs) and
unstable angina. The diagnosis for all three requires ECG changes and an appropriate history with typical corresponding features.
An acute STEMI is when there is new ST-segment elevation (≥2 mm in two contiguous chest leads or ≥1 mm in two or more limbs).
Posterior STEMI cause dominant R waves in V1 but will reveal ST elevation only if a posterior ECG is taken. New-onset LBBB with
a typical history is also considered to be STEMI until proven otherwise. Patients are typically in pain, grey and sweaty. Most of
those having STEMI have had prodrome symptoms in the previous weeks, with only a fifth having new- onset symptoms for the last
24 hours. In STEMI, the vessel is typically entirely occluded by plaque rupture and subsequent thrombus formation. STEMI has the
highest risk of inhospital mortality.
NSTEMI includes ACS that features typical symptoms, ST depression, or new T-wave inversion and troponin rise. The spectrum of
symptoms varies considerably. Patients’ risk may be assessed using GRACE or TIMI scores; most will receive dual antiplatelet
therapy, together with statins and β blockers if appropriate. The majority of NSTEMI patients are managed invasively, typically
having inpatient angiography and PCI. Vessels are less likely to be entirely occluded in NSTEMI but the presentation is variable.
Approximately 10% of patients may be referred for CABG. Although patients can appear well, NSTEMIs have a higher mortality
than STEMI at 12 months.
Unstable angina refers to a sudden acceleration of anginal symptoms on minimal activity, either as new onset or on a background
of stable angina. ECG changes may occur, but there is no troponin rise. Patients may be medically managed but those with higher
risk characteristics may undergo invasive assessment.
Diagnosis of MI
Acute, evolving or recent MI Either one of the following criteria satisfies the diagnosis for an acute,
evolving or recent MI:
Typical rise and gradual fall (troponin) or more rapid rise and fall (CK-MB) of biochemical markers of myocardial necrosis with at
least one of the following:
Ischaemic symptoms
Development of pathological Q waves on the ECG
ECG changes indicative of ischaemia (ST-segment elevation or depression)
• Pathological finding of an acute MI (eg postmortem).
Established MI
Any one of the following criteria satisfies the diagnosis of an established MI:
Development of new pathological Q waves on serial ECGs. The patient may or may not
remember previous symptoms. Biochemical markers of myocardial necrosis may have
normalised, depending on the length of time that has passed since the infarct developed
Pathological finding of a healed or healing MI
Previous MI is also suggested when coronary artery disease and a regional ventricular wall motion abnormality are seen, or
characteristic myocardial scars are observed with MRI.
Cardiac enzymes
The widespread use of troponin assays has both simplified and lowered the bar for the diagnosis of MI. A number of markers of
cardiac damage are now available. A number of markers of cardiac damage are available, the most popular being troponin which
rises 3–12 hours after the event and falls over a week, and creatine kinase (with MB being the main isoenzyme), which rises and
falls much more quickly. In general clinical practice, troponin is the mainstay for detecting myocardial
th
damage. Consensus statements agree that troponin must rise over the 99 centile upper reference range, 12 hours after the
episode of chest pain, together with symptoms and other features of ischaemia to diagnose myocardial infarction. Troponin is
usually measured twice, on admission and at the 12-hour mark; it is this second troponin for which all study data is available.
Troponin can be
•
•
raised in other conditions (see below), and as such, it adds most clinical value when the pretest probability of MI or coronary artery
disease is highest. The level of troponin is directly related to the amount of cardiac damage and is associated with the likelihood of
later adverse outcomes.
High-sensitivity troponin has recently been made available. These can measure low level troponin concentrations within the
nanogram per litre range. These assays have revealed troponin is detectable in the plasma of healthy illness-free individuals. This
means the specificity of the test has been reduced and leads to challenges in interpreting the results. The threshold for significance
will depend upon the specific assay and manufacturer. Concerns include over-diagnosis of cardiac events with subsequent
inappropriate investigation. The potential benefit is that high-sensitivity troponin assays can detect troponin rises more quickly after
the index event, enabling the confirmed diagnosis to made on serial measurements performed within 2-3 hours of each other.
Troponin rise in other conditions
Troponin assays are now incredibly sensitive and can even detect fragments of the troponin molecule. Troponin is more closely
linked to states of myocardial hypoperfusion rather than coronary occlusion as such, and it can leak from cardiomyocytes by any
state that increases myocardial membrane permeability, even without true cell necrosis. There are a multitude of conditions that
cause troponin leak (see box) and therefore high pretest probability, together with clear symptoms and new acute ECG changes, is
mandatory to make a diagnosis of MI.
Conditions that cause troponin leak
Critical illness – intensive care unit/sepsis
Hypotension
Hypertensive crisis/pre-eclampsia
Pulmonary embolism
Infective exacerbations of COPD
Abdominal aortic aneurysm rupture
Gastrointestinal bleeding
Chemotherapy: some directly cardiotoxic
Renal impairment
Neurological conditions: stroke, subarachnoid haemorrhage and
seizures
The advert of thrombolysis and then, primary percutaneous coronary intervention (PPCI) has greatly reduced complication rates.
Late presentation (with completed infarctions) or failed PPCI in the STEMI setting is still associated with significant complications
and the location of the infarct is related to the type seen.
Troponin assays in patients with renal failure
The troponin level may be elevated simply because a patient has renal failure. In patients with renal failure who present with chest
pain, it is helpful to assess the troponin level at baseline as well as 12
h after the onset of symptoms, and sometimes at later time points. In these circumstances only a rising troponin level would be
suggestive of ischaemic myocardial damage.
Complications of MI
Since the advent of thrombolysis, complication rates have been reduced (eg halved for pericarditis, conduction defects, ventricular
thrombus, fever, Dressler’s syndrome). All complications may be seen with any type of infarction, but the following are the most
common associations:
• Complications of anterior infarctions
Late VT/VF
Left ventricular aneurysm
a
Left ventricular thrombus and systemic embolism (usually 1–3 weeks post-MI)
•
• Complete heart block (rare)
Ischaemic mitral regurgitation
Congestive cardiac failure
Cardiac rupture – usually at days 4–10 with EMD
VSD with septal rupture
Pericarditis and pericardial effusion (Dressler’s syndrome with high erythrocyte
sedimentation rate [ESR], fever, anaemia, pleural effusions and anti-cardiac muscle
antibodies are seen occasionally)
• Complications of inferior infarctions
Higher re-infarction rate
Inferior aneurysm – with mitral regurgitation (rare)
Pulmonary embolism (rare)
Complete heart block and other degrees of heart block
Papillary muscle dysfunction and mitral regurgitation
Right ventricular infarcts need high filling pressures (particularly if posterior extension)
a
Although warfarin provides no general benefit, it may reduce the overall cerebrovascular accident rate (1.5–3.6%) in those
patients with ECG demonstrable mural LV thrombus after a large anterior MI, so recommended for up to 6 months after the
infarction.
Heart block and pacing after MI
Temporary pacing is indicated in anterior MI complicated by complete heart block. This presentation is associated with high
mortality due to the extensive myocardial damage affecting the AVN.
The right coronary artery is the dominant vessel (supplies the SA and AVN) in 85% of cases. Occlusion of the right coronary artery
can cause complete heart block. The decision to temporarily pace a patient with inferior infarction is primarily dictated by the
patient’s haemodynamic status. Atropine and isoprenaline may be used but most often rapid PPCI with restoration of blood flow to
the right coronary artery will resolve heart block. A temporary pacing wire may be placed in the acute situation to provide cover. An
observational period post-MI is appropriate to allow the return
of sinus rhythm before considering permanent pacing.
1.7.3 PPCI for STEMI
PPCI has replaced thrombolysis for most patients with STEMI in the UK. Centres providing PPCI services aim to take patients
presenting within 12 hours of the onset of symptoms directly from the ambulance to the catheter laboratory, which has extensive
resuscitation facilities, to reduce ‘door-to- balloon’ times. An earlier invasive strategy is patients being assessed and, if meeting the
