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We reviewed the records of 135 patients with tricuspid atresia and 120 patients with double-inlet ventricle who underwent modified Fontan procedure from 1973 to June 1985. This represents 67% of the total number of patients undergoing the procedure during this period. There were 32 (12%) in-hospital deaths (14 tricuspid atresia, 18 double-inlet ventricle) and 23 late deaths (13 tricuspid atresia, 10 double-inlet ventricle). For patients surviving to hospital dismissal, the 5 year survival was 87%. Follow-up data (mean 3.9 years) were obtained for 157 patients who survived at least 1 year after operation. At last follow-up, of the 157 patients, 92% were in NYHA class I or II, 92% claimed to be improved clinically by the operation, and 98% could work or attend school. Fifty percent were not taking medication at last follow-up. Some degree of edema or ascites was found in 16%. The study showed that (1) the modified Fontan procedure can be performed with low in-hospital mortality (12%) in patients with tricuspid atresia and double-inlet ventricle, (2) hospital survivors have an estimated 5 year survival of 87%, (3) subjectively, functional status is good for hospital survivors at intermediate follow-up, and (4) fluid retention continues to be a problem in a minority (16%) of patients.
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