Marcus F I, Fontaine G
University of Arizona Health Sciences Center, Tucson, USA.
Pacing Clin Electrophysiol. 1995 Jun;18(6):1298-314. doi: 10.1111/j.1540-8159.1995.tb06971.x.
Right ventricular dysplasia is being recognized with increasing frequency. It should be considered as a cause of ventricular tachycardia of left bundle branch block configuration and/or sudden unexpected death particularly during exercise in young men. The electrocardiogram (ECG) may show anterior precordial T wave inversion, particularly in lead V2 and/or a QRS complex duration > or = 110 ms in the right precordial leads. Echocardiographic studies focusing on the size and wall-motion abnormalities of the right ventricle are useful in confirming the diagnosis. Radionuclide angiography usually shows a moderately or markedly depressed right ventricular ejection fraction with normal or relatively well preserved left ventricular function. Cinemagnetic resonance imaging demonstrates abnormal fatty infiltration of the right ventricular myocardium and can show increased right ventricular dimensions as well as wall-motion abnormalities. Contrast ventricular angiography remains the gold standard to establish the diagnosis but must be performed with appropriate views and with care to avoid ventricular premature beats. Quantitative analysis of right ventricular dimensions can be performed in selected centers. Three-dimensional echocardiography is a promising approach to evaluate right ventricular wall-motion abnormalities as well as to demonstrate enlargement. The etiology and pathogenesis of this condition is not clear. A familial incidence has been well-documented in certain areas and an abnormal gene has been identified. Sporadic cases are the most common. In contrast to Uhl's anomaly, characterized pathologically by areas of paper thin myocardium, the right ventricular free wall is minimally decreased in thickness. Histologically there appears to be a replacement of musculature by fatty tissue. Medical therapy with sotalol or amiodarone, or combination therapy (Class Ic drugs plus beta-blocking drugs, or amiodarone plus beta-blocking drugs) is frequently effective in preventing recurrent ventricular tachycardia. Ablation using radiofrequency (RF) or direct current (DC) energy is reserved for patients who are unresponsive or intolerant of antiarrhythmic drugs. Ventricular arrhythmia recurrence of different morphology is not uncommon after apparent successful ablation. There appears to be a lower rate of successful ablation using RF energy. However, patients with this condition who have been resuscitated from sudden cardiac death or those refractory to medical treatment are candidates for ablation, implantation of an automatic cardioverter defibrillator, or cardiac transplantation. Surgery consisting of total disconnection of the right ventricle is a promising therapeutic modality.
右心室发育不良的诊断频率日益增加。应将其视为导致左束支传导阻滞型室性心动过速和/或猝死的原因，尤其是在年轻男性运动期间。心电图（ECG）可能显示胸前导联T波倒置，特别是在V2导联和/或右胸前导联的QRS波群时限≥110毫秒。关注右心室大小和室壁运动异常的超声心动图检查有助于确诊。放射性核素血管造影通常显示右心室射血分数中度或明显降低，而左心室功能正常或相对保存良好。电影磁共振成像显示右心室心肌有异常脂肪浸润，可显示右心室腔增大以及室壁运动异常。对比心室造影仍是确诊的金标准，但必须采用合适的投照角度并小心操作以避免室性早搏。在特定中心可对右心室大小进行定量分析。三维超声心动图是评估右心室壁运动异常以及显示右心室扩大的一种很有前景的方法。这种疾病的病因和发病机制尚不清楚。在某些地区家族发病率已有充分记录，并且已鉴定出一个异常基因。散发病例最为常见。与病理特征为心肌呈纸样薄区域的乌尔畸形不同，右心室游离壁厚度仅轻度减小。组织学上似乎有肌肉组织被脂肪组织替代。使用索他洛尔或胺碘酮进行药物治疗，或联合治疗（Ic类药物加β受体阻滞剂，或胺碘酮加β受体阻滞剂）通常可有效预防室性心动过速复发。对于对抗心律失常药物无反应或不耐受的患者，可采用射频（RF）或直流电（DC）能量进行消融治疗。在看似成功消融后，不同形态的室性心律失常复发并不少见。使用RF能量成功消融的比率似乎较低。然而，因心脏性猝死复苏成功的患者或对药物治疗难治的患者可考虑进行消融、植入自动心脏复律除颤器或心脏移植。由右心室完全离断组成的手术是一种很有前景的治疗方式。