diagnostic criteria of STEMI, will be consented for an immediate procedure. Urgent antiplatelets are given orally (aspirin 300 mg,
and a second antiplatelet clopidogrel 600 mg, or prasugrel 60 mg or ticagrelor 180 mg), in addition to analgesia (Table 1.10).
Arterial access is via the radial artery or femoral artery, and diagnostic angiographic images will typically reveal the occluded culprit
vessel. Intraprocedure heparin or heparin-like drugs and/or bivalirudin (a direct thrombin inhibitor) may be used; adjunctive agents
such as glycoprotein IIb/IIIa (GPIIb/IIIa) agents may be considered. If technically feasible, thrombus aspiration may be performed,
but often flow is restored by the process of passing an intracoronary wire: this is accompanied by relief of pain and haemodynamic
stabilisation. Culprit lesions are then stented, typically with the latest generation drug-eluting stents provided that there are no
contraindications. Patients are cared for on a coronary care unit environment post-procedure.
PPCI has the greatest value if performed early and should be considered in all patients presenting within 12 hours of pain. Those
who present after 12 hours should also be considered if there is ongoing pain or ECG evidence of ongoing ischaemia.
Thrombolysis
Thrombolysis is still used in many clinical settings worldwide where access to PPCI is limited. It is most effective if given within 6
hours of symptom onset, with the greatest benefit seen in the highest- risk patients (STEMI). Multiple agents are available,
including streptokinase, alteplase (tissue plasminogen activator or tPA), reteplase (recombinant or rPA) and tenecteplase (TNK-
tPA). Recanalisation occurs in 70% of patients (compared with 15% without thrombolysis). Reperfusion arrhythmias (VTs and
ectopics) are common in the first 2 hours after thrombolysis.
Although now uncommon, examination questions may consider the contraindications to thrombolysis and these are given below.
Contraindications to thrombolysis
• Absolute contraindications
Active internal bleeding or uncontrollable external bleeding
Suspected aortic dissection
Recent head trauma (<2 weeks)
Intracranial neoplasms
History of proved haemorrhagic stroke or cerebral infarction <2 months
earlier
Uncontrolled high BP (>200/120 mmHg)
• Pregnancy
• Relative contraindications
Traumatic prolonged cardiopulmonary resuscitation
Bleeding disorders
Recent surgery
Probable intracardiac thrombus (eg AF with mitral stenosis)
Active diabetic haemorrhagic retinopathy
Anticoagulation or international normalised ratio or INR >1.8
There is now a good evidence base for a range of pharmacological treatments in patients presenting with acute coronary
syndromes (Table 1.10). These are aimed at dispersing clot (aspirin, clopidogrel, heparin), preventing arrhythmias (β blockers),
stabilising plaque (statins) and preventing adverse remodelling.
1.7.4 Coronary artery interventional procedures
Percutaneous coronary intervention
PCI permits rapid and low-risk coronary revascularisation, which can be performed as an emergency (for STEMI), urgently (for
NSTEMI or unstable angina) or electively (for stable angina, failed on medical therapy and with evidence of ischaemia). The
principles are similar. Arterial access, either radial or femoral, permits catheter intubation of the coronary arteries and assessment
of stenoses on fluoroscopy. Stenoses can be further assessed using imaging (to assess anatomical severity with IVUS or OCT) or
functionally (to assess physiological significance of a stenosis using a pressure wire). Stenoses selected for intervention are then
transversed using torquable intracoronary wires, before the stenosis is balloon-dilated and stented.
Plain balloon angioplasty (POBA) is successful in relieving stenoses, but vessel recoil can cause acute occlusion which causes
endothelial injury and triggers hyperplasia; this in turn leads to ‘re- stenosis’ – typically visible by 3 months. Bare-metal stents
overcame these problems but can still undergo ‘instent re-stenosis’, where hyperplastic endothelium develops over the metal struts.
This can be difficult to treat. Drug-eluting stents are coated in anti-mitotic agents to block smooth muscle and fibrous tissue
proliferation and significantly reduce re-stenosis. However, this also means that metallic struts are exposed to blood for longer and
dual antiplatelet therapy (typically aspirin and clopidogrel) is mandatory for 12 months.
Lesions particularly amenable to PCI include those that are discrete, proximal, non-calcified, unoccluded, away from side branches
and occur in patients with a short history of angina. Although there is a small acute occlusion rate, these can usually be managed
successfully with intracoronary stenting, such that the need for emergency CABG has fallen to <1%
More challenging lesions include those within grafts, at bifurcations, calcified or long lesions,
• and those within small vessels. People with diabetes have poorer outcomes compared with those
who don’t have diabetes
Almost any lesion can be stented, including left main stem disease, three-vessel disease and even
•
chronic total occlusions, but careful evaluation and discussion with the patient are necessary, as in many situations bypass grafting
may offer patients greater freedom from repeat procedures and may have better prognostic outcome.
a
Table 1.10 Summary of clinical trials in patients with acute myocardial infarction (MI)
•
Coronary artery bypass grafting
CABG has benefits over coronary angioplasty in specific groups of patients with chronic coronary artery disease (when compared
with medical therapy alone). Analysis has previously been limited because randomised trials included small numbers and were
performed several decades ago; patients studied were usually men aged <65 years. The population now receiving CABG has
changed, but so has medical therapy. The debate of CABG versus PCI is similarly complex. The recent FREEDOM trial compared
CABG with PCI in people with diabetes with advanced coronary disease: they found that CABG had a superior reduction in a
composite of mortality and myocardial infarction at 1 year compared with PCI.
Prognostic benefits are shown for symptomatic, significant left main stem disease (Veterans’
Study), symptomatic proximal three-vessel disease and two-vessel disease that includes the
proximal left anterior descending artery (CASS data)
Patients with moderately impaired LV function show greater benefit, but those with poor LV
function have greater surgical mortality. Overall mortality rate is <2%, rising to between 5% and
10% for a second procedure. Eighty per cent of patients gain symptom relief
Perioperative vein graft occlusion remains around 10%, with 1-2% per year for the next 6 years
and then 5% per year thereafter. Intrathoracic arterial grafts (LIMA, RIMA) have much better
patency, with some studies suggesting 98% patency at 10 years. However, occlusion rates vary
considerably between studies and centres. On-pump and off-pump surgery and the use of adjuvant medications during the surgery
contribute, as well as surgical technique
A ‘Dressler-like’ syndrome may occur up to 6 months post-surgery
Minimally invasive CABG involves the redirection of internal mammary arteries to coronary vessels without the need for cardiac
bypass and full sternotomy incisions (often termed ‘off- pump’ coronary revascularisation). Recovery times after this procedure are
shorter than for conventional surgery but the procedures are technically more challenging
Post-MI rehabilitation
After MI, patients are typically kept in hospital for 5 days, but this will depend upon the management approach taken. Patients
should usually take 2 months off work and have 1 month’s abstinence from sexual intercourse and driving (see following text).
Cardiac rehabilitation is particularly important
•
for patient confidence. Depression occurs in 30% of patients. Patients who are fully revascularised or invasively investigated and
found to have no ongoing ischaemic focus may be discharged after 3 days and be rehabilitated more rapidly.
Fitness to drive
The DVLA (Driver and Vehicle Licensing Agency) provides extensive guidelines for coronary disease and interventions. Their
website (www.dvla.gov.uk) should be consulted, especially with regard to class 2 licences (for vehicles >3500 kg, minibuses and
buses) but the essential points are given in Table 1.11.
1.8 HEART FAILURE AND MYOCARDIAL DISEASES 1.8.1 Cardiac failure
Cardiac failure can be defined as the pumping action of the heart being insufficient to meet the circulatory demands of the body (in
the absence of mechanical obstructions). A broad echocardiographic definition is of an ejection fraction (EF) <40% (as in the SAVE
trial, which enrolled patients for ACE inhibitors post-MI). Overall 5-year survival rate is 60% with EF <40%, compared with 95% in
those with EF >50%.
The most common single cause of cardiac failure in the Western world is ischaemic heart disease (IHD).
• Hypertension is also a very frequent cause – either acting alone or in combination with IHD. Table 1.11 Fitness to drive

