{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,25]],"date-time":"2026-04-25T03:09:58Z","timestamp":1777086598203,"version":"3.51.4"},"reference-count":35,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"16","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2023,8,15]]},"abstract":"<jats:sec sec-type=\"background\" xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">Chronic respiratory failure and heart involvement may occur in Duchenne muscular dystrophy. We aimed to assess the prognostic value of the right ventricular (RV) systolic dysfunction in patients with Duchenne 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 studied 90 genetically proven patients with Duchenne muscular dystrophy from 2010 to 2019, to obtain respiratory function and Doppler echocardiographic RV systolic function. Prognostic value was assessed in terms of death and cardiac events. The median age was 27.5\u2009years, and median forced vital capacity was at 10% of the predicted value: 83 patients (92%) were on home mechanical ventilation. An RV systolic dysfunction was found in 46 patients (51%). In patients without RV dysfunction at inclusion, a left ventricular systolic dysfunction at inclusion was associated with a higher risk of developing RV dysfunction during follow\u2010up with an odds ratio of 4.5 (\n              <jats:italic>P<\/jats:italic>\n              =0.03). RV systolic dysfunction was significantly associated with cardiac events, mainly acute heart failure (62%) and cardiogenic shock (23%). In a multivariable Cox model, the adjusted hazard ratio was 4.96 (95% CI [1.09\u201322.6];\n              <jats:italic>P<\/jats:italic>\n              =0.04). In terms of death, we found a significant difference between patients with RV dysfunction versus patients without RV dysfunction in the Kaplan\u2013Meier curves (log\u2010rank\n              <jats:italic>P<\/jats:italic>\n              =0.045).\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">RV systolic dysfunction is frequently present in patients with Duchenne muscular dystrophy and is associated with increased risk of cardiac events, irrespective of left ventricular dysfunction and mechanical ventilation.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Registration<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              URL:\n              <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"https:\/\/www.clinicaltrials.org\">https:\/\/www.clinicaltrials.org<\/jats:ext-link>\n              ; unique identifier: NCT02501083.\n            <\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.122.027231","type":"journal-article","created":{"date-parts":[[2023,8,10]],"date-time":"2023-08-10T10:37:13Z","timestamp":1691663833000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":6,"title":["Prognosis of Right Ventricular Systolic Dysfunction in Patients With Duchenne Muscular Dystrophy"],"prefix":"10.1161","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3844-5438","authenticated-orcid":false,"given":"Abdallah","family":"Fayssoil","sequence":"first","affiliation":[{"name":"Cardiology Department Assistance Publique des H\u00f4pitaux de Paris (AP\u2010HP), Raymond Poincar\u00e9 Hospital Garches France"},{"name":"INSERM U1179, END\u2010ICAP Montigny\u2010le\u2010Bretonneux France"},{"name":"Cardiology Department, Centre de r\u00e9f\u00e9rence des cardiomyopathies et des troubles du rythme cardiaque h\u00e9r\u00e9ditaires ou rares AP\u2010HP, Ambroise Par\u00e9 Hospital, Universit\u00e9 de Versailles\u2010Saint Quentin (UVSQ) Boulogne\u2010Billancourt France"},{"name":"Service de R\u00e9animation m\u00e9dicale et unit\u00e9 de ventilation \u00e0 domicile CHU Raymond Poincar\u00e9, APHP, UVSQ Garches France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5500-1535","authenticated-orcid":false,"given":"Nicolas","family":"Mansencal","sequence":"additional","affiliation":[{"name":"Cardiology Department, Centre de r\u00e9f\u00e9rence des cardiomyopathies et des troubles du rythme cardiaque h\u00e9r\u00e9ditaires ou rares AP\u2010HP, Ambroise Par\u00e9 Hospital, Universit\u00e9 de Versailles\u2010Saint Quentin (UVSQ) Boulogne\u2010Billancourt France"},{"name":"INSERM U\u20101018, Centre de recherche en Epid\u00e9miologie et Sant\u00e9 des Populations (CESP) Equipe Epid\u00e9miologie Clinique, UVSQ Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6014-6269","authenticated-orcid":false,"given":"Lee S.","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Department of Pharmacology Sorbonne University, INSERM CIC Paris\u2010Est, AP\u2010HP, ICAN, Regional Pharmacovigilance Centre, Piti\u00e9\u2010Salp\u00eatri\u00e8re Hospital Paris France"},{"name":"CMC Ambroise Par\u00e9, Research &amp; Innovation (RICAP) Neuilly\u2010sur\u2010Seine France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4086-9766","authenticated-orcid":false,"given":"Olivier","family":"Nardi","sequence":"additional","affiliation":[{"name":"Service de R\u00e9animation m\u00e9dicale et unit\u00e9 de ventilation \u00e0 domicile CHU Raymond Poincar\u00e9, APHP, UVSQ Garches France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rabah Ben","family":"Yaou","sequence":"additional","affiliation":[{"name":"Institut de Myologie, CHU Piti\u00e9 Salpetri\u00e8re Paris France"},{"name":"Centre de Recherche en Myologie Sorbonne Universit\u00e9s, UPMC Univ Paris 06, INSERM UMRS974, Institut de Myologie, G.H. Piti\u00e9 Salp\u00eatri\u00e8re Paris France"},{"name":"Laboratoire de biochimie et g\u00e9n\u00e9tique mol\u00e9culaire h\u00f4pital Cochin, AP\u2010HP, universit\u00e9 Paris Descartes\u2010Sorbonne Paris Cit\u00e9 Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"France","family":"Leturcq","sequence":"additional","affiliation":[{"name":"Institut de Myologie, CHU Piti\u00e9 Salpetri\u00e8re Paris France"},{"name":"Centre de Recherche en Myologie Sorbonne Universit\u00e9s, UPMC Univ Paris 06, INSERM UMRS974, Institut de Myologie, G.H. 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