先天性心脏病外科治疗中国专家共识（四）：室间隔完整型肺动脉闭锁_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

 郑景浩 ¹ ,  李守军代表国家心血管病专家委员会先天性心脏病专业委员会

1. ;
2. ;

Corresponding author：

郑景浩, Email: zhengjh210@163.com; 李守军代表国家心血管病专家委员会先天性心脏病专业委员会, Email: drlishoujun@yahoo.com

Keywords：

先天性心脏病; 肺动脉闭锁; 外科治疗; 专家共识

DOI：

10.7507/1007-4848.202002110

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Citation： 郑景浩, 李守军代表国家心血管病专家委员会先天性心脏病专业委员会. 先天性心脏病外科治疗中国专家共识（四）：室间隔完整型肺动脉闭锁. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2020, 27(5): 479-483. doi: 10.7507/1007-4848.202002110 Copy

1.	Subramanian S, Wagner H, Tsehai G, et al. Pulmonary atresia with intact ventricular septum. Ann Chir Infant, 1972, 13(4): 225-228.
2.	丁文祥, 苏肇伉, 主编. 现代小儿心脏外科学. 济南: 山东科学技术出版社, 2013. 492-504.
3.	Bull C, de Leval MR, Mercanti C, et al. Pulmonary atresia and intact ventricular septum: a revised classification. Circulation, 1982, 66(2): 266-272.
4.	Hanley FL, Sade RM, Blackstone EH, et al. Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study. J Thorac Cardiovasc Surg, 1993, 105(3): 406-427.
5.	祝忠群, 刘锦纷, 苏肇伉, 等. 室间隔完整型肺动脉闭锁的个体化手术治疗. 中华医学杂志, 2008, 88(11): 738-741.
6.	Minich LL, Tani LY, Ritter S, et al. Usefulness of the preoperative tricuspid/mitral valve ratio for predicting outcome in pulmonary atresia with intact ventricular septum. Am J Cardiol, 2000, 85(11): 1325-1328.
7.	Cheung EW, Richmond ME, Turner ME, et al. Pulmonary atresia/intact ventricular septum: influence of coronary anatomy on single-ventricle outcome. Ann Thorac Surg, 2014, 98(4): 1371-1377.
8.	Ahmed AA, Snodgrass BT, Kaine S. Pulmonary atresia with intact ventricular septum and right ventricular dependent coronarycirculation through the "vessels of Wearn". Cardiovasc Pathol, 2013, 22(4): 298-302.
9.	Lewis AB, Wells W, Lindesmith GG. Right ventricular growth potenrial in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg, 1986, 91(6): 835-840.
10.	Yoshimura N, Yamaguchi M, Ohashi H, et al. Pulmonary atresia with intact ventricular septum: strategy based on right ventricular morphology. J Thorac Cardiovasc Surg, 2003, 126(5): 1417-1426.
11.	Numata S, Uemura H, Yagihara T, et al. Long-term functional results of the one and one half ventricular repair for the spectrum of patients with pulmonary atresia/stenosis with intact ventricular septum. Eur J Cardiothorac Surg, 2003, 24(4): 516-520.
12.	Zhang H, Li SJ, Li YQ, et al. Hybrid procedure for the neonatal management of pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg, 2007, 133(6): 1654-1656.
13.	Burke RP, Hannan RL, Zabinsky JA, et al. Hybrid ventricular decompression in pulmonary atresia with intact septum. Ann Thorac Surg, 2009, 88(2): 688-689.
14.	Li S, Chen W, Zhang Y, et al. Hybrid therapy for pulmonary atresia with intact ventricular septum. Ann Thorac Surg, 2011, 91(5): 1467-1471.
15.	Sano S, Ishino K, Kawada M, et al. Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum. Ann Thorac Surg, 2000, 70(5): 1501-1506.
