经右室肺动脉瓣成形术治疗室间隔完整型肺动脉闭锁的中期随访

目的 回顾经右室肺动脉瓣成形术治疗室间隔完整型肺动脉闭锁的中期随访治疗结果.方法 2010-2013年21例非冠状循环右心室依赖的室间隔完整型肺动脉闭锁患儿在我中心接受经右室肺动脉瓣成形术.平均手术体重(3.2±0.7)kg,平均手术年龄(16±8)d.三尖瓣Z值术前为-2.5～2.2(-0.9±0.4).手术在食管超声引导下,确认肺动脉瓣穿刺打孔位置.全组患儿均随访12个月以上,按治疗结果分为两组:双心室修补组和非双心室修补组.术后3、6、12、24、36、48个月分别随访经皮氧饱和度、三尖瓣Z值和再干预情况.结果 全组1例由于术后早期低氧血症,家属拒绝再手术死亡.12例患儿完成双心室纠治,8例为非双心室纠治组,其中5例完成一个半心室纠治,3例仍在随访中.平均随访时间(32.3±10.3)个月,无晚期死亡.早期4例患儿需要再干预(20％):双心室修补组1例患儿由于肺动脉瓣残余梗阻再次行球囊扩张,3例非双心室修补组由于低氧血症2例行体肺分流术,1例行动脉导管支架植入术(8.3％比37.5％,P＜0.01).术前三尖瓣Z值双心室修补组明显高于非双心室组,均值为-0.5±0.3和-1.5±0.6,(P=0.01).术后30d氧饱和度双心室修补组明显高于非双心室组,均值为88±6和74±7,(P＜0.05);PGE1维持时间双心室修补组明显短于非双心室组,均值为5.6±2.2和7.4±4.2,(P＜0.05).全组6个月、1年、3年免于再手术率分别为80％、70％和35％,两组再手术率均为75％.双心室组中9例患儿接受了房间交通关闭术;非双心室组5例患儿接受了双向腔肺分流术及房间交通关闭术,1例由于右心室流出道梗阻再次手术.三尖瓣Z值在术后3个月、6个月、1年、2年、3年和4年随访值为-2～2.2 (-0.5±0.4)、-2.1～2.2(-0.5 ±0.3)、-2.3～2.0(-0.2±0.3)、-0.4～2.2(0.5±0.8)、-0.5～2.2(0.6±0.8)、-0.2～2.2(0.7±0.3).结论 经右室肺动脉瓣成形术在右心室发育相对较好的室间隔完整型肺动脉闭锁病例治疗效果满意,安全性较高,随访证明可以促进右心室发育,是射频肺动脉瓣打孔成形等其他肺动脉瓣成形术的不错的替代方案.

Mid-term outcomes of transventricular valvotomy approach for initial treatment of pulmonary atresia with intact ventricular septum

Objective To explore the mid-term surgical outcomes of transventricular pulmonary valvotomy (TPV) in patients with pulmonary atresia-intact ventricular septum (PA/IVS) and non-right ven tricular dependant coronary circulation.Methods The clinical data were reviewed retrospectively for 21 patients with PA/IVS and non-right ventricular dependant coronary circulation undergoing TPV from 2010-2013 at our center.The mean body weight was (3.2 ± 0.7) kg and mean age (16 ± 8) days.The Z-score of tricuspid valve ranged from-2.5～2.2(-0.9 ± 0.4).TPV procedure was guided by the Micro-TEE probe for confirming the position of valvular perforation.The follow-up period was over 12 months.They were divided into two groups of Bi-ventricle repair (BVR) and non-BVR.And the parameters of percutaneous saturation,Z-score of tricuspid valve and reintervention profiles were collected at 3,6,12,24,36 and 48 months after procedure.Results One early death occurred postoperatively due to severe hyoxemia and parental refusal of reoperation.And 12 patients finally underwent BVR.Among 8 patients in non-BVR group,5/8 underwent one-and-half ventricle repair while the remainder were still followed up after TPV.The mean follow-up time was (32.3 ± 10.3) (12～60) months without late death.Early reintervention was required in 4 patients (20％).One in BVR group underwent percutaneous pulmonary valve balloon dilation for residual obstruction.And 3 in one-BVR group developed hyoxemia requiring modified B-T shunts (n =2) and patent ductus arteriosus (PDA) stenting (n =1) (8.3％ vs 37.5％,P＜0.01).At Day 30 post-operation,saturation was higher in BVR group than that in non-BVR group.And the mean values were (88 ± 6) and (74 ± 7) (P＜0.05) while PGE1 maintenance time was also shorter in BVR group than that in non-BVR group.And the mean values were (5.6 ± 2.2) and (7.4 ± 4.2) respectively (P＜0.05).Z-score was significantly greater in BVR group than that in non-BVR group.And the mean Z-scores were (-0.5 ± 0.3) and (-1.5-0.6) respectively (P =0.01).The freedom from reoperation was 80％,70％ and 35％ at 6 months,1 and 3 years respectively.And the reoperative rates of two groups were both 75％.In BVR group,9/12 underwent atrial septal defect (ASD) device closure by catheter or surgery.In non-BVR group,5 underwent bidirectional Glenn shunt for ASD closure and 1 had reconstruction of right ventricle outflow for muscular obstruction.The mean Z-score ranged from-2～ 2.2 (-0.5 ±0.4),-2.1～2.2(-0.5 ±0.3),-2.3～2.0(-0.2 ±0.3),-0.4～2.2(0.5 ±0.8),-0.5～2.2 (0.6 ±0.8) and-0.2～2.2(0.7 ± 0.3) at 3 months,6 months,1year,2 years,3 and 4 years post-TPV.Conclusions The outcomes of a hybrid approach are excellent for improving the function of right ventricle in patients with PA/IVS.And TPV may be used alternatively via a transcatheter approach.