25-Year Mortality After Surgical Repair of Congenital Heart Defect in Childhood: A Population-Based Cohort Study

Objective.—To determine long-term survival and the cause of death after repair of one of eight congenital heart defects in childhood. Design.—Cohort study. Setting.—General community. Participants.—All Oregon residents with one of eight congenital heart defects, which was repaired surgically between 1958 and 1989 when the patient was aged 18 years or younger, including (1) tetralogy of Fallot; (2) isolated ventricular septal defect; (3) isolated atrial septal defect; (4) coarctation of the aorta; (5) aortic valvular stenosis; (6) pulmonary valvular stenosis; (7) transposition of the great arteries; and (8) patent ductus arteriosus. Follow-up of this cohort of 2701 individuals was obtained from 94%. Main Outcome Measure.—Mortality from cardiac and noncardiac causes. Results.—Age at surgery and operative mortality have decreased significantly over the last 30 years. Late cardiac mortality at 25 years after surgery was 5% for tetralogy of Fallot and isolated ventricular septal defect, 10% for coarctation of the aorta, 17% for aortic stenosis, 5% for pulmonic stenosis, and less than 1% for patent ductus arteriosus; there were no late cardiac deaths after atrial septal defect repair. For transposition, late cardiac mortality was 15% at 15 years after the Mustard operation and was 2% at 10 years after the Senning operation. Conclusion.—Surgical repair of most congenital heart defects is associated with lingering cardiac mortality, particularly for aortic stenosis, coarctation, and transposition.