title: Characteristics of sudden death in inherited heart disease.
abstract: OBJECTIVE
  Cardiomyopathy and channelopathy are major causes of sudden death (SD). The little information available on the context in which SD occurs has come from only a few referral centers. The objective was to investigate the circumstances surrounding SD in families with inherited heart disease.
  
  
  METHODS
  The study included 152 SD patients (mean age 43+/-19 years) from 103 families. The reasons for inclusion were resuscitated SD in 7%, recent SD in 8%, and a diagnosis of cardiomyopathy or channelopathy in a living relative in 72%. Also, 13% were athletes. Family trees were constructed and each death's circumstances were recorded. Autopsy and medical records were reviewed.
  
  
  RESULTS
  Overall, 18% of SDs occurred during physical exercise, 32% during normal daily activities, and 37% during rest or sleep. There was a significant association between male sex and SD: 111 males (73%) vs. 41 females (27%; P=.03). Exercise-related SD was associated with young age (P=.01). The percentage of SDs associated with exercise, stress or normal daily activities was significantly greater with cardiomyopathy than channelopathy (61% vs. 41%; P=.057). All athletes were male and the majority died during exercise (50% vs. 11% of non-athletes; P=.0002). Patients with Brugada syndrome had the highest percentage of SDs during rest or sleep (i.e. 47%). No clear trigger could be identified in 33%.
  
  
  CONCLUSIONS
  SD was common in inherited heart disease, which accounted for a significant number of cases. Males clearly predominated over females (ratio 3:1) among SD cases (irrespective of pathological type). Most SDs occurred during exercise or normal daily activities in cardiomyopathies and during rest or sleep in channelopathies. The percentage of exercise-related SDs (i.e. 18%) was higher than expected.
authors.affiliation: Servicio de Cardiología, Hospital Universitario Virgen de Arrixaca, Murcia, Spain. jgimeno@secardiologia.es
authors.forename: Juan R
authors.initials: JR
authors.lastname: Gimeno
authors.forename: María J
authors.initials: MJ
authors.lastname: Oliva
authors.forename: Javier
authors.initials: J
authors.lastname: Lacunza
authors.forename: Arcadi G
authors.initials: AG
authors.lastname: Alberola
authors.forename: María
authors.initials: M
authors.lastname: Sabater
authors.forename: Juan
authors.initials: J
authors.lastname: Martínez-Sánchez
authors.forename: Daniel
authors.initials: D
authors.lastname: Saura
authors.forename: Antonio
authors.initials: A
authors.lastname: Romero
authors.forename: Mariano
authors.initials: M
authors.lastname: Valdés
mesh_terms: D000328:Adult; D016757:Death, Sudden, Cardiac / Q000453:epidemiology / Q000209:etiology*; D005260:Female; D006331:Heart Diseases / Q000150:complications* / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged