Early experience on the standard Norwood stageIprocedure for hypoplastic left heart syndrome

Objective Norwood StageⅠis the standard procedure to cope with hypoplastic left heart syndrome(HLHS), which continues to be the most challenging congenital heart disease. The aim of this study is to retrospectively analyse the perioperative management of Norwood StageⅠ. Methods Between June 2010 and August 2014, totally 5 small infants with HLHS underwent the standard Norwood StageⅠprocedure. They were all boys. Age at surgeries ranged from 29 to 75 days, and weight from 2.57-3.50 kg with median of 3.13 kg. Case 1, 2 and 3 received standard medical regimen after accessing NICU, which included intravenous prostaglandin E1 and mechanical ventilation. The 3 infants underwent emergent operations because of unstable hemodynamics. Case 4 and 5 received no medical intervention before the urgent surgeries. All 5 cases underwent the standard Norwood StageⅠprocedure under deep hypothermic circulatory arrest, including 4 cases of modified Blalock-Taussig shunt(MBTS) and 1 case of Sano shunt. Results The case with Sano shunt died from severe hypoxemia and persistent acidosis 32 hours after the operation, another case died from low cardiac output syndrome after cardiopulmonary bypass. The first case underwent bidirectional Glenn procedure 12 months after Norwood Stage I, the postoperative heart function was NYHA I and the oxygen saturation was 0.90-0.95 in room air, but he died from accidental brain injury 3 months after stage Ⅱ. The second case was followed up 3 months after stage I procedure with NYHA I and oxygen saturation of 0.78-0.83 in room air, and lost the follow-up after. The fifth case was followed up 3 months after stageⅠprocedure with NYHAⅠ, confluent MBTS and oxygen saturation of 0.84, the patient is being followed up and waiting for further evaluation for stageⅡprocedures. Conclusion The standard Norwood StageⅠprocedure is a complex procedure, which demands multidisplinary cooperation, to palliatively correct HLHS. Sharing expericences on perioperative managements of Norwood Stage I between heart centers in China will be helpful to decrease the mortality and morbidity in relatively short period. Key words: Heart defects, congenital; Hypoplastic left heart syndrome; Norwood procedures, state Ⅰ; Perioperative