{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T18:27:28Z","timestamp":1792175248185,"version":"4.4.0"},"reference-count":14,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"9","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circ: Heart Failure"],"published-print":{"date-parts":[[2020,9]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Over the last 50 years, the epidemiology of hypertrophic cardiomyopathy (HCM) has changed because of increased awareness and availability of advanced diagnostic tools. We aim to describe the temporal trends in age, sex, and clinical characteristics at HCM diagnosis over &gt;4 decades.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>We retrospectively analyzed records from the ongoing multinational Sarcomeric Human Cardiomyopathy Registry. Overall, 7286 patients with HCM diagnosed at an age \u226518 years between 1961 and 2019 were included in the analysis and divided into 3 eras of diagnosis (&lt;2000, 2000\u20132010, &gt;2010).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Age at diagnosis increased markedly over time (40\u00b114 versus 47\u00b115 versus 51\u00b116 years,\n                      <jats:italic>P<\/jats:italic>\n                      &lt;0.001), both in US and non-US sites, with a stable male-to-female ratio of about 3:2. Frequency of familial HCM declined over time (38.8% versus 34.3% versus 32.7%,\n                      <jats:italic>P<\/jats:italic>\n                      &lt;0.001), as well as heart failure symptoms at presentation (New York Heart Association III\/IV: 18.1% versus 15.8% versus 12.6%,\n                      <jats:italic>P<\/jats:italic>\n                      &lt;0.001). Left ventricular hypertrophy became less marked over time (maximum wall thickness: 20\u00b16 versus 18\u00b15 versus 17\u00b15 mm,\n                      <jats:italic>P<\/jats:italic>\n                      &lt;0.001), while prevalence of obstructive HCM was greater in recent cohorts (peak gradient &gt;30 mm\u2009Hg: 31.9% versus 39.3% versus 39.0%,\n                      <jats:italic>P<\/jats:italic>\n                      =0.001). Consistent with decreasing phenotypic severity, yield of pathogenic\/likely pathogenic variants at genetic testing decreased over time (57.7% versus 45.6% versus 38.4%,\n                      <jats:italic>P<\/jats:italic>\n                      &lt;0.001).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>Evolving HCM populations include progressively greater representation of older patients with sporadic disease, mild phenotypes, and genotype-negative status. Such trend suggests a prominent role of imaging over genetic testing in promoting HCM diagnoses and urges efforts to understand genotype-negative disease eluding the classic monogenic paradigm.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/circheartfailure.120.007230","type":"journal-article","created":{"date-parts":[[2020,9,8]],"date-time":"2020-09-08T05:00:41Z","timestamp":1599541241000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":96,"title":["Temporal Trend of Age at Diagnosis in Hypertrophic Cardiomyopathy"],"prefix":"10.1161","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9063-5576","authenticated-orcid":true,"given":"Marco","family":"Canepa","sequence":"first","affiliation":[{"name":"Cardiovascular Disease Unit, IRCCS Ospedale Policlinico San Martino \u2013 IRCCS Italian Cardiovascular Network &amp; Department of Internal Medicine, University of Genova, Italy (M.C., G.T.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlo","family":"Fumagalli","sequence":"additional","affiliation":[{"name":"Cardiomyopathy Unit and Genetic Unit, Careggi University Hospital, Florence, Italy (C.F., F.M., I.O.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8520-152X","authenticated-orcid":true,"given":"Giacomo","family":"Tini","sequence":"additional","affiliation":[{"name":"Cardiovascular Disease Unit, IRCCS Ospedale Policlinico San Martino \u2013 IRCCS Italian Cardiovascular Network &amp; Department of Internal Medicine, University of Genova, Italy (M.C., G.T.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Justin","family":"Vincent-Tompkins","sequence":"additional","affiliation":[{"name":"MyoKardia Inc, South San Francisco, CA (J.V.-T.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9802-7188","authenticated-orcid":true,"given":"Sharlene M.","family":"Day","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, University of Michigan, Ann Arbor (S.M.D.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Euan A.","family":"Ashley","sequence":"additional","affiliation":[{"name":"Stanford Center for Inherited Heart Disease, CA (E.A.A.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6159-9980","authenticated-orcid":true,"given":"Francesco","family":"Mazzarotto","sequence":"additional","affiliation":[{"name":"Cardiomyopathy Unit and Genetic Unit, Careggi University Hospital, Florence, Italy (C.F., F.M., I.O.)."},{"name":"National Heart and Lung Institute and National Institute for Health Research Royal Brompton Cardiovascular Biomedical Research Unit, Imperial College London, United Kingdom (F.M., J.S.W.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James S.","family":"Ware","sequence":"additional","affiliation":[{"name":"National Heart and Lung Institute and National Institute for Health Research Royal Brompton Cardiovascular Biomedical Research Unit, Imperial College London, United Kingdom (F.M., J.S.W.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Michels","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Thoraxcenter, Erasmus Medical Center Rotterdam, the Netherlands (M.M.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Jacoby","sequence":"additional","affiliation":[{"name":"Yale University, New Haven, CT (D.J.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolyn Y.","family":"Ho","sequence":"additional","affiliation":[{"name":"Cardiovascular Division, Brigham and Women\u2019s Hospital, Boston, MA (C.Y.H.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1751-9266","authenticated-orcid":true,"given":"Iacopo","family":"Olivotto","sequence":"additional","affiliation":[{"name":"Cardiomyopathy Unit and Genetic Unit, Careggi University Hospital, Florence, Italy (C.F., F.M., I.O.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"The SHaRe Investigators","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_4_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ahj.2005.01.005"},{"key":"e_1_3_4_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2016.02.044"},{"key":"e_1_3_4_4_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.112.136085"},{"key":"e_1_3_4_5_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.117.033200"},{"key":"e_1_3_4_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2009.12.066"},{"key":"e_1_3_4_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2015.01.019"},{"key":"e_1_3_4_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2018.04.078"},{"key":"e_1_3_4_9_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.cir.92.4.785"},{"key":"e_1_3_4_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2017.01.015"},{"key":"e_1_3_4_11_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehy730"},{"key":"e_1_3_4_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2019.01.061"},{"key":"e_1_3_4_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2019.08.1057"},{"key":"e_1_3_4_14_2","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2015-308764"},{"key":"e_1_3_4_15_2","doi-asserted-by":"publisher","DOI":"10.1002\/ejhf.541"}],"container-title":["Circulation: Heart Failure"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/CIRCHEARTFAILURE.120.007230","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,12]],"date-time":"2024-05-12T21:44:35Z","timestamp":1715550275000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/CIRCHEARTFAILURE.120.007230"}},"subtitle":["An Analysis of the International Sarcomeric Human Cardiomyopathy Registry"],"short-title":[],"issued":{"date-parts":[[2020,9]]},"references-count":14,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2020,9]]}},"alternative-id":["10.1161\/CIRCHEARTFAILURE.120.007230"],"URL":"https:\/\/doi.org\/10.1161\/circheartfailure.120.007230","relation":{},"ISSN":["1941-3289","1941-3297"],"issn-type":[{"value":"1941-3289","type":"print"},{"value":"1941-3297","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,9]]},"assertion":[{"value":"2020-04-17","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-07-06","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-09-08","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e007230"}}