Fontaine G, Guedon-Moreau L, Frank R, Lascault G, Fontaliran F, Tonet J, Himbert C, Grosgogeat Y
Service de Rythmologie et de Stimulation Cardiaque, Hôpital Jean Rostand, Ivry.
Ann Cardiol Angeiol (Paris). 1992 Sep;41(7):399-410.
Arrhythmogenic right ventricular dysplasia is responsible for ventricular tachycardia affecting an apparently healthy heart. It can sometimes lead to sudden death, which may be the presenting symptomatology of the disease. It results from fibro-adipose infiltration of the free wall of the right ventricle, and sometimes of the septum, possibly secondary to myocarditis. The prognosis depends upon the quality of the left ventricle. If it is healthy, the only risk is that of arrhythmia. Treatment using anti-arrhythmic drugs is most often effective and, with proper management, the prognosis is good and the risk of sudden death eliminated. If the left ventricle is abnormal, there is the risk that dysplasia associated with arrhythmia will progress to right then congestive cardiac failure in the context of a dilated idiopathic cardiomyopathy with ventricular tachycardias originating on the right side. Arrhythmogenic right ventricular dysplasia is a notable cause of sudden death in athletes. Routine screening of such individuals is justified, as is that of those with high risk occupations (locomotive and vehicle drivers, etc.).
致心律失常性右心室发育不良可导致影响看似健康心脏的室性心动过速。它有时会导致猝死,而猝死可能是该疾病的首发症状。它是由右心室游离壁,有时还有室间隔的纤维脂肪浸润引起的,可能继发于心肌炎。预后取决于左心室的状况。如果左心室健康,唯一的风险就是心律失常。使用抗心律失常药物进行治疗通常有效,并且通过适当的管理,预后良好,猝死风险消除。如果左心室异常,在伴有起源于右侧的室性心动过速的扩张型特发性心肌病的情况下,与心律失常相关的发育不良有进展为右心衰竭继而充血性心力衰竭的风险。致心律失常性右心室发育不良是运动员猝死的一个显著原因。对这类人群以及从事高风险职业的人群(机车和车辆驾驶员等)进行常规筛查是合理的。