Evaluation of congenital heart disease

Evaluation of congenital heart disease

• 1.
  - Dr. SandipGediya
• 2.
   Congenital heartdiseases refer to structural defects of heart which present at birth. Though some to these lesion may discovered later.  Congenital heart disease accounts for about 10% of infant deaths and nearly half of all deaths due to congenital malformationsin developed countries.  The incidence of CHD is estimated at 6–8 per 1,000 live births.  Of these, at least 25% are life threatening CHDs that require very early intervention.
• 3.
  ACYANOTIC HEART DISEASE Lesionscausing volume overload Left to right shunts (VSD, ASD, PDA, AV septal defects) Regurgitantlesions involving AV valves, aortic and pulmonary valve Cardiomyopathies Lesions causing pressure overload Ventricular outflowobstruction (PS, AS, CoA)
• 4.
  CYANOTIC HEART DISEASE Lesions withDecreased PulmonaryBlood Flow Obstruction to pulmonary blood flow (tetralogy of Fallot, tricuspid atresia andvarious lesions showingFallot'sphysiology) Shunting of systemic venous blood from right to left through PFO, VSD, ASD  Lesions withincreased PulmonaryBloodFlow Abnormal ventricular–arterial connections(TGA withoutPS) Total mixing of systemic venous and pulmonary venous blood within the heart (common atrium or ventricle, total anomalous pulmonary venous return, andtruncus arteriosus)
• 5.
  APPROACH TO ACASE Try to findanswers to these questions Does the childhave heart disease? Is it congenitalheartdisease? If it is congenitalheart disease, whatis thelesion? What is the severity of the lesion?
• 6.
  Does the childhave heart disease?  Thisis answered by applyingNADA’Scriteria.  One major or two minor criteriaindicateheart disease.  These criteriaare of limiteduse in newborns, where clinicalsigns are subtle.
• 7.
  NADA’S CRITERIA Major Criteria •Systolic murmurgrade 3 or more • Diastolicmurmur • Cyanosis • Congestivecardiac failure Minorcriteria •Systolic murmurgrade 1 or 2 •Abnormal 2nd heart sound •Abnormal ECG •Abnormal X-ray •Abnormal blood pressure
• 8.
  Is it congenitalheart disease? POINTERS of congenitalheart disease are  Presence of heart murmur withinfirst year of life  Presence of Parasternal murmur favors a congenitallesion  Presence of cyanosis  Presence of extra cardiac congenitalanomalies
• 10.
  Age of presentation This depend on severity of the lesion as wellas associated heart lesions  1st day of life  TGA withIVS, pulmonary atresia with IVS, HLHS, Ebstein’s anomaly  Newborn period  Large VSD, Large PDA, CoA, Critical AS, Critical PS, TGA with VSD withPS, Obstructed TAPVC
• 11.
   Early infancy VSD, PDA, AS, CoA, TOF, Tricuspid Atresia, Unobstructed TAPVC, ALCAPA  Childhood  ASD, CoA, TOF
• 12.
  Presenting symptoms andsigns ACYANOTICHEARTDISEASE Lesions with left to right shunts will present with complains of feeding difficulties, poor weight gain, recurrent respiratory infections, exercise intolerance and on examinations has features like tachypnea, tachycardia, sweating, chest retractions, nasal flaring, and wheezing suggestingheartfailure. Regurgitant lesions are usually associated with AV septal defects so it will add the volume overload to left to right shunt and present earlier and with moresever symptomsand signs ofCHF.
• 13.
  Obstructive lesions toventricular outflow which increases the pressure overload of the ventricles are usually asymptomatic unless the obstructionissever. Severe pulmonic stenosis in the newborn period (critical pulmonic stenosis) results in signs of right-sided heart failure (hepatomegaly, peripheral edema) as well as cyanosis from right-to-left shunting across theforamenovale. Severe aortic stenosis in the newborn period (critical aortic stenosis) is characterized by signs of left-sided heart failure (pulmonary edema, poor perfusion) andright-sided failure (hepatomegaly, peripheral edema),and it mayprogressrapidlytototalcirculatory collapse.
• 14.
  CoA may presentin newborn period as weak lower limb pulses or radio-femoral delay or total circulatory collapse depend on severity whereas in older children usual presentation is upper limbhypertension
• 15.
