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The natural history of truncus arteriosus

C Marcelletti, D C McGoon, D D Mair

    Circulation
    |July 1, 1976
    PubMed

    Insights

    Truncus arteriosus, a severe congenital heart defect, had a grave prognosis before surgical repair. Early intervention in infants and young children is crucial for better outcomes in truncus arteriosus patients.

    Area of Science:

    • Cardiology
    • Pediatric Cardiology
    • Congenital Heart Disease

    Background:

    • Truncus arteriosus is a rare congenital heart defect.
    • Outcomes for truncus arteriosus before surgical repair were historically poor.

    Purpose of the Study:

    • To review the prognosis of truncus arteriosus (Type I and II) before corrective surgery.
    • To identify factors influencing survival and disability in these patients.

    Main Methods:

    • Retrospective review of 23 patients diagnosed with truncus arteriosus (Type I or II) at Mayo Clinic before 1967.
    • Analysis of patient age at diagnosis, survival rates, and presence of pulmonary vascular obstructive disease.

    Main Results:

    • 14 out of 23 patients died.
    • Infants (≤1 year) had a 100% mortality rate.
    • Patients diagnosed after age 7 often had severe pulmonary vascular obstructive disease, impacting survival.
    • Most survivors experienced mild to moderate disability.

    Conclusions:

    • The prognosis for truncus arteriosus remains grave, underscoring the need for corrective surgery.
    • Successful surgical correction is most beneficial when performed in infancy or early childhood, before severe pulmonary complications develop.

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