本文選題：肺動脈閉鎖合并室間隔缺損 + 肺動脈發(fā)育不良�。� 參考：《北京協(xié)和醫(yī)學(xué)院》2014年博士論文

【摘要】：目的：比較常見體肺分流手術(shù)對于肺血管發(fā)育不良肺動脈閉鎖室間隔缺損病人的安全性和有效性。 方法：回顧分析2009年12月至2012年8月間連續(xù)47例在我院行體肺分流手術(shù)治療合并肺動脈發(fā)育不良的肺動脈閉鎖/室間隔缺損病例(平均年齡1.78±1.82歲,0.2-8.1; Nakata指數(shù)：86.62±45.02；McGoon比值：1.03±0.40),其中改良blalock-taussing shunt (B-T分流術(shù))28例(59.6%),中央分流19例(40.4%),主要依據(jù)術(shù)者習(xí)慣和術(shù)前肺血管發(fā)育情況決定手術(shù)方式,采用中央分流病人的肺動脈發(fā)育指數(shù)明顯小于改良B-T分流組(P0.01),部分病人同期或者分期行體肺側(cè)枝封堵術(shù),平均隨訪時(shí)間2.36±1.07年。 結(jié)果：姑息術(shù)后兩組病人肺血管均得到了明顯生長(Nakata指數(shù)：207.94±101.47,McGoon比值1.55±0.44)兩組間未見明顯統(tǒng)計(jì)數(shù)差異(P0.05),中央分流組姑息術(shù)后呼吸機(jī)使用時(shí)間和ICU滯留時(shí)間均較B-T分流組明顯延長(P0.01)。15例病人(31.9%)在姑息術(shù)后平均1.1年(0.6-1.6年)后獲得了解剖學(xué)根治,且均為改良B-T分流術(shù)后病人,根治術(shù)后無早期死亡,隨訪無遠(yuǎn)期死亡,所有病人心功能良好(NYHA Ⅰ-Ⅱ)。 結(jié)論：體肺分流雖然能夠有效的促進(jìn)PA-VSD病人發(fā)育不良的固有肺動脈發(fā)發(fā)育,但最終僅能幫助約1/3的病人獲得雙心室矯治,根治率較低,中央分流手術(shù)近遠(yuǎn)期結(jié)果略低于改良B-T分流組,可能與術(shù)前病人肺血管發(fā)育情況較差有關(guān)。 目的：評價(jià)姑息性右心室肺動脈連接手術(shù)對于肺血管發(fā)育不良肺動脈閉鎖室間隔缺損病人的安全性和有效性。 方法：回顧分析2009年12月至2012年8月間連續(xù)45例在我院行姑息性右心室肺動脈連接術(shù)治療合并肺動脈發(fā)育不良的肺動脈閉鎖/室間隔缺損病例(平均年齡1.61±1.63歲,0.2-7.2；Nakata指數(shù)：88.25±29.27；McGoon比值：0.94±0.14),部分病人同期行側(cè)枝封堵或者肺血管成型術(shù),平均隨訪時(shí)間2.06±0.83年。 結(jié)果：姑息術(shù)后無早期死亡,隨訪中因心肌炎死亡1例；所有病人肺血管均得到了明顯生長(Nakata指數(shù)：215.61±107.79；McGoon比值1.74±0.56,PO.001),其中發(fā)生分支肺動脈狹窄2例,右心室流出道狹窄1例,均通過經(jīng)胸介入治療獲得矯治。28例病人(62.2%)在姑息術(shù)后平均1.3年(0.6-2年)后獲得了解剖學(xué)根治,根治術(shù)后早期死亡1例,隨訪無遠(yuǎn)期死亡,所有病人心功能良好(NYHA I-n)。根治術(shù)后右心室/左心室壓力比大于0.6病人,呼吸機(jī)輔助時(shí)間、ICU滯留時(shí)間明顯延長。 結(jié)論：姑息性右心室肺動脈連接結(jié)合體肺側(cè)支封堵和肺動脈成型,能夠有效的促進(jìn)肺動脈發(fā)育不良病人肺血管的發(fā)育,最終幫助這些病人獲得雙心室矯治。右心室/左心室壓力比值是根治術(shù)后近期結(jié)果的重要影響因素。 目的：比較體肺分流術(shù)和姑息性右心室肺動脈連接術(shù)2種不同姑息手術(shù)方式在肺動脈閉鎖合并室間隔缺損分期矯治中的應(yīng)用效果。 方法：回顧分析2009年12月至2012年8月間連續(xù)92例在我院行分期手術(shù)治療合并肺動脈發(fā)育不良的肺動脈閉鎖／室間隔缺損病例(平均年齡1.69±1.72歲,0.2-8.1; Nakata指數(shù)：87.51±36.97；McGoon比值：0.97±0.28),部分病人同期行側(cè)枝封堵或者肺血管成形術(shù),平均隨訪時(shí)間2.01±1.02年。其中體肺分流組47例、姑息性右心室肺動脈連接術(shù)組45例。比較兩者姑息術(shù)后呼吸機(jī)輔助時(shí)間,ICU滯留時(shí)間,肺血管發(fā)育情況以及最終根治率等之間的差異。 結(jié)果：隨訪終點(diǎn)時(shí),所有病人的肺血管均較手術(shù)前得到明顯發(fā)育(P0.001),兩組間未見明顯統(tǒng)計(jì)學(xué)意義,體肺分流組有15例病人(31.9%),姑息性右心室肺動脈連接組有28例病人(62.2%)最終獲得解剖學(xué)根治。根治手術(shù)時(shí)右心室肺動脈連接組紫紺情況較體肺分流組改善明顯,根治手術(shù)時(shí)間姑息性右心室流出道連接組較體肺分流組明顯減少。ICU滯留時(shí)間和呼吸機(jī)使用時(shí)間雖有縮短但未見統(tǒng)計(jì)學(xué)差異。 結(jié)論：姑息性右心室肺動脈連接作為促進(jìn)肺動脈閉鎖室間隔缺損分期手術(shù)的姑息手術(shù)能夠更有效的提高此類患者的根治率,可能有利于二次根治手術(shù)時(shí)病人的恢復(fù)。 