COMMON HEART CONDITION IN NEWBORN

FETAL vs NEONATAL CIRCULATION

 CONGENITAL HEART DISEASES
• Congenital heart disease (CHD) encompass a spectrum of
structural abnormalities of the heart or intrathoracic vessels
• The cause of most congenital heart defects is still unknown.
Many cases of congenital heart disease are multifactorial and
result from a combination of genetic predisposition and an as-
yet-to-be-determined environmental stimulus.
• Commonly presents in the newborn with central cyanosis,
heart failure, sudden collapse or heart murmur
 approach
• History
1) Anc scan (cardiac malformation. Fetal
arrythmias, hydrops)
2) Family hx of congenital heart disease
3) Maternal illness (diabetes, rubella,
terratogenic medications)
4) Perinatal problems (prem, mas, perinatal
asphyxia)
• Physical examination
1) Dysmorphism : trisomies, Turner, di george
2) Central cyanosis
3) differential cyanosis (spo2 lower limb <upper limb)
4) Tachypnea
5) Weak or unequal pulse
6) Heart murmur
7) Hepatomegaly
• Investigations
1) Chest x ray
- cardiac size and shape, pulmonary blood flow
(vascularity), pulmonary edema, and associated
lung and thoracic anomalies that may be
associated with congenital syndromes (skeletal
dysplasias, extra or deficient number of ribs,
abnormal vertebrae, previous cardiac surgery).
- CTR ratio
- Hyperoxia test
1.Determine the pa02 while in room air
2.Give 100% o2 in 10-20mins by mask, hood or ett
3.Obtain ABG while infant breathing 100% 02, then interpret the results.
• distinguishing cyanotic congenital heart disease from pulmonary disease.
• Neonates with cyanotic congenital heart disease are usually not able to
significantly raise their arterial Pao2 (< 100mmhg) during administration of
100% oxygen.
• In patients with pulmonary disease, Pao2 generally increases significantly
with 100% oxygen as ventilation-perfusion inequalities are overcome.
• Hypoxia in many heart lesions is profound and constant, whereas in
respiratory disorders and in PPHN, arterial oxygen tension often varies
with time or changes in ventilator management. Hyperventilation may
improve the hypoxia in neonates with PPHN and only occasionally in those
with cyanotic congenital heart disease.
 ECHOCARDIOGRAM
Maternal conditions
-Diabetes, collagen vascular disease, maternal
drug/terratogen exposure

Family conditions
-h/o CHD, h/o chromosomal or genetic abnormality

Fetal condition
Abnormal HR, suspected cardiac malformation on
screening US, presence of other malformation on US,
oligo or polyhydramnios, evidence of hydrop fetalis, IUGR
 COMMON SYMPTOMS
• Central cyanosis
- bluish discolouration of lips and mucous membranes
- caused by excess deoxygenated hb (>5Gm/dl),
confirmed by pulse oxymetry (spo2 <85%) or ABG
• Sudden collapse
- Difficult to distinguished from sepsis or metabolic
disorders (hypotension, extreme cyanosis, metabolic
acidosis, oliguria)
• Heart failure
-clinical presentation may mimic pulmonary
disease or sepsis: tacypnea, tachycardia,
hepatomegaly, weak pulses
 ACYANOTIC HEART DISEASE
• Communication between the systemic and pulmonary sides of
the circulation, which results in shunting of fully oxygenated
blood back into the lungs.
• The direction and magnitude of the shunt across such a
communication depend on the size of the defect, the relative
pulmonary and systemic pressure and vascular resistances,
and the compliances of the 2 chambers connected by the
defect. These factors are dynamic and may change
dramatically with age.
A) LEFT-RIGHT SHUNT
B) OBSTRUCTIVE LESION
 ATRIAL SEPTAL DEFECT

