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Hemodynamic Consequences of Hypertrophic Cardiomyopathy with Midventricular Obstruction: Apical Aneurysm and Thrombus

Shahryar G Saba, Andrew W Ertel, Michael Siegenthaler

    Journal of General Practice (Los Angeles, Calif.)
    |March 29, 2016
    PubMed

    Insights

    Midventricular hypertrophic cardiomyopathy (HCM) can cause left ventricular obstruction and apical thrombus formation. Early recognition of this rare HCM variant is crucial due to its poor prognosis and risk of cardioembolism.

    Area of Science:

    • Cardiology
    • Cardiovascular Imaging
    • Cardiac Surgery

    Background:

    • Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
    • Midventricular hypertrophy is an uncommon HCM phenotype.
    • This variant predisposes to left ventricular outflow tract obstruction.

    Purpose of the Study:

    • To describe a case of midventricular hypertrophic cardiomyopathy with apical thrombus.
    • To highlight diagnostic findings and management strategies.
    • To emphasize the prognostic implications of this HCM variant.

    Main Methods:

    • Diagnosis initiated by incidental abdominal CT finding.
    • Transthoracic echocardiography and cardiovascular MRI for detailed assessment.
    • Surgical intervention including thrombectomy, septal myectomy, and aneurysmal ligation.

    Main Results:

    • CT revealed a left ventricular apical filling defect.
    • Echocardiography showed mid-ventricular hypertrophy, obstruction, and apical aneurysm with thrombus.
    • MRI confirmed spade-shaped left ventricle, midcavitary obliteration, apical infarction, and fibrosis.

    Conclusions:

    • Midventricular HCM causes obstruction, ischemia, and apical fibrosis.
    • Apical remodeling and fibrosis can mimic myocardial infarction.
    • Aneurysmal apex with thrombus increases cardioembolic risk, indicating a poor prognosis.
    Abstract

    Keywords:
    Aneurysmal apical chamberCardiovascular magnetic resonance imaging of hypertrophic cardiomyopathyHypertrophic cardiomyopathyMidcavity obstructionMidventricular hypertrophyParadoxic diastolic jet flow in hypertrophic cardiomyopathy

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