  Presenting symptoms andsigns CYANOTIC HEART DISEASE Lesions with decrease pulmonary blood flow usually present as cyanotic spells if obstruction to pulmonary flow is mild. Where in case of sever obstruction it manifest in the newborn period only with profound hypoxemia and shock when ductus closes. Among Lesions with increase pulmonary blood flow TGA usually present as sever cyanosis when ductus closes in new born period and other lesions which allows mixing of blood are presenting with cyanosis as well as signs of heart failure.
• 16.
  History  GESTATION ANDNATAL HISTORY  Maternal rubella infection causes multiple anomalies includingcardiac defects.  Infections by CMV, herpes virus and coxsackievirus B are suspected to be teratogenic if they occur in early in pregnancy.
• 17.
   Several medicationsuspectedteratogens  Anticonvulsant mainly phenytoin has been associated with PS, AS, CoA, and PDA. And valproic acid with ASD, VSD, AS, pulmonary artesia and CoA.  ACE inhibitors and ARB taken during 1st trimester have reported to cause congenital malformations of multiple system, including cardiac defects.  Progesterone and estrogen are also suspected causing CHD like VSD, TOF, and TGA.  Maternal Warfarin intakeis associated withTOF and VSD.
• 18.
   Lithiumhas beenassociated withEbstein’s anomaly.  Retinoicacid may causes conotruncal anomalies.  Excessive alcohol intake during pregnancy has been associated withVSD, PDA, ASD, and TOF. Infant born to diabetic mothers has high incidence of cardiomyopathy and also structural heart defects like TGA, VSD, PDA. Maternal lupusand MCTD associated withcongenitalheart block. Infants with high birth weight show a higher incidence of cardiac anomalies.
• 19.
   FAMILY HISTORY Incidence of CHD increases from about 1% in general population to as much as 15% if the mother has CHD and 3% if father has CHD.  In general, when one child is affected, the risk of recurrence in siblings is about 3%, and when two child is affected , the risk is about 4-10%.  Having a child with HLHS increases the risk of CHD in subsequent children(to ≈10%).
• 21.
  • General appearance(whether child is in distress, wellnourished or undernourishedor obese) • Heart rate • Respiratory rate • Blood pressure • Color (i.e., cyanosis, pallor, jaundice) • Pulseoximetry • Clubbing • Dimorphisms suggestive of Syndrome or chromosomal abnormalities
• 22.
  Heart rate &Respiratory rate  Thisshouldbetakenduringrestingstate.  Tachypnea, along with tachycardia, is the earliest sign of left-sided heart failure.  Bounding pulses may suggest an aortic runoff lesion such as PDA, aortic insufficiency and others whereas presence of diminished pulses in all extremities is associated with pericardial tamponade, left ventricular outflowobstruction,or cardiomyopathy.  In infants with CoA, the femoral pulses may be decreased or delayed as comparetoradialpulse.  If the child has dyspnea or retraction, this may be a sign of a more severe degreeofleft-sidedheart failureor asignificantlungpathology.
• 23.
  Blood pressure  Inall suspected case of CHDs, four extremity blood pressure measurements should be obtained.  There are various noninvasive methods of blood pressure measurement in newborns and infants like flush method, pulse oximetry, oscillatory, palpatory andauscultatory.  An automated noninvasive blood pressure instrument whichmeasure the blood pressure by oscillatory method is preferred over manual recording for four extremity blood pressure measurement.  Coarctation of the aorta in older children and adolescents is usually manifested asupper body hypertension and diminished pulses in the lower extremities.
• 24.
  Cyanosis & Pulseoximetry  Cyanosis is a bluish discoloration of the skin and mucous membranes resulting from an increased concentration of reduced hemoglobin to about 5 g/100 ml in the cutaneous veins.  The arterial saturation is usually 85% or lower before cyanosis is detectable in patients with normal hemoglobin levels.  Cyanosis that is associated with arterial desaturation is called central cyanosis. Cyanosis associated with normal arterial saturation is called peripheral cyanosis.  When in doubt, theuse of pulse oximetry is confirmatory.
• 25.