Condition
Solitary loss of consciousness likely to be of cardiovascular origin, but not confirmed, or likely vasovagal syncope
Cardiac catheter procedure (including angiography, percutaneous coronary intervention, electrophysiological studies/ablation)
Myocardial infarction Permanent pacemaker
Prophylactic ICD Secondary prevention ICD
Driving restriction
6 months from last episode or until effective treatment is given
1 week
1 month
1 month
1 month 6 months
Notes
Clear vasovagal events that occur only when the patient is erect do not preclude driving
Should be able to perform emergency stop unhindered
Only 1 week if the patient has never been syncopal
No clinical arrhythmia or syncope DVLA must be informed

ICD shock therapy

6 months
Unless an inappropriate shock is preventable by reprogramming or intervention, eg a change in the detection or therapy
programming to avoid shocks for sinus tachycardia or atrial arrhythmias

DVLA, Driver and Vehicle Licensing Agency; ICD, implantable cardioverter defibrillator.
EF is only a guide to cardiac function, which also depends on other factors including preload, afterload and tissue demand.
However, EF is reduced in patients with systolic heart failure.
Preload: will affect LVEDP
Afterload: will affect LV systolic wall tension
Other echocardiographic features of LV dysfunction include reduced fractional shortening, LV enlargement and paradoxical septal
motion.
The NYHA classification is a helpful indication of severity (Table 1.12). Heart Failure with Normal or Preserved Ejection
Fraction (HF-PEF)
Sometimes called ‘Diastolic Heart Failure’, this increasingly recognised condition should be considered in patients with
breathlessness but no signs of fluid overload. It can overlap with systolic heart failure (being a concomitant problem) or be a distinct
diagnosis. The principal problem is of impaired cardiac relaxation due to increased ventricular stiffness. This results in poor cardiac
filling and elevated diastolic pressures in the heart and lungs causing dyspnoea. Patients will typically have elevated BNP levels,
have no evidence of valve disease and have preserved systolic ejection fraction. Cardiac contractility may be reduced but since this
is poorly reflected by the ‘ejection fraction’, then that itself may be within the normal range. The diagnosis is made using a number
of echocardiographic volume and Tissue Doppler parameters that suggest it, but alone are not specific. A further difficulty is that
some of these parameters change naturally with age and they are poorly reproducible over time, meaning there is no single
parameter to confirm the diagnosis. Invasive tests and other imaging such as MRI have no role at present.
Table 1.12 New York Heart Association (NYHA) classification of heart failure
NYHA class
I II III IV
Symptoms
Asymptomatic with ordinary activity Slight limitation of physical activities Marked limitation of physical activities Dyspnoeic
symptoms at rest
One-year mortality rate (%)
5–10
15
30
50–60

