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目的评价右室流出道限制性疏通术在法洛四联症伴肺血管发育不良患者的分期矫治术中的应用价值。方法 14例重症法洛四联症患者施行右室流出道限制性疏通术,2例合并双重血供的大的体肺侧支血管,于术前行介入封堵;于体外循环下结扎动脉导管9例,术中切除流出道肥厚肌肉束,使用牛心包补片或自体心包加宽主肺动脉、肺动脉瓣环及右室流出道,加宽后其内径为正常值的1/2~2/3。结果术后入ICU时吸入氧浓度30%时血氧饱和度(Sa O2)平均(79±4)%,相比术前(57±9),提高了22.2%(P<0.01)。术后气管内插管时间(63±42)h,术后死亡1例,系合并无脾综合征患者,于术后第2天出现肺水肿,死于低氧血症。其余患者均顺利出院。所有病例均随访。随访期间所有病例未发生急性心血管意外。8例于术后6~18个月接受二次根治手术。二次手术前均行心血管CT检查,肺血管指数较第一次手术前明显增加。Mc Goon比为(2.15±0.28),Nakata指数为(260.5±80.3)mm2/m2,差异具有统计学意义(P<0.05)。结论右室流出道限制性疏通术是一种较为理想的姑息性手术方式,但仅能够应用于存在固有主肺动脉且右室流出道表面无大的冠状动脉的病例,应用有一定局限性。
Objective To evaluate the value of restrictive drainage of right ventricular outflow tract in staging of patients with tetralogy of Fallot with pulmonary vascular dysplasia. Methods A total of 14 patients with severe tetralogy of Fallot were subjected to restrictive drainage of the right ventricular outflow tract. Two large pulmonary collateral vessels with dual blood supply were involved and occluded preoperatively. Ligation of the ductus arteriosus under cardiopulmonary bypass Nine patients underwent resection of the hypertrophied outflow tract muscle bundle, the use of bovine pericardial patch or pericardial widening of the main pulmonary artery, pulmonary valve annulus and right ventricular outflow tract, after widening the inner diameter of the normal value of 1/2 ~ 2/3 . Results The oxygen saturation (Sa O2) was (79 ± 4)% at the time of 30% oxygen inhalation, and increased by 22.2% (P <0.01) compared with preoperative (57 ± 9). Postoperative intratracheal intubation time (63 ± 42) h, postoperative death in 1 case, the Department of patients without splenic syndrome, pulmonary edema on the second postoperative day, died of hypoxemia. The remaining patients were discharged smoothly. All cases were followed up. All cases did not have an acute cardiovascular accident during follow-up. Eight patients underwent 6 to 18 months postoperative radical surgery. Cardiovascular CT examination was performed before the second operation, and the pulmonary vascular index was significantly increased compared with that before the first operation. Mc Goon ratio was (2.15 ± 0.28), Nakata index was (260.5 ± 80.3) mm2 / m2, the difference was statistically significant (P <0.05). Conclusion Restrictive drainage of right ventricular outflow tract is an ideal palliative surgery. However, it can only be applied in the cases where there is no major coronary artery in the right main ventricular outflow tract with intrinsic main pulmonary artery. There are some limitations in its application.