The majority of cases of ASD are sporadic; autosomal dominant

inheritance does occur as part of the Holt-Oram syndrome or in
families with secundum ASD and heart block
• AUSCULTATION: systolic ejection murmur is heard; it is medium pitched,
without harsh qualities, seldom accompanied by a thrill, and best heard at
the left middle and upper sternal border. It is produced by the increased
flow across the right ventricular outflow tract into the pulmonary artery,
not by low-pressure flow across the ASD.
• CXR:
+ enlargement of the right ventricle and atrium, depending on the size of
the shunt
+ increased pulmonary vascularity
• ECHO: right ventricular volume overload
Small defects
- No treatment

Large defects:
- elective closure at 4-5 years age
- For most patients, the procedure of choice is percutaneous
catheter device closure using an atrial septal occlusion device,
implanted transvenously in the cardiac catheterization
laboratory
VENTRICULAR SEPTAL DEFECT
• Auscultation: loud, harsh, or blowing holosystolic murmur is present and
heard best over the lower left sternal border, and it is frequently
accompanied by a thrill. 2nd heart sound may be increased as a result of
pulmonary hypertension.
• CXR: Pulmonary vascular markings are increased, and frank pulmonary
edema, including pleural effusions, may be present.
Small defects:
• No treatment; high rate of spontaneous closure.
• SBE prophylaxis.
• Yearly follow up for aortic valve prolapse, regurgitation.
• Surgical closure indicated if prolapsed aortic valve.
Moderate defects:
• Anti-failure therapy if heart failure.
• Surgical closure if:
- Heart failure not controlled by medical therapy.
- Persistent cardiomegaly on chest X-ray.
- Elevated pulmonary arterial pressure.
- Aortic valve prolapse or regurgitation.
- One episode of infective endocarditis.
Large defects:
• Early primary surgical closure.
• Pulmonary artery banding followed by VSD closure in multiple VSDs.
 PATENT DUCTUS ARTERIOSUS

Functional closure of the ductus normally occurs soon after birth, but if the ductus
remains patent when pulmonary vascular resistance falls, aortic blood then is
shunted left-to-right into the pulmonary artery.
Balance between constricting and relaxing effects (o2 vs PG)
PDA persisting beyond the 1st few wk of life in a term infant rarely closes
spontaneously or with pharmacologic intervention, whereas if early pharmacologic
or surgical intervention is not required in a premature infant, spontaneous closure
occurs in most instances.
1) Prematurity
- smooth muscle in the wall of the preterm ductus is
less responsive to high Po2 and therefore less likely
to constrict after birth.
2) RDS and surfactant treatment
3) Fluid administration (>IV fluid load)
4) Asphyxia
5) Congenital syndromes
6) Congenital heart diseases
• Physical examination
- auscultation: systolic best heard ar 2nd, 3rd intercostal space at left sternal
border
- Hyperactive precordium (>left ventricular stroke volume), bounding pulse
and increased pulse pressure, hypotension

- Management
1) Ventilatory support
2) Fluid restrictions
3) Increasing hematocrit
4) Indomethacin and ibuprofen to promote ductal closure
c/indication: infection, NEC (decrease mesenteric blood flow), cr
>1.7mg/dl, frank renal or GIT bleed
• Small PDA:
-No treatment if there is no murmur
- If murmur present: elective closure as risk of endarteritis.
• Moderate to large PDA:
- Anti-failure therapy if heart failure
- Timing, method of closure (surgical vs transcatheter)
depends on symptom, severity, size of PDA and body weight
 PULMONARY STENOSIS

The obstruction to outflow from the right ventricle to the pulmonary

artery results in increased right ventricular systolic pressure and wall
stress, which leads to hypertrophy of the right ventricle.
When severe pulmonic stenosis occurs in a neonate, decreased right ventricular
compliance often leads to cyanosis as a result of right-to-left shunting
through a patent foramen ovale, a condition termed critical pulmonic
stenosis.
• AUSCULTATION The pulmonary component of the 2nd sound is usually inaudible. A
loud, long, and harsh systolic ejection murmur, usually accompanied by a thrill, is
maximally audible in the pulmonic area and may radiate over the entire precordium.

