Finding the correct role for heart transplant in the treatment of hypoplastic left heart syndrome

Abstract

Hypoplastic left heart syndrome (HLHS) with aortic atresia has evolved from a universally fatal disease when Norwood et al performed the first staged surgical palliations in 1979 to one with reasonable intermediate term outcomes. Despite remarkable surgical advances, transplant-free survival in this group of patients at 3 years of age is only 64%. Heart transplantation as a primary treatment for HLHS has been advocated principally by Bailey et al at Loma Linda Children’s Hospital. Although primary transplantation offers the benefit of avoiding multiple palliative surgical procedures, often with excellent long-term posttransplant survival, the mismatch between a limited supply of infant donor hearts and the large number of infants born with HLHS has relegated heart transplantation to a rescue therapy for most of these children at our institution. The current issue of The Journal of Heart and Lung Transplantation presents the contemporary experience with listing, waiting list survival, and post-transplant survival for infants and children with HLHS and other single right ventricular (RV) anomalies. Kulkarni et al used the publically available Single Ventricle Reconstruction (SVR) data set to describe the current state of heart transplantation as a rescue therapy after single-ventricle palliation in a well-characterized cohort. The outcomes for heart transplantation in this setting are disappointing. Of the 555 patients in the SVR trial, 33 were listed for heart transplant, and only 48% of those 33 patients were still alive 1 year after listing. This was due to a waiting list mortality of 39% amongst the group and a post-transplant mortality of 33%.