[Surgical treatment of total anomalous pulmonary venous connection]

Surgical experience of 70 patients with TAPVC between 1966 and 1982 were reviewed. Their age ranged from 2 days to 13 years. Fifty-two patients (74%) were below 6 months of age, 36 (51) below 3 months and 17 (24%) were neonates, respectively. Thirty-seven patients (56%) were with supracardiac type, 14 (20%) were with cardiac type and 12 (17%) were with intracardiac type. Results of operation below 6 months of age were unsatisfactory. Only 10 patients including 3 neonates out of 52 survived. However, 16 of 18 patients over 6 months of age survived. Early referral and emergency intracardiac repair is important in small babies as well as precise operation and fine bypass technique. Patients with supersystemic pulmonary hypertension immediately after surgery showed poor prognosis. Long-term results with 26 survivors were satisfactory. Eighty-one % of them showed sinus rhythm and %LVEDV and LVEF were within normal range in 6 patients investigated. In recent years, surgery without angiocardiography is recommended as the diagnostic ability of UCG developed. However, care must be taken as to some rare complicated pulmonary venous connection and associated cardiac anomaly such as VSD. Also, it should be recognized that pulmonary venous beds in neonates and early infancy demonstrated various degree of mascularization , medial hypertrophy and endothelial cell proliferation in intraacinar pulmonary veins. They were more marked in cases with intracardiac type TAPVC .