Survival after reconstructive surgery for hypoplastic left heart syndrome - A 15-year experience from a single institution

Background-There are limited data regarding the long-term survival of patie nts who have undergone reconstructive surgery for hypoplastic left heart sy ndrome (HLHS). We reviewed the 15-year experience at our institution to exa mine survival in the context of continued improvements in early operative r esults. Methods and Results-Between 1984 and 1999, 840 patients underwent stage I s urgery for HLHS. From review of medical records and direct patient contact, survival status was determined. The 1-, 2-, 5-, 10-, and 15-year survival for the entire cohort was 51%, 43%, 40%, 39%, and 39%, respectively. Late d eath occurred in 14 of the 291 patients discharged to home after the Fontan procedure, although only 1 patient has died beyond 5 years of age. Heart t ransplantation after stage I reconstruction was performed in 5 patients. La ter era of stage I surgery was associated with significantly improved survi val (P<0.001). Three-year survival for patients undergoing stage I reconstr uction from 1995 to 1998 was 66% versus 28% for those patients undergoing s urgery from 1984 to 1988. Age >14 days at stage I and weight <2.5 kg at sta ge I were also associated with higher mortality (P=0.004 and P=0.01, respec tively). Other variables, including anatomic subtype, heterotaxia, and age at subsequent staging procedures, were not associated with survival. Conclusions-Over the 15-year course of this study, early- and intermediate- term survival for patients with HLHS undergoing staged palliation increased significantly. Late death and the need for cardiac transplantation were un common.