Unrepaired Tetralogy of Fallot with Absent Pulmonary Valve in a Mildly Symptomatic 16-Year-Old Boy

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Published on: July 18, 2014

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Diana E Drogalis-Kim, Brian L Reemtsen, Leigh Christopher Reardon

    Texas Heart Institute Journal
    |January 20, 2017
    PubMed

    Insights

    Tetralogy of Fallot with absent pulmonary valve is rare and typically requires early intervention. This report details a rare case of successful surgical repair in a mildly symptomatic 16-year-old, highlighting a unique survival into late adolescence.

    Area of Science:

    • Pediatric Cardiology
    • Congenital Heart Disease
    • Thoracic Surgery

    Background:

    • Tetralogy of Fallot with absent pulmonary valve (APV) is a severe congenital heart defect, occurring in 3-6% of Tetralogy of Fallot cases.
    • Infants with this condition often require early intervention due to respiratory distress, heart failure, or failure to thrive.

    Observation:

    • A 16-year-old male presented with mild symptoms of Tetralogy of Fallot with APV.
    • This patient's presentation is unusual, as most individuals with this condition require surgical repair in infancy or early childhood.

    Findings:

    • The patient underwent successful primary surgical repair of Tetralogy of Fallot with APV.
    • Literature review identified fewer than five reported cases of Tetralogy of Fallot with APV surviving into late adolescence or adulthood without prior repair.

    Implications:

    • This case demonstrates the possibility of successful surgical outcomes for Tetralogy of Fallot with APV in older individuals.
    • Highlights the importance of considering surgical repair in select older patients with this rare condition.
    • Contributes to understanding the natural history and management of Tetralogy of Fallot with APV.

    Keywords:
    Abnormalities, multiple/diagnosis/surgeryadolescentheart defects, congenital/diagnosis/surgerypulmonary artery/pathologypulmonary valve/abnormalitiestetralogy of Fallot/complications/surgerytime factorstreatment outcome

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