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Cardiomyopathic and channelopathic causes of sudden unexplained death in infants and children

David J Tester1, Michael J Ackerman

  • 1Departments of Medicine, Pediatrics, and Molecular Pharmacology & Experimental Therapeutics, Mayo Clinic, Rochester, Minnesota 55905, USA.

Annual Review of Medicine
|October 22, 2008
PubMed

Insights

Sudden cardiac death in children can stem from inherited heart conditions like hypertrophic cardiomyopathy and channelopathies. Postmortem genetic testing aids in diagnosing these lethal disorders, especially in unexplained deaths.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • Increasing awareness of heritable cardiomyopathic and channelopathic syndromes linked to sudden cardiac death in children.
  • These conditions are significant causes of sudden cardiac death (SCD) in infants and children.

Purpose of the Study:

  • To review clinical and molecular findings of specific cardiac abnormalities and channelopathies.
  • To explore the role of molecular autopsy in sudden unexplained deaths (SUDs) and analyze channelopathies in sudden infant death syndrome (SIDS).

Main Methods:

  • Review of current clinical and molecular findings.
  • Analysis of autopsy-evident structural cardiac abnormalities and cardiac channelopathies.
  • Exploration of postmortem genetic analysis and genetic testing.

Main Results:

  • Hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy are key structural abnormalities.
  • Long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome, and short QT syndrome are significant channelopathies.
  • These channelopathies may explain up to one-third of autopsy-negative SUDs in children and adolescents.

Conclusions:

  • Heritable cardiac disorders are critical considerations in pediatric sudden cardiac death.
  • Molecular autopsy is valuable for diagnosing SUDs.
  • Genetic testing is essential for identifying and managing these potentially lethal cardiac conditions.

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