   Multiple studiesdemonstrate the benefit of routine pulse oximetry screening for all newborns to detect unsuspected critical cyanotic congenitalheart disease.  Screening is performed between 24 and 48 hr of life and before discharge in asymptomatic newborns.  A pulse oximetry saturation <90% in the right hand or either foot requires urgent echocardiography.  A pulse oximetry saturation <95% in either location or a saturation difference >3% between the right hand and either foot is considered a positive test and should be repeated in an hour; if positive again, it should be repeated in another hour. If it remains positive, echocardiography is indicated.
• 26.
   Centralcyanosis maybe caused by  cyanotic congenitalheart defects  lung disease  central nervous system depression  Methemoglibinemia,rarely.  Peripheralcyanosis caused by  Circulatory shock  Congestive heart failure  Acrocyanosis of newborns
• 27.
  Hyperoxia test  Thehyperoxia test helps differentiate cyanosis caused by cardiac disease fromthatcausedby pulmonarydisease.  In the hyperoxia test, one tests the response of arterial Po2 to 100% oxygen inhalation. Oxygen should be administered through a plastic hood (e.g., an Oxy-hood) for at least 10 minutes to completely fill the alveolar spacewithoxygen.  With pulmonary disease, arterial Po2 usually rises to a level greater than 100 mmHg.  When there is a significant intra-cardiac right-to-left shunt, the arterial Po2 does not exceed100 mm Hg, and the rise is usuallynot more than 10 to 30 mmHg.
• 28.
  Clubbing  Clubbingis characterizedby a wideningand thickeningof the ends of the fingers and toes, as well as by convex fingernails and loss of the anglebetweenthe nailand nailbed.
• 29.
  Syndrome Cardiac defectsOtherabnormalities Down’s syndrome(trisomy 21) (in 40-50%)ECD, VSD, ASD Hypotonic, flat faces, slanted palpebral fissure, small eyes, mental deficiency, simian crease Patau’s syndrome Trisomy 13 (in 80%)VSD, PDA, dextrocardia Low birth weight, central facial anomalies, polydactyly, chronichemangiomas, low-set ears, visceral and genital anomalies Edward’s syndrome Trisomy18 (in 90%)VSD, PDA, PS Low birth weight, microcephaly, micrognathia, rocker-bottom feet, closed fist with overlapping fingers Turner’ssyndrome (XO syndrome) (in 35%);COA, bicuspid aortic valve, AS Short female; broad chest with widely spaced nipples; congenital lymphedemawith residual puffiness over the dorsum of fingersand toes Noonan’s syndrome(Turner- likesyndrome) (Frequent) PS (dystrophic pulmonaryvalve), LVH (or anterior septal hypertrophy) Similar to Turner’ssyndrome but may occurboth inmales and females, without chromosomal abnormality
• 30.
  Continue… Syndrome Cardiac defectsOtherabnormalities VATERassociation (VATER or VACTERLsyndrome) (in >50%);VSD, TOF,ASD, other defects Vertebral anomalies, Anal atresia, Congenital heartdefects, TracheoEsophageal (TE) fistula, Renaldysplasia, Limb anomalies (e.g., radial dysplasia) CHARGEassociation 8q12deletion (in 65%)VSD, ASD, PDA, TOF, truncusarteriosus, aortic arch anomalies Coloboma, Heart defects, choanal Atresia, growth and mental Retardation, Genitourinaryanomalies, Ear anomalies DiGeorge syndrome Microdeletion of 22q11.2 (Frequent) interrupted aortic arch, truncusarteriosus, VSD, PDA, TOF Hypertelorism, short philtrum, down slanting eyes, hypoplasia or absence of thymusand parathyroid, hypocalcaemia, deficient cell-mediated immunity Holt-Oram syndrome (Frequent) ASD, VSD Defects or absence of thumb or radius Williams syndrome (Frequent) supravalvular AS, Peripheral pulmonaric stenosis Varyingdegreeof mental retardation, so-called elfin faces (consisting of some of the following: upturnednose, flat nasal bridge, long philtrum, flat malar area, wide mouth, full lips, widely spaced teeth, periorbital fullness)
• 31.
