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Related Experiment Videos

[Total A-V block due to tuberous sclerosis. A case report].

N Valente, J Guidugli Neto, A A De Paola

    Arquivos Brasileiros De Cardiologia
    |October 1, 1989
    PubMed
    Summary

    This report details a rare case of tuberous sclerosis (a neurologic disease) presenting with complete heart block. The patient received a pacemaker, highlighting a unique cardiac manifestation of this condition.

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    Area of Science:

    • Cardiology
    • Neurology
    • Genetics

    Background:

    • Tuberous sclerosis is a genetic disorder impacting multiple organs, often presenting with neurological symptoms.
    • Cardiac involvement in tuberous sclerosis is uncommon, making this case particularly noteworthy.

    Observation:

    • A patient with tuberous sclerosis experienced syncope due to complete atrioventricular (A-V) block.
    • Cardiac evaluations revealed dilated cardiomyopathy with left ventricular dysfunction and infra-Hisian A-V block, despite normal coronary arteries and myocardium histology.

    Findings:

    • The patient exhibited a complete A-V block with right bundle branch block morphology on ECG, later showing a left bundle branch block pattern during electrophysiologic study.
    • No inducible ventricular tachyarrhythmias were observed.
    • This represents the first reported instance of complete heart block in a patient with tuberous sclerosis in medical literature.

    Implications:

    • This case expands the understanding of potential cardiac complications associated with tuberous sclerosis.
    • Highlights the importance of comprehensive cardiac evaluation in patients with tuberous sclerosis presenting with syncope or bradycardia.
    • Suggests that complete heart block can be a rare but significant manifestation of tuberous sclerosis, requiring long-term management with pacing.

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