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Family history of premature cardiomyopathy death predicts elevated cardiomyopathy risk
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Younger people with a family history of premature cardiomyopathy death had as much as a 400-fold elevated risk for cardiomyopathy, according to data in a nationwide cohort study.
“Our general population-based results strongly support the recommendations for presymptomatic screening of relatives of patients with cardiomyopathy in order to be able to reduce morbidity and mortality through early intervention,” the researchers wrote.
They analyzed a cohort of 3.9 million people born between 1950 and 2008 from Danish national register data. Patients were stratified by family history of premature death (younger than age 60 years) from cardiomyopathy or other conditions. The outcome of interest was diagnosis of cardiomyopathy at age younger than 50 years between 1977 and 2008.
Mattis F. Ranthe
Mattis F. Ranthe, MD, PhD, and colleagues documented 3,890 cases of cardiomyopathy over 89 million person-years of follow-up and estimated incidence rate ratios for cardiomyopathy based on family history of premature death from cardiomyopathy.
Those with a first-degree relative who died prematurely from cardiomyopathy had a 29-fold increase in the rate of cardiomyopathy, whereas those with a second-degree relative who died prematurely from cardiomyopathy had a sixfold increase, Ranthe, from the department of epidemiology research, Statens Serum Institut, Copenhagen, Denmark, and colleagues found.
People with a first-degree relative who died from cardiomyopathy at age younger than 35 years had a 100-fold increase in rate of cardiomyopathy, whereas those who had two or more premature deaths from cardiomyopathy in first-degree relatives had an increase in rate of cardiomyopathy of more than 400-fold, they found.
Family history of premature death from other conditions, whether cardiac or noncardiac, increased the rate of cardiomyopathy no more than threefold, they wrote.
“The risk of serious complications in the relatives was illustrated by the finding of their three- to sevenfold increase in risk of ventricular arrhythmia,” Ranthe and colleagues wrote.
In a related editorial, Calum A. MacRae, MB, ChB, PhD, from the division of cardiovascular medicine at Brigham and Women’s Hospital and Harvard Medical School, wrote, “These data support prior proband-based family studies, which may also underestimate the heritable contributions to heart muscle disease as a result of phenotype resolution.”
However, MacRae wrote, “If we are to fully realize the potential of the human genome outside clonal neoplastic disorders, we must overcome the barriers to incorporating rigorous family structure into clinical care.” – by Erik Swain
Disclosure: Ranthe was employed by Novo Nordisk at the time of submission. The other researchers and MacRae report no relevant financial disclosures.
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