[Background] For hypoplastic left heart syndrome (HLHS), bilateral pulmonary artery banding (b-PAB) as initial intervention followed by Norwood and Glenn procedure over the neonatal period is recently promising result.
[Case] A case of HLHS, bilateral superior vena cava, interruption of inferior vena cava (IVC) with azygos connection was diagnosed during fetal period. The baby weighed 2564g was born 37 weeks at gestational age by elective cesarian section. Two days after birth b-PAB was done on continuous infusion of prostagrandin E1. Over the early neonatal period, jaundice persisted. Direct bilirubin and transaminase gradually increased. Imaging studies showed normal gall bladder and bile duct in size and appearance, so could not confirmed congenital bilirary atresia (BA).We concluded Norwood procedure followed by laparotomy and repair of BA if defined until 60 days old. However her parents decided conservative treatment and familial touching during her life. On 120 days after birth she was died being held by her mother.
[Autopsy] Polysplenia. Foramen ovale was almost closed. Clinically undiagnosed partial anomalous pulmonary venous return (right upper pulmonary vein to SVC) was only cardiac return from lung. Pulmonary hypertension because of pulmonary congestion was more severe in left lung with thickened endothelium and lymphangiectasia. Not macroscopic, but microscopic examination revealed congenital BA (III, c2, beta). [Discussions] For peritoneal dialysis immediately after Norwood procedure, laparotomy was not done. Ethical issue for severe extracardiac complication with HLHS will be discussed.