Evaluation of heart involvement in calpainopathy (LGMD2A) using cardiovascular magnetic resonance

ABSTRACT Introduction Cardiac dysfunction occurs in several forms of limb girdle muscular dystrophy (LGMD). The aim of this study was to investigate cardiac involvement in calpainopathy (LGMD2A). Methods Cardiovascular evaluation was performed in 10 patients with genetically verified LGMD2A by echocardiography, 3 Tesla ‐ cardiovascular magnetic resonance, 24‐h electrocardiography recordings with heart rate variability (HRV) analysis, and 24‐h blood pressure recordings. Results No patient with calpainopathy showed impairment of left or right ventricular function. One patient had a small amount (2% of left ventricle mass) of late gadolinium enhancement. HRV analysis revealed no significant difference compared with external reference data. Conclusions The main finding of this study is the lack of cardiac involvement in patients with calpainopathy. Cardiac involvement was not found, even in individuals with advanced age and greater disease severity. Furthermore, we did not observe an overall reduction of cardiac autonomic regulation in calpainopathy. Muscle Nerve 52: 661–663, 2015