Circulation, Vol 89, 243-251, Copyright © 1994 by American Heart Association
AJ Marelli, JK Perloff, JS Child and H Laks
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.
ARTICLES
Pulmonary atresia with ventricular septal defect in adults
Department of Medicine, University of California, Los Angeles 90024- 1679.
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