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ABSTRACT: Introduction
Little is known about the frequency of cardiopulmonary failure in limb-girdle muscular dystrophy type 2A (calpainopathy) patients, although some studies have reported severe cardiomyopathy or respiratory failure.Methods
To clarify the frequency of cardiopulmonary dysfunction in this patient population, we retrospectively reviewed the respiratory and cardiac function of 43 patients with calpainopathy.Results
Nine of the 43 patients had forced vital capacity (FVC) < 80%, and 3 used noninvasive positive pressure ventilation. Mean FVC was significantly lower in patients who were nonambulant and had normal creatine kinase levels. Only 1 patient had a prolonged QRS complex duration. Echocardiography revealed that 1 patient had very mild left ventricular dysfunction.Conclusions
These findings suggest that patients with calpainopathy may develop severe respiratory failure, but cardiac dysfunction is infrequent. Muscle Nerve 55: 465-469, 2017.
SUBMITTER: Mori-Yoshimura M
PROVIDER: S-EPMC5396288 | biostudies-literature | 2017 Apr
REPOSITORIES: biostudies-literature
Mori-Yoshimura Madoka M Segawa Kazuhiko K Minami Narihiro N Oya Yasushi Y Komaki Hirohumi H Nonaka Ikuya I Nishino Ichizo I Murata Miho M
Muscle & nerve 20161230 4
<h4>Introduction</h4>Little is known about the frequency of cardiopulmonary failure in limb-girdle muscular dystrophy type 2A (calpainopathy) patients, although some studies have reported severe cardiomyopathy or respiratory failure.<h4>Methods</h4>To clarify the frequency of cardiopulmonary dysfunction in this patient population, we retrospectively reviewed the respiratory and cardiac function of 43 patients with calpainopathy.<h4>Results</h4>Nine of the 43 patients had forced vital capacity (F ...[more]