Improving results with first-stage palliation for hypoplastic left heart syndrome.
Between January 1990 and February 1993, 73 patients underwent first-stage reconstruction for hypoplastic left heart syndrome at the University of Michigan Medical Center. During this period, surgical reconstruction remained essentially constant and consisted of a pulmonary artery-to-aorta anastomosis with allograft augmentation of the ascending, transverse, and proximal descending aorta, restriction of pulmonary blood flow with a polytetrafluoroethylene shunt from the innominate artery to the central pulmonary artery confluence, and atrial septectomy. Hospital survival was 62 of 73 patients, 85% (70% confidence limits: 80% to 89%). These results stand in marked contrast to those obtained during the earlier years of our experience from 1986 to 1989 when only 21 of 50 patients (42%, 70% confidence limits: 35% to 49%) survived (p = 0.001). Among the most recent group of patients, only 2 of 7 patients older than 1 month of age at operation survived, whereas 60 of 66 (91%, 70% confidence limits: 87% to 94%) patients younger than 1 month of age survived (p = 0.0001). Anatomic subtype and ascending aortic diameter were not predictive of survival. Actuarial survivals for those patients younger than 1 month of age at the first-stage operation, including hospital deaths and subsequent operative procedures, were 81%, 74%, and 74% at 6 months, 1 year, and 2 years, respectively. These results indicate that survival for patients after first-stage reconstruction for hypoplastic left heart syndrome has significantly improved in recent years. Older age was a strong risk factor, with a hospital survival of 91% for those patients undergoing first-stage palliation within the first month of life. These data have important implications for the type of operative intervention and its timing.
Iannettoni
M D
MD
Department of Surgery, University of Michigan Medical School, Ann Arbor.
Bove
E L
EL
Mosca
R S
RS
Lupinetti
F M
FM
Dorostkar
P C
PC
Ludomirsky
A
A
Crowley
D C
DC
Kulik
T J
TJ
Rosenthal
A
A
D000206:Actuarial Analysis; D001011:Aorta / Q000601:surgery; D001013:Aorta, Thoracic / Q000601:surgery; D001807:Blood Vessel Prosthesis; D016122:Brachiocephalic Trunk / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care / Q000379:methods*; D011651:Pulmonary Artery / Q000601:surgery; D012307:Risk Factors; D013577:Syndrome; D013997:Time Factors