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

TY - JOUR

T1 - Risk of Cardiomyopathy in Younger Persons With a Family History of Death from Cardiomyopathy

T2 - A Nationwide Family Study in a Cohort of 3.9 Million Persons

AU - Ranthe, Mattis F

AU - Carstensen, Lisbeth

AU - Øyen, Nina

AU - Jensen, Morten K

AU - Axelsson, Anna

AU - Wohlfahrt, Jan

AU - Melbye, Mads

AU - Bundgaard, Henning

AU - Boyd, Heather A

N1 - © 2015 American Heart Association, Inc.

PY - 2015/9/15

Y1 - 2015/9/15

N2 - 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.

AB - 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.

KW - Adolescent

KW - Adult

KW - Age Factors

KW - Cardiomyopathies

KW - Child

KW - Child, Preschool

KW - Cohort Studies

KW - Denmark

KW - Family

KW - Female

KW - Follow-Up Studies

KW - Humans

KW - Incidence

KW - Infant

KW - Male

KW - Medical History Taking

KW - Middle Aged

KW - Registries

KW - Regression Analysis

KW - Retrospective Studies

KW - Risk Factors

KW - Sex Factors

KW - Young Adult

U2 - 10.1161/CIRCULATIONAHA.114.013478

DO - 10.1161/CIRCULATIONAHA.114.013478

M3 - Journal article

C2 - 26276887

SN - 0009-7322

VL - 132

SP - 1013

EP - 1019

JO - Circulation

JF - Circulation

IS - 11

ER -