title: A Case of Refractory Heart Failure in Becker Muscular Dystrophy Improved With Corticosteroid Therapy.
abstract: The patient was a 26 year-old man who was referred to our hospital in June 2011 because of severe heart failure. At age 24 years, he was found to have Becker muscular dystrophy. He received enalapril for cardiac dysfunction; however, he had worsening heart failure and was thus referred to our hospital. Echocardiography showed enlargement of the left ventricle, with a diastolic dimension of 77 mm and ejection fraction of 19%. His condition improved temporarily after an infusion of dobutamine and milrinone. He was then administered amiodarone for ventricular tachycardia; however, he subsequently developed hemoptysis. Amiodarone was discontinued and corticosteroid pulse therapy was administered followed by oral prednisolone (PSL). His creatinine phosphokinase (CPK) level and cardiomegaly improved after the corticosteroid therapy. The PSL dose was reduced gradually, bisoprolol was introduced, and the catecholamine infusion was tapered. A cardiac resynchronization device was implanted; however, the patient's condition gradually worsened, which necessitated dobutamine infusion for heart failure. We readministered 30 mg PSL, which decreased the CPK level and improved the cardiomegaly. The dobutamine infusion was discontinued, and the patient was discharged. He was given 7.5 mg PSL as an outpatient, and he returned to normal life without exacerbation of the heart failure. There are similar reports showing that corticosteroids are effective for skeletal muscle improvement in Duchenne muscular dystrophy; however, their effectiveness for heart failure has been rarely reported. We experienced a case of Becker muscular dystrophy in which corticosteroid therapy was effective for refractory heart failure.
authors.affiliation: Department of Cardiology, Okinawa Prefectural Nanbu Medical Center and Children's Medical Center.
authors.forename: Makiko
authors.initials: M
authors.lastname: Nakamura
authors.forename: Osahiko
authors.initials: O
authors.lastname: Sunagawa
authors.forename: Ryo
authors.initials: R
authors.lastname: Hokama
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Tsuchiya
authors.forename: Takafumi
authors.initials: T
authors.lastname: Miyara
authors.forename: Yoji
authors.initials: Y
authors.lastname: Taba
authors.forename: Takashi
authors.initials: T
authors.lastname: Touma
mesh_terms: D000328:Adult; D005938:Glucocorticoids / Q000627:therapeutic use*; D006333:Heart Failure / Q000188:drug therapy* / Q000209:etiology*; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000188:drug therapy; D011239:Prednisolone / Q000627:therapeutic use*
chemical_list: D005938:Glucocorticoids; D011239:Prednisolone