OBJECTIVES: This study summarized patterns of presentation and attrition in complex pulmonary atresia. BACKGROUND: Assessment of the potential impact of surgical strategies for managing complex pulmonary atresia requires information about variability in age and physiology at presentation of the condition. METHODS: We performed a retrospective review of age at presentation, referral source, pulmonary artery and collateral anatomy and surgical history of 218 patients from two institutions dealing with congenital heart disease throughout life. RESULTS: Approximately 65% of pulmonary atresia appears in infancy, with 50% of patients severely symptomatic from cyanosis and 25% from heart failure. Compared with those presenting undiagnosed, patients referred secondarily for specialist management tend to be older when first seen, and care must be taken when generalizing about the natural history of the condition from their survival experience. Overall actuarial survival, including the effects of operation, suggests that 60% (95% confidence limits [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 survive to the age of 10, and 16% (95% CL 5 to 31) of those alive at 10 years old survive to age 35. CONCLUSIONS: Novel surgical approaches have generally been applied beyond infancy in patients selected by their survival through the period of greatest attrition for this disease. Unless 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.
OBJECTIVES: This study summarized patterns of presentation and attrition in complex pulmonary atresia. BACKGROUND: Assessment of the potential impact of surgical strategies for managing complex pulmonary atresia requires information about variability in age and physiology at presentation of the condition. METHODS: We performed a retrospective review of age at presentation, referral source, pulmonary artery and collateral anatomy and surgical history of 218 patients from two institutions dealing with congenital heart disease throughout life. RESULTS: Approximately 65% of pulmonary atresia appears in infancy, with 50% of patients severely symptomatic from cyanosis and 25% from heart failure. Compared with those presenting undiagnosed, patients referred secondarily for specialist management tend to be older when first seen, and care must be taken when generalizing about the natural history of the condition from their survival experience. Overall actuarial survival, including the effects of operation, suggests that 60% (95% confidence limits [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 survive to the age of 10, and 16% (95% CL 5 to 31) of those alive at 10 years old survive to age 35. CONCLUSIONS: Novel surgical approaches have generally been applied beyond infancy in patients selected by their survival through the period of greatest attrition for this disease. Unless 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.