  Continue… Syndrome Cardiac defectsOtherabnormalities Fetal alcohol syndrome (in 25-30%)VSD, ASD Prenatal growth retardation, microcephaly, short palpebral fissure, mental deficiency, irritable infant or hyperactive child Fetal warfarin syndrome (in 15–45%)TOF,VSD Facial asymmetryand hypoplasia, hypoplasia, or aplasia of the pinnawith blind or absent external ear canal (microtia); ear tags; cleft lip orpalate; epitubular dermoid; hypoplastic vertebrae Infantof motherwith diabetes CHDs (3%–5%); TGA,VSD, COA; cardiomyopathy (10%–20%) Macrosomia, hypoglycemiaand hypocalcaemia, polycythemia,hyperbilirubinemia,other congenital anomalies Rubella syndrome (in >95%);PDA and Peripheral pulmonic stenosis Triadof the syndrome:deafness, cataract, and CHDs; others includeintrauterinegrowth retardation, microcephaly, microphthalmia, hepatitis, neonatal thrombocytopenic purpura
• 32.
  Cardiovascular system examination INSPECTIONPALPATION Precordium Apex impulse Visible Pulsations Dilated veins Apex beat Left Parasternal heave Pulsations Thrills
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  AUSCULTATION Heart sounds Heart murmurs Frictionrub or venous hum Systolic clicks and opening snaps
• 34.
  Secondheart sound  Splittingof the 2nd heart sound increases during inspiration and decreases during expiration.  Wide splitting is noted in atrial septal defect, pulmonary stenosis, Ebstein's anomaly, total anomalous pulmonary venous return, and right bundle branch block.  An accentuated pulmonic component of the 2nd sound with narrow splitting is a sign of pulmonary hypertension.  A single 2nd sound occurs in pulmonary or aortic atresia or severe stenosis, truncus arteriosus, and, often, transposition of the great arteries.
• 37.
   The absenceof a precordial murmur does not rule out significant congenitalor acquired heart disease.  In contrast, loud murmurs may be present in theabsence of structural heart disease, for example,in patients with a large noncardiac arteriovenous malformation, myocarditis, severe anemia,or hypertension.  Many murmurs are not associated with significanthemodynamic abnormalities.These murmurs are referred to as functional, insignificant,or innocentmurmurs.
• 38.
  Innocent murmurs  Classicvibratory murmur (still’s murmur)(systolic)  Pulmonaryejectionmurmur (systolic)  Pulmonaryflow murmur of newborn (systolic)  Carotid bruit or Supraclavicular murmur (systolic)  Venous hum (continuous)
• 40.
   Typical diagnosticX rays are rare  Interpret with a backdrop of a clinical diagnosis  Always proceed systematically  Give due importance to lungfields
• 41.
  What all tolook for in chest x-ray?  Situs  Cardiac Apex  Heart Size  Chamber Enlargement  PulmonaryVasculature  Lungs  Bones & soft tissues  Typical contour, if present
• 42.
  X-ray quality –“Ideal” X-ray  Correct penetration/exposure  3rd/4th thoracic I/V spacesvisible  Correct centering  Relationof clavicle tosternoclavicular joint  Positionof diaphragm  9th rib (posteriorly) or 6th rib (anteriorly)
• 43.
  Diagnosis of atrialarrangement (Situs)  Liver right sided,gas leftsided Situssolitus-RAright,LAleft  Liver leftsided, gas right sided Situsinversus- RAleft,LAright  Midline liver Rightor left isomerism (asplenia/polysplenia syndrome) Situs inversus
• 44.
  Apex RVAPEX LV APEX
• 45.
  Heart Size  Cardio-ThoracicRatio  < 50%- adults  < 55%- infants  < 60%- newborns A B C
• 46.
  • Sometimes, CTRis more than50% BUT HEARTIS NORMAL  Expiratory Film  AP Films  Ascites  PectusExcavatum • Sometimes, CTR is less than50% butHEARTIS ABNORMAL  Obstruction to outflow of ventricles (must look at cardiac contours to rulethis out)
• 47.
  Vascularity • Decreased Vascularity Dark LungFields  Thinperipheral vessels (stringy)  Small Hila • Increased Vascularity  MPA dilated  ProminentHilar PAs  PA traced tilllateral 3rd of lungfield  Externaldiameter of RPA visible in theright hilusis wider than the internaldiameterof the trachea.
• 48.
  Right pulmonary artery narrower than trachea
• 49.
  Dilated right pulmonary artery Pulmonary arteriescan be traced till lateral 1/3 of the lung
• 51.
