{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T15:29:05Z","timestamp":1790436545682,"version":"4.1.0"},"reference-count":32,"publisher":"Ovid Technologies (Wolters Kluwer Health)","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"abstract":"<jats:sec xml:lang=\"en\">\n                    <jats:title>Background<\/jats:title>\n                    <jats:p xml:lang=\"en\">Original apical hypertrophic cardiomyopathy was characterized by left ventricular hypertrophy confined to the apex below the papillary muscle level. In contrast, apical hypertrophic cardiomyopathy in Western countries often includes hypertrophy extending to the midventricular septum. Recognizing these phenotypic differences is essential as they may influence the clinical prognosis. The aim of this study was to delineate the clinical and genetic disparities between the pure\u2010apical form, according to the original definition, and the distal\u2010dominant form, in which hypertrophy extends to the ventricular septum without basal septal hypertrophy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p xml:lang=\"en\">A retrospective analysis was conducted for 111 consecutive patients with apical hypertrophic cardiomyopathy with assessment of hypertrophic cardiomyopathy\u2013related adverse events including hypertrophic cardiomyopathy\u2013related death, heart failure admission, embolic stroke admission, and sustained ventricular tachycardia with hemodynamic instability or appropriate implantable cardioverter\u2010defibrillator discharge. Genetic testing for hypertrophic cardiomyopathy\u2010associated genes was performed in 72 patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Results<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      Among the patients, 60 were classified as pure\u2010apical form, and 51 were classified as distal\u2010dominant form. The median age at diagnosis was 63\u2009years, with a predominance of men in both groups. Over a follow\u2010up period of 11.0\u2009years, the incidence of hypertrophic cardiomyopathy\u2013related adverse events was significantly higher in the distal\u2010dominant group than in the pure\u2010apical group (log\u2010rank,\n                      <jats:italic>P<\/jats:italic>\n                      &lt;0.001). The detection rate of pathogenic or likely pathogenic variants was also significantly higher in the distal\u2010dominant group than in the pure\u2010apical group (26% versus 3%;\n                      <jats:italic>P<\/jats:italic>\n                      =0.005).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p xml:lang=\"en\">Distinct clinical and genetic profiles of the 2 apical hypertrophic cardiomyopathy phenotypes warrant their recognition and differentiation in clinical practice due to distinct prognoses and genetic backgrounds.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/jaha.124.038208","type":"journal-article","created":{"date-parts":[[2025,3,7]],"date-time":"2025-03-07T23:52:01Z","timestamp":1741391521000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":8,"title":["Unveiling Clinical and Genetic Distinctions in Pure\u2010Apical Versus Distal\u2010Dominant Apical Hypertrophic Cardiomyopathy"],"prefix":"10.1161","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3414-0434","authenticated-orcid":false,"given":"Kenta","family":"Sugiura","sequence":"first","affiliation":[{"name":"Department of Cardiology and Geriatrics, Kochi Medical School Kochi University Kochi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5681-8854","authenticated-orcid":false,"given":"Toru","family":"Kubo","sequence":"additional","affiliation":[{"name":"Department of Cardiology and Geriatrics, Kochi Medical School Kochi University Kochi 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