Cardiac Arrhythmias in Muscular Dystrophies Associated with Emerinopathy and Laminopathy: A Cohort Study.

INTRODUCTION

Cardiac involvement in patients with muscular dystrophy associated with Lamin A/C mutations () is characterized by atrioventricular conduction abnormalities and life-threatening cardiac arrhythmias. Little is known about cardiac involvement in patients with emerin mutation (). The aim of our study was to describe and compare the prevalence and time distribution of cardiac arrhythmias at extended follow-up.

PATIENTS AND METHODS

45 consecutive patients affected by muscular dystrophy associated to laminopathy or emerinopathy were examined. All patients underwent clinical evaluation, 12-lead surface electrocardiogram (ECG), 24 h electrocardiographic monitoring, and cardiac implanted device interrogation.

RESULTS

At the end of 11 (5.0-16.6) years of follow-up, 89% of the patients showed cardiac arrhythmias. The most prevalent was atrial standstill (AS) (31%), followed by atrial fibrillation/flutter (AF/Afl) (29%) and ventricular tachycardia (22%). patients presented more frequently AF/AFl compared to (50% vs. 20%, ). Half of the patients presented with AS, whilst there was no occurrence of such in the ( = 0.001). Ventricular arrhythmias were found in 60% of patients with laminopathy compared to 3% in patients with emerinopathy ( < 0.001). The age of AVB occurrence was higher in the group (32.8 +/- 10.6 vs. 25.1 +/- 9.1, = 0.03).

CONCLUSIONS

Atrial arrhythmias are common findings in patients with muscular dystrophy associated with / mutations; however, they occurred earlier in patients. Ventricular arrhythmias were very common (60%) in and occurred definitely earlier compared to the group.