Update on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C).
Update on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C).
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DOI：
10.1007/s11936-013-0251-8
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发表时间：
2013-08-01
影响因子：
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通讯作者：
Calkins, Hugh
中科院分区：
文献类型：
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作者：
James, Cynthia A;Calkins, Hugh
意见声明：致心律失常性右心室发育不良/心肌病（ARVD/C）是一种遗传性心肌病，其特征是危及生命的室性心律失常和缓慢进行性心室功能障碍。对ARVD/C家族的治疗旨在预防心源性猝死，预防或延缓疾病进展，并适应终身慢性遗传性疾病的生活。由于尚无ARVD/C治疗或筛查方案的随机试验，因此治疗决策基于临床专业知识、基于回顾性登记研究的结果以及对疾病病理机制的理解。治疗的第一步是通过仔细应用诊断工作组标准来确保准确的诊断。在索引病例诊断后，对家庭成员进行筛查。我们建议受影响的个人和基因携带者大大减少运动，并强烈建议ARVD/C患者停止竞技运动。我们通常建议对所有符合工作组标准的先证者放置植入式心律转复除颤器（ICD），特别是如果他们有持续性室性心动过速（VT）、致心律失常性晕厥或频繁的心室异位和/或非持续性VT病史。对于通过家庭筛查确诊为ARVD/C的家庭成员，我们更谨慎地建议植入ICD，因为这些人现在在疾病的早期阶段就被识别出来，而不是以前。抗心律失常药物和导管消融用于降低室性心动过速的频率，并使用相关的植入式心律转复除颤器治疗。如果心内膜消融失败或作为初始心内膜/心外膜联合消融策略的一部分，则提供心外膜消融，具体取决于患者的偏好。我们尝试启动β受体阻滞剂治疗和血管紧张素转换酶抑制剂治疗，以限制结构性进展和预防室性心律失常。未受影响的家庭成员从青春期开始通过非侵入性心脏检查定期筛查疾病发作。通过持续的遗传咨询、基于网络的患者-家庭支持和家庭面对面研讨会，向家庭提供支持。
OPINION STATEMENT: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterized by life-threatening ventricular arrhythmias and slowly progressive ventricular dysfunction. Treatment for families with ARVD/C is directed toward prevention of sudden cardiac death, prevention or delay of disease progression, and adjustment to life with a lifelong chronic genetic disease. As there have been no randomized trials of ARVD/C treatments or screening regimens, treatment decisions are based on clinical expertise, results of retrospective registry-based studies, and understanding of pathologic mechanisms of disease. The first step for treatment is securing an accurate diagnosis through careful application of the diagnostic Task Force criteria. Following diagnosis in an index case, family members are screened. We advise affected individuals and gene carriers to dramatically reduce exercise and strongly recommend that ARVD/C patients discontinue competitive athletics. We generally recommend implantable cardioverter defibrillator (ICD) placement in all probands who meet Task Force criteria, especially if they have a history of sustained ventricular tachycardia (VT), arrhythmogenic syncope, or frequent ventricular ectopy and/or nonsustained VT. We are more circumspect about recommending implantation of an ICD in a family member who has been diagnosed with ARVD/C through family screening, as these individuals are now being identified at a much earlier stage in their disease than was possible previously. Anti-arrhythmic medications and catheter ablation are used to decrease the frequency of ventricular tachycardia and associated implantable cardioverter defibrillator therapies. Epicardial ablation is offered if endocardial ablation fails or as part of an initial combined endocaridal/epicardial ablation strategy, depending on patient preference. We attempt to initiate beta-blocker therapy and therapy with angiotensin-converting enzyme inhibitors to limit structural progression and prevent ventricular arrhythmias. Unaffected family members are screened for disease onset regularly from adolescence through noninvasive cardiac testing. Support to families is provided through ongoing genetic counseling, patient-family web-based support, and in-person seminars for families.