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Pulmonary atresia with intact ventricular septum has continued to have a high surgical mortality and morbidity. This mAy attribute to the non-uniformity of the anomaly. We reviewed a total of 34 infants with pulmonary atresla and intact ventricular septum managed in this hospital between 1987 and 19...

Pulmonary atresia with intact ventricular septum has continued to have a high surgical mortality and morbidity. This mAy attribute to the non-uniformity of the anomaly. We reviewed a total of 34 infants with pulmonary atresla and intact ventricular septum managed in this hospital between 1987 and 1995. Mean age and body weight were 57.2 (range, 3-208) days and 4.1 (range, B.3∼6.8) kg. The preoperative Z-value of the diameter of the tricuspid valve was less than -2 in 85.2% of patients and less than -4 in 33. 3% . It is well correlated w th right ventricular cavity size (n=27. r10.68, p< 0.05). Coronary artery-right ventricular fistulas were identified in 3 patients, and right ventricular dependency was suspected in 1 Over All hospital mortality was 23.5%(8/34), although it decreased to 16.6%(4124) in 1990s. Subsequent procedures were performed in 6 patients between 3 days and 58 months after Initial palliation : one bidirectional cavopulmonary shunt and 1 Fontan operation after systemic-pulmonary shunt, 3 transannular patch + atrial septal defect closure and 1 additional systemic-pulmonary shunt after polmonary valvectomy or valvotomy. Changes of Z-values of the diameter of tricuspid valve have been followed up in 11 patients between 1 and 66 months postoperatively. Z-values were increased In 5 out of 8 transannular right ventriculAr outflow tract enlargement group and in 1 out of 3 pulmonary valvectomy or valvotomy group. Our data suggest that tailoring a treatment to right ventricular cavity size and coronary anom lies may improve the surgical outcome. A Z-value of the tricuspid valve diameter could be used.

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