16.	McLean KM, Pearl JM. Pulmonary atresia with intact ventricular septum: initial management. Ann Thorac Surg, 2006, 82(6): 2214-2220.
17.	Marasini M, Gorrieri PF, Tuo G, et al. Long-term results of catheter-based treatment of pulmonary atresia and intact ventricular septum. Heart, 2009, 95(18): 1520-1524.
18.	Hasan BS, Bautista-Hernandez V, McElhinney DB, et al. Outcomes of transcatheter approach for initial treatment of pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv, 2013, 81(1): 111-118.
19.	Hirata Y, Chen JM, Quaegebeur JM, et al. Pulmonary atresia with intact ventricular septum: limitations of catheter-based intervention. Ann Thorac Surg, 2007, 84(2): 574-580.
20.	郑景浩, 徐志伟, 刘锦纷, 等. 室间隔完整型肺动脉闭锁手术治疗的个体化方案. 中华胸心血管外科杂志, 2013, 29(2): 69-72.
21.	Laks H, Pearl JM, Drinkwater DC, et al. Partial biventricular repair of pulmonary atresia with intact ventricular septum. Use of an adjustable atrial septal defect. Circulation, 1992, 86(5 Suppl): Ⅱ159-Ⅱ166.
22.	Guleserian KJ, Armsby LB, Thiagarajan RR, et al. Natural history of pulmonary atresia with intact ventricular septum and right-ventricle-dependent coronary circulation managed by the single-ventricle approach. Ann Thorac Surg, 2006, 81(6): 2250-2258.
23.	Ashburn DA, Blackstone EH, Wells WJ, et al. Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg, 2004, 127(4): 1000-1008.
24.	Liava'a M, Brooks P, Konstantinov I, et al. Changing trends in the management of pulmonary atresia with intact ventricular septum: the Melbourne experience. Eur J Cardiothorac Surg, 2011, 40(6): 1406-1411.
25.	Zheng JH, Gao BT, Zhu ZQ, et al. Surgical results for pulmonary atresia with intact ventricular septum: a single-centre 15-year experience and medium-term follow-up. Eur J Cardiothorac Surg, 2016, 50(6): 1083-1088.
26.	Elias P, Poh CL, du Plessis K, et al. Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricularseptum: Fontan survivors remain at risk of late myocardial ischaemia and death. Eur J Cardiothorac Surg, 2018, 53(6): 1230-1236.
27.	Mi YP, Cheung YF. Assessment of right and left ventricular function by tissue Doppler echocardiography in patients after biventricular repair of pulmonary atresia with intact ventricular septum. Int J Cardiol, 2006, 109(3): 329-334.
28.	Romeih S, Groenink M, van der Plas MN, et al. Effect of age on exercise capacity and cardiac reserve in patients with pulmonary atresia with intact ventricular septum after biventricular repair. Eur J Cardiothorac Surg, 2012, 42(1): 50-55.
29.	Romeih S, Groenink M, Roest AA, et al. Exercise capacity and cardiac reserve in children and adolescents with corrected pulmonary atresia with intact ventricular septum after univentricular palliation and biventricular repair. J Thorac Cardiovasc Surg, 2012, 143(3): 569-575.
30.	Karamlou T, Poynter JA, Walters HL 3rd, et al. Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: a Congenital Heart Surgeons Society study. J Thorac Cardiovasc Surg, 2013, 145(4): 1018-1027.

