Chen S M, Sun C, Wang X Y, Zhang Y, Liu S W
Department of Cardiology and Institute of Vascular Medicine, Peking University Third Hospital; Key Laboratory of Cardiovascular Molecular Biology and Regulatory Peptides, Ministry of Health; Key Laboratory of Molecular Cardiovascular Science, Ministry of Education; Beijing Key Laboratory of Cardiovascular Receptors Research, Beijing 100191, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2021 Oct 18;53(5):1002-1006. doi: 10.19723/j.issn.1671-167X.2021.05.032.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a kind of inherited cardio-myopathy, which is characterized by fibro-fatty replacement of right ventricular myocardium, leading to ventricular arrhythmia. However, rapid atrial arrhythmias are also common, including atrial fibrillation, atrial flutter and atrial tachycardia. Long term rapid atrial arrhythmia can lead to further deterioration of cardiac function. This case is a 51-year-old male. He was admitted to Department of Cardiology, Peking University Third Hospital with palpitation and fatigue after exercise. Electrocardiogram showed incessant atrial tachycardia. Echocardiography revealed dilation of all his four chambers, especially the right ventricle, with the left ventricular ejection fraction of 40% and the right ventricular hypokinesis. Cardiac magnetic resonance imaging found that the right ventricle was significantly enlarged, and the right ventricular aneurysm had formed; the right ventricular ejection fraction was as low as 8%, and the left ventricular ejection fraction was 35%. The patients met the diagnostic criteria of ARVC, and both left and right ventricles were involved. His physical activities were restricted, and metoprolol, digoxin, spironolactone and ramipril were given. Rivaroxaban was also given because atrial tachycardia could cause left atrial thrombosis and embolism. His atrial tachycardia converted spontaneously to normal sinus rhythm after these treatments. Since the patient had severe right ventricular dysfunction, frequent premature ventricular beats and non-sustained ventricular tachycardia on Holter monitoring, indicating a high risk of sudden death, implantable cardioverter defibrillator (ICD) was implanted. After discharge from hospital, physical activity restriction and the above medicines were continued. As rapid atrial arrhythmia could lead to inappropriate ICD shocks, amiodarone was added to prevent the recurrence of atrial tachycardia, and also control ventricular arrhythmia. After 6 months, echocardiography was repeated and showed that the left ventricle diameter was reduced significantly, and the left ventricular ejection fraction increased to 60%, while the size of right ventricle and right atrium decreased slightly. According to the clinical manifestations and outcomes, he was diagnosed with ARVC associated with arrhythmia induced cardiomyopathy. According to the results of his cardiac magnetic resonance imaging, the patient had left ventricular involvement caused by ARVC, and the persistent atrial tachycardia led to left ventricular systolic dysfunction.
致心律失常性右室心肌病(ARVC)是一种遗传性心肌病,其特征为右心室心肌被纤维脂肪组织替代,进而导致室性心律失常。然而,快速房性心律失常也较为常见,包括心房颤动、心房扑动和房性心动过速。长期快速房性心律失常可导致心功能进一步恶化。该病例为一名51岁男性。他因运动后心悸、乏力入住北京大学第三医院心内科。心电图显示持续性房性心动过速。超声心动图显示其四个心腔均有扩大,尤其是右心室,左心室射血分数为40%,右心室运动减弱。心脏磁共振成像发现右心室明显增大,已形成右心室室壁瘤;右心室射血分数低至8%,左心室射血分数为35%。该患者符合ARVC的诊断标准,左右心室均受累。其体力活动受限,给予美托洛尔、地高辛、螺内酯和雷米普利治疗。因房性心动过速可导致左心房血栓形成和栓塞,故还给予了利伐沙班治疗。经上述治疗后,其房性心动过速自发转为正常窦性心律。由于该患者右心室功能严重受损,动态心电图监测显示频发室性早搏和非持续性室性心动过速,提示猝死风险高,遂植入了植入式心律转复除颤器(ICD)。出院后,继续限制体力活动并服用上述药物。由于快速房性心律失常可能导致ICD不适当放电,加用胺碘酮以预防房性心动过速复发,并控制室性心律失常。6个月后复查超声心动图显示左心室直径明显缩小,左心室射血分数增至60%,而右心室和右心房大小略有减小。根据临床表现和治疗结果,他被诊断为ARVC合并心律失常性心肌病。根据其心脏磁共振成像结果,该患者存在由ARVC导致的左心室受累,持续性房性心动过速导致左心室收缩功能障碍。