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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
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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(P<0.01),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(P>0.05),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(P<0.01)。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,P     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(P<0.001),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(P<0.001)和2.0±0.36(P<0.001),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(P<0.01);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(P<0.01)。
     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。
Objective: To compare the differences of the effect and safeness in the common systemic-to-pulmonary artery shunts to rehabilitate the hypoplastic pulmonary arteries in patients with pulmonary atresia and ventricular septal defect.
     Methods:From December2009to August2012,47consecutive patients (mean age1.78±1.82years, range0.2-8.1years) diagnosed with pulmonary atresia, ventricular septal defect and pulmonary artery hypoplasia (Nakata index86.62±45.02mm2/m2; McGoon ratio1.03±0.40) were included in this retrospective study.28(59.6%) patients underwent the procedure of modified blalock-taussing shunt (BT shunt)and the left19paitents receiewed the central shunt, mainly based on the surgeon habits and preoperative pulmonary vascular development. The nakata index in the BT group was significantly smaller than that in the central pulmonary shunt group(P<0.01). Some patients received transcatheter occlusion of major aortopulmonary collaterals and/or pulmonary angioplasty. Mean follow-up was2.36±1.07years.
     Result:After the systemic-to-pulmonary artery shunts,two groups'pulmonary vascular had significant growth (Nakata index:207.94±101.47, McGoon ratio of1.55±0.44),but there was no difference between the two groups(P>0.05).The central shunt group had more prolonged mechanical ventilation time and ICU monitoring time than the BT shunt group(p<0.01).15(31.9%)patients underwent the anatomical repair after palliative surgery in an average of1.1years(0.6-1.6years).They were all in the BT shunt group. There were no early dearth and late dearth. All survivors who underwent anatomical repair were in New York Heart Association class Ⅰ-Ⅱ.
     Conclusion:Although the systemic-to-pulmonary artery shunts can effectively promote the hypoplastic pulmonary arteries growth in the PA-VSD patients, but ultimately only1/3of the patients can get biventricular repiar. The central shunt group had worse early and late results than the BT group,maybe for poorer preoperative pulmonary vascular development.
     Objective:To determine the effect and safeness of the right ventricle to pulmonary artery connection to rehabilitate the hypoplastic pulmonary arteries in patients with pulmonary atresia and ventricular septal defect.
     Methods:From December2009to August2012,45consecutive patients (mean age1.61±1.63years, range0.2-7.2years) diagnosed with pulmonary atresia, ventricular septal defect and pulmonary artery hypoplasia (Nakata index88.25±29.27mm2/m2; McGoon ratio0.94±0.14) were included in this retrospective study. All patients underwent the procedure of right ventricle to pulmonary artery connection, during which most of them received transcatheter occlusion of major aortopulmonary collaterals and/or pulmonary angioplasty. Mean follow-up was2.06±0.83years.
     Results:There were no early deaths, but one patient died of myocarditis. Significant pulmonary artery growth was obtained (Nakata index215.1±107.79mm2/m2, P<0.001; McGoon ratio1.6±0.5, P<0.001) in all of the47patients.Restenosis occurs in2patients'pulmonary arterial brach and1parient's outflow tract of right ventricle.All of the restenosis were relieved though percutneous trnsluminl blloon dilttion.28patients (62.2%) whose pulmonary growth was considered adequate obtained a complete repair.There were1early dearth and no late dearth. All survivors who underwent anatomical repair were in New York Heart Association class Ⅰ-Ⅱ.The patients with right ventricle/left ventricle pressure ratio more than0.6after the anatomical repair had prolonged mechanical ventilation time and ICU monitoring time.
     Conclusions:Connection of the right ventricle to the pulmonary artery is safe and effective to promote the growth of the native pulmonary arteries in patients with pulmonary atresia, ventricular septal defect and hypoplastic native pulmonary arteries who ultimately have anatomical repair. The right ventricle/left ventricle pressure ratio is the important impact of early results
     Object: To compare the effect between the right ventricle to pulmonary artery connection and the systemic-to-pulmonary artery shunts in patients with pulmonary atresia and ventricular septal defect.