No treatment has been shown to reduce the morbidity or mortality

mainstay, to reduce sodium and water retention and relieve dyspnoea. Controlling blood pressure and any ischaemia is important.
in HF-PEF. Diuretics remain the
Drug therapy for cardiac failure
After diagnosis and during investigation for a cause of cardiac failure, drug therapy is commenced:
If oedema is present a loop diuretic is used (eg Furosemide). If no peripheral oedema is present,
care must be taken to avoid hypovolaemia; prolonged use of loop diuretics is associated with
increased mortality and they should be stopped where possible.
If oedema persists despite good doses, consider diuretic resistance or poor absorption of drug.
Limited data suggests bumetanide has better bioavailability due to better absorption.
Alternatively, changing the route of administration (oral to IV bolus or infusion) can overcome
resistance, or adding a thiazide diuretic (bendroflumetazide or metolazone). Note this last combination may trigger a significant
diuresis and renal function may temporarily suffer.
Nitrates give symptomatic benefit and may be needed in the acute phase of decompensation;
longer term, a nitrate-free period should be maintained. Hydralazine is another vasodilator that
appeared to show benefit when combined with nitrates in African-American heart failure
patients (A-HeFT). This combination is often considered when impaired renal function precludes ACE inhibitors.
ACE inhibitors should be started early and titrated up rapidly to the maximum tolerated dose
(CONSENSUS, SOLVD and ATLAS studies showed significant improvements in mortality and
morbidity).
Angiotensin receptor blockers should be considered in ACE-inhibitor intolerant patients (eg cough). Val-HeFT, VALIANT and
CHARM-added studies support their use. Note that they should not be used in combination with ACE inhibitors, as there is little
additional benefit and harm may occur from worsening renal function.
Beta blockers should be started once the patient is diuresed. Newer agents such as metoprolol,
bisoprolol and carvedilol should be used. The majority of stable patients tolerate these well if
started at low dose and up-titrated to the maximum dose. They achieve significant mortality and
morbidity improvements (COPERNICUS, MERIT-HF, CIBIS-II). Beta blockers can be safely started in hospital in even those with
recent decompensation.
Cessation of previously stable ACE inhibitor and β-blocker therapy (eg when the patient is admitted with intercurrent infection with
hypotension and/or acute renal impairment) is associated with increased mortality. These drugs should be continued unless
profoundly hypotensive, or at least restarted after clinical stability has been restored.
Aldosterone receptor antagonists: Spironolactone and eplerenone should be added if patients
continue to have symptoms despite ACE inhibitors and β blockers. RALES (spironolactone) and
EMPHASIS-HF (eplerenone) both showed significant mortality and morbidity benefit in severe
HF. Eplerenone is additionally indicated in patients who suffer heart failure after myocardial infarction (EPHESUS). Observe for
hyperkalaemia and impaired renal function.
Ivabradine is a rate-limiting drug with a novel mode of action, selectively inhibiting the I f
channel in the sinus node, to slow the sinus rhythm (but not arrhythmias). It has use-dependency meaning faster heart rates are
most effected. The SHIFT study showed ivabradine added to HF patients with HR ≥70 bpm, despite other optimal medical therapy,
reduced a composite of cardiovascular mortality and admission. It is recommended β blockers are at maximal doses before
ivabradine is considered.
•
•
•
Digoxin may help improve symptoms in patients with systolic heart failure and sinus rhythm. A single study, DIG, did not show a
mortality benefit but showed reduction in hospitalisation. However this was before the β-blocker era and digoxin should only be
considered once other therapies are maximal.
Omega-3 polyunsaturated fatty acids (Omacor): these are purified fatty acids at high dose without the characteristic taste of fish
oils. In the GISSI-HF PUFA study, patients randomised to these drugs had a just-significant reduction in cardiovascular mortality,
felt to be due to reduction in arrhythmias.
Heart failure patients should avoid glitazones (eg rosiglitazone and pioglitazone), calcium • channel blockers (diltiazem, verapamil),
NSAIDs and COX-2 inhibitors, since all cause
worsening of fluid retention and heart failure.
Summary of therapeutic interventions in heart failure
A summary of therapeutic interventions in heart failure is given in Table 1.13. Table 1.13 Summary of therapeutic interventions in
heart failure
•
•

Inte rve ntion

Diuretics
ACE inhibitors
Angiotensin receptor blockers
β blockers
Aldosterone antagonists Ivabradine
Digoxin
Vasodilators
Cardiac resynchronisation therapy
Implantable cardioverter defibrillator
Anticoagulation
ACE, angiotensin-converting enzyme; AF, atrial fibrillation; NYHA, New York Heart Association.
1.8.2 Hypertrophic cardiomyopathy
Benefit
Symptom
Mortality
Mortality
Mortality
Mortality Mortality Symptom Symptom
control
and symptoms
and symptoms
and symptoms
and symptoms and symptoms control control
Comme nt
Loop diuretic or loop diuretic with thiazide
Should be titrated to maximum tolerated dose
In place of or in addition to ACE inhibitors
Bisoprolol and carvedilol have the best evidence base
NYHA III–IV NYHA II–IV NYHA III–IV
NYHA III–IV, see Section 1.6.5 See Section 1.6.5

Mortality Mortality
Stroke morbidity
and symptoms

In AF or if there are prior thromboemboli

HCM is a spectrum disorder with a diverse range of gene mutations that lead to myocardial disarray
and hypertrophy. The hypertrophy can be apical, global or predominantly in the outflow tract. There is dynamic outflow tract
obstruction, which causes an audible ESM and accounts for exertional symptoms and risk of sudden death.
Characteristic features of HCM
Jerky pulse with large tidal wave as outflow obstruction is overcome
Large a waves in the JVP
Double apical impulse (palpable atrial systole, S4, in sinus rhythm)
LSE systolic thrill (turbulence) with harsh ESM radiating to axilla
Often accompanied by mitral regurgitation
Often paradoxical splitting of second heart sound
The ESM increases with Valsalva’s manoeuvre and decreases with squatting.
Important points to remember
Autosomal dominant in half the patients, associated with chromosomes 1, 11, 14 or 15. May also result from a gene mutation that
leads to myocardial disarray and varying expression of hypertrophy. Prevalence <0.2% in the general population. Life expectancy is
variable and the
risk of sudden cardiac death (SCD) can be estimated from the presence of family history of SCD, severe symptoms including prior
cardiac arrest or unexplained syncope, and key findings on investigation (including: non-sustained ventricular tachycardia on holter
monitoring, severe LVH with wall thickness >30mm, BP fall during exercise)
Associations with Friedreich’s ataxia, WPW, phaeochromocytoma, familial lentiginosis
ESM increases with: glyceryl trinitrate (GTN), digoxin and standing, due to volume reduction in diastole; ESM decreases with:
squatting, βblockers, Valsalva’s release, handgrip
Cardiac catheterisation abnormalities include a ‘banana’ or ‘spade-shaped’ LV cavity in systole, MR and ‘swordfish’ narrowing of
the left anterior descending artery
Therapeutic options include β blockers, calcium antagonists, amiodarone, dual-chamber pacing, internal defibrillators, surgical
myomectomy or therapeutic septal infarction
Avoid digoxin (if in sinus rhythm), nitrates, atropine, inotropes, diuretics (unless in left ventricular failure)
Sudden death may be due to catecholamine-driven extreme outflow obstruction, VF related to
accessory pathway-transmitted AF, or massive MI. Sudden death may occur without hypertrophy.
Annual mortality rate of 2.5% in adults and 6% in children
Poor prognostic features include young age at diagnosis, family history of sudden death and syncopal symptoms, but there is no
correlation with the LVOT gradient
Pregnancy is possible, but haemorrhage, prolonged vaginal delivery effort and epidural analgesia are best avoided; antibiotic
prophylaxis and counselling are advised.
1.8.3 Dilated cardiomyopathy
•
•
•
•
••
Dilated cardiomyopathy (DCM) is a syndrome of global ventricular dysfunction and dilatation,
usually with macroscopically normal coronary arteries (if causes of ischaemic cardiomyopathy are excluded). The aetiology is often
undetermined and the condition is more common in male and African-Caribbean patients (this association is possibly because of
undiagnosed hypertensive cardiomyopathy). There is often LBBB or poor R-wave progression on ECG, and anticoagulation may be
warranted as the incidence of AF and ventricular thrombus is high. DCM is treated as other forms of cardiac failure, with ACE
inhibitors and βblockers, with escalating therapies, including cardiac resynchronisation therapy, as the NYHA class worsens.
Causes of DCM
Alcohol
Undiagnosed hypertension
Autoimmune disease
Nutritional deficiency (eg thiamine and selenium)
Muscular dystrophies
Viral infections (eg Coxsackie virus and HIV)
Peripartum
Drugs (eg doxorubicin)
Infiltration (eg haemochromatosis, sarcoidosis)
Tachycardia-mediated cardiomyopathy (uncontrolled fast heart rates, eg AF)
Diabetes – combination of epicardial coronary disease, microvascular ischaemia, metabolic derangement of myocyte
function
•
1.8.4 Restrictive cardiomyopathy
This produces identical symptoms to constrictive pericarditis (see Section 1.9.1) but surgery is of little use in restrictive
cardiomyopathy. The ventricles are excessively rigid and impede diastolic filling. AF may supervene and stagnation of blood leads
to thrombus formation.

