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Cardiomyopathy in First-Degree Relatives of Patients Presenting With Acute Myocarditis: Prevalence and Prognostic
Jacinthe Boulet1, Neal K Lakdawala2, Mia Nielsen Christiansen3,4
1Department of Medicine, Division of Cardiology, Montreal Heart Hospital, Université de Montréal, QC, Canada (J.B.).
Insights
Acute myocarditis is linked to dilated cardiomyopathy (DCM) and heart failure (HF), carrying a worse long-term prognosis for patients and their families. Family history of DCM or HF increases mortality risk in myocarditis patients.
Area of Science:
- Cardiology
- Genetics
- Public Health
Background:
- Acute myocarditis is genetically linked to dilated cardiomyopathy (DCM), but its clinical significance and familial implications are unclear.
- Investigating the prevalence of DCM and heart failure (HF) in acute myocarditis patients and their relatives is crucial for understanding long-term prognosis.
Purpose of the Study:
- To determine the prevalence of DCM and HF in patients hospitalized with acute myocarditis and their first-degree relatives.
- To compare the long-term prognosis of DCM and HF in these patients versus a matched general population cohort.
Main Methods:
- An observational study using Danish nationwide registries from 1995 to 2018.
- Identified patients with first-time myocarditis and matched them with 10 controls based on birth year and sex.
- Analyzed the incidence of DCM, HF, and all-cause mortality over 20 years.
Main Results:
- Patients with acute myocarditis showed higher baseline prevalence of DCM and HF compared to controls.
- Myocarditis patients had a significantly increased 20-year incidence of DCM, HF, and all-cause mortality.
- A family history of DCM or HF in first-degree relatives was associated with increased long-term mortality in myocarditis patients.
Conclusions:
- Acute myocarditis aggregates with DCM within families and is associated with a worsened prognosis.
- The findings suggest that environmental factors may play a role in myocarditis development, particularly given the sibling preponderance observed.
- Early identification and management of cardiovascular risks in myocarditis patients with a family history are warranted.
Background:
Acute myocarditis has been genetically linked to dilated cardiomyopathy (DCM), but the clinical significance remains uncertain. We investigated the prevalence and long-term prognosis of DCM and heart failure (HF) among unselected patients hospitalized with acute myocarditis and their first-degree relatives compared with an age- and sex-matched cohort.
Methods:
This was an observational study utilizing the Danish nationwide registries, where all patients with a first-time myocarditis diagnosis from 1995 to 2018 were identified and matched (on birth year and sex) with 10 controls from the general population.
Results:
Totally 3176 patients with acute myocarditis and 31 760 controls were included (median age, 49.8 [Q1-Q3, 32.5-70.2] years; 35.6% female). At baseline, patients with myocarditis had a higher prevalence of DCM (7 [0.2%] versus 8 [0.0%]) and HF (336 [10.6%] versus 695 [2.2%]) than controls; P<0.0001 for both. Patients with myocarditis more often had siblings with DCM (12 [0.4%] versus 17 [0.05%]) or HF (36 [1.1%] versus 89 [0.3%]); P<0.0001, odds ratios 7.09 (3.38-14.85) and 2.92 (1.25-6.80), respectively, whereas parental DCM and HF did not differ among patients with myocarditis and controls. Patients with myocarditis had greater 20-year incidence of DCM, HF, and all-cause mortality (0.5% [0.3%-0.9%], 15% [13%-17%], and 47% [44%-50%]) compared with controls (0.06% [0.03%-0.11%], 6.8% [6.4%-7.3%], and 34% [33%-35%]; P<0.0001). Having a first-degree relative with DCM or HF was associated with increased long-term mortality among the patients with myocarditis (hazard ratio, 1.40 [1.11-1.77]) but not among the controls (hazard ratio, 0.90 [0.81-1.01]; Pdifference=0.0008).
Conclusions:
Acute myocarditis aggregates with DCM within families, where it carries a worsened prognosis. A differential association between parents and siblings (with sibling preponderance) could suggest that additional environmental factors are important for myocarditis development even in predisposed individuals.
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