1. Subramanian S, Wagner H, Tsehai G, et al. Pulmonary atresia with intact ventricular septum. Ann Chir Infant, 1972, 13(4): 225-228.
2. 丁文祥, 苏肇伉, 主编. 现代小儿心脏外科学. 济南: 山东科学技术出版社, 2013. 492-504.
3. Bull C, de Leval MR, Mercanti C, et al. Pulmonary atresia and intact ventricular septum: a revised classification. Circulation, 1982, 66(2): 266-272.
4. Hanley FL, Sade RM, Blackstone EH, et al. Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study. J Thorac Cardiovasc Surg, 1993, 105(3): 406-427.
5. 祝忠群, 刘锦纷, 苏肇伉, 等. 室间隔完整型肺动脉闭锁的个体化手术治疗. 中华医学杂志, 2008, 88(11): 738-741.
6. Minich LL, Tani LY, Ritter S, et al. Usefulness of the preoperative tricuspid/mitral valve ratio for predicting outcome in pulmonary atresia with intact ventricular septum. Am J Cardiol, 2000, 85(11): 1325-1328.
7. Cheung EW, Richmond ME, Turner ME, et al. Pulmonary atresia/intact ventricular septum: influence of coronary anatomy on single-ventricle outcome. Ann Thorac Surg, 2014, 98(4): 1371-1377.
8. Ahmed AA, Snodgrass BT, Kaine S. Pulmonary atresia with intact ventricular septum and right ventricular dependent coronarycirculation through the "vessels of Wearn". Cardiovasc Pathol, 2013, 22(4): 298-302.
9. Lewis AB, Wells W, Lindesmith GG. Right ventricular growth potenrial in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg, 1986, 91(6): 835-840.
10. Yoshimura N, Yamaguchi M, Ohashi H, et al. Pulmonary atresia with intact ventricular septum: strategy based on right ventricular morphology. J Thorac Cardiovasc Surg, 2003, 126(5): 1417-1426.
11. Numata S, Uemura H, Yagihara T, et al. Long-term functional results of the one and one half ventricular repair for the spectrum of patients with pulmonary atresia/stenosis with intact ventricular septum. Eur J Cardiothorac Surg, 2003, 24(4): 516-520.
12. Zhang H, Li SJ, Li YQ, et al. Hybrid procedure for the neonatal management of pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg, 2007, 133(6): 1654-1656.
13. Burke RP, Hannan RL, Zabinsky JA, et al. Hybrid ventricular decompression in pulmonary atresia with intact septum. Ann Thorac Surg, 2009, 88(2): 688-689.
14. Li S, Chen W, Zhang Y, et al. Hybrid therapy for pulmonary atresia with intact ventricular septum. Ann Thorac Surg, 2011, 91(5): 1467-1471.
15. Sano S, Ishino K, Kawada M, et al. Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum. Ann Thorac Surg, 2000, 70(5): 1501-1506.
16. McLean KM, Pearl JM. Pulmonary atresia with intact ventricular septum: initial management. Ann Thorac Surg, 2006, 82(6): 2214-2220.
17. Marasini M, Gorrieri PF, Tuo G, et al. Long-term results of catheter-based treatment of pulmonary atresia and intact ventricular septum. Heart, 2009, 95(18): 1520-1524.
18. Hasan BS, Bautista-Hernandez V, McElhinney DB, et al. Outcomes of transcatheter approach for initial treatment of pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv, 2013, 81(1): 111-118.
19. Hirata Y, Chen JM, Quaegebeur JM, et al. Pulmonary atresia with intact ventricular septum: limitations of catheter-based intervention. Ann Thorac Surg, 2007, 84(2): 574-580.
20. 郑景浩, 徐志伟, 刘锦纷, 等. 室间隔完整型肺动脉闭锁手术治疗的个体化方案. 中华胸心血管外科杂志, 2013, 29(2): 69-72.
21. Laks H, Pearl JM, Drinkwater DC, et al. Partial biventricular repair of pulmonary atresia with intact ventricular septum. Use of an adjustable atrial septal defect. Circulation, 1992, 86(5 Suppl): Ⅱ159-Ⅱ166.
22. Guleserian KJ, Armsby LB, Thiagarajan RR, et al. Natural history of pulmonary atresia with intact ventricular septum and right-ventricle-dependent coronary circulation managed by the single-ventricle approach. Ann Thorac Surg, 2006, 81(6): 2250-2258.
23. Ashburn DA, Blackstone EH, Wells WJ, et al. Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg, 2004, 127(4): 1000-1008.
24. Liava'a M, Brooks P, Konstantinov I, et al. Changing trends in the management of pulmonary atresia with intact ventricular septum: the Melbourne experience. Eur J Cardiothorac Surg, 2011, 40(6): 1406-1411.
25. Zheng JH, Gao BT, Zhu ZQ, et al. Surgical results for pulmonary atresia with intact ventricular septum: a single-centre 15-year experience and medium-term follow-up. Eur J Cardiothorac Surg, 2016, 50(6): 1083-1088.
26. Elias P, Poh CL, du Plessis K, et al. Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricularseptum: Fontan survivors remain at risk of late myocardial ischaemia and death. Eur J Cardiothorac Surg, 2018, 53(6): 1230-1236.
27. Mi YP, Cheung YF. Assessment of right and left ventricular function by tissue Doppler echocardiography in patients after biventricular repair of pulmonary atresia with intact ventricular septum. Int J Cardiol, 2006, 109(3): 329-334.
28. Romeih S, Groenink M, van der Plas MN, et al. Effect of age on exercise capacity and cardiac reserve in patients with pulmonary atresia with intact ventricular septum after biventricular repair. Eur J Cardiothorac Surg, 2012, 42(1): 50-55.
29. Romeih S, Groenink M, Roest AA, et al. Exercise capacity and cardiac reserve in children and adolescents with corrected pulmonary atresia with intact ventricular septum after univentricular palliation and biventricular repair. J Thorac Cardiovasc Surg, 2012, 143(3): 569-575.
30. Karamlou T, Poynter JA, Walters HL 3rd, et al. Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: a Congenital Heart Surgeons Society study. J Thorac Cardiovasc Surg, 2013, 145(4): 1018-1027.

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