PRESENTATION AND ATTRITION IN COMPLEX PULMONARY ATRESIA

Objectives. This study summarized patterns of presentation and attriti on in complex pulmonary atresia. Background. Assessment of the potenti al impact of surgical strategies for managing complex pulmonary atresi a requires information about variability in age and physiology at pres eotation of the condition. Methods. We performed a retrospective revie w of age at presentation, referral source, pulmonary artery and collat eral anatomy and surgical history of 218 patients from two institution s dealing with congenital heart disease throughout life. Results. Appr oximately 65% of pulmonary atresia appears in infancy, with 50% of pat ients severely symptomatic from cyanosis and 25% from heart failure. C ompared with those presenting undiagnosed, patients referred secondari ly for specialist management tend to be older when first seen, and car e must be taken when generalizing about the natural history of the con dition from their survival experience. Overall actuarial survival, inc luding the effects of operation, suggests that 60% (95% confidence lim its [CL] 43 to 73) of patients presenting in infancy survive to their first birthday, 65% (95% CL 51 to 74) of those alive at 1 year old sur vive to the age of 10, and 16% (95% CL 5 to 31) of those alive at 10 y ears old survive to age 35. Conclusions. Novel surgical approaches hav e generally been applied beyond infancy in patients selected by their survival through the period of greatest attrition for this disease. Un less successful application in symptomatic infants is demonstrated, we cannot assume that these serial and complicated operations will have a major impact on the outlook of most patients with complex pulmonary atresia.