Pulmonary atresia with ventricular septal defect in adults.

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Abstract
BACKGROUND: Multistage surgery culminating in completed hemodynamic repair is now performed for pulmonary atresia with ventricular septal defect (PA-VSD). Justification for operation in patients with an adequate collateral pulmonary circulation is controversial. Data on natural adult survival are scant but are necessary to provide the rationale for multistage reconstructive procedures. METHODS AND RESULTS: All cyanotic adults with PA-VSD in the UCLA Adult Congenital Heart Disease Center Registry from 1978 through 1992 formed the basis for this study. Registry data and echocardiographic, hemodynamic, and angiographic information were used to determine longevity, clinical course, and operative feasibility. Of 26 patients, 16 were unoperated when referred (group A), and 10 had been palliated before age 18 years (group B). Two thirds were 18 to 29 years old. Only 2 patients survived beyond age 40 years. Six died during follow-up at a mean age of 31 years (+/- 12.1 SD). Eight group A patients were in New York Heart Association class II, and 8 were in class III. Of the 26 group A and B patients, 20 had aortic regurgitation, which was moderate or severe in 10. Eight had cardiac failure. Of 11 group A patients who remained unoperated, 5 died. Twelve patients were considered eligible for surgery at > or = 18 years of age. Ten underwent completed hemodynamic repair with a mean post-operative right ventricular-to-left ventricular systolic pressure ratio of 0.45 (+/- 0.16 SD), and there were no early or late deaths. CONCLUSIONS: Even when collateral blood flow permits adult survival, all such patients are symptomatic. Mean life expectancy without operation did not exceed three decades. Aortic regurgitation and cardiac failure are significant negative variables. Nearly half of unoperated adults died during follow-up. Staged hemodynamic repair can be performed with a low surgical risk in properly selected adults with PA/VSD.