Myocardial causes
Idiopathic
Scleroderma
Amyloid (see below)
Sarcoid
Haemochromatosis
Glycogen storage disorders
Gaucher’s disease
Endomyocardial causes
• Endomyocardial fibrosis
Hypereosinophilic syndromes (including Löffler’s)
Carcinoid
Malignancy or radiotherapy
Toxin-related
Cardiac amyloidosis
Cardiac amyloidosis is caused by extracellular deposition of insoluble amyloid fibrils. The protein deposition can be due to a
primary disorder (AL amyloidosis, a clonal plasma cell disorder with light chain production to form the fibrils) or a secondary
disorder (any chronic inflammatory disorder). The deposition and resultant fibrosis cause the ventricles to become thickened and
stiff, with poor systolic and diastolic function. The atria typically dilate in response and may develop AF. Infiltration of the conducting
tissues leads to heart block. ECGs characteristically have low-voltage QRS complexes and the echocardiographic appearances of
the myocardium are classically ‘speckled’. Diagnosis can be confirmed by rectal biopsy with Congo red staining, which can
demonstrate amyloid infiltration. Cardiac involvement is a marker of poor prognosis in amyloid disease, with congestive heart
failure, syncope, pulmonary hypertension and conduction problems being causes of death. Treatment is palliative. Negative
inotropic drugs (eg diltiazem) must be avoided and care taken with digoxin, which binds avidly to the fibrils and can reach toxic
levels even within the therapeutic range.
1.8.5 Myocarditis
Myocarditis may be due to many different aetiological factors (eg viral, bacterial, fungal, protozoal, autoimmune, allergic and drugs).
It may be difficult to differentiate from DCM, but the following features may help:
Usually a young patient
Acute history
Prodrome of fever, arthralgia, respiratory tract infection, myalgia
Neutrophilia
Slight cardiomegaly on chest radiograph
Episodes of VT, transient AV block and ST/T-wave changes
Elevated viral titres
Cardiac enzymes raised (with normal coronary arteries).
Myocarditis is associated with severe morbidity and mortality and needs careful support and management in the intensive care
environment. Transplantation may be necessary if there is poor response to therapy. Those who recover may be left with significant
cardiac impairment.
Rheumatic fever
This follows a group A streptococcal infection; pancarditis usually occurs and valvular defects are long-term sequelae. The cardiac
histological marker is Aschoff’s nodule. Patients are treated with
penicillin and salicylates or steroids.
Criteria for diagnosis include the need for evidence of preceding α-haemolytic streptococcal infection (raised antistreptolysin O titre
[ASOT], positive throat swab or history of scarlet fever), together with two major (or one major and two minor) Duckett–Jones
criteria (see below).
Rheumatic fever (Duckett–Jones diagnostic
criteria)
Major criteria
Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Minor criteria
Fever
Arthralgia
Previous rheumatic heart disease
High ESR and C-reactive protein (CRP)
Prolonged PR interval on ECG
1.8.6 Cardiac tumours
Myxomas are the most common cardiac tumours, comprising 50% of most pathological series.
Atrial myxomas
Post-mortem incidence of <0.3%; more common in females (2 : 1) and in the left atrium (75% of myxomas); usually benign and
occasionally familial
Signs: fever and weight loss occur in 25%, and this may be due to release of interleukin-6 (IL-6). There may be transient mitral
stenosis, early diastolic ‘plop’, clubbing, Raynaud’s phenomenon (rare) or pulmonary hypertension. Usually the rhythm is sinus.
Atrial myxomas may present with the classic triad of systemic embolism, intracardiac obstruction and systemic symptoms
Investigations: white cell count high, platelets low, haemolytic anaemia or polycythaemia, raised immunoglobulins, raised ESR in
60% (thought to be due to secretion of IL-6)
Avoid left ventricular catheterisation; use TOE to diagnose. They occur most commonly on the interatrial septum
Atrial myxomas grow rapidly with a risk of embolisation and sudden death, so they should be resected surgically without delay.
•
•
•••
Other primary cardiac tumours
These include papillomas, fibromas, lipomas, angiosarcomas, rhabdomyosarcomas and mesotheliomas, which are all rare lesions.
1.8.7 Alcohol and the heart
Acute alcoholic intoxication is the most common cause of paroxysmal AF among younger individuals. Chronic excessive intake
over 10 years is responsible for a third of the cases of DCM in Western populations; alcohol is also aetiologically related to
hypertension, cerebrovascular accident (CVA), arrhythmias and sudden death. AF may be the first presenting feature (usually
between the ages of 30 and 35 years).
Pathological mechanisms
Direct myocardial toxic effect of alcohol and its metabolites
Toxic effect of additives (eg cobalt)
Secondary effect of associated nutritional deficiencies (eg
thiamine)
Effect of hypertension
Treatment includes nutritional correction and – most importantly – complete abstinence from alcohol, without which 50% will die
within 5 years. Abstinence can lead to a marked recovery of resting cardiac function.
Beneficial mechanisms of modest amounts of alcohol
Favourable effects on lipids (50% of this benefit is due to raised HDL levels)
Antithrombotic effects (perhaps by raising natural levels of tissue plasminogen
activator)
Antiplatelet effects (changes in prostacyclin:thromboxane ratios)
Increase in insulin sensitivity
Antioxidant effects of red wine (flavonoids and polyphenols)
1.8.8 Cardiac transplantation
Six UK centres currently conduct heart transplantations with approximately 150 operations carried out per year, most often for
intractable coronary disease and cardiomyopathy (44%); survival rates have been estimated at 80% at 1 year, 75% at 3 years and
40–50% at 10 years. Myocarditis is yet another indication; transplantation during the acute phase does not worsen prognosis, but
myocarditis may recur in the donor heart.
The major complications encountered after transplantation include accelerated coronary atheroma, lymphoma, skin cancer (and
other tumours) and chronic kidney disease (due to ciclosporin A or
tacrolimus toxicity).
1.9 PERICARDIAL DISEASE 1.9.1 Constrictive pericarditis
Rare in clinical practice, this presents in a similar way to restrictive cardiomyopathy, ie with signs of right-sided heart failure
(cachexia, hepatomegaly, raised JVP, ascites and oedema) due to restriction of diastolic filling of both ventricles. It is treated by
pericardial resection.
Other specific features include:
A diastolic pericardial knock occurs after the third heart sound, at the time of the y descent of the
JVP, and this reflects the sudden reduction of ventricular filling – ‘the ventricle slaps against the
rigid pericardium’
Soft heart sounds and impalpable apex beat
Severe pulsus paradoxus occurs rarely and indicates the presence of a coexistent tense effusion
Thickened, bright pericardium on echocardiography.
Causes of constrictive pericarditis
Tuberculosis (usually post-pericardial effusion)
Mediastinal radiotherapy
Pericardial malignancy
Drugs (eg hydralazine, associated with a lupus-like
syndrome)
Post-viral (especially haemorrhagic) or bacterial pericarditis
Following severe uraemic pericarditis
Trauma/post-cardiac surgery
Connective tissue disease
Recurrent pericarditis
Signs common to constrictive pericarditis and restrictive
cardiomyopathy
• Raised JVP with prominent x + y descents • AF
Non-pulsatile hepatomegaly
Normal systolic function
Some key features distinguish constrictive pericarditis from restrictive cardiomyopathy: • Absence of LVH in constrictive pericarditis
Absent calcification on chest radiograph, prominent apical impulse and conduction abnormalities on ECG, which are features of
restrictive cardiomyopathy.
However, a combination of investigations, including cardiac CT, MRI and cardiac biopsy, may be necessary to differentiate
between the two conditions.
1.9.2 Pericardial effusion
A slowly developing effusion of 2 L can be accommodated by pericardial stretching and without raising the intrapericardial
pressure. The classic symptoms of chest discomfort, dysphagia, hoarseness or dyspnoea (due to compression) may be absent. A
large effusion can lead to muffled heart sounds, loss of apical impulse, occasional pericardial rub, small ECG complexes and
eventually EMD.
Other key features are:
Pulsus alternans: variable left ventricular output and right ventricular filling
Pulsus paradoxus: exaggerated inspiratory fall in systolic BP (mechanism described in section 1.2.2)
Electrical alternans on ECG: ‘swinging QRS axis’
Globular cardiac enlargement on chest radiograph
•
•

