NEONATAL CARDIAC

PROBLEMS

Fetal Cardiac Physiology

Foramen Ovale
Ductus Arteriosus
Right heart is the dominant ventricle
pumps 2/3 of cardiac output
Relative RVH in utero

Responses of Pulmonary artery (PA) and

Ductus arteriosus to various stimuli
PA responds to oxygen and acidosis in the
opposite manner as the ductus arteriosus:
1. hypoxia & acidosis relaxes the ductus and
constricts the pulmonary arterioles
2. Oxygen relaxes the pulmonary arterioles
but constricts the ductus
3. sympathetic stimulation and -adrenergic
stimulation constricts the PA; whle vagal
stimulation & adrenergic stimulation
dilate the PA

Changes in circulation after birth

Primary change: shift of blood flow for gas
exchange from placenta to the lungs
1. interruption of umbilical cord results in:
Increase SVR as a result of removal of very low
resistance placenta
Closure of ductus venosus as a result of lack of
blood return from the placenta

Changes in circulation after birth

2. Lung expansion results in:
Decrease PVR, increase pulmonary blood flow and
decrease pulmonary arterial pressure
Functional closure FO occurs because of increase
in LA pressure as a result of increased pulmonary
venous return to LA; Ra pressure falls as a result
of closure of ductus venosus
Closure of ductus arteriosus as a result of
increased arterial oxygen saturation

Changes after Birth

Left heart now the dominant side
Process continues for up to 6-8 weeks

Nursery is the ideal time to diagnose

congenital heart disease (if not
prenatally diagnosed) in order to
assure early appropriate care

Cyanosis in the newborn

Most are cyanotic at birth
Where to look? Lips, tongue, oral mucous
membrane, nailbeds , even the tips of the
nose
Usually recognized when arterial oxygen
saturation is 80% or lower
In the newborn, cyanosis may occur even at a
high oxygen sat of 90% because Hgb is often
high and peripheral circulation is sluggish

CYANOSIS
Occurs when the amount of reduced
hemoglobin reaches critical levels of 5gm/dl in
the cutaneous veins

CENTRAL CYANOSIS
Maybe seen in:
CNS depression
Pulmonary disease
Cyanotic cardiac disease
Hematologic methemoglobinemia, severe blood
loss
endocrine disease

Always significant

PERIPHERAL CYANOSIS
Maybe seen in acrocyanosis ( hands &feet);
exposure to cold (vasoconstriction),
hypovolemia and circulatory shock

Approach to neonate with central cyanosis

Take ABG to confirm type of cyanosis
Do hyperoxia test
Get CXR to assess heart size and shape; asses
pulmonary vascular markings
Get ECG to assess chamber enlargement
Request for 2D echo with Doppler color
studies
If cyanotic and ductal dependent, start
prostaglandin E

PGE
RE-opens PDA and prevents it from closing
Allows partially desaturated systemic arterial
blood to enter the pulmonary artery and be
oxygenated
Initial dose 0.1mg/kg/min
Side effects- apnea, fever, hypotension

CONGENITAL HEART DISEASE

occurs in 8 per 1000 births
The spectrum of lesions ranges from
asymptomatic to fatal
Although most cases of congenital heart
disease are multifactorial, some lesions
are associated with chromosomal
disorders, single gene defects,
teratogens, or maternal metabolic
disease

Cardiac S/S:
Cyanosis - 1st sign to appear
Irregular rhythm
Variation in amplitude of heart
sounds
Murmurs
Cardiac enlargement
Especially if any symptoms with
feeding (exercise for babies)

CHD can be divided into three

pathophysiologic groups :
I.

Left-to-right shunts
(acyanotic)
-

Atrial septal defect

Ventricular septal defect
Patent ductus arteriosus

II. Right-to-left shunts (cyanotic)

- tetralogy of Fallot
- transposition of great
arteries
- tricuspid atresia
- truncus arteriosus
- TAPVR

III. Obstructive stenotic

lesions
- coarctation of aorta
- valvular stenosis :
aortic,
pulmonary
mitral

** although not all cyanosis is of cardiac origin

BUT cyanosis + murmur = cardiac problem
especially if with abnormal cardiac rate ad
rhythm, (+) signs of heart failure
** However, absence of cyanosis during the
newborn period DOES NOT exclude cardiac
anomaly

Cardiac Evaluation
History
Prematurity
Maternal pregnancy complications (DM, PIH,
infections, teratogen exposure)
Abnormal ultrasounds
Family history of congenital heart disease

Exam:
Inspection
Palpation
Auscultation

Assessment - History
Maternal history
Age
Advanced? risk of Downs
w/associated endocardial cushion
defect
Comorbidities
Diabetes? Think ventricular septal
hypertrophy
Lupus? Risk of complete heart block

Assessment - History
Medications/Drugs
Valproic Acid? Associated with CoA,
HLHS, AS
Illness
Rubella? Causes PDA
Family history
Previously affected children?

