Progressive left ventricular dysfunction and myocardial fibrosis in Duchenne and Becker muscular dystrophy: a longitudinal cardiovascular magnetic resonance study

Aikawa, Tadao; Takeda, Atsuhito; Oyama‐Manabe, Noriko; Naya, Masanao; Yamazawa, Hirokuni; Koyanagawa, Kazuhiro; Ito, Yoichi M.; Anzai, Toshihisa

doi:10.1007/s00246-018-2046-x

Cited by 22 publications

(24 citation statements)

References 25 publications

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“…Other observational studies suggest it is unclear if the progressive decline in LV function that characterizes DMD cardiomyopathy can be attenuated by ACEI and/or BB. Aikawa et al 7 reported that fibrosis, as measured by late gadolinium enhancement with CMR, was not attenuated by ACEI, and that only subjects with a reduced LVEF at baseline exhibited an increase in LVEF with therapy 7 . Similarly, Hor et al 11 found that the addition of an ACEI to chronic steroid therapy did not reduce the decline of LV circumferential strain as measured by CMR.…”

Section: Resultsmentioning

confidence: 99%

A systematic review of pharmacologic therapies for the cardiomyopathy of Duchenne muscular dystrophy

Kipke

Birnkrant

Jin

et al. 2021

Pediatric Pulmonology

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Objective To perform a systematic review of studies evaluating pharmacologic therapies for the cardiomyopathy of Duchenne muscular dystrophy (DMD). Methods: PubMed, Google Scholar, and Embase were searched through October 8, 2020. Articles were selected using pre‐determined criteria; 26 underwent detailed review by two co‐authors. Study quality was assessed with the Newcastle–Ottawa scoring system (NOS); GRADE assessment evaluated their overall clinical importance. Results There were few randomized controlled trials. Two of four trials of angiotensin converting enzyme inhibitors (ACEI) or ACEI plus beta‐blockers (BB) found improved LV function. Two of two randomized trials of aldosterone antagonists (AA), when added to ACEI and BB therapy, demonstrated less decline of LV circumferential strain over 1 year of treatment. Observational studies of ACEI and BB had differing patient ages, symptomatology, cohort size, study duration and baseline heart function. LV function, assessed via unblinded imaging, was the most frequent outcome measure. LV dysfunction improved in some trials but was unconfirmed in others. Class IV heart failure patients had transient improvement of symptoms and LVEF. Most NOS scores reflected a low level of study quality. The Grade certainty rating, used for the summation of studies, was between “low” and “moderate.” Conclusion Randomized trial evidence was inconsistent that either ACEI or BB or their combination improve LV function and/or alter progressive LV dysfunction. When ACEI and BB therapy are initiated for symptomatic Class IV heart failure, symptoms and LVEF improve transiently. AAs retard the rate of decline of LV function when initiated in younger DMD patients.

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Section: Resultsmentioning

confidence: 99%

A systematic review of pharmacologic therapies for the cardiomyopathy of Duchenne muscular dystrophy

Kipke

Birnkrant

Jin

et al. 2021

Pediatric Pulmonology

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“…Twelve studies were included in our meta-analysis ( Duboc et al, 2005 ; Jefferies et al, 2005 ; Kajimoto et al, 2006 ; Rhodes et al, 2008 ; Matsumura et al, 2010 ; Viollet et al, 2012 ; Raman et al, 2015 ; Silva et al, 2017 ; Aikawa et al, 2019 ; Adorisio et al, 2019 ; Dittrich et al, 2019 ; Kim et al, 2020 ). Four studies were RCTs, ( Duboc et al, 2005 ; Raman et al, 2015 ; Silva et al, 2017 ; Dittrich et al, 2019 ) seven were observational, ( Jefferies et al, 2005 ; Kajimoto et al, 2006 ; Rhodes et al, 2008 ; Viollet et al, 2012 ; Adorisio et al, 2019 ; Aikawa et al, 2019 ; Kim et al, 2020 )and one study was open-label. Study characteristics are summarized in Table 1 ( Matsumura et al, 2010 ).…”