     Method:From December2009to August2012,92consecutive patients (mean age1.69±1.72years, range0.2-8.1years) diagnosed with pulmonary atresia, ventricular septal defect and pulmonary artery hypoplasia (Nakata index87.51±36.97mm2/m2; McGoon ratio0.97±0.28) were included in this retrospective study.45patients underwent the procedure of right ventricle to pulmonary artery connection and47underwent the systemic-to-pulmonary artery shunts,during which part of them received transcatheter occlusion of major aortopulmonary collaterals and/or pulmonary angioplasty. The mechanical ventilation time and ICU monitoring time,the pulmonary vascular growth and the anatomical repair rate were compared between the two groups.
     Results:All the patients'pulmonary vascular had significant growth(p<0.001),but there was no sinificant difference between the two groups.15(31.9%) patients in the BT group and28(62.2%) patients in the RVPA group ultimately had anatomical repair.3patients were awaiting for the anatomical repair.At the anatomical repair, the cyanosis in the RVPA group were improved more than the patients in the BT group. The RVPA group had less mechanical ventilation time and ICU monitoring time than the BT group,but there was no statistical difference.
     Conclusion:The right ventricle to pulmonary artery connection has higher rate of staging anatomical repiar in patients with pulmonary atresia and ventricular septal defect and maybe help the patients reconver after the anatomical repair.
     Objective:This study retrospectively analysised the clinical data of patients with pulmonary atresia with ventricular septal defect who underwent the staging right ventricle to pulmonary artery connection and anatomical repair to find the evaluation index for the postoperative pulmonary vascular development.
     Methods:From December2009to July2013,28consecutive patients diagnosed with pulmonary atresia, ventricular septal defect and pulmonary artery hypoplasia (Nakata index89.39±17.91mm2/m2; McGoon ratio0.96±0.11) were included in this retrospective study. All of them underwent the staging right ventricle to pulmonary artery connection and anatomical repair. They were divided into two groups according to the right ventricle/left ventricle pressure ratio(one was more than0.6, the other was less than0.6) to compare the difference of Preoperative and postoperative Nakata index,Mcgoon ratio,Oxygen saturation and hemoglobin concentration.
     Results:After the right ventricle to pulmonary artery connection,alll the28patients receiewed the anatomical with the rising of nakata index and Mcgoon ratio (nakata index:264.25±85.84mm2/m2(P<0.001),Mcgoon ratio2.0±0.36(P<0.001)).10patients'postoperative right ventricle/left ventricle pressure ratio were more than0.6(Group A),the left18patents were less than0.6(Group B).In the grop A,there was1early death for pulmonary infection and right heart dysfunction,and another need ECMO assitance for acute respiratory distress syndrome and ultimately discharged. Group A had significantly prolonged mechanical ventilation time and ICU monitoring time than Group B(p<0.01).There was no statistical difference between the two groups in the preoperative Nakata index and Mcgoon ratio.SpO2and HB were significanly improved after the anatomical repair(p<0.01).
     Conclusions:The right ventricle/left ventricle pressure ratio was independent risk factor for patients with pulmonary atresia with ventricular septal defect who underwent the staging right ventricle to pulmonary artery connection and anatomical repair.There was limitation for Nakata index and Mcgoon ratio to evaluate the development of pulmonary vascular after the palliative right ventricle to pulmonary connection.The improvement of preoperative SPO2and decline of preoperative HB can reflect the development of pulmonary vascular after the right ventricle to pulmonary artery connection.
引文
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    [12]Grosse-Wortmann L, Yoo SJ, van Arsdell G, Chetan D, Macdonald C, Benson L et al. Preoperative total pulmonary blood flow predicts right ventricular pressure in patients early after complete repair of tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2013.
