肺动脉发育不良的肺动脉闭锁合并室间隔缺损的外科治疗

发布时间：2018-04-24 17:09

本文选题：肺动脉闭锁合并室间隔缺损 + 肺动脉发育不良　；参考：《北京协和医学院》2014年博士论文

【摘要】：目的：比较常见体肺分流手术对于肺血管发育不良肺动脉闭锁室间隔缺损病人的安全性和有效性。方法：回顾分析2009年12月至2012年8月间连续47例在我院行体肺分流手术治疗合并肺动脉发育不良的肺动脉闭锁/室间隔缺损病例(平均年龄1.78±1.82岁,0.2-8.1; Nakata指数：86.62±45.02；McGoon比值：1.03±0.40),其中改良blalock-taussing shunt (B-T分流术)28例(59.6%),中央分流19例(40.4%),主要依据术者习惯和术前肺血管发育情况决定手术方式,采用中央分流病人的肺动脉发育指数明显小于改良B-T分流组(P0.01),部分病人同期或者分期行体肺侧枝封堵术,平均随访时间2.36±1.07年。结果：姑息术后两组病人肺血管均得到了明显生长(Nakata指数：207.94±101.47,McGoon比值1.55±0.44)两组间未见明显统计数差异(P0.05),中央分流组姑息术后呼吸机使用时间和ICU滞留时间均较B-T分流组明显延长(P0.01)。15例病人(31.9%)在姑息术后平均1.1年(0.6-1.6年)后获得了解剖学根治,且均为改良B-T分流术后病人,根治术后无早期死亡,随访无远期死亡,所有病人心功能良好(NYHA Ⅰ-Ⅱ)。结论：体肺分流虽然能够有效的促进PA-VSD病人发育不良的固有肺动脉发发育,但最终仅能帮助约1/3的病人获得双心室矫治,根治率较低,中央分流手术近远期结果略低于改良B-T分流组,可能与术前病人肺血管发育情况较差有关。目的：评价姑息性右心室肺动脉连接手术对于肺血管发育不良肺动脉闭锁室间隔缺损病人的安全性和有效性。方法：回顾分析2009年12月至2012年8月间连续45例在我院行姑息性右心室肺动脉连接术治疗合并肺动脉发育不良的肺动脉闭锁/室间隔缺损病例(平均年龄1.61±1.63岁,0.2-7.2；Nakata指数：88.25±29.27；McGoon比值：0.94±0.14),部分病人同期行侧枝封堵或者肺血管成型术,平均随访时间2.06±0.83年。结果：姑息术后无早期死亡,随访中因心肌炎死亡1例；所有病人肺血管均得到了明显生长(Nakata指数：215.61±107.79；McGoon比值1.74±0.56,PO.001),其中发生分支肺动脉狭窄2例,右心室流出道狭窄1例,均通过经胸介入治疗获得矫治。28例病人(62.2%)在姑息术后平均1.3年(0.6-2年)后获得了解剖学根治,根治术后早期死亡1例,随访无远期死亡,所有病人心功能良好(NYHA I-n)。根治术后右心室/左心室压力比大于0.6病人,呼吸机辅助时间、ICU滞留时间明显延长。结论：姑息性右心室肺动脉连接结合体肺侧支封堵和肺动脉成型,能够有效的促进肺动脉发育不良病人肺血管的发育,最终帮助这些病人获得双心室矫治。右心室/左心室压力比值是根治术后近期结果的重要影响因素。目的：比较体肺分流术和姑息性右心室肺动脉连接术2种不同姑息手术方式在肺动脉闭锁合并室间隔缺损分期矫治中的应用效果。