Infant cardiac transplantation for hypoplastic left heart syndrome (HLHS) can be performed with a high success rate, but it remains unclear whether variations in anatomy or degree of illness before transplantation can affect ultimate survival.Methods and Results
We analyzed the initial echocardiograms and pretransplant courses of 21 infants whose families chose transplantation as a primary therapy option for factors influencing survival. Sixteen of 21 were transplanted (mean wait, 18 days; range, 3 to 97 days) with 15 current (6- to 36-month) survivors, leading to a survival rate from presentation of 71% (70%o confidence limit [CL], 61% to 81%) and an operative survival of 94% (70% CL, 81% to 99%). Seven of 20 listed for transplant (35%) had a severely restrictive interatrial communication, defined as a color flow jet width of 3 mm or less across the interatrial septum. Three of these 7 infants died at 4 (2) and 7 (1) weeks from congestive heart failure and progressive hypoxemia before transplantation. One died 2 months after transplant with an autopsy demonstrating pulmonary venous hypertensive disease, leading to a significantly higher relative risk for death (relative risk=7.4, P=.01) for those infants with a severely restrictive interatrial communication versus those infants without severe restriction. The size and function of the right ventricle, left atrium, aorta, and tricuspid valve, as well as the magnitude of support on presentation, were not related to ultimate survival.Conclusions
A severely restrictive interatrial communication is a frequent component of HLHS. When primary cardiac transplantation is the chosen therapy for HLHS, it is a significant negative risk factor for death primarily before transplantation.