Dr Adeena ,Dr Jayasree(Neo), Dr Jayakumar
Department of Pediatrics
AIMS KOCHI
Seven hour old ba was referred to AIMS in view of congenital heart disease detected antenatally for a 30 year old G2P1L1 mother, GDM on oral hypoglycemics
Third trimester evaluation showed mitral atresia , hypoplastic left heart syndrome.
Ba was born at 30 wk+2/7 of gestation vaginal delivery. (Spontaneous expulsion, ?cord presentation).
Ba didnot cry immediately after birth, and was started on Positive pressure ventilation had a weak cry 4 minutes after birth and heart rate improved to > 100/min, continued on CPAP & later intubated
Caffeine loading and prostaglandin infusion was given and referred here.
At admission,
wt 1.05kg, HC-25 cm, L 38cm
RS- Air entry equal , tachypneic, bilateral intercostal retractions, Transillumination negative
CVS -S1,S2 heard.Systolic murmur noted.
Bilateral femorals palpated
Pre and post ductal saturation >94%
Bilateral descended testis
Hypoplastic toe nails
Bilateral hazy corneas
Cxr showed well expanded lung fields with mild haziness.
He was continued on SIMV mode.Echo showed hypoplastic mital valve, aortic valve and LV with severe TR and large PDA supplying descending aorta.
PgE1 infusion was continued.
Management with multiple staged correction surgery was discussed.
Eyes- congenital galucoma and aniridia, left eye microspaerophakia with cataract.
Neuro sonogram normal.In view of symmetrical SGA and CHD ,DNA isolation and karyotyping was sent.
In view of prematurity, complex heart disease and anomalies, with a guarded prognosis, parents opted for DNI/DNR.He progressively deteriorated , had desaturations, bradycardia and expired.Parents were advised genetic counselling and ECHO for the elder sibling .
HYPOPLASTIC LEFT HEART SYNDROME
Heterogenoces group of anatomic abnormalities with small to absent left ventricle with hypoplastic to atretic mitral or aortic valves.RV supplies both pulmonary and systemic blood flow through the PDA
As PVR falls, blood is directed to pulmonary circulation- early onset CCF IN 1st week of life.
Management is maintaining ductal patency with PgE1 infusion.Ventilatory manouevere to increase PVR.In severely restrictive PFO,baloon dilation of atrial septum.Surgical therapy-neonatal Norwood procedure followed Glenn in infancy and Fontan in childhood.