  • PulmonaryArterial Hypertension ProminentMPA (Normal GV)  Dilated Hilar PAs  Sudden cut off(pruning) of pulmonary arterial shadow of junction of medial &middle1/3rd of lungfields • Pulmonaryvenous hypertension  Cephalization  Perihilar Haze  Fluid in fissures  Kerley’s Lines
• 52.
  Pulmonary venous hypertension Kerleylines CephalizationPerihilar haze
• 53.
  Yellow arrow showing sudden cutoff of pulmonary vasculature (pruning) Red arrow showing dilated MPA Pulmonary arterial hypertension
• 54.
  Chest X-Ray inL to R shunt with increase PBF ATRIAL SEPTAL DEFECT
• 55.
  VENTRICULAR SEPTAL DEFECT
• 56.
  Chest X-ray inobstructive lesions Valvular PS Valvular AS Dilated MPA Dilated Ascending Aorta
• 57.
  Coarctation of Aorta Ascendingaorta may bedilated, normal orsmall Convexity above aortic knob is due to dilated LSCA or aorta proximal to Coarctation Red Arrows shows rib notching (seen after ageof 4-5 years)
• 58.
  Chest X-ray ofcyanotic CHD with decrease PBF Tetrology of fallot Ebstein’s anomaly
• 59.
  Chest X-ray ofcyanotic CHD with increase PBF D - TGA Truncus Arteriosus
• 60.
  TAPVC (supra-cardiac)
• 62.
  ECG graph paper
• 63.
  The 12 CameraPhotography • ThereSIX cameras photographing frontal plane • Lead 1 and aVL are horizontal left sided cameras • Lead 2, aVF, Lead 3 are vertical inferior cameras • aVR is horizontal Rt. sided camera • ThereSIX cameras photographing in transverse or anterio-posterior plane • V1 and V2 record events of septum • V3and V4record events of the anterior wall • V5 and V6 record events of left lateralwall
• 65.
  ECG Complex
• 66.
   P wave-Atrial contraction 0.10 sec(2.5)  PR interval – start of P to begin. of QRS 0.20 sec(5)  QRS complex – Ventricular contraction 0.08 sec(2)  ST segment - Electricalsilence Isoelectric  T wave -repolarization 0.12 sec(3)  QT interval -From Q to T end 0.40 sec(10)  TP segment -Electricalsilence 0.20 sec(5) The wave orinterval Duration
• 67.
  Interpretation  Rate  Rhythm Axis  Intervals  Atrial enlargement  Ventricular hypertrophy  ST/T wave evaluation
• 68.
  Rate  Measured inbeats per minute  60 / RR interval (in seconds)  300 / number of “big boxes” betweenconsecutive QRS complexes  1500 / numberof “small boxes” betweenconsecutive QRS complexes Newborn 145 (90–180) 6 mo 145 (105–185) 1 yr 132 (105–170) 2 yr 120 (90–150) 4 yr 108 (72–135) 6 yr 100 (65–135) 10 yr 90 (65–130) 14 yr 85 (60–120)
• 69.
  Rhythm  Normal sinusrhythm Pwavesprecedingeach QRS complex  NormalP axis(0 to+90degrees)  ThePR interval isregular butdoesnot have tobeofnormal interval
• 70.
  Axis determination  Successiveapproximation  Locate quadrant with leads I and aVF  Narrow down by using leads withinquadrant  Use most equiphasiclead  Axis is perpendicular to thatlead, in thequadrant previously identified  Equal amplitudes  If two leads with equal net QRS amplitudesexist, themean axis lies midway between theaxis of thesetwo leads
• 71.
  Quadrant determination Normal axis Leftaxis Right axis Extreme R/L axis
• 72.
  Successive approximation
• 73.
  Normal QRS Axis Normalranges of QRS axis vary with age.  Newborns normallyhave RADcomparedwith theadult standard.By 3 years ofage,theQRS axisapproaches theadult meanvalue of +50 degrees.  The meanand ranges of anormalQRS axisaccording to ageare  1 wk–1mo : +110°(+30 to+180)  1–3 mo : +70°(+10 to+125)  3 mo–3yr : +60°(+10 to+110)  Older than 3yr : +60°(+20 to+120)  Adult : +50°(–30 to+105)
• 74.