• Mild (peak systolic gradient < 50 mmHg)

- No treatment.
• Moderate-severe (gradient > 50 mmHg)
- Transcatheter balloon valvuloplasty is treatment of choice.
• Neonatal critical PS:
- Characterized with cyanosis and RV dysfunction.
- Temporary stabilization with IV Prostaglandin E infusion.
- Early transcatheter balloon valvuloplasty.

Note: SBE prophylaxis is indicated in all cases

COARCTATION OF AORTA
The classic sign of coarctation of
the aorta is a disparity in pulsation
and blood pressure in the arms
and legs. The femoral, popliteal,
posterior tibial, and dorsalis pedis
pulses are weak (or absent in up to
40% of patients), in contrast to the
bounding pulses of the arms and
carotid vessels.
• Neonatal severe CoA:
- Frequently associated with large malaligned VSD, intractable
heart failure.
- Sick infants require temporary stabilization:
• Mechanical ventilation.
• Correction of metabolic acidosis, hypoglycaemia,
electrolyte disorders.
• IV Prostaglandin E infusion (to reopen the ductus and
reestablish adequate lower extremity blood flow)
- Early surgical repair (single-stage CoA repair + VSD closure or 2
stage CoA repair followed by VSD closure at later date).
CYANOTIC HEART
 TETRALOGY OF FALLOT

1. obstruction to right ventricular outflow (pulmonary stenosis)

2. a malalignment type of ventricular septal defect (VSD)
3. Dextroposition of the aorta so that it overrides the ventricular
septum, and
4. right ventricular hypertrophy
Physical Examination
Cyanotic with systolic ejection murmur along left sternal border. Loud
murmur are associated with more flow across right ventricular outflow tract
and milder degree of desaturation.
CXR
Small, boot shaped heart with decrease pulmonary vascular
Markings
ECG
Normal or RVH. T wave in v4r or v1 after 72h ol
• Most TOFs suitable for single stage surgical repair at 1 to 2 years age
• Indications for modified Blalock Taussig shunt:
- Hypercyanotic spells or severe cyanosis < 6 months age when child is too
young for total repair.
- Small pulmonary arteries; to promote growth before definitive repair
- Anomalous coronary artery crossing in front of right ventricular outflow
tract - precludes transannular incision; repair with conduit required at later
age.
• Following surgical repair, patients need life-long follow up for late right
ventricular dysfunction; some may require pulmonary valve replacement.
Tet spells
• Sudden severe episodes of intense cyanosis caused by reduction of pulmonary
flow in patients with underlying Tetralogy of Fallot or other cyanotic heart lesions.,
due to spasm of the right ventricular outflow tract or reduction in systemic
vascular resistance (e.g. hypovolaemia) with resulting increased in right to left
shunt across the VSD.
• Clinical Presentation
- Often in the morning, can be precipitated by crying, feeding, defaecation.
- Severe cyanosis, hyperpnoea, metabolic acidosis.
- In severe cases, may lead to syncope, seizure, stroke or death.
- There is a reduced intensity of systolic murmur during spell.
• Management
• Knee-chest/squatting position:
• Place the baby on the mother’s shoulder with the knees tucked up underneath.
(reduces systemic venous return and increases systemic vascular resistance.)
• Administer 100% oxygen
• Give IV/IM/SC morphine 0.1 – 0.2 mg/kg to reduce distress and hyperpnoea.
 TRANSPOSITION OF GREAT ARTERIES
Cyanosis and tachypnea are most often
recognized within the 1st hr or days of life.
In d-transposition of the great arteries
(d-TGA), the aorta is anterior and
to the right of the pulmonary artery.
Desaturated blood returning from the
body to the right side of
the heart goes inappropriately out the
aorta and back to the body again,
whereas oxygenated pulmonary
venous blood returning to the left side
of the heart is returned directly to the
lungs. Thus, the systemic and
pulmonary circulations exist as 2
parallel circuits. Survival in the
immediate newborn period is
provided by the foramen ovale and
the ductus arteriosus, which permit
some mixture of oxygenated and
deoxygenated blood
Physical Examination
-typical infant is large and vigorous, with cyanosis but little or no respiratory
distress. There may be no murmur or a soft systolic murmur
CXR
Normal or narrow upper mediastinal shadow (egg on stick appearance)
• Simple TGA (intact ventricular septum)
- IV Prostaglandin E infusion promotes intercirculatory mixing at PDA.
- Early balloon atrial septostomy (BAS) if restrictive interatrial communication.
- Surgical repair of choice: arterial switch operation at 2 to 4 weeks age
- Left ventricular regression may occur if repair not performed within 4
weeks of life.
• TGA with VSD:
- Does not usually require intervention during early neonatal period; may
develop heart failure at 1 to 2 months age.
- Elective one-stage arterial switch operation + VSD closure < 3 months age.
• TGA with VSD and PS:
- Blalock Taussig shunt during infancy followed by Rastelli repair at 4 to 6 years
age
 TRUNCUS ARTERIOSUS
a single arterial trunk (truncus arteriosus)
arises from the heart and supplies the
systemic, pulmonary, and coronary
circulations.

