右室减压术伴改良体肺分流术治疗室间隔完整型肺动脉膜性闭锁

Trealment of membranous pulmonary atresia with intact ventricular septum by right ventricular decompression and modified Blalock-Taussig shunting

目的比较体外循环下行右心室流出道切开后跨肺动脉瓣补片术加改良体肺分流术（术式a）与经右室表面行肺动脉瓣球囊扩张术加改良体肺分流术（术式b）两种术式治疗室间隔完整型肺动脉膜性闭锁的临床效果。方法2003年7月至2014年7月，共收治57例室间隔完整型肺动脉闭锁患儿，从中随机选出22例室间隔完整型肺动脉膜性闭锁患儿，其中11例患儿行术式a（a组），另外11例行术式b（b组），比较两组患儿的病死率、呼吸机辅助时间、CCU住院时间。术后随访6个月，监测出院当日、术后1个月、3个月、6个月的经皮氧饱和度、三尖瓣Z值、McGoon比值、肺动脉跨瓣压差、肺动脉瓣反流率（肺动脉反流束宽度与肺动脉瓣环内径的比值），比较两组患儿上述指标的差异。结果a组患儿共死亡4例，b组患儿共死亡2例。a组患儿术后呼吸机辅助呼吸平均时间为（127．09±46．81）h、CCU平均住院时间为（7．91±2．5）d。b组患儿术后呼吸机辅助呼吸平均时间为（81．55±26．25）h、平均CCU住院时间为（4．82±1.08）d。两组比较，b组时间明显短于a组。出院当日，a组的肺动脉瓣反流率明显低于b组，两组间差异具有统计学意义（P〈0．01）；a、b两组的经皮氧饱和度、三尖瓣Z值、McGoon比值、肺动脉跨瓣压差之间差异无统计学意义。术后1个月，a、b两组患儿的经皮氧饱和度、三尖瓣z值、McGoon比值、肺动脉瓣反流率之间差异无统计学意义；a组患儿肺动脉跨瓣压差低于b组，差异有统计学意义（P〈0．05）。术后3个月，a组患儿经皮氧饱和度明显高于b组，a组患儿肺动脉跨瓣压差明显低于b组，两组间差异具有统计学意义（P〈0．01）；a组患儿三尖瓣z值、McGoon比值均高于b组，两组间差异有统计学意义（P〈0．05）；a、b两组问肺动脉瓣反流率之间差异无统计学意义。术后6个月，a组肺动脉瓣反流率低于b组，两组间差异有统计学意义（P〈0．05）；a、b两组的经皮氧饱和度、三尖瓣z值、McGoon比值、肺动脉跨瓣压差之间差异具有统计学意义（P〈0．01）。结论b组呼吸机辅助时间、CCU住院时间均短于a组，但术后3个月以后随访，a组患儿右心室及肺血管发育优于b组患儿。

Objective To compare the surgical efficacies of membranous pulmonary atresia with intact ventricular septum （PAIVS） by a transannular patch from pulmonary valve after right ventricular outflow-tract cutting with BT shunt under extracorporeal circulation （operation a） and by balloon valvuloplasty via right ventricular pulmonary valve with BT shunt （operation b）. Methods A total of 57 patients with PAIVS were treated between July 2003 and July 2014. And 22 patients with membranous PAIVS were selected randomly. The first 11 patients were surgically treated by operation a （group a） while the remainder by operation b （group b）. Both groups were compared in mortality, breathing machine auxiliary time and time in cardiac care unit （CCU）. The follow up period was up to 6 months. Transcutaneous oxygen saturation, tricuspid Z valve, MeGoon ratio pulmonary arterial pressure gradient and pulmonary valve regurgitation （ratio of width of pulmonary artery to diameter of pulmonary valve ring） were monitored and compared at discharging day, 1 month, 3 and 6 months post-operation. Results Four children died of operation a with a mortality rate of 36. 4%. And another 2 died of operation b with a mortality rate of 18. 2%. No inter-group statistical difference existed in mortality（P〈0. 05）. After operation a, breathing machine auxiliary average time was （127. 09 ± 46. 81） hours and average time in CCU （7. 91 ± 2. 5） days. After operation b, breathing machine auxiliary average time was （1.55± 26. 25） hours and average time in CCU （4. 82 ± 1. 08） days. The time was obviously shorter for operation b than that for operation a. At the day of discharging, pulmonary valve regurgitation was significantly lower in group a than that in group b. Andthe inter-group difference was significant （P〈0. 01）. The values of transcutaneous oxygen saturation, tricuspid Z valve, McGoon ratio and pulmonary arterial pressure had no inter-group statistical difference. At 1 month post-operation, transcutaneous oxygen saturation, tricuspid Z valve and McGoon ratio had no inter-group statistical difference. The pulmonary arterial pressure difference of group a was lower than that of group b. And the inter-group difference had statistical difference（P 〈0. 05）. At 3 months post-operation, transcutaneous oxygen saturation of group a was higher the that of group b. And pulmonary arterial pressure gradient of group a was lower than that of group b. And the inter-group differences had obvious statistical difference （P〈0. 01）. The values of tricuspid Z valve and McGoon ratio of group a were higher than those of group b. And the difference had statistical significance （P 〈 0. 05 ）. Pulmonary valve regurgitation had no inter-group statistical difference. At 6 months, pulmonary valve regurgitation of group a was lower than that of group b. And the inter-group difference had statistical difference （P〈 0. 05）. The values of transcutaneous oxygen saturation, tricuspid Z valve, McGoon ratio and pulmonary arterial pressure gradient had significant inter-group differences （P〈0. 01 ）. Conclusions The mortality was lower for operation b than that for group a. And breathing machine auxiliary time and time in CCU were shorter in group b than those in group a. However, at 3 months, right ventricle and pulmonary vascular growth was better in group a than that in group b.