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The obstructive subaortic conus.

J D Waldman, A Schneeweiss, W D Edwards

    Circulation
    |September 1, 1984
    PubMed
    Summary
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    Subaortic stenosis can occur in children with a malposed aorta, often associated with ventricular septal defects and coarctation of the aorta. This obstruction can worsen over time and may affect the left coronary artery.

    Area of Science:

    • Pediatric Cardiology
    • Congenital Heart Disease
    • Cardiovascular Surgery

    Background:

    • Congenital heart defects can lead to complex anatomical variations.
    • Subaortic stenosis is a significant obstruction below the aortic valve.

    Purpose of the Study:

    • To describe the characteristics and progression of subaortic stenosis in children with a malposed aorta.
    • To highlight potential complications, including coronary artery compression.

    Main Methods:

    • Retrospective case series of eleven children with subaortic stenosis and malposed aorta.
    • Review of clinical data, echocardiography, and cardiac catheterization findings.
    • Serial catheterization in four patients to assess stenosis progression.

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    Main Results:

    • Eleven children presented with subaortic stenosis (gradients 20-76 mm Hg) due to infundibular hypertrophy and septal malalignment.
    • Nine patients had ventricular septal defects; seven had coarctation of the aorta.
    • Progressive stenosis was observed in serial studies; one patient experienced left main coronary artery compression.

    Conclusions:

    • Subaortic stenosis is a potential complication in patients with malposed aorta, possibly occurring in over 5% of cases.
    • The obstruction can be progressive and may lead to coronary artery compromise, especially after surgical repair.
    • Early recognition and monitoring are crucial for managing this condition.