title: Improving results with first-stage palliation for hypoplastic left heart syndrome.
abstract: 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.
authors.affiliation: Department of Surgery, University of Michigan Medical School, Ann Arbor.
authors.forename: M D
authors.initials: MD
authors.lastname: Iannettoni
authors.forename: E L
authors.initials: EL
authors.lastname: Bove
authors.forename: R S
authors.initials: RS
authors.lastname: Mosca
authors.forename: F M
authors.initials: FM
authors.lastname: Lupinetti
authors.forename: P C
authors.initials: PC
authors.lastname: Dorostkar
authors.forename: A
authors.initials: A
authors.lastname: Ludomirsky
authors.forename: D C
authors.initials: DC
authors.lastname: Crowley
authors.forename: T J
authors.initials: TJ
authors.lastname: Kulik
authors.forename: A
authors.initials: A
authors.lastname: Rosenthal
mesh_terms: 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