方法：回顾分析2009年12月至2012年8月间连续92例在我院行分期手术治疗合并肺动脉发育不良的肺动脉闭锁／室间隔缺损病例(平均年龄1.69±1.72岁,0.2-8.1; Nakata指数：87.51±36.97；McGoon比值：0.97±0.28),部分病人同期行侧枝封堵或者肺血管成形术,平均随访时间2.01±1.02年。其中体肺分流组47例、姑息性右心室肺动脉连接术组45例。比较两者姑息术后呼吸机辅助时间,ICU滞留时间,肺血管发育情况以及最终根治率等之间的差异。结果：随访终点时,所有病人的肺血管均较手术前得到明显发育(P0.001),两组间未见明显统计学意义,体肺分流组有15例病人(31.9%),姑息性右心室肺动脉连接组有28例病人(62.2%)最终获得解剖学根治。根治手术时右心室肺动脉连接组紫绀情况较体肺分流组改善明显,根治手术时间姑息性右心室流出道连接组较体肺分流组明显减少。ICU滞留时间和呼吸机使用时间虽有缩短但未见统计学差异。结论：姑息性右心室肺动脉连接作为促进肺动脉闭锁室间隔缺损分期手术的姑息手术能够更有效的提高此类患者的根治率,可能有利于二次根治手术时病人的恢复。目的：本研究通过回顾性分析我院经姑息性右心室肺动脉连接手术获得分期矫治的合并肺动脉发育不良的肺动脉闭锁室间隔缺损病人临床资料分析,探讨术后肺血管发育情况的评价指标,为临床决策提供依据。方法：回顾分析我院2009年12月至2013年7月28例,通过姑息性右心室肺动脉连接手术促进肺血管发育而获得根治的合并肺动脉发育不良的肺动脉闭锁室间隔缺损病人(Nakata index:89.39±17.91mm2/m2; McGoon:0.96±0.11),根据右心室／左心室压力比值是否大于0.6分为两组,比较两组间手术前后Nakata指数、McGoon比值、氧饱和度、血红蛋白浓度。结果：28例病人术前发育不良的肺动脉均通过姑息性右心室肺动脉连接获得显著的发育,Nakata指数和McGoon比值分别上升至264.25±85.84mm2/m2(P0.001)和2.0±0.36(P0.001),最终获得解剖学矫治,右心室肺动脉连接加室间隔缺损修补。10例根治术后右心室／左心室压力比大于0.6,18例小于0.6,其中比值大于0.6组：1例因肺部感染,右心功能衰竭死亡；1例术后呼吸功能衰竭,急性呼吸窘迫综合症通过ECMO辅助康复出院,呼吸机辅助时间以及ICU滞留时间均较后者明显延长(P0.01)；两组间术前Nakata指数,McGoon比值以及根治术前较姑息术前Nakata指数以及McGoon比值均未见明显差别,前者根治术前Sp02较姑息术后增加值以及根治术前Hb较姑息术前降低值均小于后者,存在显著统计学意义(P0.01)。结论：右心室／左心室压力比值是肺动脉闭锁合并室间隔缺损病人根治术后近期结果的独立危险因素,受肺血管发育情况的影响。Nakata指数以及McGoon比值在评价肺动脉闭锁合并室间隔缺损病人姑息术后肺血管发育情况存在一定的局限性,而根治术前Sp02及其上升值以及血红蛋白下降值作为参考指标能够较好的反映姑息术后肺血管发育的改善情况。
[Abstract]:Objective : To compare the safety and efficacy of common pulmonary shunt surgery for patients with pulmonary vascular dysplasia .

