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Left ventricular anomalies associated with Ebstein's malformation of the tricuspid valve

A A Monibi, W H Neches, C C Lenox

    Circulation
    |February 1, 1978
    PubMed

    Insights

    Ebstein's malformation can affect left ventricular function, with many patients showing contraction abnormalities. This highlights the need for thorough left ventricular evaluation in Ebstein's anomaly cases.

    Area of Science:

    • Cardiology
    • Pediatric Cardiology
    • Congenital Heart Disease

    Background:

    • Ebstein's malformation of the tricuspid valve is a rare congenital heart defect.
    • Presentation varies, with neonates often showing cyanosis and cardiomegaly, while others are diagnosed later due to murmurs or cardiomegaly.

    Purpose of the Study:

    • To evaluate left ventricular function in patients with Ebstein's malformation.
    • To determine the prevalence of left ventricular abnormalities in this patient population.

    Main Methods:

    • Retrospective review of 17 patients with Ebstein's malformation.
    • Cardiac catheterization, including right and left heart studies, performed on 13 patients.
    • Angiocardiography to assess left ventricular contraction.

    Main Results:

    • 11 out of 17 patients presented in the newborn period with cyanosis and cardiomegaly.
    • 3 patients died, including 2 sudden deaths in adolescence.
    • 92% (12/13) of catheterized patients exhibited left ventricular contraction abnormalities.
    • 5 patients had associated mitral valve prolapse and left ventricular dysfunction.

    Conclusions:

    • Significant left ventricular abnormalities are common in Ebstein's malformation.
    • Systematic evaluation of left ventricular function is crucial for managing patients with Ebstein's anomaly.
    • Early identification of left ventricular dysfunction may impact patient outcomes.

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