目的：本研究通過回顧性分析我院經(jīng)姑息性右心室肺動脈連接手術(shù)獲得分期矯治的合并肺動脈發(fā)育不良的肺動脈閉鎖室間隔缺損病人臨床資料分析,探討術(shù)后肺血管發(fā)育情況的評價(jià)指標(biāo),為臨床決策提供依據(jù)。 方法：回顧分析我院2009年12月至2013年7月28例,通過姑息性右心室肺動脈連接手術(shù)促進(jìn)肺血管發(fā)育而獲得根治的合并肺動脈發(fā)育不良的肺動脈閉鎖室間隔缺損病人(Nakata index:89.39±17.91mm2/m2; McGoon:0.96±0.11),根據(jù)右心室／左心室壓力比值是否大于0.6分為兩組,比較兩組間手術(shù)前后Nakata指數(shù)、McGoon比值、氧飽和度、血紅蛋白濃度。 結(jié)果：28例病人術(shù)前發(fā)育不良的肺動脈均通過姑息性右心室肺動脈連接獲得顯著的發(fā)育,Nakata指數(shù)和McGoon比值分別上升至264.25±85.84mm2/m2(P0.001)和2.0±0.36(P0.001),最終獲得解剖學(xué)矯治,右心室肺動脈連接加室間隔缺損修補(bǔ)。10例根治術(shù)后右心室／左心室壓力比大于0.6,18例小于0.6,其中比值大于0.6組：1例因肺部感染,右心功能衰竭死亡；1例術(shù)后呼吸功能衰竭,急性呼吸窘迫綜合癥通過ECMO輔助康復(fù)出院,呼吸機(jī)輔助時(shí)間以及ICU滯留時(shí)間均較后者明顯延長(P0.01)；兩組間術(shù)前Nakata指數(shù),McGoon比值以及根治術(shù)前較姑息術(shù)前Nakata指數(shù)以及McGoon比值均未見明顯差別,前者根治術(shù)前Sp02較姑息術(shù)后增加值以及根治術(shù)前Hb較姑息術(shù)前降低值均小于后者,存在顯著統(tǒng)計(jì)學(xué)意義(P0.01)。 結(jié)論：右心室／左心室壓力比值是肺動脈閉鎖合并室間隔缺損病人根治術(shù)后近期結(jié)果的獨(dú)立危險(xiǎn)因素,受肺血管發(fā)育情況的影響。Nakata指數(shù)以及McGoon比值在評價(jià)肺動脈閉鎖合并室間隔缺損病人姑息術(shù)后肺血管發(fā)育情況存在一定的局限性,而根治術(shù)前Sp02及其上升值以及血紅蛋白下降值作為參考指標(biāo)能夠較好的反映姑息術(shù)后肺血管發(fā)育的改善情況。
[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 鹵 1.82 years , 0.2 - 8.1 ; Nakata index : 86.62 鹵 45.02 ) were analyzed .
McGoon ratio : 1.03 鹵 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 鹵 1.07 years .

Results : There was no significant difference between the two groups after palliative operation ( Nakata index : 207.94 鹵 101.47 , McGoon ratio 1.55 鹵 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 鹵 1 . 63 years , 0.2 - 7.2 ) were analyzed .
Nakata index : 88.25 鹵 29.27 ;
The mean follow - up time was 2.06 鹵 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 鹵 107.79 ) .
McGoon ratio ( 1.74 鹵 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 鹵 1 . 72 years , 0.2 - 8.1 ; Nakata index : 87.51 鹵 36.97 ) were analyzed .
The average follow - up time was 2 . 01 鹵 1 . 02 years . The mean follow - up time was 2 . 01 鹵 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 鹵 17.91mm2 / m2 ; McGoon : 0.96 鹵 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 鹵 85.84mm2 / m2 ( P0.001 ) and 2.0 鹵 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 .

【學(xué)位授予單位】：北京協(xié)和醫(yī)學(xué)院
【學(xué)位級別】：博士
【學(xué)位授予年份】：2014
【分類號】：R726.5

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1 張永輝;肺動脈發(fā)育不良的肺動脈閉鎖合并室間隔缺損的外科治療[D];北京協(xié)和醫(yī)學(xué)院;2014年

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