先天性心臟病外科治療中國專家共識（三）：肺動脈閉鎖合并室間隔缺損_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

 陳欣欣 ¹ ,  李守軍代表國家心血管病專家委員會先天性心臟病專業委員會

1. ;
2. ;

Corresponding?author：

陳欣欣, Email: zingerchen@163.com

Keywords：

外科治療; 先天性心臟病; 肺動脈閉鎖; 肺血管單源化; 專家共識

DOI：

10.7507/1007-4848.202002040

Video：

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Citation： 陳欣欣, 李守軍代表國家心血管病專家委員會先天性心臟病專業委員會. 先天性心臟病外科治療中國專家共識（三）：肺動脈閉鎖合并室間隔缺損. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2020, 27(4): 401-407. doi: 10.7507/1007-4848.202002040 Copy

1.	Chan KC, Fyfe DA, McKay CA, et al. Right ventricular outflow reconstruction with cryopreserved homografts in pediatric patients: intermediate-term follow-up with serial echocardio-graphic assessment. J Am Coll Cardiol, 1994, 24(2): 483-489.
2.	Amark KM, Karamlou T, O'Carroll A, 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(7): 1448-1456.
3.	Zheng S, Yang K, Li K, et al. Establishment of right ventricle-pulmonary artery continuity as the first-stage palliation in older infants with pulmonary atresia with ventricular septal defect may be preferable to use of an arterial shunt. Interact Cardiovasc Thorac Surg, 2014, 19(1): 88-94.
4.	Meinel FG, Huda W, Schoepf UJ, et al. Diagnostic accuracy of CT angiography in infants with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Cardiovasc Comput Tomogr, 2013, 7(6): 367-375.
5.	Mackie AS, Gauvreau K, Perry SB, et al. Echocardiographic predictors of aortopulmonary collaterals in infants with tetralogy of Fallot and pulmonary atresia. J Am Coll Cardiol, 2003, 41(5): 852-857.
6.	Asija R, Roth SJ, Hanley FL, et al. Reperfusion pulmonary edema in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries undergoing unifocalization procedures: A pilot study examining potential pathophysiologic mechanisms and clinical significance. J Thorac Cardiovasc Surg, 2014, 148(4): 1560-1565.
7.	Tchervenkov CI, Roy N. Congenital Heart Surgery Nomenclature and Database Project: pulmonary atresia--ventricular septal defect. Ann Thorac Surg, 2000, 69(4 Suppl): S97-S105.
8.	Barbero-Marcial M, Jatene AD. Surgical management of the anomalies of the pulmonary arteries in the tetralogy of Fallot with pulmonary atresia. Semin Thorac Cardiovasc Surg, 1990, 2(1): 93-107.
9.	Smyllie JH, Sutherland GR, Keeton BR. The value of Doppler color flow mapping in determining pulmonary blood supply in infants with pulmonary atresia with ventricular septal defect. J Am Coll Cardiol, 1989, 14(7): 1759-1765.
10.	McGoon DC, Baird DK, Davis GD. Surgical management of large bronchial collateral arteries with pulmonary stenosis or atresia. Circulation, 1975, 52(1): 109-118.
11.	Faletra F, Giardina A, Petroni R, et al. Evaluation of pulmonary atresia with 64-slice multidetector computed tomography (MDCT). Echocardiography, 2007, 24(9): 998-999.
12.	Ryan JR, Moe TG, Richardson R, et al. A novel approach to neonatal management of tetralogy of Fallot, with pulmonary atresia, and multiple aortopulmonary collaterals. JACC Cardiovasc Imaging, 2015, 8(1): 103-104.
13.	Mainwaring RD, Margetson TD, McCarthy P, et al. Measurement of residual collateral flow in pulmonary atresia with major aortopulmonary collaterals. Ann Thorac Surg, 2019, 108(1): 154-159.
14.	Reddy VM, Petrossian E, McElhinney DB, et al. One-stage complete unifocalization in infants: when should the ventricular septal defect be closed. J Thorac Cardiovasc Surg, 1997, 113(5): 858-868.
15.	Geva T, Greil GF, Marshall AC, et al. Gadolinium-enhanced 3-dimensional magnetic resonance angiography of pulmonary blood supply in patients with complex pulmonary stenosis or atresia: comparison with x-ray angiography. Circulation, 2002, 106(4): 473-8.
16.	Kersting-Sommerhoff BA, Sechtem UP, Higgins CB. Evaluation of pulmonary blood supply by nuclear magnetic resonance imaging in patients with pulmonary atresia. J Am Coll Cardiol, 1988, 11(1): 166-171.
