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Ebstein's anomaly: editorial comments and personal observations

G K Danielson

    The Annals of Thoracic Surgery
    |October 1, 1982
    PubMed
    Summary
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    Surgical repair for Ebstein's anomaly, including plastic reconstruction, shows improved outcomes. This treatment is recommended for patients with advanced symptoms or significant cardiac issues, offering good long-term results.

    Area of Science:

    • Cardiology
    • Congenital Heart Disease
    • Cardiac Surgery

    Background:

    • Ebstein's anomaly is a rare congenital heart defect affecting the tricuspid valve.
    • Recent surgical advancements have renewed interest in managing this condition.
    • Treatment options include tricuspid valve replacement and plastic reconstruction.

    Purpose of the Study:

    • To evaluate the outcomes of surgical repair for Ebstein's anomaly.
    • To assess the effectiveness of different surgical techniques, including plastic repair and valve replacement.
    • To identify patient groups who would benefit most from surgical intervention.

    Main Methods:

    • Retrospective review of 42 patients with Ebstein's anomaly who underwent surgical repair at the Mayo Clinic.
    • Surgical procedures included plastic repair (34 patients), right ventricular plication and valve replacement (6 patients), and Fontan procedure (2 patients).

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  • Long-term follow-up was obtained for 22 survivors.

    • Main Results:

    • Overall mortality was low, with no deaths in the most recent 25 patients.
    • Significant improvement in New York Heart Association (NYHA) functional class was observed in most survivors (22/25 improved to Class I or II).
    • Plastic repair was successful in the majority of patients, demonstrating good long-term results.

    Conclusions:

    • Surgical repair, particularly plastic procedures, is advisable for Ebstein's anomaly patients with NYHA Class III or IV symptoms.
    • Patients with moderate to severe cyanosis, paradoxical emboli, or progressive cardiomegaly are also candidates for surgery.
    • The low mortality and favorable long-term outcomes support surgical intervention for selected patients.