Lower Body Mass Indexed in Non-Ambulatory Duchenne Muscular Dystrophy is Associated with Worse Disease Severity by Cardiac Magnetic Resonance Imaging


Clinical Management

Poster Number: 9


Kan Hor, MD, Nationwide Children's Hospital and The Ohio State University, Selina Lao, Nationwide Children's Hospital, William Hor, Pittsburgh University College of Pharmacy, Kathleen Lao, DO, Loyola University Medical Center

Background: Cardiomyopathy is universal in Duchenne muscular dystrophy (DMD). Previous study reported no correlation of body mass index (BMI) on cardiomyopathy1. Obesity (OB) is a well-established risk for cardiovascular disease (CVD). A recent large study reported that underweight (UW, BMI < 18.5 kg/m) was the strongest risk factor for CVD2. We sought to determine the impact of BMI on non-ambulatory DMD patients on prevalence of late gadolinium enhancement (LGE) and cardiomyopathy by Cardiac MRI (CMR).
Method: A retrospective review of CMRs of DMD patient ? 15 years with LGE and LVEF data. Patients were divided underweight (UW, BMI <18.5 kg/m), normal (NW, BMI ? 18.5 < 24.9 kg/m), overweight (OW, BMI ? 25 < 30 kg/m) and obese (OB, BMI ? 30 kg/m). The prevalence of LGE and LVEF was compared between groups.
Results: 273 DMD patients who met inclusion criteria are detailed in Table 1. There is no difference in age with expect difference in weight and BMI between groups. LV dilation is uncommon but LGE is prevalent in all groups (UW=87.8%, NW=79.8%, OW=94.7% and OB=79.4%). LVEF is lowest in UW compared to both NW and OW group (49.8±11.5 vs 53.8±9.5 vs 54.8±10.3%, p =0.02 and 0.006 respectively) with no difference compared to OB group (52.4±11.9%, p = 0.15).
Conclusion: UW is associated with lower LVEF and more cardiomyopathy compared to NW or OW patients. This study highlights the importance of lower BMI as a potential sign of advance disease. Future longitudinal study is needed to better understand how BMI affects cardiomyopathy.