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Evaluation of heart involvement in calpainopathy (LGMD2A) using cardiovascular magnetic resonance
Author(s) -
Quick Silvio,
Schaefer Jochen,
Waessnig Nadine,
Schultheiss Thorsten,
Reuner Ulrike,
Schoen Steffen,
Reichmann Heinz,
Strasser Ruth,
Speiser Uwe
Publication year - 2015
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.24717
Subject(s) - cardiology , medicine , ventricle , magnetic resonance imaging , electrocardiography , heart rate , cardiac function curve , heart rate variability , blood pressure , radiology , heart failure
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