Sarcoglycanopathies are autosomic recessive muscular dystrophies, secondary to mutations of the sarcoglycan complex. Heart can be involved in sarcoglycanopathies. Herein, the authors aimed to analyse left ventricular function in patients with alpha-sarcoglycanopathy and gamma-sarcoglycanopathy. They conducted a retrospective study that aimed to analyse clinical and echocardiographic data of patients with sarcoglycanopathies. They included 19 patients: eight patients with alpha-sarcoglycanopathy and 11 patients with gamma-sarcoglycanopathy. Mean age was 37.8 ± 8.7 years in alpha-sarcoglycanopathy and 36 ± 7.3 years in gamma-sarcoglycanopathy. Mean VC was, respectively, 36.3 ± 18 % in alpha-sarcoglycanopathy and 23.5 ± 6.8 % in gamma-sarcoglycanopathy (p 0.05). 1/8 patients disclosed a left ventricular dysfunction with a left ventricular ejection fraction (LVEF) <50 % in alpha-sarcoglycanopathy, whereas 5/11 patients disclosed a left ventricular dysfunction (LVEF < 50 %) in gamma-sarcoglycanopathy. LV was altered in gamma-sarcoglycanopathy than in alpha-sarcoglycanopathy (LVEF at 45.6 ± 18 vs. 59.6 ± 5.9 % p 0.018). A significant alteration of the left ventricular function in gamma-sarcoglycanopathy compared to alpha-sarcoglycanopathy was observed.
