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Isolated ventricular noncompaction mimicking arrhythmogenic right ventricular cardiomyopathy--a study of nine

Elzbieta K Wlodarska, Olgierd Wozniak, Marek Konka

    International Journal of Cardiology
    |July 8, 2009
    PubMed

    Insights

    Isolated noncompaction can affect both ventricles, mimicking arrhythmogenic right ventricular cardiomyopathy (ARVC). This study highlights the importance of considering ventricular noncompaction in ARVC evaluations, especially when excessive trabeculation is present.

    Area of Science:

    • Cardiology
    • Cardiovascular Imaging
    • Pathology

    Background:

    • Isolated ventricular noncompaction typically affects the left ventricle.
    • Characterized by deep trabecular recesses and increased wall thickness.
    • This study focuses on cases mimicking arrhythmogenic right ventricular cardiomyopathy (ARVC).

    Purpose of the Study:

    • To present patients with isolated noncompaction of both ventricles.
    • To highlight the differential diagnosis between noncompaction and ARVC.
    • To emphasize the importance of recognizing noncompaction in ARVC evaluations.

    Main Methods:

    • Retrospective analysis of 9 patients initially diagnosed with ARVC.
    • Clinical evaluation, Cardiac Magnetic Resonance (CMR) in 8 patients.
    • Cardiac catheterization and endomyocardial biopsy in 2 patients each.

    Main Results:

    • All 9 patients showed morphologic/functional RV changes: enlargement and hypertrabeculation.
    • 4 patients had global RV hypokinesis; 5 had focal wall motion abnormalities.
    • Biopsies revealed thickened endocardium, fibrosis, myocardial damage, and lymphocyte infiltration.

    Conclusions:

    • Ventricular noncompaction should be considered in right ventricular cardiomyopathies with excessive trabeculation.
    • Utilizing Task Force criteria for ARVC can improve diagnostic accuracy in complex cases.
    • This finding broadens the understanding of noncompaction's presentation and diagnostic challenges.
    Abstract

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