Syndromes
Turner: aortic disease
Downs: VSD
Rubella: PDA

ACYANOTIC HEART DISEASES

(LEFT TO RIGHT SHUNTS)

VENTRICULAR SEPTAL DEFECT

A congenital abnormality
in which blood flows from
high pressure LV low
pressure RV through a
hole
the most common
congenital heart defect,
accounts for 25% of all
congenital heart disease

VENTRICULAR SEPTAL DEFECT

Perimembranous VSDs are
the most common of all
VSDs (67%)
PANSYSTOLIC/
HOLOSYSTOLIC murmur
Typically loudest left lower
sternal border

VENTRICULAR SEPTAL DEFECT

Large VSDs are not symptomatic at birth
because the pulmonary vascular resistance is
normally elevated at this time.
As the pulmonary vascular resistance
decreases over the first 4 to 6 weeks of life
the amount of shunt increases, and symptoms
may develop.

Patent Ductus Arteriosus

Persistent functioning
connection between
the pulmonary artery
and the beginning of
descending aorta
CONTINUOUS,
MACHINE-LIKE murmur
Heard best under the
clavicle (left)

Patent Ductus Arteriosus

The physical examination findings
depend on the size of the shunt.
A widened pulse pressure is often
present as a result of the runoff of blood
into the pulmonary circulation during
diastole.

ATRIAL SEPTAL DEFECT

Most common
congenital heart disease
encountered in adults
The ECG may show right
axis deviation and right
ventricular enlargement

CYANOTIC HEART DISEASES

(RIGHT TO LEFT SHUNTS)

Five Ts

Tetralogy of Fallot
Transposition of Great arteries
Tricuspid valve atresia
Truncus arteriosus
Total anomalous pulmonary venous return

Tetralogy of Fallot
Most common of the cyanotic cardiac
diseases
10% of CHDs

Tetraology of Fallot
1. VSD
2. RVOT Obstruction
( PA stenosis)
3. RVH
4. Overriding aorta

Tetralogy of Fallot
Pulmonary Artery
Stenosis determinant
factor related to
severity

Clinical Presentation of Cyanotic TOF

Cyanosis, clubbing, dyspnea on exertion,
squatting, hypoxic spells.
Loud systolic ejection murmur, systolic thrill
at middle LSB

Tetralogy of Fallot
Boot shaped heart
on x-ray
couer en sabot
Uplifted apex, with
concave MPA
segment and
decreased PVM

Hypoxic Spell
(TET Spell)
Peak incidence of 2-4 months
Characterized by:
Hyperapnea (Rapid and deep respirations)
Irritability and prolonged crying
Increased cyanosis
Decreased heart murmur

Pathophysiology TET spells

Lower SVR or inc
resistance of RVOT can
increase the R-L shunt
Stimulates the
respiratory center to
produce hyperpnea
Results in an increase
SVR
In turn, increases R-L
shunt through VSD

TET Spell Treatment

1. Hold infant in knee-chest position
2. Morphine
3. Sodium bicarbonate to treat acidosisdecreases resp stimulating effect of acidosis
4. Vasoconstrictor (phenylephrine)
5. Propranolol
6. Surgical: Blalock Taussig shunt

Complete Transposition of the Great

Arteries
5% of all CHD
Boys 3:1
Most common cyanotic condition that
requires hospitalization in the first two
weeks of life

Complete Transposition of the Great

Arteries
Aorta arises from RV and
Pulmonary Arteries from
LV.
Without an abnormality,
life would not be possible.
ASD
VSD (30-40%)
PDA

Transposition of the Great

Arteries

Cyanosis always present at birth

CHF develop during NB period
Often no murmur
(+) family history of diabetes
LAB:
CXR: cardiomegaly with egg shaped cardiac
silhouette

Complete Transposition of the

Great Arteries
Egg on side
on x-ray

Tricuspid Atresia
Absence or
imperforation of
tricuspid valve
RV hypoplastic
HUGE heart on x-ray
(mainly right atrium)

