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A case of tuberous sclerosis presenting with dysrhythmia in the first day of life

Figen Akalin1, Gökhan Baysoy, Birsen Oztürk

  • 1Department of Pediatrics, Marmara University Faculty of Medicine, Turkey.

The Turkish Journal of Pediatrics
|April 13, 2004
PubMed

Insights

Cardiac rhabdomyoma, a common childhood heart tumor, can cause arrhythmias. Neonatal arrhythmia warrants investigation for cardiac rhabdomyoma, which may indicate tuberous sclerosis.

Area of Science:

  • Pediatric Cardiology
  • Neonatal Medicine
  • Genetics

Background:

  • Cardiac rhabdomyoma (CR) is the most frequent primary cardiac tumor in children.
  • While often asymptomatic, CR can lead to significant complications like arrhythmias, heart failure, and fetal hydrops.

Observation:

  • Neonatal arrhythmias necessitate evaluation for structural heart diseases, including CR.
  • Cardiac rhabdomyomas can occur in isolation or be associated with tuberous sclerosis (TS).

Findings:

  • Arrhythmias resulting from CR can be the earliest clinical manifestation of tuberous sclerosis.
  • This case highlights tuberous sclerosis presenting with ventricular premature beats and second-degree atrioventricular block on the first day of life, with multiple CRs identified via echocardiography.

Implications:

  • Early detection of CR in neonates with arrhythmias is crucial for identifying potential underlying conditions like tuberous sclerosis.
  • This underscores the importance of comprehensive cardiac evaluation in newborns presenting with arrhythmias.

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