    [13]Talwar S, Saxena R, Choudhary SK, Airan B. Staged repair of pulmonary atresia, ventricular septal defect, and major systemic to pulmonary artery collaterals. Ann Pediatr Cardiol 2010;3:136-139.
    [14]Song SW, Park HK, Park YH, Cho BK. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries. Circ J 2009;73:516-522.
    [15]Malhotra SP, Hanley FL. Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals:a protocol-based approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009:145-151.
    [16]Duncan BW, Mee RB, Prieto LR, Rosenthal GL, Mesia CI, Qureshi A et al. Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2003; 126:694-702.
    [17]Ishibashi N, Shin'oka T, Ishiyama M, Sakamoto T, Kurosawa H. Clinical results of staged repair with complete unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Eur J Cardiothorac Surg 2007;32:202-208.
    [18]Potapov EV, Alexi-Meskishvili VV, Dahnert I, Ivanitskaia EA, Lange PE, Hetzer R. Development of pulmonary arteries after central aortopulmonary shunt in newborns. Ann Thorac Surg 2001;71:899-905; discussion 905-896.
    [19]Dirks V, Pretre R, Knirsch W, Valsangiacomo Buechel ER, Seifert B, Schweiger M et al. Modified Blalock Taussig shunt: a not-so-simple palliative procedure. Eur J Cardiothorac Surg 2013;44:1096-1102.
    [20]Mainwaring RD, Reddy VM, Perry SB, Peng L, Hanley FL. Late outcomes in patients undergoing aortopulmonary window for pulmonary atresia/stenosis and major aortopulmonary collaterals. Ann Thorac Surg 2012;94:842-848.
    [21]Di Donato RM. Late outcomes in patients undergoing aortopulmonary window for pulmonary atresia/stenosis and major aortopulmonary collaterals Invited commentary. Ann Thorac Surg 2012;94:848-849.
    [22]Petrucci O, O'Brien SM, Jacobs ML, Jacobs JP, Manning PB, Eghtesady P. Risk factors for mortality and morbidity after the neonatal Blalock-Taussig shunt procedure. Ann Thorac Surg 2011;92:642-651; discussion 651-642.
    [23]O'Connor MJ, Ravishankar C, Ballweg JA, Gillespie MJ, Gaynor JW, Tabbutt S et al. Early systemic-to-pulmonary artery shunt intervention in neonates with congenital heart disease. J Thorac Cardiovasc Surg 2011;142:106-112.
    [24]Martinez-Quintana E, Rodriguez-Gonzalez F. Vestibulopathy after a modified Blalock-Taussig procedure in a cyanotic congenital heart disease patient. Am J Otolaryngol 2013.
    [25]Fang M, Wang H, Jin Y, Wang Z, Wang Z, Zhang C. Development of Pulmonary Arteries after a Central End-to-Side Shunt in Patients with Pulmonary Atresia, Ventricular Septal Defect, and Diminutive Pulmonary Arteries. Thorac Cardiovasc Surg 2013.
    [26]DeCampli WM, Argueta-Morales IR, Zabinsky J, Hannan RL, Burke RP. An institutional approach to, and results for, patient with tetralogy with pulmonary atresia and major systemic-to-pulmonary collateral arteries. Cardiol Young 2010;20 Suppl 3:128-134.
    [1]Cho JM, Puga FJ, Danielson GK, Dearani JA, Mair DD, Hagler DJ et al. Early and long-term results of the surgical treatment of tetralogy of Fallot with pulmonary atresia, with or without major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2002; 124:70-81.
    [2]Duncan BW, Mee RB, Prieto LR, Rosenthal GL, Mesia CI, Qureshi A et al. Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2003; 126:694-702.
    [3]Gupta A, Odim J, Levi D, Chang RK, Laks H. Staged repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries:experience with 104 patients. J Thorac Cardiovasc Surg 2003;126:1746-1752.
