Carrel T, Pfammatter J P
Clinic for Cardiovascular Surgery, University Hospital Berne, Switzerland.
Thorac Cardiovasc Surg. 2000 Aug;48(4):224-7. doi: 10.1055/s-2000-6894.
One of the most serious late complications of the intraatrial baffle procedure (Mustard or Senning correction) in patients suffering from transposition of the great arteries, (TGA) is the late systemic right ventricular failure. Nearly all patients presenting with right ventricular dysfunction have severe associated tricuspid regurgitation. The surgical options for these patients include tricuspid valve reconstruction or replacement, staged conversion to the arterial switch operation and orthotopic heart transplantation. Review of 189 operative survivors who underwent the Mustard or Senning operation for TGA between 1970 and 1993 in our institution revealed 12 patients (6.3%) who died from severe systemic right ventricular dysfunction (mean follow-up 16+/-3.5 years), which was the most common cause of late death. All of them had concomitant severe tricuspid regurgitation. 7 patients (3.7%) died from sudden cardiac death. The actuarial survival at 10 years is 93% for simple TGA and 85% for TGA associated with ventricular septum defect or coarctation. At our institution, 4 adolescent or adult patients underwent tricuspid valve surgery; tricuspid valve replacement was performed in 2 patients and valve repair in 2 patients. In the mid-term follow-up, 2 of these patients died. Two additional patients underwent orthotopic heart transplantation, and one died on the waiting list. Staged conversion from the Senning/Mustard atrial repair to the arterial switch operation was initially reported by Mee. The procedure for pulmonary artery banding starts with inducing left ventricular reconditioning with subsequent arterial switch. The mortality of this two-staged procedure was as high as 20% to 30% in our early experience, and some of the candidates underwent heart transplantation. Tricuspid valve repair or replacement do not improve right ventricular function in patients with a failing right ventricle following the Mustard/Senning operation. Staged conversion to arterial switch may improve right ventricular function by decreasing the work load of the right ventricle and provides anatomic repair with left ventricle-to-aorta continuity. Orthotopic heart transplantation is the only alternative if the left ventricle does not respond to pulmonary artery banding.
大动脉转位（TGA）患者行心房内挡板手术（Mustard或Senning矫正术）最严重的晚期并发症之一是晚期体循环右心室衰竭。几乎所有出现右心室功能障碍的患者都伴有严重的三尖瓣反流。这些患者的手术选择包括三尖瓣重建或置换、分期转换为动脉调转手术和原位心脏移植。回顾1970年至1993年间在我院接受Mustard或Senning手术治疗TGA的189例手术幸存者，发现12例（6.3%）死于严重的体循环右心室功能障碍（平均随访16±3.5年），这是晚期死亡的最常见原因。他们均伴有严重的三尖瓣反流。7例（3.7%）死于心源性猝死。单纯TGA患者10年实际生存率为93%，合并室间隔缺损或主动脉缩窄的TGA患者为85%。在我院，4例青少年或成年患者接受了三尖瓣手术；2例行三尖瓣置换术，2例行瓣膜修复术。在中期随访中，这些患者中有2例死亡。另外2例患者接受了原位心脏移植，1例在等待名单上死亡。Mee最初报道了从Senning/Mustard心房修复分期转换为动脉调转手术。肺动脉环扎术的步骤是先诱导左心室重塑，随后进行动脉调转。在我们早期的经验中，这种两阶段手术的死亡率高达20%至30%，一些患者接受了心脏移植。在Mustard/Senning手术后右心室功能衰竭的患者中，三尖瓣修复或置换并不能改善右心室功能。分期转换为动脉调转可能通过减轻右心室的工作负荷来改善右心室功能，并提供左心室与主动脉连续性的解剖修复。如果左心室对肺动脉环扎无反应，原位心脏移植是唯一的选择。
