Becker muscular dystrophy–related cardiomyopathy: a favorable response to medical therapy

Doing, Anthony; Renlund, Dale G.; Smith, Ruth Ann

doi:10.1016/s1053-2498(01)00316-3

Cited by 17 publications

(11 citation statements)

References 9 publications

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“…33 Our study findings indicate that, in the outpatient setting, ACE inhibitor therapy was safely initiated either alone or in combination with ␤-blocker therapy. Although the use of carvedilol and metoprolol in our study was not randomized, both drugs appeared to have the desired effects on cardiac function and were tolerated equally well.…”

Section: Discussionmentioning

confidence: 59%

Genetic Predictors and Remodeling of Dilated Cardiomyopathy in Muscular Dystrophy

et al. 2005

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Background-Dystrophin gene mutations cause 2 common muscular dystrophies, Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). Both are frequently associated with dilated cardiomyopathy (DCM) and premature death. We hypothesized that early diagnosis and treatment of DCM in DMD/BMD patients would lead to ventricular remodeling and that specific dystrophin gene mutations would predict cardiac involvement. Methods and Results-Sixty-nine boys with DMD (nϭ62) and BMD (nϭ7) (mean age, 12.9 and 13.7 years, respectively) were referred to our Cardiovascular Genetics Clinic for evaluation, including echocardiography and DNA analysis. Follow-up evaluations were scheduled yearly until the first abnormal echocardiogram indicative of DCM and quarterly thereafter. After the first abnormal echocardiogram, angiotensin-converting enzyme inhibitor or ␤-blocker therapy was started. ␤-Blockers were added if echocardiography showed no ventricular remodeling in angiotensin-converting enzyme inhibitor-treated patients after 3 months. DCM was diagnosed in 31 subjects (DMD, 27/62, 44%; BMD, 4/7, 57%) (mean age at onset, 15.4Ϯ2.8 years; range, 10.4 to 21.2 years). All 31 subjects were begun on pharmacological therapy after diagnosis. On follow-up (nϭ29), 2 subjects (both DMD) showed stable DCM, 8 subjects (all DMD) showed improvement, and 19 subjects (16 DMD; 3 BMD) showed normalization of left ventricular size and function (total improvement, 27/29 [93%]). DNA analysis in 47 cases (68%) revealed a significant association between DCM and exon 12 and 14 to 17 mutations, possible protection against DCM by exon 51 to 52 mutations, and a trend toward significant association between onset of DCM and exon 31 to 42 mutations. Statistical significance was based on nominal probability values. Conclusions-Early

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Section: Discussionmentioning

confidence: 59%

Genetic Predictors and Remodeling of Dilated Cardiomyopathy in Muscular Dystrophy

et al. 2005

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“…7 The present study of the use of a -blocker in muscular dystrophy raises questions regarding the types of muscular dystrophy amenable to the therapy and the optimal timing of the therapy. Many types of muscular dystrophy involve the cardiac muscles, [20][21][22][23][24][25][26][27][28] but because the number of patients with Becker muscular dystrophy, Fukuyama muscular dystrophy, and Emery-Dreifuss muscular dystrophy was small in the present study, we could not conclude if there was a difference in the effectiveness of -blockers among them.…”

Section: Discussionmentioning

confidence: 70%

Beta-Blocker Therapy for Cardiac Dysfunction in Patients With Muscular Dystrophy

Kajimoto

Ishigaki

Okumura

et al. 2006

Circ J

102

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eart failure is one of the major causes of death in patients with Duchenne muscular dystrophy (DMD). 1 It has been shown that angiotensin-converting enzyme inhibitors (ACEI) and -blockers effectively improve symptoms and cardiac function, and decrease mortality in adult patients with congestive heart failure caused by ischemic heart disease or dilated cardiomyopathy. 2-5 However, whether these drugs are effective in the management of patients with muscular dystrophy has not been well studied. We investigated whether a -blocker, carvedilol, in addition to an ACEI is effective to improve cardiac function in patients with muscular dystrophy. Methods PatientsBetween August 1999 and December 2002, 28 patients, age 17±5 years (range 7-29 years), were started on cilazapril or enalapril because of ventricular dysfunction. Diagnoses were DMD in 25 patients; Fukuyama-type muscular dystrophy in 2 patients, and Emery-Dreifuss muscular dystrophy in 1. The decision to start an ACEI was based on echocardiographic findings; these drugs were started when left Circulation Journal Vol.70, August 2006 ventricular (LV) fractional shortening (FS) was <0.26. After the dosage of the ACEI reached its final value, the 28 patients were randomly allocated into 2 groups: carvedilol plus an ACEI (carvedilol group: 13 patients; age 18±6 years, range 7-27 years), or an ACEI only (ACEI group: 15 patients; age 15±4 years, range 8-29 years).Because all patients were physically not active, it was difficult to evaluate cardiac symptoms, so in the present study, they were considered to be present if patients complained of fatigue, palpitations, sweating, and/or chest discomfort.There were no significant differences in age, body weight, the ratio of patients with cardiac symptoms, ratio of patients with concomitant use of diuretics, heart rate, blood pressure, and plasma brain natriuretic peptide (BNP) levels between the 2 groups before drug administration (Tables 1,2 Background In muscular dystrophy, cardiac function deteriorates with time and heart failure is one of the major causes of death. Although the combination of angiotensin-converting enzyme inhibitors (ACEI) and -blockers improves cardiac function in adults, little is known about the efficacy of those drugs in patients with muscular dystrophy. Methods and ResultsThe effect of the -blocker, carvedilol, and/or ACEI on ventricular function in patients with muscular dystrophy was studied. Carvedilol and an ACEI were given to 13 patients (ACEI group; mean age 18 years, range 7-27 years), and an ACEI only to 15 patients (carvedilol group; mean age 15 years, range 8-29 years). Diagnoses included Duchenne muscular dystrophy (n=25), Fukuyama muscular dystrophy (n=2), and Emery-Dreifuss muscular dystrophy (n=1). Echocardiographic parameters of the left ventricle were measured during the 2-3 years of follow-up. In the carvedilol group, combination therapy of carvedilol and an ACEI for 2 years resulted in a significant increase in left ventricular fractional shortening (LVFS). In the ACEI group, th...

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“…CI in BMD requires adequate treatment, depending on the type of cardiac abnormality. Overall, the prognosis of CI is favourable in the majority of BMD patients if CI is recognized early and adequately treated (42,80). …”

Section: Discussionmentioning

confidence: 99%

Cardiac involvement in Becker muscular dystrophy

Finsterer¹,

Stöllberger²

2008

Canadian Journal of Cardiology

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Becker muscular dystrophy–related cardiomyopathy: a favorable response to medical therapy

Cited by 17 publications

References 9 publications

Genetic Predictors and Remodeling of Dilated Cardiomyopathy in Muscular Dystrophy

Genetic Predictors and Remodeling of Dilated Cardiomyopathy in Muscular Dystrophy

Beta-Blocker Therapy for Cardiac Dysfunction in Patients With Muscular Dystrophy

Cardiac involvement in Becker muscular dystrophy

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