Progressive cardiac conduction defect and cardiomyopathy in siblings with syncope

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Fetal Mouse Cardiovascular Imaging Using a High-frequency Ultrasound (30/45MHZ) System
07:34

Fetal Mouse Cardiovascular Imaging Using a High-frequency Ultrasound (30/45MHZ) System

Published on: May 5, 2018

B P Fuhrman, J L Bass, C Lawrence

    Pediatric Cardiology
    |January 1, 1982
    PubMed

    Insights

    Three siblings with syncope were diagnosed with left bundle-branch block, progressing to complete heart block. Pacemaker implantation resolved symptoms, suggesting a familial cardiomyopathy affecting cardiac conduction.

    Area of Science:

    • Cardiology
    • Genetics
    • Pediatrics

    Background:

    • Syncope in children can indicate serious underlying cardiac conditions.
    • Left bundle-branch block (LBBB) is a significant finding requiring thorough investigation.

    Observation:

    • Three siblings presented with syncope and were diagnosed with LBBB.
    • All three siblings experienced progression to complete heart block.
    • Echocardiography revealed cardiomyopathy despite normal heart size on X-ray.

    Findings:

    • Pacemaker implantation successfully eliminated syncopal episodes in all affected siblings.
    • The family study did not reveal other members with conduction defects.
    • These cases suggest a novel form of familial cardiomyopathy.

    Implications:

    • This familial cardiomyopathy predominantly affects the cardiac conduction system.
    • Early diagnosis and intervention, such as pacemaker implantation, are crucial.
    • Further research into the genetic basis of this condition is warranted.

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