  Abnormal QRS Axis •LAD :QRS Axis less than lower limit of normal for that age that can be seen in • Left Ventricular Hypertrophy (volume Overload) • Left Bundle Branch Block. • RAD :QRSAxis greater than upper limit of normal for that agethat canbe seen in • Right Ventricular Hypertrophy. • Right Bundle Branch Block. • SAD : ‘S’wave is greater than ‘R’ wave inaVFthat is seen in • Endocardial Cushion Defect. • Tricuspid Atresia. • Right Bundle Branch Block.
• 75.
  Atrial hypertrophy  TheP-wave duration and amplitude are important in the diagnosis of atrial hypertrophy. Normally, the P amplitude is less than 3 mm. The duration of P waves is shorter than 0.09 second in children and shorter than 0.07 second in infants.  Right Atrial Hypertrophy : TallP waves (>3 mm) indicate right atrial hypertrophy (RAH) or “P pulmonale”.  Left Atrial Hypertrophy : Widened and often notched P wave is seen in left atrialhypertrophy (LAH) or “P mitrale.” The P wave duration hasto be abnormally prolonged (with the P duration >0.10 second in children and >0.08 second in infants). In V1, the P wave is diphasic with a prolonged negative segment. A notched P wave in V1 is not diagnostic of LAH.
• 76.
  Ventricular hypertrophy • Ventricularhypertrophy produces abnormalities in one or more of the following  Changes inQRS axis The QRS axis is usually directed toward the ventricle that is hypertrophied.  Changes inQRS voltage Anatomically, the RV occupies the right and anterior aspect, and the LV occupies the left, inferior, and posterior aspect of the ventricular mass. With ventricular hypertrophy, the voltage of the QRS complex increases in the direction of the respective ventricle.
• 77.
   ChangesinR/Sratio The R/Sratio represents the relative electromotive force of opposing ventricles ina givenlead. An increase in the R/S ratio in the rightprecordial leads suggests RVH; a decrease inthe R/S ratio in these leads suggests LVH. Likewise, an increase in the R/S ratio in the left precordial leads suggests LVH,andadecrease intheratio suggests RVH.  Miscellaneousnonspecificchanges  RVH:A) q waveinV1 (qR or qRs pattern) suggests RVH. B) An upright T wave in V1 after 3 days of age is a sign of probableRVH.  LVH : Deep Q waves (>5 mm) and tall T waves in V5 and V6 are signs of LVH of the “volume overload” type. These may be seen with a large- shunt ventricular septal defect (VSD).
• 78.
  Right ventricular hypertrophy Tracingfroma 10-month-old infant with severe tetralogy ofFallot
• 79.
   The SYSTOLICOVERLOAD PATTERN (typically seenin pulmonary stenosis) is characterized by tall,pure R waves in the right precordial leads.  The DIASTOLIC OVERLOAD PATTERN (typically seen in patientswithASDs) is characterized by an rsR′ patternand a slightlyincreased QRS duration (minor right ventricular conduction delay).
• 80.
  Left ventricular hypertrophy Tracingfroma 4-year-old child with a moderate ventricular septal defect
• 81.
   SEVERE SYSTOLICOVERLOAD (i.e. AS, CoA) of the left ventricle is suggestedby straighteningof the ST segmentsand inverted T waves over the left precordial leads  DIASTOLIC OVERLOAD (i.e. MR, AR) may result in tallR waves, a large Q wave, and normal T waves over the left precordial leads.
• 82.
  Biventricular hypertrophy Tracing froma 2-month-old infant with large-shunt ventricular septal defect, patent ductus arteriosus, and severe pulmonary hypertension Large equiphasic QRS complexes in two or more of the limb leads and in the midprecordial leads (i.e.,V2 through V5), called the Katz-Wachtel phenomenon(with normal QRSduration)
• 84.
  Echocardiography  Echocardiography (echo)uses ultrasound beams reflected by cardiovascular structures to produce characteristic lines or shapes caused by normal or abnormal cardiac anatomy in one, two, or three dimensions, which are called M-mode, 2D, and three-dimensional (3D) echocardiography, respectively.  INDICATIONS : 1. Screen newborns who appear to have cardiac defects 2. Dx PDA or ventricular dysfunction in a preterm on ventilator for pulmonary disease
• 85.