Section: Resultsmentioning

confidence: 99%

Anti-Remodeling Cardiac Therapy in Patients With Duchenne Muscular Dystrophy, Meta-Analysis Study

et al. 2022

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Background: Almost all Duchenne muscular dystrophy (DMD) patients that reach their 30s present cardiomyopathy. As a result, this population remains under-treated. There is no sufficient proof of the efficacy of anti-remodeling cardiac therapy for DMD cardiomyopathy (DMDCM). We aim to assess the efficacy of anti-remodeling cardiac therapy for DMDCM by using meta-analysis.Methods: PubMed (MEDLINE), Embase, and Cochrane library were searched through January 2021. Randomized control trials, case-control studies, and observational studies that reported assessments of cardiovascular outcomes and death of participants using angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, beta-blockers, mineralocorticoid-receptor antagonists and Ivabradine, were included. The primary outcome was total mortality. Secondary outcomes included changes in left ventricular ejection fraction (LVEF), serum natriuretic peptide levels (BNP), and heart rate (HR). Data were extracted for eligibility by two independent reviewers. Random-effects meta-analysis was used to pool results.Results: Twelve studies with 439 patients were included in our meta-analysis. Treated patients have lower HR, mean difference of −17 beats per minute (CI [−25]–[−9], p < 0.01). The LVEF was improved in treated patients, with a mean difference of LVEF of 3.77% (CI 0.44–7.12, p < 0.03). Although mortality rates did not reach statistical significance there was a trend for total mortality reduction (hazard ratio 0.36, CI (0.1–1.25), p = 0.107) and for BNP reduction (SSMD: 0.141, CI ([−0.19]–[0.47]), p = 0.3).Conclusion: Pharmacologic treatment for DMDCM patients is associated with decreased HR and improved LVEF. Therefore, DMDCM patients may benefit from implementing guideline therapy for HF.

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“…12 Both readers were blinded to clinical information. The extent of LGE was expressed as a percentage of LV mass using a 5-standard deviation (SD) threshold above the mean of normal myocardium 3 because the extent of LGE on PSIR sequence quantified using the "n"-SD method has been reported to be comparable to that on inversion-recovery balanced steadystate free precession sequence in both ischemic and nonischemic cardiomyopathies. 13 Global ECV was calculated using pre-and post-contrast T 1 values of myocardium and blood pool (T 1 myo pre , T 1 myo post , T 1 blood pre , and T 1 blood post , respectively) as follows: ECV (%) = (1 − hematocrit) × (1/T 1 myo post − 1/T 1 myo pre )/(1/T 1 blood post − 1/T 1 blood pre ).…”

Section: Methodsmentioning

confidence: 99%

“…1 The most common form of cardiac involvement in muscular dystrophy is dilated cardiomyopathy, 2 presenting as an age-related progression of left ventricular (LV) dysfunction and myocardial fibrosis detected by late gadolinium enhancement (LGE) cardiovascular MR (CMR) imaging. 3 Accurate evaluation of myocardial fibrosis could be useful for risk stratification 4 and determining early initiation of cardioprotective treatment in patients with muscular dystrophy. 5,6 Cardiomyopathy associated with muscular dystrophy has also been reported in female carriers of Duchenne and Becker muscular dystrophy.…”

Section: Introductionmentioning

confidence: 99%

Myocardial T<sub>1</sub>-mapping and Extracellular Volume Quantification in Patients and Putative Carriers of Muscular Dystrophy: Early Experience

et al. 2021

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To assess myocardial fibrosis associated with muscular dystrophy, T 1 -mapping and extracellular volume fraction (ECV) quantification was prospectively performed using cardiovascular MR (CMR) imaging in 6 male patients with muscular dystrophy and 5 female putative carriers of Duchenne or Becker muscular dystrophy. Five patients and all putative carriers had an elevated ECV (>29.5% for men and >35.2% for women), suggesting that ECV has a potential to detect diffuse fibrotic changes in patients and putative carriers of muscular dystrophy.

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Progressive left ventricular dysfunction and myocardial fibrosis in Duchenne and Becker muscular dystrophy: a longitudinal cardiovascular magnetic resonance study

Cited by 22 publications

References 25 publications

A systematic review of pharmacologic therapies for the cardiomyopathy of Duchenne muscular dystrophy

A systematic review of pharmacologic therapies for the cardiomyopathy of Duchenne muscular dystrophy

Anti-Remodeling Cardiac Therapy in Patients With Duchenne Muscular Dystrophy, Meta-Analysis Study

Myocardial T<sub>1</sub>-mapping and Extracellular Volume Quantification in Patients and Putative Carriers of Muscular Dystrophy: Early Experience

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