PULMONARY ATRESIA WITH VENTRICULAR SEPTAL-DEFECT IN ADULTS

Background Multistage surgery culminating in completed hemodynamic rep air is now performed for pulmonary atresia with ventricular septal def ect (PA-VSD). Justification for operation in patients with an adequate collateral pulmonary circulation is controversial. Data on natural ad ult 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. Regis try data and echocardiographic, hemodynamic, and angiographic informat ion were used to determine longevity, clinical course, and operative f easibility. 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). Eig ht 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 re gurgitation, which was moderate or severe in 10. Eight had cardiac fai lure. Of 11 group A patients who remained unoperated, 5 died. Twelve p atients were considered eligible for surgery at greater than or equal to 18 years of age. Ten underwent completed hemodynamic repair with a mean postoperative right ventricular-to-left ventricular systolic pres sure ratio of 0.45 (+/-0.16 SD), and there were no early or late death s. Conclusions Even when collateral blood flow permits adult survival, an such patients are symptomatic. Mean life expectancy without operat ion did not exceed three decades. Aortic regurgitation and cardiac fai lure are significant negative variables. Nearly half of unoperated adu lts died during follow-up. Staged hemodynamic repair can be performed with a low surgical risk in properly selected adults with PA/VSD.