{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T11:57:43Z","timestamp":1753876663107,"version":"3.41.2"},"reference-count":54,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2025,4,18]],"date-time":"2025-04-18T00:00:00Z","timestamp":1744934400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["R01 HL 102090","R01 HL147035"],"award-info":[{"award-number":["R01 HL 102090","R01 HL147035"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,5,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>Incidence of sudden cardiac death (SCD) is 1%\/year in cohorts with hypertrophic cardiomyopathy (HCM), but this estimate presumes arrhythmic cause and misses occult cases dying before diagnosis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>POST SCD (POstmortem Systematic InvesTigation of Sudden Cardiac Death) is a prospective cohort study using autopsy, clinical records, and toxicology to adjudicate arrhythmic or non-arrhythmic causes among presumed SCDs (pSCDs) meeting WHO criteria aged 0\u201390 years in San Francisco County. We included all incident cases 2\/1\/2011\u20133\/1\/2014 (n = 525) and approximately every third day 3\/1\/2014\u20139\/1\/2022 (n = 497) based on medical examiner call schedule. We identified HCM victims via three approaches: (i) pathology; (ii) echocardiogram [transthoracic echocardiogram (TTE)]; (iii) genetic criteria. Incidence calculations used county data and estimated HCM prevalence of 1:500 from studies of persons aged 23\u201335 years old. Of 1022 pSCDs [558 (54.6%) arrhythmic deaths] during the study period, 13 had HCM: 10 met pathology criteria; 2 via review of 203 TTEs (missed on initial report); 1 via genetic testing. Of these, 11 were arrhythmic deaths, yielding 1.3% burden of sudden death (pSCD) and 2% of arrhythmic death. Only 2 of 13 (15%) pSCDs with HCM had pre-mortem diagnosis. Incidence for persons with HCM 18\u201335 years old was 0.2% pSCDs\/year and 0.1% SADs\/year. pSCDs with HCM had a higher proportion of arrhythmic cause [11\/13 (85%) vs. 547\/1009 (54%), P = 0.03] than those without. pSCD burden due to HCM decreased with age (P = 0.003), highest among victims &amp;lt;35 years old, for whom HCM accounted for 7.1% of pSCD and 9.4% of arrhythmic death. Genetic testing of 317 consented pSCDs yielded pathogenic or likely pathogenic variants in 40% (2\/5) and identified one additional case without clinical phenotype.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>In this 11-year countywide post-mortem study, HCM meeting pathologic, clinical, or genetic criteria was associated with autopsy-confirmed arrhythmic cause of sudden death, accounting for 2% of SADs up to age 90, highest in cases &amp;lt;35 years old. Since 85% of cases were undiagnosed before pSCD, the true burden of HCM-related sudden death may be substantially underestimated.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/europace\/euaf088","type":"journal-article","created":{"date-parts":[[2025,4,18]],"date-time":"2025-04-18T17:43:22Z","timestamp":1744998202000},"source":"Crossref","is-referenced-by-count":0,"title":["Countywide burden, pathology, and genetics of lethal hypertrophic cardiomyopathy: from the POST SCD study"],"prefix":"10.1093","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7857-0789","authenticated-orcid":false,"given":"Leila","family":"Haghighat","sequence":"first","affiliation":[{"name":"Division of Cardiology, Department of Medicine, University of California-San Francisco , San Francisco, 500 Parnassus Avenue, Box 1354, CA 94143-1354 ,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrew","family":"Connolly","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, University of California-San Francisco , San Francisco, CA ,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5224-4096","authenticated-orcid":false,"given":"Francesca Nesta","family":"Delling","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine, University of California-San Francisco , San Francisco, 500 Parnassus Avenue, Box 1354, CA 94143-1354 ,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3411-1334","authenticated-orcid":false,"given":"Theodore Pravinchandra","family":"Abraham","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine, University of California-San Francisco , San Francisco, 500 Parnassus Avenue, Box 1354, CA 94143-1354 ,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ellen","family":"Moffatt","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, University of California-San Francisco , San Francisco, CA ,","place":["USA"]},{"name":"Office of the Chief Medical Examiner , San Francisco, CA ,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5996-4099","authenticated-orcid":false,"given":"Zian H","family":"Tseng","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine, University of California-San Francisco , San Francisco, 500 Parnassus Avenue, Box 1354, CA 94143-1354 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