    [4]Ishibashi N, Shin'oka T, Ishiyama M, Sakamoto T, Kurosawa H. Clinical results of staged repair with complete unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Eur J Cardiothorac Surg 2007;32:202-208.
    [5]Talwar S, Saxena R, Choudhary SK, Airan B. Staged repair of pulmonary atresia, ventricular septal defect, and major systemic to pulmonary artery collaterals. Ann Pediatr Cardiol 2010;3:136-139.
    [6]Amark KM, Karamlou T, O'Carroll A, MacDonald C, Freedom RM, Yoo S J et al. Independent factors associated with mortality, reintervention, and achievement of complete repair in children with pulmonary atresia with ventricular septal defect. J Am Coll Cardiol 2006;47:1448-1456.
    [7]Dragulescu A, Kammache I, Fouilloux V, Amedro P, Metras D, Kreitmann B et al. Long-term results of pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect, pulmonary artery hypoplasia, and major aortopulmonary collaterals. J Thorac Cardiovasc Surg 2011;142:1374-1380.
    [8]Bockeria LA, Podzolkov VP, Makhachev OA, Alekyan BG, Khiriev T, Zelenikin MA et al. Palliative surgical treatment of congenital heart defects associated with unilateral absence of the pulmonary artery. Interact Cardiovasc Thorac Surg 2013; 16:286-292.
    [9]Bradley SM, Erdem CC, Hsia TY, Atz AM, Bandisode V, Ringewald JM. Right ventricle-to-pulmonary artery shunt: alternative palliation in infants with inadequate pulmonary blood flow prior to two-ventricle repair. Ann Thorac Surg 2008;86:183-188; discussion 188.
    [10]Fouilloux V, Bonello B, Kammache I, Fraisse A, Mace L, Kreitmann B. Management of patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries and major aorto-pulmonary collaterals:Focus on the strategy of rehabilitation of the native pulmonary arteries. Arch Cardiovasc Dis 2012;105:666-675.
    [11]Davies B, Mussa S, Davies P, Stickley J, Jones TJ, Barron DJ et al. Unifocalization of major aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect is essential to achieve excellent outcomes irrespective of native pulmonary artery morphology. J Thorac Cardiovasc Surg 2009;138:1269-1275 e1261.
    [12]d'Udekem Y, Alphonso N, Norgaard MA, Cochrane AD, Grigg LE, Wilkinson JL et al. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries: unifocalization brings no long-term benefits. J Thorac Cardiovasc Surg 2005; 130:1496-1502.
    [13]Metras D, Chetaille P, Kreitmann B, Fraisse A, Ghez O, Riberi A. Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals. Eur J Cardiothorac Surg 2001;20:590-596; discussion 596-597.
    [14]Grosse-Wortmann L, Yoo SJ, van Arsdell G, Chetan D, Macdonald C, Benson L et al. Preoperative total pulmonary blood flow predicts right ventricular pressure in patients early after complete repair of tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2013.
    [15]Song SW, Park HK, Park YH, Cho BK. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries. Circ J 2009;73:516-522.
    [16]Malhotra SP, Hanley FL. Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a protocol-based approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009:145-151.
    [17]Rome J J, Mayer JE, Castaneda AR, Lock JE. Tetralogy of Fallot with pulmonary atresia. Rehabilitation of diminutive pulmonary arteries. Circulation 1993;88:1691-1698.
    [18]Brizard CP, Liava'a M, d'Udekem Y. Pulmonary atresia, VSD and Mapcas: repair without unifocalization. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009:139-144.
    [19]Carotti A, Albanese SB, Di Donato RM. Unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Acta Paediatr Suppl 2006;95:22-26.
    [20]DeCampli WM, Argueta-Morales IR, Zabinsky J, Hannan RL, Burke RP. An institutional approach to, and results for, patient with tetralogy with pulmonary atresia and major systemic-to-pulmonary collateral arteries. Cardiol Young 2010;20 Suppl 3:128-134.
