Risk of Cardiomyopathy in Younger Persons With a Family History of Death from Cardiomyopathy: A Nationwide Family Study in a Cohort of 3.9 Million Persons

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Mattis F Ranthe, Lisbeth Carstensen, Nina Øyen, Morten K Jensen, Anna Axelsson, Jan Wohlfahrt, Mads Melbye, Henning Bundgaard, Heather A Boyd

BACKGROUND: Recommendations for presymptomatic screening of relatives of cardiomyopathy patients are based on findings from tertiary centers. Cardiomyopathy inheritance patterns are fairly well understood, but how cardiomyopathy in younger persons (<50 years) aggregates in families at the population level is unclear. In a nationwide cohort, we examined the risk of cardiomyopathy by family history of premature death (<60 years) from cardiomyopathy.

METHODS AND RESULTS: By linking Danish national register data, we constructed a cohort of 3.9 million persons born from 1950 to 2008. We ascertained family history of premature (<60 years) death from cardiomyopathy or other conditions, and cohort members were followed from 1977 to 2008 for cardiomyopathy diagnosed at <50 years. We identified 3890 cardiomyopathies in 89 million person-years of follow-up. Using Poisson regression, we estimated incidence rate ratios for cardiomyopathy by family history of premature death. Premature cardiomyopathy deaths in first- and second-degree relatives were associated with 29- and 6-fold increases in the rate of cardiomyopathy, respectively. If the first-degree relative died aged <35 years, the rate of cardiomyopathy increased 100-fold; given ≥2 premature deaths in first-degree relatives, the rate increased more than 400-fold. In contrast, a family history of premature death from other cardiac or noncardiac conditions increased the rate of cardiomyopathy 3-fold at most.

CONCLUSIONS: A family history of premature cardiomyopathy death was associated with an increase in risk of cardiomyopathy ranging from 6- to 400-fold, depending on age, kinship, gender and number of affected family members. Our general population-based results support recommendations for presymptomatic screening of relatives of cardiomyopathy patients.

Original languageEnglish
Issue number11
Pages (from-to)1013-9
Number of pages7
Publication statusPublished - 15 Sep 2015

    Research areas

  • Adolescent, Adult, Age Factors, Cardiomyopathies, Child, Child, Preschool, Cohort Studies, Denmark, Family, Female, Follow-Up Studies, Humans, Incidence, Infant, Male, Medical History Taking, Middle Aged, Registries, Regression Analysis, Retrospective Studies, Risk Factors, Sex Factors, Young Adult

ID: 162340587