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Pacemaker malfunctions in Danon's disease.

Elena Marras1, Luigi Sciarra, Manuela Bocchino

  • 1Department of Cardiology, Santa Maria dei Battuti Hospital, Conegliano, Treviso, Italy. elena.marras@alice.it

Pacing and Clinical Electrophysiology : PACE
|January 10, 2008
PubMed
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Danon disease, a genetic disorder, can cause severe cardiac issues like hypertrophic cardiomyopathy and conduction problems. This case highlights cardiac manifestations and pacemaker challenges in a young patient with Danon disease.

Area of Science:

  • Genetics
  • Cardiology
  • Cell Biology

Background:

  • Danon disease is an X-linked genetic disorder.
  • It results from a deficiency in lysosomal-associated membrane protein 2 (LAMP2).
  • This deficiency leads to glycogen and autophagic material accumulation in cells.

Observation:

  • The case involves a 30-year-old male patient.
  • He presented with cardiac involvement including hypertrophic cardiomyopathy, preexcitation, and atrioventricular block.
  • The patient received an implantable cardioverter defibrillator for primary prevention of sudden death.

Findings:

  • Cardiac involvement in Danon disease can manifest as hypertrophic cardiomyopathy at a young age.
  • Conduction disorders, such as atrioventricular block, are significant cardiac manifestations.

Related Experiment Videos

  • Patients may experience multiple pacemaker malfunctions.

    • Implications:

    • Early diagnosis and cardiac monitoring are crucial for patients with Danon disease.
    • Understanding cardiac involvement aids in managing conduction disorders and preventing sudden cardiac death.
    • This case underscores the complex cardiac phenotype and potential device-related complications in Danon disease.