    [21]Liava'a M, Brizard CP, Konstantinov IE, Robertson T, Cheung MM, Weintraub R et al. Pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals:neonatal pulmonary artery rehabilitation without unifocalization. Ann Thorac Surg 2012;93:185-191.
    [22]Carotti A, Albanese SB, Filippelli S, Rava L, Guccione P, Pongiglione G et al. Determinants of outcome after surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2010;140:1092-1103.
    [23]Rabinovitch M, Herrera-deLeon V, Castaneda AR, Reid L. Growth and development of the pulmonary vascular bed in patients with tetralogy of Fallot with or without pulmonary atresia. Circulation 1981;64:1234-1249.
    [24]Batra AS, Starnes VA, Wells WJ. Does the site of insertion of a systemic-pulmonary shunt influence growth of the pulmonary arteries? Ann Thorac Surg 2005;79:636-640.
    [1]McCrary AW, Clabby ML, Mahle WT. Patient and practice factors affecting growth of infants with systemic-to-pulmonary shunt. Cardiol Young 2013;23:499-506.
    [2]Fang M, Wang H, Jin Y, Wang Z, Wang Z, Zhang C. Development of Pulmonary Arteries after a Central End-to-Side Shunt in Patients with Pulmonary Atresia, Ventricular Septal Defect, and Diminutive Pulmonary Arteries. Thorac Cardiovasc Surg 2013.
    [3]Mainwaring RD, Reddy VM, Perry SB, Peng L, Hanley FL. Late outcomes in patients undergoing aortopulmonary window for pulmonary atresia/stenosis and major aortopulmonary collaterals. Ann Thorac Surg 2012;94:842-848.
    [4]Di Donato RM. Late outcomes in patients undergoing aortopulmonary window for pulmonary atresia/stenosis and major aortopulmonary collaterals Invited commentary. Ann Thorac Surg 2012;94:848-849.
    [5]Baruteau AE, Serraf A, Levy M, Petit J, Bonnet D, Jais X et al. Potts shunt in children with idiopathic pulmonary arterial hypertension: long-term results. Ann Thorac Surg 2012;94:817-824.
    [6]Mumtaz MA, Rosenthal G, Qureshi A, Prieto L, Preminger T, Lorber R et al. Melbourne shunt promotes growth of diminutive central pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and systemic-to-pulmonary collateral arteries. Ann Thorac Surg 2008;85:2079-2083; discussion 2083-2074.
    [7]Ong K, Boone R, Gao M, Carere R, Webb J, Kiess M et al. Right Ventricle to Pulmonary Artery Conduit Reoperations in Patients With Tetralogy of Fallot or Pulmonary Atresia Associated With Ventricular Septal Defect. Am J Cardiol 2013.
    [8]Fouilloux V, Bonello B, Kammache I, Fraisse A, Mace L, Kreitmann B. Management of patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries and major aorto-pulmonary collaterals:Focus on the strategy of rehabilitation of the native pulmonary arteries. Arch Cardiovasc Dis 2012;105:666-675.
    [9]Fiore AC, Tobin C, Jureidini S, Rahimi M, Kim ES, Schowengerdt K. A comparison of the modified Blalock-Taussig shunt with the right ventricle-to-pulmonary artery conduit. Ann Thorac Surg 2011;91:1479-1484; discussion 1484-1475.
    [10]Metras D, Chetaille P, Kreitmann B, Fraisse A, Ghez O, Riberi A. Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals. Eur J Cardiothorac Surg 2001;20:590-596; discussion 596-597.
    [11]Ohye RG, Ludomirsky A, Devaney EJ, Bove EL. Comparison of right ventricle to pulmonary artery conduit and modified Blalock-Taussig shunt hemodynamics after the Norwood operation. Ann Thorac Surg 2004;78:1090-1093.