Tricuspid Atresia

Tricuspid Atresia
Presents with cyanosis and heart failure in the
first few weeks, as pulmonary vascular
resistance falls
CXR- boot shaped heart

Total Anomalous Pulmonary Venous Return

All four
pulmonary veins
drain to the right
side.
ASD is present to
sustain life

Total Anomalous Pulmonary Venous Return

Infant presents in
first days with
cyanosis, circulatory
and respiratory
failure and collapse.
If obstructed, is the
only pediatric cardiac
surgical EMERGENCY

Total Anomalous Pulmonary

Venous Return (TAPVR)
CXR is snowman
figure of 8
appearance

Truncus Arteriosus
A single trunk leaves
the heart
Gives rise to pulm,
systemic, and
coronary circulations
Large VSD is always
present

Clinical Signs
Cyanosis immediately after birth
Early signs of CHF
2-4/6 systolic murmur at LSB suggestive of
VSD

OBSTRUCTIVE / STENOTIC
LESIONS

COARCTATION OF AORTA
Localized narrowing
of aorta
Systolic ejection
murmur best LUSB
and over back
Decreased femoral
pulses

COARCTATION OF AORTA
Diagnosed by a
difference in blood
pressure between lower
extremities and upper
ones.
Pressure in upper
extremities > lower

Coarctation of the Aorta

Pre-Ductal
Less common but more serious
Associated with VSD, PDA, Transposition

Post-Ductal
More common
Often associated with collateral circulation
beyond coarctation, which minimizes effect.

Coarctation of the Aorta

CXR:
Rib notching may also be seen in older
children (>8 years of age) with large
collaterals.

Key Points
A normal neonatal examination does not
guarantee that the baby is normal and
certainly does not exclude life threatening
cardiovascular malformation
The absence of a murmur does not exclude
serious heart disease

Key Points
Most murmurs are benign, but if its loud,
harsh, diastolic, or the infant has symptoms,
be concerned
A persistent murmur or any other sign of
congenital heart disease should warrant
prompt pediatric cardiac evaluation
Genetic syndromes have commonly associated
heart defects

GASTROINTESTINAL ANOMALIES

Common congenital GI anomalies

in the newborn
- Anomalies of the mouth
cleft lip & cleft palate
- Anomalies of the esophagus
esophageal atresia
chalasia of the esophagus

 Anomalies of the stomach and

duodenum:
- pyloric stenosis
- duodenal obstruction
- hiatus hernia

 Anomalies of the intestine:

- Imperforate anus.
- Omphalocele.
- Intestinal atresia.
- Diaphragmatic hernia.
- Hirschsprungs disease (congenital
aganglionic megacolon)
- Intussusception

FETUS : Gastrointestinal Function

12th week: swallowing
16th week: Meconium present
Digestive enzymes are all active EXCEPT
amylase
20th week: gut in final position, non nutritive
sucking present
32nd -34th week: nutritive sucking

Things to remember:
the baby at birth
Stomach holds 15-30ml
Gastric emptying time 2-3 hours
Presence of mucus in the stomach delays
emptying further
Enzymes are less effective than an adults
Initiation of feeding stimulates growth of
the intestinal mucosa

GASTROINTESTINAL S/S

VOMITING
1st few hrs after birth - mucus,
occasionally blood streaked
Should disappear after first few feedings

If protracted do gastric lavage

If persistent pathologic

VOMITING
If with early onset
If with maternal
and persistent:
polyhydramnios
Intestinal
Upper
obstruction
gastrointestinal
(esophageal,
Metabolic
duodenal,
ileal)
CNS - increased
atresia
ICP

VOMITUS
If blood stained:
If bile stained:
? Maternal or infant
Intestinal obstruction
Perform APTS TEST
beyond the duodenum
- mix vomitus with equal
amounts of water
centrifuge to 5 parts of
supernatant , add 1 part of
0.25% NaOH
if it turns YELLOW within 2
minutes - maternal blood
no change - infants blood

REGURGITATION
Effortless movement of stomach contents
into the esophagus and mouth
Not associated with distress
Result of gastroesophageal reflux through an
incompetent LES
mainly due to stomach distention because of
overfeeding or excessive swallowed air in the
rapid act of sucking
Resolves with maturity

CONSTIPATION
99% of healthy full-term infants pass their first
stool or meconium within 24 hours of birth
and all healthy term neonates should do so by
48 hours.
With preterm infants, the length of time can
extend up to 9 days.
Failure to pass meconium consider
intestinal obstruction
Seldom in breast fed infants

