Pulmonary overcirculation following left atrial decompression in hypoplastic left heart syndrome with intact/restrictive atrial septum

Patients with hypoplastic left heart syndrome (HLHS) with intact or severely restrictive atrial septum have slow resolution of their elevated pulmonary vascular resistance after left atrial decompression. We report two neonates who required urgent surgical intervention because of unusually rapid fall in their pulmonary vascular resistance following left atrial decompression leading to pulmonary overcirculation and severe congestive heart failure. HLHS with intact or severely restrictive atrial septum is a predictor of poor outcome. The need for left atrial decompression can be predicted by well defined fetal echocardiographic indicators. However evolution of pulmonary resistance can be difficult to predict. In some patients decompression of the left atrium can lead to early excessive pulmonary blood flow and congestive heart failure requiring urgent intervention. Here, we report two cases of HLHS with highly restrictive atrial septum that after atrial septostomy had an accelerated transition requiring urgent intervention. Because of retrospective nature of the study, Children's National Medical Center approved waiver of informed consent.