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

Circulation. 2000 Nov 7;102(19 Suppl 3):III136-41. doi: 10.1161/01.cir.102.suppl_3.iii-136.


Background: There are limited data regarding the long-term survival of patients who have undergone reconstructive surgery for hypoplastic left heart syndrome (HLHS). We reviewed the 15-year experience at our institution to examine survival in the context of continued improvements in early operative results.

Methods and results: Between 1984 and 1999, 840 patients underwent stage I surgery 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 death 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 transplantation after stage I reconstruction was performed in 5 patients. Later era of stage I surgery was associated with significantly improved survival (P:<0.001). Three-year survival for patients undergoing stage I reconstruction from 1995 to 1998 was 66% versus 28% for those patients undergoing surgery from 1984 to 1988. Age >14 days at stage I and weight <2.5 kg at stage I were also associated with higher mortality (P:=0.004 and P:=0.01, respectively). 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 uncommon.

MeSH terms

  • Body Weight
  • Cardiovascular Surgical Procedures / mortality*
  • Cardiovascular Surgical Procedures / statistics & numerical data
  • Cohort Studies
  • Female
  • Follow-Up Studies
  • Fontan Procedure / mortality
  • Fontan Procedure / statistics & numerical data
  • Heart Transplantation / mortality
  • Hospitals, Urban / statistics & numerical data
  • Humans
  • Hypoplastic Left Heart Syndrome / surgery*
  • Infant
  • Infant, Newborn
  • Male
  • Palliative Care
  • Retrospective Studies
  • Risk Factors
  • Survival Rate / trends
  • Time
  • Treatment Outcome