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Double outlet right ventricle and left-sided aorta. A previously undescribed association with cor triatriatum and

J R Büchler, M N Cano

    Japanese Heart Journal
    |January 1, 1986
    PubMed

    Insights

    This study details a rare congenital heart defect: double-outlet right ventricle with unusual chamber anatomy. The findings highlight a previously undescribed combination of cardiac anomalies in a single patient.

    Area of Science:

    • Cardiology
    • Congenital Heart Disease
    • Pediatric Cardiology

    Background:

    • Double-outlet right ventricle (DORV) is a complex congenital heart defect.
    • Viscero-atrial situs solitus with a right-sided right ventricle and left-sided aorta is an uncommon presentation of DORV.

    Observation:

    • A case of DORV with situs solitus presented with a unique combination of anomalies.
    • This included dextrocardia, a subaortic ventricular septal defect, and an ostium secundum atrial septal defect.

    Findings:

    • The patient exhibited a previously undescribed condition: cor triatriatum with a double right ventricular chamber.
    • Absence of pulmonary and aortic stenosis was noted at all levels.

    Implications:

    • This case expands the spectrum of known DORV presentations.
    • It underscores the importance of comprehensive cardiac evaluation in complex congenital heart disease.
    • Further research may elucidate the embryological basis for this unique combination of defects.

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