Nimkhedkar K, Hilton C J, Furniss S S, Bourke J P, Glenville B, McComb J M, Campbell R W
Regional Cardiothoracic Centre, Freeman Hospital, Newcastle Upon Tyne, England.
J Am Coll Cardiol. 1992 Apr;19(5):1079-84. doi: 10.1016/0735-1097(92)90299-3.
Ten patients (nine men, one woman; mean age 39 years) with arrhythmogenic right ventricular dysplasia underwent surgery to control life-threatening drug refractory ventricular arrhythmias. All had ventricular tachycardia causing syncope and six had a history of cardiac arrest. In all a minimum of three antiarrhythmic drugs (mean five) had been ineffective. At operation, the right ventricle was grossly diseased in all patients. Ventricular tachycardias were induced and mapped intraoperatively in all patients. The surgical plan was to ablate the arrhythmogenic focus if it was less than 4 cm2; one patient was so managed. Of the remaining nine, four underwent partial (approximately 40% of the right ventricular free wall) and five underwent total right ventricular disarticulation. All survived the operation and are alive at a mean follow-up interval of 24 months (range 5 to 67). Two patients developed new sustained ventricular tachycardias. These were well tolerated and, unlike the original arrhythmias, were easily controlled by drug treatment. All patients who underwent right ventricular disarticulation manifested signs of right heart failure in the early postoperative period, but these lessened progressively with the development of systolic septal movement into the right ventricular cavity. All 10 patients are in New York Heart Association class I or II at last review. In selected patients with arrhythmogenic right ventricular dysplasia, surgery offers a curative treatment for ventricular tachycardia and should be considered for patients whose arrhythmias are life-threatening and refractory to drug treatment.
10例致心律失常性右室发育不良患者（9例男性，1例女性；平均年龄39岁）接受了手术，以控制危及生命的药物难治性室性心律失常。所有患者均有导致晕厥的室性心动过速，6例有心脏骤停史。所有患者至少使用三种抗心律失常药物（平均五种）均无效。手术时，所有患者的右心室均有严重病变。所有患者术中均诱发并标测了室性心动过速。如果致心律失常灶面积小于4平方厘米，则手术方案是消融该病灶；1例患者采用了这种治疗方法。其余9例中，4例行部分右心室游离壁切除术（约占右心室游离壁的40%），5例行全右心室离断术。所有患者均存活，平均随访24个月（5至67个月）。2例患者出现了新的持续性室性心动过速。这些易于耐受，与原来的心律失常不同，药物治疗很容易控制。所有接受右心室离断术的患者术后早期均出现右心衰竭体征，但随着收缩期室间隔向右心室腔内移动，这些体征逐渐减轻。最后一次复查时，所有10例患者的纽约心脏协会心功能分级均为I级或II级。对于选定的致心律失常性右室发育不良患者，手术为室性心动过速提供了一种治愈性治疗方法，对于心律失常危及生命且药物治疗无效的患者应考虑手术治疗。
