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The Effect of Adiposity on Cardiovascular Function and Myocardial Fibrosis in Patients With Duchenne Muscular
Sarah E Henson1, Sean M Lang1,2, Philip R Khoury1,3
1Heart Institute Cincinnati Children's Hospital Medical Center Cincinnati OH.
Insights
Adiposity is linked to heart problems in Duchenne muscular dystrophy (DMD) patients. Managing body fat may help prevent cardiac dysfunction in individuals with DMD.
Area of Science:
- Cardiology
- Genetics
- Metabolic Disorders
Background:
- Duchenne muscular dystrophy (DMD) causes cardiomyopathy due to dystrophin deficiency, leading to fibrofatty myocardial replacement.
- Patients with DMD are at risk for increased adiposity from corticosteroid use and limited mobility.
- The relationship between adiposity and cardiovascular dysfunction in DMD requires further investigation.
Purpose of the Study:
- To determine the association between adiposity and cardiovascular dysfunction in patients with Duchenne muscular dystrophy.
- To investigate the link between body fat indices and cardiac magnetic resonance imaging (cardiac dysfunction and late gadolinium enhancement) in DMD patients.
Main Methods:
- Retrospective review of 324 patients with DMD who underwent cardiac MRI and dual-energy x-ray absorptiometry within one year.
- Cardiac MRI parameters included left ventricular ejection fraction and late gadolinium enhancement (LGE).
- Adiposity indices measured by DXA included body fat percentage, height-indexed fat mass, and BMI.
Main Results:
- 36% of patients had cardiac dysfunction (LVEF <55%) and 52% had LGE+.
- Patients with cardiac dysfunction exhibited higher height-indexed fat mass and BMI on univariate analysis.
- Height-indexed fat mass remained independently associated with cardiac dysfunction in multivariable analysis, adjusting for age, LGE+, and corticosteroid duration.
- High height-indexed fat mass and body fat percentage were associated with LGE+ on univariate analysis.
- High body fat percentage remained independently associated with LGE+ in multivariable analysis, adjusting for age and cardiac dysfunction.
Conclusions:
- Adiposity is independently associated with cardiac dysfunction and LGE+ in patients with Duchenne muscular dystrophy.
- Preventing or managing adiposity may be a strategy to mitigate the development of ventricular dysfunction in DMD.
- These findings highlight the importance of monitoring and managing body composition in DMD patients to support cardiovascular health.
Abstract:
Background Patients with Duchenne muscular dystrophy (DMD) develop cardiomyopathy because of a dystrophin deficiency causing fibrofatty replacement of the myocardium. Corticosteroid use and mobility limitations place these patients at risk for increased adiposity. We sought to determine the association of adiposity with cardiovascular dysfunction in patients with DMD. Methods and Results This was a retrospective review of patients with DMD who underwent both cardiac magnetic resonance imaging and dual-energy x-ray absorptiometry within 1 year. The cardiac magnetic resonance imaging parameters included left ventricular ejection fraction and the presence of late gadolinium enhancement (LGE positive [LGE+]). The adiposity indices, measured by dual-energy x-ray absorptiometry, included percentage of body fat, whole body fat mass indexed to height, and body mass index. A total of 324 patients were identified. Fifty-two percent had LGE+, and 36% had cardiac dysfunction (left ventricular ejection fraction <55%). Patients with cardiac dysfunction had higher whole body fat mass indexed to height and body mass index on univariate analysis (mean difference between patients with and without cardiac dysfunction: +2.9 kg/m, P=0.001; and +1.5 kg/m2, P=0.03, respectively). whole body fat mass indexed to height remained independently associated with cardiac dysfunction on multivariable analysis after adjusting for age, LGE+, and corticosteroid duration. High whole body fat mass indexed to height and percentage of body fat were associated with LGE+ on univariate analysis (mean difference between patients with and without LGE+: +2.0 kg/m, P=0.02; and +2.4%, P=0.02, respectively). Using multivariable analysis, including age and cardiac dysfunction, high percentage of body fat remained independently associated with LGE+. Conclusions This study demonstrates an independent association of adiposity with cardiac dysfunction and LGE+ in patients with DMD. Preventing adiposity may mitigate the later development of ventricular dysfunction in DMD.
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