Causes of pericardial effusion

All causes as listed for constrictive pericarditis
Aortic dissection
Iatrogenic due to pacing or cardiac
catheterisation
Ischaemic heart disease with ventricular rupture
Anticoagulation associated with acute pericarditis
1.9.3 Cardiac tamponade
In contrast, if a small amount of intrapericardial fluid (eg <200 mL) accumulates rapidly, it can significantly limit ventricular filling,
reduce cardiac output and elevate intracardiac pressures (particularly right sided initially). Thus the y descent due to right
ventricular filling with tricuspid valve opening is lost as right ventricular pressures are high, and the x descent of right atrium filling
due to right ventricular contraction is prominent. The right atrium collapses in diastole as a result of impaired filling and high
intrapericardial pressures. In early diastole even the right ventricle may collapse.
Occasionally the stretched pericardium may compress the lingular lobe of the left lung, causing bronchial breathing at the left base
(Ewart’s sign). The QRS axis of the ECG may also be altered (electrical alternans).
Common signs of cardiac
tamponade
Elevated jugular venous pressure
Kussmaul’s sign
Tachypnoea
Systolic hypotension
Pulsus paradoxus
Tachycardia
Diminished heart sounds
Impalpable apex beat
Treatment is by urgent drainage – usually under echocardiographic control. Surgical ‘pericardial’ windows may be necessary for
chronic (eg malignant) effusions.
1.10 DISORDERS OF MAJOR VESSELS 1.10.1 Pulmonary hypertension
It is important to determine whether pulmonary hypertension is secondary to an underlying condition because this may be treatable.
The most common cause of secondary pulmonary hypertension is COPD.
WHO Venice classification of pulmonary hypertension
WHO group 1: pulmonary arterial hypertension
Idiopathic/Sporadic
Familial
Associated with pulmonary venous or capillary disease
Associated with systemic diseases:
Collagen vascular disease (eg scleroderma)
HIV
Drugs/Toxins (eg anorexigens)
Portal hypertension
Congenital heart disease with shunting left to right (ASD, VSD)
Persistent pulmonary hypertension of newborn babies
WHO group 2: pulmonary hypertension associated with left heart
disease
• Atrial or ventricular disease (DCM, IHD, HCM)
Valvular disease (eg MS)
Extrinsic compression of the central pulmonary veins
WHO group 3: pulmonary hypertension associated with lung diseases and/or hypoxemia
COPD
Interstitial lung disease
Obstructive sleep apnoea
Alveolar hypoventilation disorders
Chronic high altitude
Chronic hypoxia (polio, myasthenia)
WHO group 4: pulmonary hypertension due to chronic thrombotic and/or embolic disease
Pulmonary emboli of proximal arteries
Obstruction of distal pulmonary arteries
Embolisation of thrombus, tumour, ova, parasites or foreign material
In situ thrombosis
Sickle cell disease
WHO group 5: pulmonary hypertension caused by disorders directly affecting the pulmonary vasculature
Inflammatory – schistosomiasis, sarcoidoisis
Pulmonary capillary haemangiomatosis
Primary pulmonary hypertension
Primary pulmonary hypertension (PPH) is a rare disease with an incidence of 2/million per year; it is a disease of children and
young adults, with a female:male ratio of 2 : 1. PPH constitutes <1% of all cases of pulmonary hypertension and is characterised by
a mean pulmonary artery pressure >25 mmHg at rest, in the absence of another demonstrable cause. One in ten cases is familial.
PPH is associated with connective tissue disease, vasculitis and HIV infection, and also the use of appetite suppressants (eg
fenfluramine). The pulmonary arteries become dilated and abnormally thickened; there is dilatation of the proximal pulmonary
vessels with thick-walled, obstructed, ‘pruned’ peripheral vessels. As a consequence of the high pulmonary pressure the right
ventricle undergoes marked hypertrophy.
Patients present with gradually worsening exertional dyspnoea and, in the later stages, angina of effort and syncope occur. Fatigue
is common and haemoptysis may occur.
Signs include: cyanosis, right ventricular heave, loud P2, tricuspid regurgitation, peripheral oedema and ascites
•
Diagnosis may be suggested on echocardiography with dilated right ventricle with impaired
function; estimated pulmonary pressures will be elevated. Confirmation requires cardiac catheterisation and direct measure of
mean pulmonary arterial pressure (>25 mmHg), in the presence of normal wedge pressure (≤15 mmHg)
•
• Untreated, the median survival is approximately 3 years.
Treatment of PPH
This would usually be undertaken at a specialist centre. The chief aspects involve:
Advise avoidance of strenuous exercise and recommend contraception, because pregnancy is harmful
patients to transplantation. The drug has an extremely short half-life and has to be given by continuous intravenous infusion,
usually through a tunnelled central venous catheter. It is also very expensive
Endothelin receptor blockade (eg bosentan)
Calcium channel antagonists have been used to lower pulmonary (and systemic) pressure
Diuretics are helpful in the management of right heart failure
Continuous ambulatory inhaled nitric oxide is being developed, and this would provide good pulmonary vasodilatation, but without
systemic effect.
The chief therapeutic option is transplantation, because other treatments are of limited benefit or are difficult to administer.
•
Anticoagulation to avoid thrombus formation in situ in the pulmonary arteries and also pulmonary embolism
•
Prostacyclin (PGI2), a potent pulmonary and systemic vasodilator, is used, particularly to bridge
•
•
Summary of available treatments for pulmonary
hypertension
General
Address secondary causes where possible
Digoxin, even in patients with sinus rhythm
Diuretics for symptoms
Ambulatory supplemental oxygen for some
Anticoagulation
Vasodilator therapy (only helps some patients)
• Endothelin receptor blockers (eg bosentan)
PGI2 or PGE (chronic infusion)
•
• Adenosine infusions or boluses
Nitric oxide inhalation, nitrates (chronic infusion)
Calcium channel blockers
• Surgical options (in selected cases)
• Heart–lung or single/double lung transplantation
• Atrial septostomy (only if no resting hypoxia)
1.10.2 Venous thrombosis and pulmonary embolism
The true incidence of pulmonary embolism (PE) is unknown but PE probably accounts for 1% of all admissions. Predisposing
factors are discussed in Chapter 9, Haematology.
One or more predisposing risk factors are found in 80–90% of cases. The oral contraceptive increases the risk of deep vein
thrombosis (DVT)/PE two to four times. However, thromboembolism is rare in women taking oestrogens without other risk factors.
Clinical features
Nearly all patients have one or more of the following symptoms: dyspnoea, tachypnoea or pleuritic chest pain. With a large PE
patients may present with collapse. Hypoxaemia may be present with moderate or large pulmonary emboli.

