Publication: Characteristics of sudden death in inherited heart disease
Authors
Gimeno, Juan Ramón ; Oliva, María José ; Lacunza, Javier ; García Alberola, Arcadio ; Martínez Sánchez, Juan José ; Saura, Daniel ; Romero, Antonio ; Valdés, Mariano ; Sabater Molina, María
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Publisher
Elsevier
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DOI
https://doi.org/10.1016/S1885-5857(10)70059-1
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info:eu-repo/semantics/article
Description
©2009.
This document is the Accepted, version of a Published Work that appeared in final form in Revista Española de Cardiología. To access the final edited and published work see https://doi.org/10.1016/S1885-5857(10)70059-1
Abstract
Introduction and objectives
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 (73.0%) versus 41 females (27.0%; 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 (ie, 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.
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Citation
Revista Española de Cardiología 63(3) 2010: 268-276
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