Sudden Death in the Young in the Netherlands: Towards Optimal Care for the Victims Relatives

概要

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When an young, apparently healthy, individual dies suddenly, autopsy unmasks a cardiac cause of death in approximately 70%. In approximately 18% the autopsy is negative (sudden unexplained death (SUD)), which is suggestive of primary arrhythmia syndromes. In case of autopsy-negative SUD or when autopsy is not performed (making this SUD as well), surviving first-degree relatives are advised cardiological and genetic examination. However, in the Netherlands and many other countries autopsy is not mandatory, autopsy may not be performed as recommended, and not all of the families are referred. In all consecutive families who presented to our Cardiogenetics department for examination because of a first degree related SUD victim aged 1–50 years from 1996–2009 for comprehensive cardiological and genetic examination, a certain or probable diagnosis was made in 47 of 140 (33%) SUD families. In conclusion, accurate autopsy in young sudden death victims, followed by cardiological and genetic examination in the victims first-degree relatives seems to the optimal approach to indentify relatives at risk of sudden death. Efforts are made to introduce this approach in the Netherlands.
日本不整脈学会の論文