Univentricular atrioventricular connection to a dominant left ventricle with a concordant ventriculo-arterial...

Double inlet left ventricle with concordant ventriculo-arterial connection, first described by Holmes, is an unusual cardiac malformation which includes a right-sided rudimentary right ventricle and, frequently, subpulmonary stenosis. We have now encountered six patients, aged 1 month to 13 years, with this basic combination. In 5 cases, cross-sectional echocardiography, using parasternal, apical and subcostal views was diagnostic. Subpulmonary stenosis was seen in two of them. The sixth case shown to have atresia of the left atrioventricular valve, a concordant ventriculo-arterial connection, a left-sided rudimentary right ventricle, mild subpulmonary stenosis and a right-sided aorta. The combination of the left atrioventricular valve, left-sided rudimentary right ventricle and a concordant ventriculo-arterial connection has not, to our knowledge, been previously reported. Cross-sectional echocardiography always provided the correct morphologic diagnosis.