Hypoplastic Left Heart Syndrome, or HLHS, is among the most severe forms of congenital heart disease a newborn can be born with. The left side of the heart is underdeveloped to the point where it cannot support the body’s circulation, and without immediate intervention, the condition becomes life-threatening within days.This is exactly the situation a ten-day-old baby from Nanded arrived in at the hospital. The infant came in critically unstable, already intubated and on prostaglandin E1 to keep a crucial blood vessel open. The cardiac anatomy was complex, a hypoplastic mitral valve and left ventricle, a severely underdeveloped aortic arch close to interruption, large septal defects with bidirectional shunting, and a moderate patent ductus arteriosus. The measurements reflected just how extreme the underdevelopment was, with the aortic arch recording a Z-score of -8.58.Over the following days, the baby was stabilised with ventilatory support, inotropic medications, antibiotics, and parenteral nutrition. Advanced CT imaging was used to map the vascular anatomy before planning surgery.The baby underwent a Norwood procedure with a Sano shunt on 29th May 2026. The surgery lasted 227 minutes on cardiopulmonary bypass, with no intraoperative complications. Post-surgical imaging showed good flow across the shunt, a well-open outflow tract, preserved ventricular function, and no fluid accumulations around the heart or lungs.The postoperative period had its challenges. The chest was initially left open given intraoperative bleeding concerns and was successfully closed on day two. The baby was extubated on day five, but the following day brought a setback, respiratory deterioration, rising infection markers, and a positive culture for Pseudomonas aeruginosa. The infant was re-intubated and started on targeted antibiotics. Within 24 hours, there was clear improvement. Lactate levels normalised, haemodynamics stabilised, and the baby was gradually stepped down from ventilatory support.By postoperative day eight, all inotropic support had been stopped. The infant was breathing room air with stable saturations, and the recovery from that point was uneventful. The baby was discharged on postoperative day 23, feeding orally and on medications, with follow-up planned for the next stages of surgical management.According to Dr Smruti Rajan Mohanty, Consultant, Paediatric Cardiovascular Surgery at Kokilaben Dhirubhai Ambani Hospital, outcomes like this reflect the importance of early stabilisation, precise anatomical assessment, and careful surgical execution. “The Norwood-Sano procedure is one of the most complex neonatal cardiac surgeries, where success depends not only on surgical precision but also on coordinated intensive care and timely postoperative decision-making,” he noted.Every case of this nature is managed through a multidisciplinary team of paediatric cardiac surgeons, anaesthetists, intensivists, cardiologists, perfusionists, and neonatal intensive care specialists at Kokilaben Dhirubhai Ambani Hospital. Advances in neonatal cardiac surgery, Dr Smruti Mohanty added, have meaningfully changed what is possible even in conditions that were once considered uniformly fatal.
