Al-Ghamdi Bandar, Mallawi Yaseen, Shafquat Azam, AlRuwaili Nadiah, Alhazaymeh Ayman, Al-Manea Waleed, Al-Fayyadh Majid
Heart Centre, King Faisal Specialist Hospital and Research Centre (KFSH&RC), Riyadh, Saudi Arabia.
Alfaisal University, Riyadh, Saudi Arabia.
Cardiol Res. 2018 Aug;9(4):204-214. doi: 10.14740/cr734w. Epub 2018 Aug 10.
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited cardiomyopathy characterized histologically by the replacement of ventricular myocardium with fibrous and fatty tissue, and clinically by ventricular tachycardia arrhythmias primarily of right ventricular (RV) origin. Implantable cardioverter defibrillator (ICD) is the only proven therapy to reduce mortality in ARVC/D patients. However, it has the risk of inappropriate anti-tachycardia pacing (ATP) or shocks. This study aimed to assess the occurrence of appropriate and inappropriate ICD therapies in ARVC/D patients who underwent ICD implantation in a single Cardiac Centre.
Retrospective analysis of the data of patients with the diagnosis of ARVC/D based on the 2010 revised Task Force Criteria, who underwent ICD implantation in the Heart Centre, at King Faisal Specialist Hospital and Research Center (KFSH&RC), Riyadh between January 1997 and May 2016. The clinical data and information about appropriate and inappropriate ICD therapies were obtained from medical records with the review of the available intra-cardiac electrograms (EGMs).
Twenty-two ARVC/D patients with ICD implantation (20 males (91%), mean age at ICD implantation: 32 ± 14 years). ICD was implanted for secondary prevention of sudden cardiac death (SCD) in 15 patients (68.2%), and for primary prevention in 7 patients (31.8%). At mean follow-up of 9.4 ± 4.8 years, 11 patients (50%) had appropriate ICD therapies, and five patients (22.7%) had inappropriate ICD therapies. Out of 950 ICD therapies, 865 (91%) were appropriate (586 episodes of VT/VF treated with ATP (61.3%), and 279 episodes treated with shocks (29.37%)) and 85 (9.4%) were inappropriate (45 episodes treated with ATP (4.73%), and 40 treated with shocks (4.21%)).
ARVC/D patients are at risk of VT/VF arrhythmias. ICD therapy is the only proven life-saving therapy in those patients. Most of ICD therapies in our patient's population are appropriate, and ATP therapy is effective in terminating most of VT episodes. Although we do not have any patient with subcutaneous ICD, the high success rate of ATP suggests that transvenous ICD would be more appropriate in ARVC/D patients.
致心律失常性右室心肌病/发育不良（ARVC/D）是一种遗传性心肌病，组织学特征为心室心肌被纤维和脂肪组织替代，临床特征为主要起源于右心室（RV）的室性心动过速心律失常。植入式心脏复律除颤器（ICD）是唯一经证实可降低ARVC/D患者死亡率的治疗方法。然而，它存在不适当的抗心动过速起搏（ATP）或电击风险。本研究旨在评估在单一心脏中心接受ICD植入的ARVC/D患者中适当和不适当的ICD治疗的发生率。
回顾性分析1997年1月至2016年5月在利雅得法赫德国王专科医院和研究中心（KFSH&RC）心脏中心根据2010年修订的工作组标准诊断为ARVC/D并接受ICD植入的患者数据。通过查阅现有心内电图（EGM），从病历中获取临床数据以及关于适当和不适当ICD治疗的信息。
22例植入ICD的ARVC/D患者（20例男性（91%），ICD植入时平均年龄：32±14岁）。15例患者（68.2%）植入ICD用于心脏性猝死（SCD）的二级预防，7例患者（31.8%）用于一级预防。平均随访9.4±4.8年，11例患者（50%）接受了适当的ICD治疗，5例患者（22.7%）接受了不适当的ICD治疗。在950次ICD治疗中，865次（91%）是适当的（586次室性心动过速/心室颤动发作接受ATP治疗（61.3%），279次发作接受电击治疗（29.37%）），85次（9.4%）是不适当的（45次发作接受ATP治疗（4.73%），40次接受电击治疗（4.21%））。
ARVC/D患者有发生室性心动过速/心室颤动心律失常的风险。ICD治疗是这些患者中唯一经证实的挽救生命的治疗方法。我们患者群体中的大多数ICD治疗是适当的，并且ATP治疗在终止大多数室性心动过速发作方面是有效的。尽管我们没有任何皮下ICD患者，但ATP的高成功率表明经静脉ICD对ARVC/D患者可能更合适。
