Guiraudon G M, Klein G J, Gulamhusein S S, Painvin G A, Del Campo C, Gonzales J C, Ko P T
Circulation. 1983 Feb;67(2):463-70. doi: 10.1161/01.cir.67.2.463.
Arrhythmogenic right ventricular dysplasia is a myopathy that affects the right ventricular free wall (RVFW) and gives rise to recurrent reentrant ventricular tachycardia (VT). Because the entire right ventricle is potentially arrhythmogenic, ablating a single site of VT may not eliminate the arrhythmia. We developed an operation to confine any arrhythmic activity arising from the right ventricle to that chamber: total disconnection of the RVFW from the left ventricle. We performed RVFW disconnection in two patients with refractory VT associated with arrhythmogenic right ventricular dysplasia. At least two sites or origin of morphologically distinct VT were identified in the RVFW in each patient. RVFW disconnection was carried out under normothermic cardiopulmonary bypass. An encircling incision was made along the attachment of the RVFW to the aortoventricular unit and the tricuspid annulus; the right coronary artery and its RVFW branches were left intact. Electrical activity of the two chambers became dissociated, and VT arising from the RVFW was confined to that chamber. Postoperatively, there was no clinical evidence of hemodynamic impairment (follow-up 4 months and 3 months). Left ventricular function was unchanged and right ventricular flow was maintained by atrial contraction and motion of the septum toward the RVFW during left ventricular systole. One patient had incessant right ventricular tachycardia confined to the RVFW for 3 weeks. We conclude that RVFW disconnection is feasible and applicable to patients with refractory VT originating in the diffusely diseased RVFW.
致心律失常性右心室发育不良是一种影响右心室游离壁(RVFW)并引发反复折返性室性心动过速(VT)的心肌病。由于整个右心室都有潜在的致心律失常性,消融单个室性心动过速部位可能无法消除心律失常。我们开发了一种手术,将右心室产生的任何心律失常活动限制在该心腔内:将右心室游离壁与左心室完全断开连接。我们对两名患有与致心律失常性右心室发育不良相关的难治性室性心动过速的患者进行了右心室游离壁断开连接手术。在每名患者的右心室游离壁中至少确定了两个形态学上不同的室性心动过速起源部位。右心室游离壁断开连接手术在常温体外循环下进行。沿着右心室游离壁与主动脉心室单位和三尖瓣环的附着处做一个环形切口;右冠状动脉及其右心室游离壁分支保持完整。两个心腔的电活动分离,右心室游离壁产生的室性心动过速被限制在该心腔内。术后,没有血流动力学损害的临床证据(随访4个月和3个月)。左心室功能未改变,右心室血流通过心房收缩和左心室收缩期间室间隔向右心室游离壁的运动得以维持。一名患者持续3周出现局限于右心室游离壁的右心室心动过速。我们得出结论,右心室游离壁断开连接是可行的,适用于起源于广泛病变的右心室游离壁的难治性室性心动过速患者。