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Review article. Congenital complete heart block

E Esscher

    Acta Paediatrica Scandinavica
    |January 1, 1981
    PubMed
    Summary

    Congenital complete heart block has varied causes, often linked to maternal connective tissue disease. Prognosis is challenging, with neonatal factors like low heart rate and prolonged QT time indicating poor outcomes.

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    Area of Science:

    • Cardiology
    • Pediatrics
    • Genetics

    Background:

    • Isolated congenital complete heart block (CCHB) presents a heterogeneous etiology.
    • Maternal connective tissue disease is identified in approximately one-third of CCHB cases.
    • Mortality rates for CCHB vary significantly by age, being highest in neonates.

    Purpose of the Study:

    • To investigate the etiology and long-term outcomes of isolated congenital complete heart block.
    • To identify prognostic indicators for patients with CCHB.
    • To evaluate the need for and indications of pacemaker implantation in CCHB patients.

    Main Methods:

    • Retrospective analysis of patients with isolated congenital complete heart block.
    • Review of maternal and patient medical histories, including signs of connective tissue disease.
    • Assessment of mortality, pacemaker implantation rates, and clinical presentation across different age groups.

    Main Results:

    • Congenital complete heart block etiology is diverse, with maternal connective tissue disease being a significant factor.
    • Mortality is highest in the neonatal period, decreasing in childhood and adolescence, then slowly increasing in adulthood.
    • Pacemaker implantation rates were approximately 10% for patients under 15 and 25% for those over 15, with neonatal heart failure being a primary indication.
    • Stokes-Adams attacks can occur at any age.
    • Poor prognostic signs include a fixed or decreasing low neonatal ventricular rate and prolonged QT interval.

    Conclusions:

    • Congenital complete heart block requires careful prognostic evaluation due to its varied etiology and clinical course.
    • Neonatal factors such as low ventricular rate and prolonged QT time are critical indicators of poor prognosis.
    • Pacemaker implantation is a common intervention, particularly for heart failure in neonates and for managing arrhythmias in older patients.

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