{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T21:24:10Z","timestamp":1781299450338,"version":"3.54.1"},"reference-count":26,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2025,12,5]],"date-time":"2025-12-05T00:00:00Z","timestamp":1764892800000},"content-version":"vor","delay-in-days":1298,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2022,5,17]],"date-time":"2022-05-17T00:00:00Z","timestamp":1652745600000},"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":[[2022,8,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Despite advances in contemporary cardiopulmonary therapies, cardiomyopathy remains the leading cause of death in patients with Duchenne muscular dystrophy (DMD). Also, the long-term clinical outcomes of patients with DMD and cardiomyopathy is unknown. This study investigated long-term clinical outcomes and their associated factors in patients with late-stage DMD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>A total of 116 patients with late-stage DMD (age &amp;gt; 15 years) were enrolled in this retrospective study. All enrolled patients were followed up at a single tertiary referral hospital. LV systolic dysfunction was dichotomously defined as reduced [left ventricular ejection fraction (LVEF) \u2264 40%] vs. preserved [&amp;gt;40%] based on the initial echocardiographic result. The primary endpoint was all-cause death. The secondary endpoint was a composite event defined as death or unexpected hospitalization due to cardiovascular reasons including chest pain, dyspnoea, and generalized oedema. The patients were divided into preserved (n = 84, 72.4%) and reduced LVEF groups (n = 32, 27.6%). The mean age was 20.8 \u00b1 5.9 years, the mean disease duration, 8.8 \u00b1 3.7 years, and the mean follow-up duration, 1708 \u00b1 659 days. For primary endpoint, the reduced LVEF group showed a lower rate of overall survival (Reduced LVEF vs. Preserved LVEF; 81.3% vs. 98.8%, log-rank P = 0.005). In the multivariable Cox regression analysis, brain-natriuretic peptide (BNP) level (adjusted hazard ratio [HR] 1.088, 95% confidence interval [CI] 1.019\u20131.162, P = 0.011) and diuretic use (adjusted HR 9.279, 95%CI 1.651\u201352.148, P = 0.011) were significant predictors of all-cause death in patients with DMD. For the secondary endpoint, the reduced LVEF group had a lower rate of freedom from composite events than the preserved LVEF group (65.6% vs. 86.9%, log-rank P = 0.005). In the multivariable Cox regression analysis, BNP level (adjusted HR 1.057, 95%CI 1.005\u20131.112, P = 0.032) and diuretic use (adjusted HR 4.189, 95% CI 1.704\u201310.296, P = 0.002) were significant predictors of the composite event in patients with DMD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Patients with DMD and reduced LVEF had worse clinical outcomes than those with preserved LVEF. BNP level and diuretic use were associated with adverse clinical outcomes in patients with late-stage DMD, irrespective of LVEF.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ehf2.13970","type":"journal-article","created":{"date-parts":[[2022,5,17]],"date-time":"2022-05-17T05:39:06Z","timestamp":1652765946000},"page":"2199-2206","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["The Association Between Cardiac Involvement and Long-Term Clinical Outcomes in Patients with Duchenne Muscular Dystrophy"],"prefix":"10.1093","volume":"9","author":[{"given":"Jung-Joon","family":"Cha","sequence":"first","affiliation":[{"name":"Department of Cardiology, Cardiovascular Centre, Korea University Anam Hospital, Korea University College of Medicine , Seoul ,","place":["Korea"]},{"name":"Department of Internal Medicine, Yonsei University College of Medicine , Seoul 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,","place":["Korea"]},{"name":"Division of Cardiology, Gangnam Severance Hospital, Yonsei University College of Medicine , Seoul ,","place":["Korea"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pil-Ki","family":"Min","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Yonsei University College of Medicine , Seoul ,","place":["Korea"]},{"name":"Division of Cardiology, Gangnam Severance Hospital, Yonsei University College of Medicine , Seoul ,","place":["Korea"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Young Won","family":"Yoon","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Yonsei University College of Medicine , Seoul ,","place":["Korea"]},{"name":"Division of Cardiology, Gangnam Severance Hospital, Yonsei University College of Medicine , Seoul ,","place":["Korea"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Byoung Kwon","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Yonsei University College of Medicine , Seoul ,","place":["Korea"]},{"name":"Division of Cardiology, Gangnam Severance Hospital, Yonsei University College of Medicine , Seoul ,","place":["Korea"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bum-Kee","family":"Hong","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Yonsei University College of Medicine , Seoul ,","place":["Korea"]},{"name":"Division of Cardiology, Gangnam Severance Hospital, Yonsei University College of Medicine , Seoul ,","place":["Korea"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hyuck Moon","family":"Kwon","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Yonsei University College of Medicine , Seoul ,","place":["Korea"]},{"name":"Division of Cardiology, Gangnam Severance Hospital, Yonsei University College of Medicine , Seoul ,","place":["Korea"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Han Eol","family":"Cho","sequence":"additional","affiliation":[{"name":"Rehabilitation Institute of Neuromuscular Disease, Yonsei University College of Medicine , Seoul ,","place":["Korea"]},{"name":"Department of Rehabilitation Medicine and Pulmonary Rehabilitation Center, Gangnam Severance Hospital, Yonsei University College of Medicine , Seoul ,","place":["Korea"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Won Ah","family":"Choi","sequence":"additional","affiliation":[{"name":"Rehabilitation Institute of Neuromuscular Disease, Yonsei University College of Medicine , Seoul ,","place":["Korea"]},{"name":"Department of Rehabilitation Medicine and Pulmonary Rehabilitation Center, Gangnam Severance Hospital, Yonsei University College of Medicine , Seoul 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