The late-onset dilated cardiomyopathy. (25th November 2020)

• Record Type: Journal Article
• Authors: Cannata, A; Merlo, M; Manca, P; Dal Ferro, M; Paldino, A; Artico, J; Gentile, P; Jirikowic, J; Todd, E; Salcedo, E; Graw, S; McDonagh, T; Taylor, M; Mestroni, L; Sinagra, G
• Is Part Of: European heart journal. Volume 41:(2020)Supplement 2
• Publication Date: 2020-11-25
• Subjects: Dilative Cardiomyopathy; Cardiology -- Periodicals; Heart -- Diseases -- Periodicals; 616.12005
• DOI: 10.1093/ehjci/ehaa946.2096
• Languages: English
• ISSNs: 0195-668X

Abstract

Abstract: Background: Dilated Cardiomyopathy (DCM) represents a specific subgroup of non-ischemic cardiomyopathies. Little is known about the genotypic characterization of dilated cardiomyopathy (DCM) patients diagnosed over 60 years of age. Aim: To investigate prevalence, characterization and prognostic impact of the genetic background of late-onset DCM patients. Methods: We analyzed a study population of 566 DCM patients from two international referral centers. Genetic background was analyzed and patients were grouped into typical-onset DCM (<60 years of age at diagnosis) or late-onset DCM (>60 years of age at diagnosis). Results: Approximately 12% of patients (n=70) had late-onset DCM and female sex was significantly more frequent in the late-onset DCM cohort (p<0.001). Diagnostic yield of genetic testing was comparable between typical- and late-onset DCM (53% vs 50%, respectively p=0.438) whereas the prevalence of Titin gene truncation variants (TTNtv) was higher in the late-onset DCM group compared to the younger cohort (23% vs 13% respectively; p<0.05). Notably, patients with late-onset genetic DCM had comparable long-term outcomes to those with typical-onset DCM. Conclusions: Late-onset DCM patients have nearly double the rate of TTNtv mutations and are more likely to be female compared to younger DCM patients. These observed differences in mutational makeup and sex may reveal insights into age and sex dependent mechanisms for TTNtv and should prompt further study. Notably, the increased prevalence of TTNtv and female sex did not translate into noticeable differences in rates of measurable cardiac events. Funding Acknowledgement: Type of funding source: None