{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,6]],"date-time":"2026-05-06T11:27:18Z","timestamp":1778066838706,"version":"3.51.4"},"reference-count":61,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"21","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2023,11,7]]},"abstract":"<jats:sec sec-type=\"background\" xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">The prognostic utility of cardiovascular magnetic resonance imaging, including strain analysis and tissue characterization, has not been comprehensively investigated in adult patients with muscular dystrophy.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Methods and Results<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              We prospectively enrolled 148 patients with dystrophinopathies (including heterozygotes), limb\u2010girdle muscular dystrophy, and type 1 myotonic dystrophy (median age, 36.0 [interquartile range, 23.0\u201350.0] years; 51 [34.5%] women) over 7.7\u2009years in addition to an age\u2010 and sex\u2013matched healthy control cohort (n=50). Cardiovascular magnetic resonance markers, including 3\u2010dimensional strain and fibrosis, were assessed for their respective association with major adverse cardiac events. Our results showed that markers of contractile performance were reduced across all muscular dystrophy groups. In particular, the dystrophinopathies cohort experienced reduced left ventricular (LV) ejection fraction and high burden of replacement fibrosis. Patients with type 1 myotonic dystrophy showed a 26.8% relative reduction in LV mass with corresponding reduction in chamber volumes. Eighty\u2010two major adverse cardiac events occurred over a median follow\u2010up of 5.2\u2009years. Although LV ejection fraction was significantly associated with major adverse cardiac events (adjusted hazard ratio [aHR], 3.0 [95% CI, 1.4\u20136.4]) after adjusting for covariates, peak 3\u2010dimensional strain amplitude demonstrated greater predictive value (minimum principal amplitude: aHR, 5.5 [95% CI, 2.5\u201311.9]; maximum principal amplitude: aHR, 3.3 [95% CI, 1.6\u20136.8]; circumferential amplitude: aHR, 3.4 [95% CI, 1.6\u20137.2]; longitudinal amplitude: aHR, 3.4 [95% CI, 1.7\u20136.9]; and radial strain amplitude: aHR, 3.0 [95% CI, 1.4\u20136.1]). Minimum principal strain yielded incremental prognostic value beyond LV ejection fraction for association with major adverse cardiac events (change in\n              <jats:italic>\u03c7<\/jats:italic>\n              <jats:sup>2<\/jats:sup>\n              =13.8;\n              <jats:italic>P<\/jats:italic>\n              &lt;0.001).\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">Cardiac dysfunction is observed across all muscular dystrophy subtypes; however, the subtypes demonstrate distinct phenotypic profiles. Myocardial deformation analysis highlights unique markers of principal strain that improve risk assessment over other strain markers, LV ejection fraction, and late gadolinium enhancement in this vulnerable patient population.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.123.030229","type":"journal-article","created":{"date-parts":[[2023,11,6]],"date-time":"2023-11-06T11:30:58Z","timestamp":1699270258000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":9,"title":["Prognostic Utility of Cardiovascular Magnetic Resonance\u2013Based Phenotyping in Patients With Muscular Dystrophy"],"prefix":"10.1161","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-1109-0206","authenticated-orcid":false,"given":"Niharika","family":"Kashyap","sequence":"first","affiliation":[{"name":"Division of Cardiology University of Alberta Edmonton Alberta Canada"},{"name":"Department of Medicine, Faculty of Medicine and Dentistry Mazankowski Alberta Heart Institute, University of Alberta Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anish","family":"Nikhanj","sequence":"additional","affiliation":[{"name":"Division of Cardiology University of Alberta Edmonton Alberta Canada"},{"name":"Department of Medicine, Faculty of Medicine and Dentistry Mazankowski Alberta Heart Institute, University of Alberta Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7108-8554","authenticated-orcid":false,"given":"Dina","family":"Labib","sequence":"additional","affiliation":[{"name":"Department of Cardiac Sciences University of Alberta Edmonton Alberta Canada"},{"name":"Libin Cardiovascular Institute of Alberta, University of Calgary Calgary Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Easter","family":"Prosia","sequence":"additional","affiliation":[{"name":"Department of Cardiac Sciences University of Alberta Edmonton Alberta Canada"},{"name":"Libin Cardiovascular Institute of Alberta, University of Calgary Calgary Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sandra","family":"Rivest","sequence":"additional","affiliation":[{"name":"Department of Cardiac Sciences University of Alberta Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7985-9907","authenticated-orcid":false,"given":"Jacqueline","family":"Flewitt","sequence":"additional","affiliation":[{"name":"Libin Cardiovascular Institute of Alberta, University of Calgary Calgary Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7657-7098","authenticated-orcid":false,"given":"Gerald","family":"Pfeffer","sequence":"additional","affiliation":[{"name":"Department of Clinical Neurosciences University of Calgary Calgary Alberta Canada"},{"name":"Hotchkiss Brain Institute, University of Calgary Calgary Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3658-2554","authenticated-orcid":false,"given":"Jeffrey A.","family":"Bakal","sequence":"additional","affiliation":[{"name":"Alberta Strategy for Patient Oriented Research Unit University of Calgary Calgary Alberta Canada"},{"name":"Provincial Research Data Services University of Alberta Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zaeem A.","family":"Siddiqi","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Medicine, Faculty of Medicine and Dentistry University of Alberta Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6920-8833","authenticated-orcid":false,"given":"Richard A.","family":"Coulden","sequence":"additional","affiliation":[{"name":"Department of Radiology and Diagnostic Imaging University of Alberta Hospital Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3319-4377","authenticated-orcid":false,"given":"Richard","family":"Thompson","sequence":"additional","affiliation":[{"name":"Division of Cardiology University of Alberta Edmonton Alberta Canada"},{"name":"Department of Medicine, Faculty of Medicine and Dentistry Mazankowski Alberta Heart Institute, University of Alberta Edmonton Alberta Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5297-4507","authenticated-orcid":false,"given":"James A.","family":"White","sequence":"additional","affiliation":[{"name":"Department of Cardiac Sciences University of Alberta Edmonton Alberta Canada"},{"name":"Libin Cardiovascular Institute of Alberta, University of Calgary Calgary 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