CONGENITAL GIT ANOMALIES

ESOPHAGEAL ATRESIA
Resistance is encountered during an attempt to
pass a catheter into the stomach
Should be diagnosed early
s/s : Unusual drooling from the mouth
copious saliva
Vomiting occurs with the 1st feeding
choking, coughing
cyanosis on food intake
Complication: aspiration pneumonia

ESOPHAGEAL ATRESIA

CHALASIA
Continuous relaxation of
esophageal-gastric
sphincter
Presents with
regurgitation of feedings
Management:
keep the infant in semiupright position
Thicken the feedings
Administer prokinetic
drugs

Anomalies of the stomach and

duodenum:
Pyloric Stenosis
hypertrophy of the pylorus,
with elongation and
thickening of tissue that
progresses to nearcomplete obstruction of
the gastric outlet.
most cases arising at 3-6
weeks, but it can present
even in the first few
months.

Rate of 3 in 1,000 live births,

more commonly in males (4-6:1).

Anomalies of the stomach and

duodenum:
DUODENAL ATRESIA
congenital absence or
complete closure of a
portion of the lumen of
the duodenum
The diagnosis confirmed
by radiography.
An X-ray of the abdomen
shows two large air filled
spaces, the so-called
"double bubble" sign.

DUODENAL ATRESIA
Approximately 2040% of all infants with
duodenal atresia have Down syndrome.
Approximately 8% all infants with Down
syndrome have duodenal atresia.
It is also associated with polyhydramnios,
which is increased amniotic fluid in the uterus.

 Anomalies of the intestine:

- Imperforate anus
- Abdominal wall defects:
1. Omphalocele
2. gastroschisis more common
- Intestinal malrotation/volvulus
- Diaphragmatic hernia
- Hirschsprungs disease (congenital
aganglionic megacolon)
- Meconium ileus

IMPERFORATE ANUS
signs & symptoms
absence of anal opening
misplaced anal opening
no passage of first stool
within 24 to 48 hours after
birth
stool passed by way of
vagina or urethra
Abdominal distention

OMPHALOCELE
an abdominal wall
defect at the base of the
umbilical cord
(umbilicus); the infant is
born with sac protruding
through the defect which
contains small intestine,
liver, and large intestine.

GASTROSCHISIS
an abdominal wall
defect to the side of the
umbilical cord
is rarely associated with
other birth defects.
a life-threatening event
requiring immediate
intervention.

DIAPHRAGMATIC HERNIA
two types of diaphragmatic
hernia:
1. Bochdalek hernia usually involves an opening
on the left side (85%) of the
diaphragm.
2.Morgagni hernia - involves
an opening on the right side
of the diaphragm.

MECONIUM ILEUS
Incidence
Almost always associated with cystic fibrosis
Reported to be the presenting symptom in 1520% of cases

MECONIUM ILEUS
Plain radiographs
Varying sized loops
of distended bowel
Absence of air fluid
levels
Soap bubble
appearance
particularly in the
right lower quadrant

MECONIUM PLUG SYNDROME

most common form of functional bowel
obstruction in the newborn, with an incidence
of 1/500.
Lower colonic or anorectal plugs with a lower
normal water content causing intestinal
obstruction
Treatment: glycerin suppository
rectal irrigation with isotonic saline

MECONIUM PLUG SYNDROME

Supine frontal view
of the abdomen in a
newborn with
meconium plug
syndrome
demonstrates
multiple dilated
loops of bowel but
no rectal gas.

NECROTIZING ENTEROCOLITIS
(NEC)
Characterized by mucosal or transmural
necrosis
Life threatening
? Cause - UNCLEAR
May be multifactorial
Incidence: 1-5%

NECROTIZING ENTEROCOLITIS
(NEC)
Occurrence:
20% premature births
Males = Females
common in LBW who had perinatal distress
most frequently involved: distal part of
ileum and proximal segment of colon

NECROTIZING ENTEROCOLITIS
(NEC)
Clinical triad:
intestinal ischemia
early formula feeding
bacterial colonization
Greatest risk factor: prematurity

NECROTIZING ENTEROCOLITIS
S/S: nonspecific
lethargy
temp instability
abdominal distention
bloody stools (25%)

NECROTIZING ENTEROCOLITIS
Diagnosis: high index of
suspicion
Abdo Xray:
pneumatosis
intestinalis (air in
the bowel wall)
portal venous gas
indicates severity
seen on ultrasound
pneumoperitoneum
(perforation)