CAJ | 학술논문

目的：分析肺动脉瓣直视切开术治疗右心室轻、中度发育不良室间隔完整肺动脉闭锁（PA/IVS）的中期疗效。方法回顾性分析我院自1995年1月至2013年10月收治的15例肺动脉瓣直视切开术治疗PA/IVS患儿临床资料。手术时中位年龄4个月（7 d至32个月），其中女8例，男7例；术前根据超声检测三尖瓣Z值以判断右心室发育程度，患儿三尖瓣Z值皆＞－4，即右心室轻、中度发育不良。其中12例施行肺动脉瓣切开，3例肺动脉瓣切开和体-肺动脉分流术。结果手术后死亡1例（死亡率为6.7%），患儿血氧饱和度明显改善（0.82±0.04vs.0.91±0.05，P＜0.01），右心室压力与体循环压力比值明显下降（1.85±0.14 vs.0.78±0.15，P＜0.01）。平均随访5.2年（1～8年），死亡1例。其中9例患儿施行二期手术（7例两心室修复，2例双向腔肺动脉分流术），无手术死亡，近期随访满意。术后6个月、1年、2年和3年的二次手术免除率分别为92.9%、85.7%、62.9%和39.3%。所有患儿心功能NYHA分级Ⅰ级。结论对于PA/IVS患儿的外科治疗，依据术前三尖瓣Z值，选取右心室轻、中度发育不全患儿，应用肺动脉瓣直视切开术，可获得满意的疗效。
목적：분석폐동맥판직시절개술치료우심실경、중도발육불량실간격완정폐동맥폐쇄（PA/IVS）적중기료효。방법회고성분석아원자1995년1월지2013년10월수치적15례폐동맥판직시절개술치료PA/IVS환인림상자료。수술시중위년령4개월（7 d지32개월），기중녀8례，남7례；술전근거초성검측삼첨판Z치이판단우심실발육정도，환인삼첨판Z치개＞－4，즉우심실경、중도발육불량。기중12례시행폐동맥판절개，3례폐동맥판절개화체-폐동맥분류술。결과수술후사망1례（사망솔위6.7%），환인혈양포화도명현개선（0.82±0.04vs.0.91±0.05，P＜0.01），우심실압력여체순배압력비치명현하강（1.85±0.14 vs.0.78±0.15，P＜0.01）。평균수방5.2년（1～8년），사망1례。기중9례환인시행이기수술（7례량심실수복，2례쌍향강폐동맥분류술），무수술사망，근기수방만의。술후6개월、1년、2년화3년적이차수술면제솔분별위92.9%、85.7%、62.9%화39.3%。소유환인심공능NYHA분급Ⅰ급。결론대우PA/IVS환인적외과치료，의거술전삼첨판Z치，선취우심실경、중도발육불전환인，응용폐동맥판직시절개술，가획득만의적료효。
ObjectiveTo evaluate the mid-term outcome of pulmonary valvotomy for pulmonary atresia with intact ventricular septum (PA/IVS) with mild and moderate dysplasia of the right ventricle.Methods A retrospective analysis of 15 cases of pulmonary valvotomy in our hospital from January 1995 to October 2013. The median age was 4 months (7 days-32 months), 8 cases were female, 7 cases were male. TheZ value of tricuspid valve was detected preoperatively, and theZ value of all the patients were larger than-4. Twelve patients underwent pulmonary valvotomy, while pulmonary valvotomy plus systemic pulmonary shunt for 3 cases.Results One patient (6.7%) died in hospital. The blood oxygen saturation was significantly improved (0.82±0.04vs. 0.91±0.05,P<0.01), and the ratio of right ventricular pressure and body circulation pressure decreased significantly (1.85±0.14vs. 0.78±0.15, P<0.01). Mean follow-up time of 5.2 years (1-8 years), one patient died. Nine cases performed second stage surgery (7 cases of biventricular repair, 2 cases of bidirectional cavopulmonary shunt). There was no operative death, the rates of freedom from reoperation at the 6th month, 1st year, 2nd year and 3rd year postoperative were 92.9%, 85.7%, 62.9% and 39.3% respectively. The New York Heart Association (NYHA) classification of all patients heart function was classⅠ.Conclusion The application of pulmonary valvotomy for PA/IVS patients with mild and moderate dysplasia of the right ventricle can obtain satisfactory outcome.