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[Interventricular defect with discrete aortic stenosis below the defect]

G Calcaterra, G Bonnì, D Pieri

    Giornale Italiano Di Cardiologia
    |January 1, 1981
    PubMed
    Summary
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    Discrete subaortic stenosis with ventricular septal defect (VSD) requires careful diagnosis. Surgical outcomes for corrected cases were favorable, highlighting the importance of precise evaluation for optimal treatment in this rare congenital heart anomaly.

    Area of Science:

    • Cardiology
    • Pediatric Cardiology
    • Congenital Heart Disease

    Background:

    • Discrete subaortic stenosis (DSS) associated with ventricular septal defect (VSD) is a rare congenital anomaly.
    • Distinguishing between supravalvular and subvalvular obstruction relative to the VSD is crucial for management.
    • DSS often presents with other complex cardiac anomalies.

    Observation:

    • Four cases of subaortic stenosis below the VSD were analyzed.
    • Diagnosis utilized clinical examination, ECG, chest X-rays, echocardiography, cardiac catheterization, and contrastography.
    • One patient also had associated subpulmonary stenosis (SPS).

    Findings:

    • Three of four patients with DSS and VSD underwent successful surgical correction with good outcomes at 6-18 months post-op.

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  • One patient with associated SPS also had a successful correction.
  • Surgical approaches included right atriotomy and right ventriculotomy; trans-aortic approach was deemed less suitable.
  • Conservative management was chosen for one patient due to small VSD size, mild pressure gradient, and mild aortic insufficiency.

    • Implications:

    • Accurate pre-operative assessment, including pressure gradients and angiography, is vital for managing DSS and VSD.
    • Surgical intervention can yield favorable long-term results for selected patients.
    • The choice of surgical approach impacts safety and efficacy, particularly regarding conduction tissue preservation.