Methods : From December 2009 to August 2012 , 47 cases of pulmonary atresia / ventricular septal defect ( mean age : 1.78 卤 1.82 years , 0.2 - 8.1 ; Nakata index : 86.62 卤 45.02 ) were analyzed .
McGoon ratio : 1.03 卤 0.40 ) , in which the modified blalock - taproshunt ( B - T shunt ) 28 cases ( 59.6 % ) , central shunt in 19 cases ( 40.4 % ) mainly depended on the surgeon ' s habit and the preoperative pulmonary vascular development . The pulmonary artery development index was significantly smaller than that in the modified B - T shunt group ( P0.01 ) . The mean follow - up time was 2.36 卤 1.07 years .

Results : There was no significant difference between the two groups after palliative operation ( Nakata index : 207.94 卤 101.47 , McGoon ratio 1.55 卤 0.44 ) .

Conclusion : Although pulmonary shunt can effectively promote the development of PA - VSD in patients with dysplasia , it can only help patients with about 1 / 3 to obtain double - ventricle correction , with a low radical cure rate . The near - long - term results of central shunt operation are slightly lower than that of modified B - T shunt group , which may be related to the poor pulmonary vascular development in the pre - operative patients .

Objective : To evaluate the safety and efficacy of palliative right - ventricle pulmonary artery ligation in patients with pulmonary vascular dysplasia .

Methods : From December 2009 to August 2012 , 45 cases of pulmonary atresia / ventricular septal defect ( mean age , 1 . 61 卤 1 . 63 years , 0.2 - 7.2 ) were analyzed .
Nakata index : 88.25 卤 29.27 ;
The mean follow - up time was 2.06 卤 0.83 years .

Results : There was no early death after palliative operation , 1 case died due to myocarditis in follow - up .
All patients had significant growth ( Nakata index : 215.61 卤 107.79 ) .
McGoon ratio ( 1.74 卤 0.56 , PO.001 ) , in which 2 cases of branch pulmonary stenosis occurred and 1 case of right ventricular outflow tract stenosis were treated by percutaneous intervention . 28 patients ( 62.2 % ) were treated with anatomic radical after palliative operation . One case died early after radical operation . All patients died without long - term death . All patients had good cardiac function ( NYHA I - n ) . After radical operation , the pressure ratio of the right ventricle to the left ventricle was greater than 0.6 , the auxiliary time of ventilator and the stay time of ICU were significantly prolonged .

Conclusion : Palliative right ventricle pulmonary artery ligation combined with pulmonary artery occlusion and pulmonary artery formation can effectively promote the development of pulmonary vessels in patients with pulmonary artery dysplasia .

Objective : To compare the effect of two different palliative operation methods on pulmonary atresia with ventricular septal defect ( VSD ) in two different palliative operation modes .

Methods : From December 2009 to August 2012 , 92 cases of pulmonary atresia / ventricular septal defect ( mean age , 1 . 69 卤 1 . 72 years , 0.2 - 8.1 ; Nakata index : 87.51 卤 36.97 ) were analyzed .
The average follow - up time was 2 . 01 卤 1 . 02 years . The mean follow - up time was 2 . 01 卤 1 . 02 years .

Results : At the end of follow - up , the pulmonary vessels of all patients were significantly higher than those before operation ( P 0.001 ) . There were 15 patients ( 31.9 % ) in the pulmonary shunt group and 28 patients ( 62.2 % ) in the palliative right ventricle pulmonary artery group .

Conclusion : Palliative right ventricular pulmonary artery connection can improve the radical cure rate of patients with pulmonary atresia and septal defect , which may be beneficial to the recovery of patients during secondary radical operation .

Objective : To analyze the clinical data of patients with pulmonary atresia and ventricular septal defect ( VSD ) treated by stage correction by palliative right - ventricle pulmonary artery ligation in our hospital , and to discuss the evaluation index of pulmonary vascular development after operation and provide the basis for clinical decision - making .

Methods : From December 2009 to July 28 , 2013 , the patients with pulmonary atresia ( Nakata index : 89.39 卤 17.91mm2 / m2 ; McGoon : 0.96 卤 0.11 ) were divided into two groups according to whether the ratio of right ventricle / left ventricle pressure was greater than 0.6 . The Nakata index , McGoon ratio , oxygen saturation and hemoglobin concentration were compared between the two groups .

Results : All 28 patients with pre - operative dysplasia of the pulmonary artery obtained significant development through palliative right ventricular pulmonary artery connection . The ratio of Nakata index and McGoon increased to 264.25 卤 85.84mm2 / m2 ( P0.001 ) and 2.0 卤 0.36 ( P0.001 ) .
1 patient with respiratory failure and acute respiratory distress syndrome were discharged from hospital with ECMO assisted rehabilitation , ventilator - assisted time and ICU stay time were significantly prolonged ( P0.01 ) .
The preoperative Nakata index , McGoon ratio and pre - operative Nakata index and McGoon ratio were not significantly different between the two groups .

Conclusion : The ratio of right ventricle / left ventricle pressure is an independent risk factor after radical operation in patients with pulmonary atresia and ventricular septal defect . The effects of Nakata index and McGoon ratio in evaluating pulmonary vascular development in patients with pulmonary atresia and ventricular septal defect are limited , and Sp02 and its upper value and hemoglobin decrease value can reflect the improvement of pulmonary vascular development after palliative operation .

【学位授予单位】：北京协和医学院
【学位级别】：博士
【学位授予年份】：2014
【分类号】：R726.5

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