OUTCOME OF PULMONARY ATRESIA AND VENTRICULAR SEPTAL-DEFECT DURING INFANCY

We evaluated 54 patients with pulmonary atresia and ventricular septal defect who were referred during the first year of life between 1972 a nd 1992. Particular emphasis was given to the nature of the pulmonary blood supply and its influence on outcome. Ductal supply of confluent pulmonary arteries was present in 30 patients (55.6%, group I), wherea s 24 patients (44.4%, group II) had a pulmonary blood supply that was entirely (31.4%) or predominantly (13.0%) dependent on systemic collat eral arteries. Over the 20 years there was no significant difference i n actuarial survival between the two groups. Corrective surgery was pe rformed in 8 of 30 patients in group I (26.7%)-significantly more than in group II (4 of 24, 16.7%). Arborization abnormalities of the pulmo nary arteries (stenosis of unbranched and intrapulmonary arteries) wer e almost exclusively present in patients with systemic collateral arte ries (p < 0.03), accounting for the lower probability of undergoing co rrective surgery in group II patients. During the first decade of this study (1973-1983) corrective surgery was attempted in 9.6% of patient s, with 42% mortality; and during the second decade (1983-1993) surger y was performed in 39.1% of patients, with 26% mortality, a significan tly lower figure. Improving surgical results, complete preoperative de marcation of the pulmonary blood supply, and a more aggressive approac h with early unifocalization of the pulmonary blood supply may invalid ate comparison with retrospective data on the advisability of attempti ng to correct this anomaly. The present paper provides data against wh ich treatment of infants with pulmonary atresia and ventricular septal defect presenting during the next decade can be compared.