Inve stigations
•
•
Chest radiograph: may be normal; pleurally based, wedge-shaped defects described classically are rare and areas of oligaemia
may be difficult to detect
•
• D-Dimer: will be raised in PE but the test is non-specific
Helical CT scanning: will demonstrate pulmonary emboli in the large pulmonary arteries but may not show small peripheral emboli
CTPA: the test of choice but really is to exclude major PE and subsegmental PE. True small
peripheral PEs can be missed – and first generation scanners were only 70% sensitive compared
to invasive pulmonary angiography
ECG: may show sinus tachycardia and, in massive PE, features of acute right heart strain; non- specific ST-segment and T-wave
changes occur
Arterial blood gases: show a low or normal PCO2 and may show a degree of hypoxaemia
Ventilation/perfusion ( ) scanning: shows one or more areas of V/Q mismatching
Pulmonary angiography: remains the ‘gold standard’, but this is underused
In each case a clinical assessment of the probability of PE should be made. As demonstrated in the PIOPED study:
Cases of high clinical probability combined with a high-probability scan are virtually diagnostic of PE
•
Similarly, cases of low clinical suspicion combined with low-probability or normal V/Q scans make the diagnosis of PE very unlikely
•
• All other combinations of clinical probability and V/Q scan result should be investigated further
• Patients who present with collapse need urgent echocardiography, helical CT scan or pulmonary angiogram to demonstrate PE.
Manage me nt
In all cases of moderate or high clinical probability of PE, anticoagulation with heparin or low- molecular heparin should be started
immediately after baseline coagulation studies have been taken. If unfractionated heparin is used, the dose should be adjusted to
maintain the activated partial thromboplastin time (APTT) to 1.5–2.5 times the control). Low-molecular-weight heparin has the
advantage of once-daily subcutaneous injections that do not need monitoring. Warfarin should be started concurrently and heparin
can be discontinued once the INR is 2–3.
Warfarin is continued for 3–6 months in most cases; for PE occurring postoperatively, 6 weeks’
anticoagulation is adequate. In recurrent PE, anticoagulation should be for longer periods (eg 1
year) and consideration should be given to lifelong treatment
In cases of collapse due to massive PE, thrombolysis with streptokinase or recombinant tPA
given by peripheral vein should be considered. This should be avoided when the embolic
material is an infected vegetation (eg intravenous drug abusers)
Occasionally, pulmonary embolectomy is used for those with massive PE where thrombolysis is unsuccessful or contraindicated
Inferior vena caval filters should be considered in patients in whom anticoagulation is contraindicated or in those who continue to
embolise despite anticoagulation.
1.10.3 Systemic hypertension
Overall, 30% of the UK adult population have hypertension, with the prevalence increasing with age to 70% in the eighth decade.
Guidelines for treatment continually adapt to new clinical evidence, but the British Hypertension Society (BHS) has issued
guidelines (Table 1.14) to identify those in need of treatment.
Suggested treatment targets are <140/85 mmHg in general, and <130/80 mmHg for high-risk patients, such as patients with
diabetes.
A suggested treatment algorithm is given in Table 1.15. Other considerations in hypertension management
Investigation of phaeochromocytomas: recommend three 24-h urinary catecholamine
• derivatives of all drugs on a vanilla-free diet (and off all drugs). Urinary metadrenalines may
also be measured
••

Hypertension increases the risk (Framingham data) of: stroke (37); cardiac failure (34); coronary artery disease (33); peripheral
vascular disease (32)
•
• Potassium salt should be substituted for sodium salt where possible
Drugs to avoid in pregnancy: diuretics, ACE inhibitors, angiotensin II receptor blockers. Drugs • with well-identified risks
preferred in pregnancy: β blockers (especially labetalol),
methyldopa and hydralazine
Young Black men have a poor response to ACE inhibitors and β blockers because they are salt
•
develop the side-effect of impotence.
manipulation and particularly likely to
conservers by background, and so are resistant to renin Table 1.14 British Hypertension Society Guidelines
Systolic pressure (mmHg)
Diastolic pressure (mmHg)
Observation
Cofactor
DM, TOD, 10- year risk >20%
R e c o mm e ndation
Treat Treat
Treat
Reas
sess annually
Reas
sess annually
Reas
sess annually

180 110 160–179 100–109

140–159 90–99
140–159 90–99
<140 <90
130–139 80–85
>130 >0
Single reading Multiple readings
Multiple readings Multiple readings Multiple readings Single reading
None of the above
Reas
sess in 5 years
DM, diabetes mellitus; TOD, target organ damage (eg any cardiovascular disease, left ventricular hypertrophy by echocardiography
or ECG, proteinuria).
Table 1.15 Treatment algorithm for hypertension First line for those aged >55 years or Black
patients
First-line treatment for others
Second-line treatment
Third-line treatment
Additional possibilities
ACE, angiotensin-converting enzyme; ARB, angiotensin receptor blocker.
1.10.4 Aortic dissection
Two-thirds of tears occur in the ascending aorta with about a fifth occurring in the descending aorta. Mortality is highest in the first
few hours if the dissection is untreated. The differential diagnosis for ascending dissection includes MI if the vulnerable right
coronary ostium is involved (giving rise to an inferior infarct pattern). This is particularly important when considering thrombolysis;
aortic regurgitation provides supportive evidence of the diagnosis.

