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 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.


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.
Mahle
W T
WT
Divisions of Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Spray
T L
TL
Wernovsky
G
G
Gaynor
J W
JW
Clark
B J
BJ
D001835:Body Weight; D013504:Cardiovascular Surgical Procedures / Q000401:mortality* / Q000706:statistics & numerical data; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000401:mortality / Q000706:statistics & numerical data; D016027:Heart Transplantation / Q000401:mortality; D006786:Hospitals, Urban / Q000706:statistics & numerical data; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D013995:Time; D016896:Treatment Outcome