    [12]Rome JJ, Mayer JE, Castaneda AR, Lock JE. Tetralogy of Fallot with pulmonary atresia. Rehabilitation of diminutive pulmonary arteries. Circulation 1993;88:1691-1698.
    [13]Bockeria LA, Podzolkov VP, Makhachev OA, Alekyan BG, Khiriev T, Zelenikin MA et al. Palliative surgical treatment of congenital heart defects associated with unilateral absence of the pulmonary artery. Interact Cardiovasc Thorac Surg 2013; 16:286-292.
    [14]Dragulescu A, Kammache I, Fouilloux V, Amedro P, Metras D, Kreitmann B et al. Long-term results of pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect, pulmonary artery hypoplasia, and major aortopulmonary collaterals. J Thorac Cardiovasc Surg 2011;142:1374-1380.
    [15]Davies B, Mussa S, Davies P, Stickley J, Jones TJ, Barron DJ et al. Unifocalization of major aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect is essential to achieve excellent outcomes irrespective of native pulmonary artery morphology. J Thorac Cardiovasc Surg 2009;138:1269-1275 e1261.
    [16]Brizard CP, Liava'a M, d'Udekem Y. Pulmonary atresia, VSD and Mapcas: repair without unifocalization. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009:139-144.
    [17]Carotti A, Albanese SB, Di Donato RM. Unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Acta Paediatr Suppl 2006;95:22-26.
    [18]Malhotra SP, Hanley FL. Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a protocol-based approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009:145-151.
    [19]DeCampli WM, Argueta-Morales IR, Zabinsky J, Hannan RL, Burke RP. An institutional approach to, and results for, patient with tetralogy with pulmonary atresia and major systemic-to-pulmonary collateral arteries. Cardiol Young 2010;20 Suppl 3:128-134.
    [1]Metras D, Chetaille P, Kreitmann B, Fraisse A, Ghez O, Riberi A. Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals. Eur J Cardiothorac Surg 2001;20:590-596; discussion 596-597.
    [2]Amark KM, Karamlou T, O'Carroll A, MacDonald C, Freedom RM, Yoo S J et al. Independent factors associated with mortality, reintervention, and achievement of complete repair in children with pulmonary atresia with ventricular septal defect. J Am Coll Cardiol 2006;47:1448-1456.
    [3]Cho JM, Puga FJ, Danielson GK, Dearani JA, Mair DD, Hagler DJ et al. Early and long-term results of the surgical treatment of tetralogy of Fallot with pulmonary atresia, with or without major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2002; 124:70-81.
    [4]Dragulescu A, Kammache I, Fouilloux V, Amedro P, Metras D, Kreitmann B et al. Long-term results of pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect, pulmonary artery hypoplasia, and major aortopulmonary collaterals. J Thorac Cardiovasc Surg 2011;142:1374-1380.
    [5]Ong K, Boone R, Gao M, Carere R, Webb J, Kiess M et al. Right Ventricle to Pulmonary Artery Conduit Reoperations in Patients With Tetralogy of Fallot or Pulmonary Atresia Associated With Ventricular Septal Defect. Am J Cardiol 2013.
    [6]Martinez-Quintana E, Rodriguez-Gonzalez F. Vestibulopathy after a modified Blalock-Taussig procedure in a cyanotic congenital heart disease patient. Am J Otolaryngol 2013.
    [7]Mainwaring RD, Reddy VM, Peng L, Kuan C, Palmon M, Hanley FL. Hemodynamic assessment after complete repair of pulmonary atresia with major aortopulmonary collaterals. Ann Thorac Surg 2013;95:1397-1402.
    [8]Grosse-Wortmann L, Yoo SJ, van Arsdell G, Chetan D, Macdonald C, Benson L et al. Preoperative total pulmonary blood flow predicts right ventricular pressure in patients early after complete repair of tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2013.
    [9]Chowdhury UK, Bishnoi AK, Ray R, Kalaivani M, Kapoor PM, Reddy SM et al. Central pulmonary artery histopathology in patients with cyanotic congenital heart diseases. Ann Thorac Surg 2009;87:589-596,596 e581-583.