Calcium blocker or thiazide

ACE inhibitor or ARB
ACE inhibitor or ARB + calcium blocker or thiazide
ACE Inhibitor or ARB + calcium blocker + thiazide
Other diuretic, α/β blockers

Associations with aortic dissection

Systemic hypertension (present in 80%)
Marfan’s syndrome
Cystic medial degeneration (rare in the absence of Marfan’s syndrome)
Noonan’s and Turner’s syndromes
Trauma
Aortic coarctation
Congenital bicuspid aortic valve (present in 10–15% and dissection is therefore associated with aortic
stenosis)
Giant-cell arteritis
Pregnancy (particularly in patients with Marfan’s syndrome)
Cocaine abuse
•
Involvement of ascending aorta may cause
Aortic regurgitation
Inferior MI
Pericardial effusion (including cardiac
tamponade)
Carotid dissection
Absent or decreased subclavian pulse
Medical therapy to be considered for
Old, stable dissections (>2 weeks)
Uncomplicated dissection of descending
aorta
Isolated arch dissections
Investigations for aortic dissection
TOE, aortic MRI and contrast-enhanced spiral CT all have a high diagnostic sensitivity, but CT • rarely identifies the site of the tear
or the presence of aortic regurgitation or coronary
involvement
MRI is of the highest quality but is contraindicated in patients with pacemakers and certain vascular clips
TOE is probably the most widely used investigation because it is available in the acute situation and has high sensitivity and
specificity
Aortography is no longer the gold standard and coronary angiography is applicable only when deciding on the need for concomitant
CABG.
•••
Management of aortic dissection
Suspected aortic dissection is a medical and surgical emergency and requires prompt response,
with mortality increasing by 1% for every hour. Long delays for diagnosis should be minimised.
Untreated proximal dissection are almost always fatal. Descending aortic dissections have a
better prognosis with 85% surviving their hospital stay but can still cause significant mortality if shock, organ ischaemia and renal
failure occurs.
Medical therapy with intravenous agents to reduce the blood pressure may help prevent
extension of the dissection flap. Medical therapy remains the first-line therapy for Type B
dissections (descending aorta only), with subsequent treatment based upon the clinical course.
The mainstay of treatment of proximal dissections (Type A) is urgent surgery and even if
successful, mortality can be as high as 20% at 2 weeks from complications of the dissection,
including aortic rupture, stroke, visceral ischaemia, cardiac tamponade and circulatory failure.
The aim of the surgery is to resect the region of intimal tear to prevent aortic rupture and cardiac tamponade. If the tear is
extensive, partial or total aortic arch replacement may be required. The aortic valve may be suitable to be resuspended or may
require replacement. Medical hypothermia is used but has not significantly improved post-operative mortality.
Endovascular stent-graft placement has no role in Type A dissections but may help in Type B dissections when key aortic branches
are compromised.
APPENDIX I
Normal cardiac physiological values
ECG
PR interval 0.12–0.20 s
QRS duration >0.10 s
QTc (males) 380 ms, (females) 420 ms
QRS axis −308 to +908.
Indices of cardiac function
2
Cardiac index = Cardiac output/body surface area (BSA) = 2.5–4.0 L/min per m Stroke volume index = stroke volume/BSA = 40–
2
70 mL/m
5
Systemic vascular resistance (SVR) = 80 × (Ao − RA)/cardiac output = 770–1500 dyn s/cm , where Ao is the mean aortic pressure
and RA the mean right atrial pressure
•
•
••
•
• Ejection fraction = Proportion of blood ejected from left ventricle = 50–70%.

Cardiac catheterisation pressures mmHg

Criteria for significant oxygen saturation set- up
Mean right atrial
Right ventricular systolic
0–8 SVC/IVC to RA >7% (eg ASD) 15–30 RA to RV >5% (eg VSD)
End-diastolic
Pulmonary artery systolic
End-diastolic 3–12
Mean
Pulmonary artery wedge
A 3–15
V 3–12 Left atrial mean 1–10 Left ventricular systolic 100–140 End-diastolic 3–12 Aortic systolic 100–140 End-diastolic 60–90
Mean 70–105
APPENDIX II
Summary of further trials in cardiology
Major trials Condition
Heart failure
Heart failure
Heart failure
Intervention
β blockers
ACE inhibitors
Cardiac resynchro nisation therapy (CRT)
Trials
MERIT-HF,
II, COPERNICUS
SOLVD, ATLAS, CONCENSUS
MUSTIC, MIRACL, COMPA NION, CARE-HF
Main finding
β blockers reduce mortality and morbidity
ACE inhibitors reduce mortality and morbidity
CRT reduces mortality and morbidity
References
Lancet 1999;353:2 001–7, Lancet 199 9;353:9–13, N Engl J Med 2001; 344:1659–67
N Engl J Med 199 1;325:293–302, Circulation 1999;100: 2312– 18
N Engl J Med 200 1;344:873–80, N Engl J Med 2002; 346:1845–53, N Engl J Med 2004; 350:2140–50, N Engl J Med
2005;352:1539–49
0–8 RV to PA Any level:
>5% (eg PDA) >7%
15–30 SVC to PA
Usual saturations (SaO2)
9–16 Venous 65–75% Arterial 96–98%
CIBIS
Ventricular arrhy thmia
Acute MI
Acute MI
Ischaemic heart disease
Ischaemic heart disease
Ischaemic heart disease
Implantable defibri llators (ICD)
Aspirin
Thrombolysis
β blockers
ACE inhibitors
Statins
AVID, MADIT II, COMPANION
ISIS 2
ISIS 2, ISIS 3, GUSTO, GISSI 1
ISIS, BHAT, CAP RICORN
AIRE, SAVE, GISSI 3, TRACE
SSSS, WOSCOPS, HPS
ICD reduce mortality in primary and secondary prevention
N Engl J Med
1997;337:1576– 83, N Engl J Med 2002;346:877–83, N Engl J Med 2004;350:2140–50
Lancet
1988;ii:349–60
Lancet
1988;ii:349–60, Lancet 1992;339:7 53–70, New Engl J Med 1993;329:673 –82, Lancet 1986;i:397–401
Lancet 1986;ii:57– 66, JAMA 1982;24 7:1707–14, Lancet 2001;357:1385–90
Lancet 1993;342:8 21–8, N Engl J Med 1992;327:669 –77, Lancet 1994; 343:1115–22, N Engl J Med 1995;333:1670–6
Lancet 1994;344:1 383–89, N Engl J Med 1995;333:130 1–7, Lancet 2002;360:7–22
Aspirin reduces mortality
Thrombolysis reduces mortality
β blockers reduce mortality
ACE inhibitors reduce mortality
Statins are effective in primary and secondary prevention