Nikhanj Anish, Sivakumaran Soori, Miskew-Nichols Bailey, Siddiqi Zaeem A, Oudit Gavin Y
Division of Cardiology, Department of Medicine, University of Alberta, 8440 112 Street NW, Edmonton, Alberta, Canada.
Mazankowski Alberta Heart Institute, University of Alberta, 11220 83 Avenue NW, Edmonton, Alberta, Canada.
Eur Heart J Case Rep. 2019 Jun 1;3(2). doi: 10.1093/ehjcr/ytz095.
Type 1 myotonic dystrophy (DM1) is associated with a variety of cardiac conduction abnormalities and the frequent need for permanent pacing. However, the role of ventricular tachycardia (VT) and the implied risk of sudden cardiac death (SCD) is poorly understood.
This study examined a 56-patient DM1 cohort of men and women, and identified five patients (two females and three males) with ventricular arrhythmias (8.9%). Patients were reviewed on a case-by-case basis, with their clinical presentation and management of VT and the associated cardiomyopathy indicated. Patient cardiac function was determined by 12-lead electrocardiogram, 48-h Holter monitor, and transthoracic echocardiography. These patients were therefore suitable candidates for implantable cardioverter-defibrillator implantation and received these devices; four of the five patients also received cardiac resynchronization therapy. Medical therapies included angiotensin converting enzyme inhibition, mineralocorticoid receptor antagonist, and following device implantation, beta-blocker therapy was initiated.
Our case series demonstrates the prevalence of VT in patients with DM1 highlighting the associated risks of SCD in this patient population. The burden of ventricular arrhythmias, advanced conduction disease, and cardiomyopathy are best treated with a combination of device and medical therapies.
1型强直性肌营养不良症（DM1）与多种心脏传导异常有关，且常常需要永久起搏治疗。然而，室性心动过速（VT）的作用以及由此隐含的心脏性猝死（SCD）风险尚未得到充分了解。
本研究对56例DM1患者（包括男性和女性）进行了队列研究，确定了5例室性心律失常患者（2例女性和3例男性），患病率为8.9%。对这些患者进行了逐例评估，说明了他们的临床表现、VT的治疗及相关的心肌病情况。通过12导联心电图、48小时动态心电图监测和经胸超声心动图来确定患者的心脏功能。因此，这些患者适合植入植入式心脏复律除颤器，并接受了该装置；5例患者中有4例还接受了心脏再同步治疗。药物治疗包括血管紧张素转换酶抑制剂、盐皮质激素受体拮抗剂，在植入装置后，开始使用β受体阻滞剂治疗。
我们的病例系列表明，DM1患者中VT的患病率较高，突出了该患者群体中SCD的相关风险。室性心律失常、严重传导疾病和心肌病的负担最好通过装置和药物治疗相结合的方式来治疗。