    [10]Mumtaz MA, Rosenthal G, Qureshi A, Prieto L, Preminger T, Lorber R et al. Melbourne shunt promotes growth of diminutive central pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and systemic-to-pulmonary collateral arteries. Ann Thorac Surg 2008;85:2079-2083; discussion 2083-2074.
    [11]Nakata S, Imai Y, Takanashi Y, Kurosawa H, Tezuka K, Nakazawa M et al. A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg 1984;88:610-619.
    [12]Batra AS, Starnes VA, Wells WJ. Does the site of insertion of a systemic-pulmonary shunt influence growth of the pulmonary arteries? Ann Thorac Surg 2005;79:636-640.
    [13]Zhang Y, Hua Z, Yang K, Zhang H, Yan J, Wang X et al. Outcomes of the rehabilitative procedure for patients with pulmonary atresia, ventricular septal defect and hypoplastic pulmonary arteries beyond the infant period. Eur J Cardiothorac Surg 2014.
    [14]Liava'a M, Brizard CP, Konstantinov IE, Robertson T, Cheung MM, Weintraub R et al. Pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals:neonatal pulmonary artery rehabilitation without unifocalization. Ann Thorac Surg 2012;93:185-191.
    [15]Honjo O, Al-Radi OO, MacDonald C, Tran KC, Sapra P, Davey LD et al The functional intraoperative pulmonary blood flow study is a more sensitive predictor than preoperative anatomy for right ventricular pressure and physiologic tolerance of ventricular septal defect closure after complete unifocalization in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. Circulation 2009;120:S46-52.
    [16]Fouilloux V, Bonello B, Kammache I, Fraisse A, Mace L, Kreitmann B. Management of patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries and major aorto-pulmonary collaterals:Focus on the strategy of rehabilitation of the native pulmonary arteries. Arch Cardiovasc Dis 2012;105:666-675.
    [17]Carotti A, Albanese SB, Filippelli S, Rava L, Guccione P, Pongiglione G et al. Determinants of outcome after surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2010;140:1092-1103.
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    [10]Talwar S, Saxena R, Choudhary SK, Airan B. Staged repair of pulmonary atresia, ventricular septal defect, and major systemic to pulmonary artery collaterals. Ann Pediatr Cardiol 2010;3:136-139.
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    [18]Duncan BW, Mee RB, Prieto LR, Rosenthal GL, Mesia CI, Qureshi A et al. Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2003;126:694-702.
    [19]Metras D, Chetaille P, Kreitmann B, Fraisse A, Ghez O, Riberi A. Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals. Eur J Cardiothorac Surg 2001;20:590-596; discussion 596-597.
    [20]d'Udekem Y, Alphonso N, Norgaard MA, Cochrane AD, Grigg LE, Wilkinson JL et al. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries: unifocalization brings no long-term benefits. J Thorac Cardiovasc Surg 2005;130:1496-1502.
    [21]Amark KM, Karamlou T, O'Carroll A, MacDonald C, Freedom RM, Yoo SJ et al. Independent factors associated with mortality, reintervention, and achievement of complete repair in children with pulmonary atresia with ventricular septal defect. J Am Coll Cardiol 2006;47:1448-1456.
    [22]Honjo O, Al-Radi OO, MacDonald C, Tran KC, Sapra P, Davey LD et al. The functional intraoperative pulmonary blood flow study is a more sensitive predictor than preoperative anatomy for right ventricular pressure and physiologic tolerance of ventricular septal defect closure after complete unifocalization in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. Circulation 2009;120:S46-52.
    [23]Cho JM, Puga FJ, Danielson GK, Dearani JA, Mair DD, Hagler DJ et al. Early and long-term results of the surgical treatment of tetralogy of Fallot with pulmonary atresia, with or without major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2002;124:70-81.
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