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@article{Canepa:2020:10.1161/CIRCHEARTFAILURE.120.007230,
author = {Canepa, M and Fumagalli, C and Tini, G and Vincent-Tompkins, J and Day, SM and Ashley, EA and Mazzarotto, F and Ware, J and Michels, M and Jacoby, M and Ho, CY and Olivotto, I and The, SHaRe Investigators},
doi = {10.1161/CIRCHEARTFAILURE.120.007230},
journal = {Circulation},
pages = {376--381},
title = {Temporal trend of age at diagnosis in hypertrophic cardiomyopathy: an analysis of the international SHaRe Registry},
url = {http://dx.doi.org/10.1161/CIRCHEARTFAILURE.120.007230},
volume = {13},
year = {2020}
}

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TY  - JOUR
AB  - BACKGROUNDOver the last 50 years, the epidemiology of hypertrophic cardiomyopathy (HCM) has changed dueto increased awareness and availability of advanced diagnostic tools. We aim to describe thetemporal trends in age, gender and clinical characteristics at HCM diagnosis over >4 decades.METHODSWe retrospectively analyzed records from the ongoing multinational SHaRe registry. Overall, 7,286HCM patients diagnosed at an age ≥18 years between 1961 and 2019 were included in the analysisand divided into three eras of diagnosis (<2000, 2000-2010, >2010).RESULTSAge at diagnosis increased markedly over time (40±14 vs. 47±15 vs. 51±16 years, p<0.001), both inUS and non-US sites, with a stable male-to-female ratio of about 3:2. Frequency of familial HCMdeclined over time (38.8% vs. 34.3% vs. 32.7%, p<0.001), as well as heart failure symptoms atpresentation (NYHA III/IV: 18.1% vs. 15.8% vs. 12.6%, p<0.001). Left ventricular hypertrophybecame less marked over time (maximum wall thickness: 20±6 vs. 18±5 vs. 17±5 mm, p<0.001),while prevalence of obstructive HCM was greater in recent cohorts (peak gradient >30 mmHg:31.9% vs. 39.3% vs. 39.0%, p=0.001). Consistent with decreasing phenotypic severity, yield ofpathogenic/likely-pathogenic variants at genetic testing decreased over time (57.7% vs. 45.6% vs.38.4%, p<0.001).CONCLUSIONSEvolving HCM populations include progressively greater representation of older patients withsporadic disease, mild phenotypes and genotype-negative status. Such trend suggests a prominentrole of imaging over genetic testing in promoting HCM diagnoses and urges efforts to understandgenotype-negative disease eluding the classic monogenic paradigm
AU  - Canepa,M
AU  - Fumagalli,C
AU  - Tini,G
AU  - Vincent-Tompkins,J
AU  - Day,SM
AU  - Ashley,EA
AU  - Mazzarotto,F
AU  - Ware,J
AU  - Michels,M
AU  - Jacoby,M
AU  - Ho,CY
AU  - Olivotto,I
AU  - The,SHaRe Investigators
DO  - 10.1161/CIRCHEARTFAILURE.120.007230
EP  - 381
PY  - 2020///
SN  - 0009-7322
SP  - 376
TI  - Temporal trend of age at diagnosis in hypertrophic cardiomyopathy: an analysis of the international SHaRe Registry
T2  - Circulation
UR  - http://dx.doi.org/10.1161/CIRCHEARTFAILURE.120.007230
UR  - https://www.ahajournals.org/doi/10.1161/CIRCHEARTFAILURE.120.007230
UR  - http://hdl.handle.net/10044/1/82914
VL  - 13
ER  -

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