Spadotto Alberto, Morabito Domenico, Carecci Alessandro, Massaro Giulia, Statuto Giovanni, Angeletti Andrea, Graziosi Maddalena, Biagini Elena, Martignani Cristian, Ziacchi Matteo, Diemberger Igor, Biffi Mauro
IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, Italy.
Department of Experimental, Diagnostic and Specialty Medicine, 40138 Bologna, Italy.
Rev Cardiovasc Med. 2022 Aug 15;23(8):283. doi: 10.31083/j.rcm2308283. eCollection 2022 Aug.
we sought to review the evolution in the diagnosis and treatment of Arrhythmogenic Cardiomyopathy (ACM), a clinically multifaceted entity beyond the observation of ventricular arrhythmias, and the outcome of therapies aiming at sudden death prevention in a single center experience.
retrospective analysis of the data of consecutive patients with an implanted cardioverter-defibrillator (ICD) and a confirmed diagnosis of ACM according to the proposed Padua Criteria, who were referred to our center from January 1992 to October 2021.
we enrolled 72 patients (66% males, mean age at implant 46 16 years), 63.9% implanted for primary prevention. At the time of ICD implant, 29 (40.3%) patients had a right ventricular involvement, 24 (33.3%) had a dominant LV involvement and 19 (26.4%) had a biventricular involvement. After a median follow-up of 6,1 years [IQR: 2.5-9.9], 34 patients (47.2%) had 919 sustained episodes of ventricular arrhythmias (VA). 27 patients (37.5%) had 314 episodes of life-threatening arrhythmias (LT-VA), defined as sustained ventricular tachycardia 200 beats/min. Considering only the patients with an ICD capable of delivering ATP, 80.4% of VA and 65% of LT-VA were successfully terminated with ATP. 16 (22.2%) patients had an inappropriate ICD activation, mostly caused by atrial fibrillation, while in 9 patients (12.5%) there was a complication needing reintervention (in 3 cases there was a loss of ventricular sensing dictating lead revision). During the follow-up 11 (15.3%) patients died, most of them due to heart failure, and 8 (11.1%) underwent heart transplantation.
ACM is increasingly diagnosed owing to heightened suspicion at ECG examination and to improved imaging technology and availability, though the diagnostic workflow is particularly challenging in the earliest disease stages. ICD therapy is the cornerstone of sudden death prevention, albeit its efficacy is not based on controlled studies, and VT ablation/medical therapy are complementary to this strategy. The high burden of ATP-terminated VA makes shock-only devices debatable. The progressive nature of ACM leads to severe biventricular enlargement and refractory heart failure, which pose significant treatment issues when a predominant RV dysfunction occurs owing to the reduced possibility for mechanical circulatory assistance.
我们试图回顾致心律失常性心肌病（ACM）的诊断和治疗进展，ACM是一种临床多层面的疾病，不仅仅是观察室性心律失常，同时回顾在单一中心经验中旨在预防猝死的治疗结果。
对1992年1月至2021年10月转诊至我们中心的连续植入心脏复律除颤器（ICD）且根据拟议的帕多瓦标准确诊为ACM的患者数据进行回顾性分析。
我们纳入了72例患者（66%为男性，植入时平均年龄46±16岁），63.9%为一级预防植入。在植入ICD时，29例（40.3%）患者右心室受累，24例（33.3%）左心室为主受累，19例（26.4%）双心室受累。中位随访6.1年[四分位间距：2.5 - 9.9]后，34例患者（47.2%）发生919次持续性室性心律失常（VA）。27例患者（37.5%）发生314次危及生命的心律失常（LT - VA），定义为持续性室性心动过速≥200次/分钟。仅考虑能够进行抗心动过速起搏（ATP）的ICD患者，80.4%的VA和65%的LT - VA通过ATP成功终止。16例（22.2%）患者发生不适当的ICD激活，主要由心房颤动引起，而9例患者（12.5%）出现需要再次干预的并发症（3例因心室感知丧失需要更换导线）。随访期间11例（15.3%）患者死亡，大多数死于心力衰竭，8例（11.1%）接受了心脏移植。
由于心电图检查时怀疑度增加以及成像技术和可及性的改善，ACM的诊断越来越多，尽管在疾病最早阶段诊断流程极具挑战性。ICD治疗是预防猝死的基石，尽管其疗效并非基于对照研究，室性心动过速消融/药物治疗是该策略的补充。ATP终止的VA负担较高使得仅具备电击功能的设备存在争议。ACM的进行性导致严重双心室扩大和难治性心力衰竭，当主要为右心室功能障碍时，由于机械循环辅助可能性降低，会带来重大治疗问题。
