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(Circulation. 2000;102:III-136.)
© 2000 American Heart Association, Inc.

Surgery for Congenital Heart Disease

Survival After Reconstructive Surgery for Hypoplastic Left Heart Syndrome

A 15-Year Experience From a Single Institution

William T. Mahle, MD; Thomas L. Spray, MD; Gil Wernovsky, MD; J. William Gaynor, MD; Bernard J. Clark, III, MD

From the Divisions of Cardiology (W.T.M., G.W., B.J.C.) and Cardiothoracic Surgery (T.L.S., J.W.G.), The Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pa.

Correspondence to Bernard J. Clark IIII, MD, The Children’s Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104.

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.

Key Words: surgery • mortality • heart defects, congenital • survival

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