Abstract

This report provides follow - up data on 37 patients, aged 7 days to 25 years [median: 6.5 months], who underwent repair of total anomalous pulmonary venous connection at Seoul national University Hospital between May, 1978 and June, 1987. The patients were 22 males and 17 females and the sex ratio was 1.6 to 1, showing a male predominance. Sixteen patients had supracardiac, 13 cardiac, 3 infracardiac and 5 had a mixed type. The duration of follow up was from 1 month to 60 months [median: 14 months] There were eight early and one late deaths, and the overall mortality was 24%. The deaths during 1 year of life were eight [89%] and only one death [11%] occurred above 1 year of age. The mortality of cardiac type was unusually high, accounting for 56 percent of the total death, which was probably due to the preoperative poor clinical condition such as pulmonary edema and congestive heart failure. The major cause of death was the perioperative myocardial failure, and the survival was closely related to the preoperative clinical status, age and moderately elevated pulmonary arterial pressure, the sign of the elevated pulmonary vascular resistance and pulmonary venous obstruction. Early diagnosis and early application of surgical intervention is essential to the improved postoperative survival

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