Rago Anna, Papa Andrea Antonio, Arena Giulia, Mosella Marco, Cassese Antonio, Palladino Alberto, Golino Paolo
Department of Cardiology, University of Campania "L. Vanvitelli", Monaldi Hospital, Naples, Italy.
Cardiomyology and Medical Genetics, University Hospital "L. Vanvitelli", Naples, Italy.
Acta Myol. 2017 Dec 1;36(4):218-222. eCollection 2017 Dec.
Myotonic Dystrophy type 1 (DM1) is the most common muscular dystrophy in adult life characterized by muscle dysfunction and cardiac conduction abnormalities. Atrial fibrillation frequently occurs in DM1 patients. It's related to the discontinuous and inhomogeneous propagation of sinus impulses and to the prolongation of atrial conduction time, caused by progressive fibrosis and fatty replacement of the myocardium. AF predisposes to a hyper-coagulable state and to an increased risk of thromboembolism. We report the first case of complete resolution of left atrial appendage thrombosis with oral dabigatran etexilate in a myotonic dystrophy type I patient with atrial fibrillation scheduled for transesophageal echocardiogram-guided direct current cardioversion.
1型强直性肌营养不良(DM1)是成人中最常见的肌营养不良症,其特征为肌肉功能障碍和心脏传导异常。心房颤动在DM1患者中经常发生。它与窦性冲动的不连续和不均匀传播以及心房传导时间延长有关,这是由心肌的进行性纤维化和脂肪替代引起的。房颤易导致高凝状态和血栓栓塞风险增加。我们报告了首例使用口服达比加群酯使1型强直性肌营养不良合并心房颤动患者左心耳血栓完全溶解的病例,该患者计划接受经食管超声心动图引导下的直流电复律。