Seipelt Ralf G, Vazquez-Jimenez Jaime F, Sachweh Jörg S, Seghaye Marie-Christin, Messmer Bruno J
Department of Thoracic and Cardiovascular Surgery, University Hospital RWTH Aachen, Paulwelsstrasse 30, G-52074 Aachen, Germany.
Eur J Cardiothorac Surg. 2002 Apr;21(4):721-4; discussion 724. doi: 10.1016/s1010-7940(02)00050-7.
The purpose of this study was to evaluate the outcome following palliative reconstruction of right ventricular outflow tract in Tetralogy of Fallot (TOF) with diminutive pulmonary arteries with central and peripheral stenosis.
Between 1986 and 1999 in 15 children with the diagnosis of TOF palliative reconstruction of the right ventricular outflow tract without closure of the ventricular septal defect (VSD) was performed. All patients were not suitable for an AP-Shunt because of a diminutive pulmonary vascular bed. Six patients were younger than 1 year at operation.
There was one hospital death (6.7%) in a child with additional aortic valve insufficiency in multi-organ failure. Although the postoperative course was prolonged (median duration on ICU: 8 days) and complicated by congestive heart failure, clinically the 14 patients discharged improved significantly. The arterial oxygen saturation increased from 67 to 93% (P<0.001), the hemoglobin decreased from 16.1 to 13.3g/l (P=0.02) and hematocrit from 0.52 to 0.40 (P=0.06). In control angiography, the McGoon Index increased in the average from 1.01 to 1.95 (P<0.001). VSD closure was performed in 12 patients (median: 2.5 years after initial operation) with one perioperative death. A homograft had to be implanted in seven patients and a mechanical prosthesis in the right ventricular outflow tract in one. One late death occurred due to ventricular arrhythmia 12 years after antegrade palliation (11 years after corrective operation).
The antegrade palliation seems to be an adequate strategy for the treatment of selected children with diminutive pulmonary arteries in TOF, who were not candidates for primary correction or an AP-Shunt.
本研究旨在评估法洛四联症（TOF）合并细小肺动脉且存在中央和外周狭窄的患者，在进行右心室流出道姑息性重建后的结局。
1986年至1999年间，对15例诊断为TOF的儿童进行了右心室流出道姑息性重建，未闭合室间隔缺损（VSD）。由于肺血管床细小，所有患者均不适合进行体肺分流术。6例患者手术时年龄小于1岁。
1例合并主动脉瓣关闭不全的患儿在多器官功能衰竭中发生医院死亡（6.7%）。尽管术后病程延长（在重症监护病房的中位时间：8天）且并发充血性心力衰竭，但14例出院患者的临床症状有显著改善。动脉血氧饱和度从67%升至93%（P<0.001），血红蛋白从16.1g/l降至13.3g/l（P=0.02），血细胞比容从0.52降至0.40（P=0.06）。在对照血管造影中，麦戈恩指数平均从1.01升至1.95（P<0.001）。12例患者（中位时间：初次手术后2.5年）进行了VSD闭合术，其中1例围手术期死亡。7例患者植入了同种异体移植物，1例患者在右心室流出道植入了机械假体。1例患者在顺行姑息治疗12年后（矫正手术后11年）因室性心律失常发生晚期死亡。
对于TOF合并细小肺动脉且不适合进行一期矫正或体肺分流术的特定患儿，顺行姑息治疗似乎是一种合适的治疗策略。
