{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T12:00:42Z","timestamp":1770897642940,"version":"3.50.1"},"reference-count":29,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2026,1,19]],"date-time":"2026-01-19T00:00:00Z","timestamp":1768780800000},"content-version":"vor","delay-in-days":3168,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2017,5,18]],"date-time":"2017-05-18T00:00:00Z","timestamp":1495065600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2017,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Duchenne muscular dystrophy (DMD) is characterized by respiratory and heart involvements. In the context of permanently wheelchair bound and on mechanical ventilation (MV) patients, the clinical presentation of acute heart failure (AHF) syndrome may be atypical. We sought to describe clinical and genetic profiles and to determine prognosis of DMD and Becker muscular dystrophy (BMD) patients on home MV (HMV), hospitalized for AHF.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>We included genetically proven DMD and BMD patients on HMV admitted for AHF. A total of 13 patients (11 DMD and 2 BMD) fulfilled the inclusion criteria. Median age was 34.0 [interquartile range (IQR) 26.0; 40.0] years. Median pulmonary vital capacity was 9.0% (6.0; 15.0) of predicted value. Long-term invasive ventilation was performed in 69% of patients. All the 11 DMD patients carried out-of-frame DMD gene mutations. At admission, dyspnoea was present in 46%, lipothymia in 23%, and abdominal discomfort in 38.4% of patients. A total of 53.8% of patients showed anasarca. Cardiogenic shock presentation was found in six patients (46%). Ejection fraction was severely altered [median 25% (IQR 20; 30)]. Intra-hospital mortality rate was 30%, reaching 53.8 % after 1 year. Previous episodes of AHF \u2265 2 were associated with intra-hospital mortality (P = 0.025). In patients with cardiogenic shock, intra-hospital mortality rate was 66.6%, reaching 83.3% after 1 year.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>In adult DMD and BMD patients with severe ejection fraction alteration and on HMV, admitted for AHF, right cardiac signs are frequent. The intra-hospital and 1 year mortality rate was high and was associated with previous episodes of AHF \u2265 2.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ehf2.12165","type":"journal-article","created":{"date-parts":[[2017,5,18]],"date-time":"2017-05-18T06:59:20Z","timestamp":1495090760000},"page":"527-534","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Clinical Profiles and Prognosis of Acute Heart Failure in Adult Patients with Dystrophinopathies on Home Mechanical Ventilation"],"prefix":"10.1093","volume":"4","author":[{"given":"Abdallah","family":"Fayssoil","sequence":"first","affiliation":[{"name":"Service de R\u00e9animation m\u00e9dicale et unit\u00e9 de ventilation \u00e0 domicile, centre de r\u00e9f\u00e9rence neuromusculaire GNMH, CHU Raymond Poincar\u00e9, APHP, Universit\u00e9 de Versailles Saint Quentin en Yvelines , Garches ,","place":["France"]},{"name":"Centre d'Investigation clinique et Innovation technologique CIC 14.29, INSERM , Garches ,","place":["France"]},{"name":"UPMC INSERM UMRS974, CNRS FRE3617, Centre de recherche en myologie, Institut de Myologie, Groupe hospitalier Piti\u00e9 Salpetri\u00e8re , Paris ,","place":["France"]},{"name":"APHP, Centre de r\u00e9f\u00e9rence maladies neuromusculaires Paris-Est, Groupe hospitalier Piti\u00e9-Salp\u00eatri\u00e8re , Paris ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rabah Ben","family":"Yaou","sequence":"additional","affiliation":[{"name":"UPMC INSERM UMRS974, CNRS FRE3617, Centre de recherche en myologie, Institut de Myologie, Groupe hospitalier Piti\u00e9 Salpetri\u00e8re , Paris ,","place":["France"]},{"name":"APHP, Centre de r\u00e9f\u00e9rence maladies neuromusculaires Paris-Est, Groupe hospitalier Piti\u00e9-Salp\u00eatri\u00e8re , Paris ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adam","family":"Ogna","sequence":"additional","affiliation":[{"name":"Service de R\u00e9animation m\u00e9dicale et unit\u00e9 de ventilation \u00e0 domicile, centre de r\u00e9f\u00e9rence neuromusculaire GNMH, CHU Raymond Poincar\u00e9, APHP, Universit\u00e9 de Versailles Saint Quentin en Yvelines , Garches ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"France","family":"Leturcq","sequence":"additional","affiliation":[{"name":"UPMC INSERM UMRS974, CNRS FRE3617, Centre de recherche en myologie, Institut de Myologie, Groupe hospitalier Piti\u00e9 Salpetri\u00e8re , Paris ,","place":["France"]},{"name":"Laboratoire de g\u00e9n\u00e9tique et biologie mol\u00e9culaires, h\u00f4pital Cochin, AP-HP, Universit\u00e9 Paris Descartes-Sorbonne Paris Cit\u00e9 , Paris ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olivier","family":"Nardi","sequence":"additional","affiliation":[{"name":"Service de R\u00e9animation m\u00e9dicale et unit\u00e9 de ventilation \u00e0 domicile, centre de r\u00e9f\u00e9rence neuromusculaire GNMH, CHU Raymond Poincar\u00e9, APHP, Universit\u00e9 de Versailles Saint Quentin en Yvelines , Garches ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bernard","family":"Clair","sequence":"additional","affiliation":[{"name":"Service de R\u00e9animation m\u00e9dicale et unit\u00e9 de ventilation \u00e0 domicile, centre de r\u00e9f\u00e9rence neuromusculaire GNMH, CHU Raymond Poincar\u00e9, APHP, Universit\u00e9 de Versailles Saint Quentin en Yvelines , Garches ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karim","family":"Wahbi","sequence":"additional","affiliation":[{"name":"APHP, Centre de r\u00e9f\u00e9rence maladies neuromusculaires Paris-Est, Groupe hospitalier Piti\u00e9-Salp\u00eatri\u00e8re , Paris ,","place":["France"]},{"name":"Service de Cardiologie, H\u00f4pital Cochin, APHP, Universit\u00e9 Paris Descartes-Sorbonne Paris Cit\u00e9 , Paris ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Frederic","family":"Lofaso","sequence":"additional","affiliation":[{"name":"Service de Physiologie - Exploration fonctionnelles, CHU Raymond Poincar\u00e9, APHP, Universit\u00e9 de Versailles saint Quentin en Yvelines , Garches ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pascal","family":"Laforet","sequence":"additional","affiliation":[{"name":"APHP, Centre de r\u00e9f\u00e9rence maladies neuromusculaires Paris-Est, Groupe hospitalier Piti\u00e9-Salp\u00eatri\u00e8re , Paris ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Denis","family":"Duboc","sequence":"additional","affiliation":[{"name":"Service de Cardiologie, H\u00f4pital Cochin, APHP, Universit\u00e9 Paris Descartes-Sorbonne Paris Cit\u00e9 , Paris ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Orlikowski","sequence":"additional","affiliation":[{"name":"Service de R\u00e9animation m\u00e9dicale et unit\u00e9 de ventilation \u00e0 domicile, centre de r\u00e9f\u00e9rence neuromusculaire GNMH, CHU Raymond Poincar\u00e9, APHP, Universit\u00e9 de Versailles Saint Quentin en Yvelines , Garches ,","place":["France"]},{"name":"Centre d'Investigation clinique et Innovation technologique CIC 14.29, INSERM , Garches ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Djillali","family":"Annane","sequence":"additional","affiliation":[{"name":"Service de R\u00e9animation m\u00e9dicale 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