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[Ebstein's anomaly in adults].

P Presbitero, E Aruta, F Orzan

    Cardiologia (Rome, Italy)
    |March 1, 1989
    PubMed
    Summary
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    Ebstein's anomaly diagnosed in adults is generally stable, especially when asymptomatic. Surgery is recommended for symptomatic patients experiencing paradoxical embolism or worsening tricuspid regurgitation.

    Area of Science:

    • Cardiology
    • Congenital Heart Disease

    Background:

    • Ebstein's anomaly is a rare congenital heart condition affecting the tricuspid valve.
    • Diagnosis in adulthood often presents with varying degrees of cyanosis, palpitations, and arrhythmias.

    Purpose of the Study:

    • To evaluate the long-term clinical course and outcomes of adult patients diagnosed with Ebstein's anomaly.
    • To identify predictors of adverse events and assess the efficacy of medical and surgical management.

    Main Methods:

    • A retrospective analysis of 18 adult patients (18-55 years) with Ebstein's anomaly followed for 3-13 years.
    • Clinical, echocardiographic, and cardiac catheterization data were collected and analyzed.
    • Pregnancy outcomes were also documented.

    Main Results:

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    • Mild cyanosis and palpitations were common initial findings. Wolff-Parkinson-White (WPW) syndrome occurred in 4 patients.
    • Moderate-to-severe tricuspid regurgitation was present in 7 patients. Systemic embolism occurred in 3 patients with patent foramen ovale.
    • Three patients underwent successful surgery for worsening tricuspid incompetence. The remaining 14 patients remained clinically stable.

    Conclusions:

    • Adult-onset Ebstein's anomaly is often a benign and stable condition, particularly in asymptomatic individuals.
    • Surgical intervention is indicated for symptomatic patients, especially those with paradoxical embolism or significant tricuspid regurgitation.
    • Medical management effectively controlled symptoms in patients with WPW syndrome.