A VSD is always present, with the truncus

overriding the defect and receiving blood
from both the right and left ventricles.

When pulmonary vascular resistance is

relatively high immediately after birth,
pulmonary blood flow may be normal; as
pulmonary resistance drops in the 1st mo
of life, blood flow to the lungs is greatly
increased and heart failure ensues
• Surgical repair (VSD closure and RV-to-PA
conduit) before 3 months of age
 HEART FAILURE
Definition
inability to provide adequate cardiac output to meet the metabolic demand of the
body

• Clinical presentation
Varies with age of presentation.
Symptoms of heart failure in infancy:
• Feeding difficulty: poor suck, prolonged time to feed, sweating during feed.
• Recurrent chest infections.
• Failure to thrive.
Signs of heart failure in infancy:
• Resting tachypnoea, subcostal recession.
• Tachycardia, Poor peripheral pulses, poor peripheral perfusion.
• Hyperactive praecordium, praecordial bulge.
• Hepatomegaly.
• Wheezing.
Common signs of heart failure in adults, i.e. increased jugular venous pressure, leg
oedema and basal lung crackles are not usually found in children.
 TREATMENT
General measures
• Oxygen supplementation, propped up position
• Keep warm, gentle handling.
• Fluid restriction to ¾ normal maintenance if not dehydrated
or in shock
• Optimize caloric intake; low threshold for nasogastric
feeding;
- consider overnight continuous infusion feeds.
• Correct anaemia, electrolyte imbalance, treat concomitant
chest infections .
Antifailure medications
• Frusemide (loop diuretic)
- Dose: 1 mg/kg/dose OD to QID, oral or IV
- Continuous IV infusion at 0.1 – 0.5 mg/kg/hour if severe fluid overload
- Use with potassium supplements (1 - 2 mmol/kg/day) or add potassium sparing
diuretics.
• Spironolactone (potassium sparing diuretic, modest diuretic effect)
- Dose: 1 mg/kg/dose BD
• Captopril
- Angiotensin converting enzyme inhibitor, afterload reduction agent
- Dose: 0.1 mg/kg/dose TDS, gradual increase up to 1 mg/kg/dose TDS
- Monitor potassium level (risk of hyperkalaemia)
• Digoxin
- Role controversial
- Useful in heart failure with excessive tachycardia, supraventricular tachyarrhythmias.
• IV inotropic agents - i.e. Dopamine, Dobutamine, Adrenaline, Milrinone
- Use in acute heart failure, cardiogenic shock, post-op low output syndrome.
• Specific management
- Establishment of definitive aetiology is of crucial
importance
- Specific treatment targeted to underlying aetiology.