  3. ConfirmDx inpatientsdiagnosed clinically 4. R/Ostructural heartdisease in syndromic situations. 5. R/Ostructural heartdisease in patients with arrhythmia. 6. Follow up conditions thatmay change with timeand treatment 7. Evaluation after surgery
• 86.
  Cardiac catheterization  Thecatheterization laboratory has become the site of high- technology interventional procedures, allowing for the nonsurgical repair or palliation of heart defects that once required open heart surgery •DIAGNOSTIC CATHETERIZATION isperformed (1) to assist in the initial diagnosis of some complex congenital heart lesions (2) incases in which otherimagingstudiesare equivocal (3) inpatientsfor whomhemodynamicassessmentis critical (4) betweenstagesofrepair ofcomplexcongenitalheartdisease (5) for myocardial biopsy in the diagnosis of cardiomyopathy or in screeningforcardiac rejectionaftercardiactransplantation (6) forelectrophysiologicstudyin theevaluationofcardiac arrhythmias
• 87.
   INTERVENTIONAL CARDIACCATHETERIZATION  Cathetertreatmentis the standard of practice for most cases of isolated pulmonary or aortic valve stenosis as wellas for recoarctation of the aorta.  The clinicalresults of this procedure are similar to those obtainedby open heart surgery, but withouttheneed for sternotomy or prolonged hospitalization.  Interventionalcatheterizationtechniques are being adapted for use in thefetuswithlesions such as aortic stenosis inan attemptto prevent their progression to more complex lesions such as hypoplastic left heart syndrome.
• 88.
   Closure ofa small PDA is routinelyachieved withcatheter- delivered coils , whereas a largerPDA can be closed witha variety of sandwich-type devices.  Closure of anomalousvascular connections (coronary fistulas, veno-venous collaterals in cyanotic heart lesions) can also be achieved usingcoils.  SecundumASDs are now routinelyclosed witha double-disc occluder device
• 89.
  MRI and MRA Magneticresonance imaging(MRI) andmagneticresonance angiography (MRA)are extremelyhelpfulin thediagnosisand managementofpatients withcongenitalheartdisease.  These techniquesproduce tomographicimagesofthe heartin any projection,with excellentcontrastresolution of fat,myocardium,and lung, as wellas moving blood frombloodvessel walls.  MRI is usefulin evaluatingareas thatare lesswellvisualizedby echocardiography,such as distalbranch pulmonary arteryanatomyand anomaliesin systemicand pulmonary venous return.
• 90.
   Computer processingof MRA images allows thenoninvasive visualization of the cardiovascular system from inside of the heart or vessels, a techniqueknown as FLY-THROUGH IMAGING.These images allowthe cardiologist to imagethe interiors of various cardiovascular structures
• 91.
  CT Scan  CTscanningcan now be used to perform rapid, respiration- gated cardiac imagingin children with resolutions down to 0.5 mm.  Three-dimensionalreconstruction of electron-beam CT images are especially usefulin evaluatingbranch pulmonary arteries, anomaliesin systemic and pulmonary venous return, and great vessel anomaliessuch as Coarctation of theaorta.
• 92.
  Radionuclide angiography  Radionuclideangiography may be used to detect and quantify shuntsand toanalyze thedistributionof blood flow toeach lung.  This technique is particularly useful in quantifying the volume of blood flow distribution between the 2 lungs in patients with abnormalities of the pulmonary vascular tree or after a shunt operation or to quantify the success of balloon angioplasty and intravascular stentingprocedures.
• 93.
  TAKE HOSPITAL MESSAGE The initial evaluation for suspected congenital heart disease involves a systematic approach with 3 majorcomponents.  First, congenital cardiac defects can be divided into 2 major groups based on the presence or absence of cyanosis, which can be determined by physical examination aided by pulse oximetry.  Second, these 2 groups can usually be further subdivided according to whether the chest radiograph shows evidenceof increased,normal, ordecreased pulmonary vascular markings.  Finally, the electrocardiogram can be used to determine whether right, left, or biventricular hypertrophy exists.  The character of the heart sounds and the presence and character of any murmurs further narrow the differential diagnosis.  The final diagnosis is then confirmed by echocardiography, CT or MRI, or cardiac catheterization.
• 94.
  THANK YOU