Left ventricular function in alpha-sarcoglycanopathy and gamma-sarcoglycanopathy.

Sarcoglycanopathies are autosomic recessive muscular dystrophies, secondary to mutations of the sarcoglycan complex. Heart can be involved in sarcoglycanopathies. We sought to analyse left ventricular function in patients with alpha-sarcoglycanopathy and gamma-sarcoglycanopathy. We conducted a retrospective study that aimed to analyse clinical and echocardiographic data of patients with sarcoglycanopathies. Our study 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). We found a significant alteration of the left ventricular function in gamma-sarcoglycanopathy compared to alpha-sarcoglycanopathy.