PMID: 8940853Oct 1, 1996Paper

A case of total cavopulmonary connection with preceding tricuspid valve closure in pulmonary atresia and intact ventricular septum, right ventricular-coronary artery fistulae, and coronary artery stenoses

[Zasshi] [Journal]
Y ChoA Aki

Abstract

More than half of the patients with pulmonary atresia and intact ventricular septum (PA/IVS) are known to complicate right ventricular-coronary artery fistula (fistulae) and particularly those with coronary artery stenoses bear a high mortality rate and remain in a surgical challenge. A 4-year-old girl was first admitted to our hospital at 5 days of age and right ventriculogram revealed markedly hypoplastic tripartite ventricle and multiple fistulae through which both coronary arteries and aortic root were retrogradely opacified. Echocardiographically measured diameter of the tricuspid valve was 5 mm (Z value: 4). She underwent pulmonary valvotomy and central aorto-pulmonary (AP) shunt at 16 days of age. Repeat right ventriculogram at 1.9 years of age disclosed multiple stenoses in left anterior descending coronary artery (LAD) with proximal dilatation and tortuosity. Additional findings of left ventricular dysfunction (LVEF of 61.5%) and depressed ST segment in left precordial leads prompted us to proceed to the second palliation which comprised take-down of central AP shunt, bidirectional cavopulmonary shunt and closure of tricuspid and pulmonary valves. Catheterization at 3.4 years of age disclosed antegradely filled LAD wit...Continue Reading

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