Univentricular atrioventricular connection to a dominant left ventricle with a concordant ventriculo-arterial connection.
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.
Cifarelli
A
A
Department of Paediatric Cardiology, Bambino Gesù Hospital, Rome, Italy.
Di Carlo
D
D
Pasquini
L
L
Marino
B
B
Ballerini
L
L
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D006325:Heart Atria / Q000002:abnormalities; D006330:Heart Defects, Congenital / Q000473:pathology*; D006351:Heart Valves / Q000002:abnormalities; D006352:Heart Ventricles / Q000002:abnormalities; D006801:Humans; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities; D011662:Pulmonary Subvalvular Stenosis / Q000151:congenital