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

Alberta Cifarelli, Duccio Di Carlo, Luciano Pasquini, Bruno Marino, Luigi Ballerini

Research output: Contribution to journalArticle

Abstract

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 was 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 atresia 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.

Original languageEnglish
Pages (from-to)21-26
Number of pages6
JournalInternational Journal of Cardiology
Volume25
Issue number1
DOIs
Publication statusPublished - 1989

Keywords

  • Double inlet left ventricle
  • Holmes heart
  • Left atrioventricular valve atresia

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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