Sudden death due to catecholaminergic polymorphic ventricular tachycardia following negative stress-test outcome: genetics and clinical implications

Forensic Sci Med Pathol. 2017 Jun;13(2):217-225. doi: 10.1007/s12024-017-9862-9. Epub 2017 Apr 13.

Abstract

This paper discusses the case of a young boy who died suddenly during a football match. The victim's personal and family medical histories were negative for cardiac events. He had undergone a cardiological investigation some months before his death, enabling him to participate in competitive sports. Only post-mortem molecular analysis allowed for a clearer determination of the most plausible cause of death, which was identified as inherited arrhythmogenic heart disease, known as catecholaminergic polymorphic ventricular tachycardia. It was possible to detect a novel, previously undescribed, variant in the RYR2 gene. This case report highlights the importance of a meaningful forensic multidisciplinary investigation in such cases, and also discusses possible medical malpractice claims.

Keywords: Catecholaminergic polymorphic ventricular tachycardia; Provocation test; Rare variant; Stress electrocardiogram; Sudden cardiac death.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Amino Acid Substitution*
  • Death, Sudden, Cardiac / etiology*
  • Echocardiography, Stress
  • Electrocardiography, Ambulatory
  • Humans
  • Male
  • Mutation, Missense*
  • Ryanodine Receptor Calcium Release Channel / genetics*
  • Tachycardia, Ventricular / genetics*

Substances

  • Ryanodine Receptor Calcium Release Channel

Supplementary concepts

  • Polymorphic catecholergic ventricular tachycardia