17.	Baque J, Paul JF. Evaluation of pulmonary atresia with magnetic resonance imaging. Heart, 2002, 87(2): 159.
18.	Hofbeck M, Rauch A, Buheitel G, et al. Monosomy 22q11 in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Heart, 1998, 79(2): 180-185.
19.	Momma K, Kondo C, Matsuoka R. Tetralogy of Fallot with pulmonary atresia associated with chromosome 22q11 deletion. J Am Coll Cardiol, 1996, 27(1): 198-202.
20.	Bauser-Heaton H, Borquez A, Han B, et al. Programmatic approach to management of tetralogy of Fallot with major aortopulmonary collateral arteries: a 15-year experience with 458 patients. Circ Cardiovasc Interv, 2017, 10(4): e004952.
21.	Gerelli S, van Steenberghe M, Murtuza B, et al. Neonatal right ventricle to pulmonary connection as a palliative procedure for pulmonary atresia with ventricular septal defect or severe tetralogy of Fallot. Eur J Cardiothorac Surg, 2014, 45(2): 278-88.
22.	Kostolny M, Schreiber C, Hess J, et al. Successful primary correction of tetralogy of Fallot with pulmonary atresia and aortopulmonary window in a 2, 220-g neonate with a valved bovine jugular vein conduit. Her, 2004, 29(7): 710-712.
23.	Di Donato RM, Jonas RA, Lang P, et al. Neonatal repair of tetralogy of Fallot with and without pulmonary atresia. J Thorac Cardiovasc Surg, 1991, 101(1): 126-137.
24.	Watanabe N, Mainwaring RD, Reddy VM, et al. Early complete repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. Ann Thorac Surg, 2014, 97(3): 909-915.
25.	Mainwaring RD, Reddy VM, Perry SB, et al. Late outcomes in patients undergoing aortopulmonary window for pulmonary atresia/stenosis and major aortopulmonary collaterals. Ann Thorac Surg, 2012, 94(3): 842-848.
26.	Zhu J, Meza J, Kato A, et al. Pulmonary flow study predicts survival in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg, 2016, 152(6): 1494-1503.
27.	Kim H, Sung SC, Choi KH, et al. A central shunt to rehabilitate diminutive pulmonary arteries in patients with pulmonary atresia with ventricular septal defect. J Thorac Cardiovasc Surg, 2015, 149(2): 515-520.
28.	Soquet J, Liava'a M, Eastaugh L, et al. Achievements and limitations of a strategy of rehabilitation of native pulmonary vessels in pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Ann Thorac Surg, 2017, 103(5): 1519-1526.
29.	Reddy VM, McElhinney DB, Amin Z, et al. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. Circulation, 2000, 101(15): 1826-1832.
30.	Metras D, Chetaille P, Kreitmann B, et al. Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals. Eur J Cardiothorac Surg, 2001, 20(3): 590-597.
31.	Zahorec M, Hrubsova Z, Skrak P, et al. A comparison of Blalock-Taussig shunts with and without closure of the ductus arteriosus in neonates with pulmonary atresia. Ann Thorac Surg, 2011, 92(2): 653-658.
32.	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: 139-44.
33.	Mumtaz MA, Rosenthal G, Qureshi A, 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(6): 2079-2084.
34.	Duncan BW, Mee RB, Prieto LR, et al. Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg, 2003, 126(3): 694-702.
35.	Barozzi L, Brizard CP, Galati JC, et al. Side-to-side aorto-GoreTex central shunt warrants central shunt patency and pulmonary arteries growth. Ann Thorac Surg, 2011, 92(4): 1476-1482.
36.	Fouilloux V, Bonello B, Kammache I, et al. 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(12): 666-675.
37.	Yuan SM, Mishaly D, Shinfeld A, et al. Right ventricular outflow tract reconstruction: valved conduit of choice and clinical outcomes. J Cardiovasc Med (Hagerstown), 2008, 9(4): 327-337.
38.	Miyazaki T, Yamagishi M, Maeda Y, et al. Long-term outcomes of expanded polytetrafluoroethylene conduits with bulging sinuses and a fan-shaped valve in right ventricular outflow tract reconstruction. J Thorac Cardiovasc Surg, 2018, 155(6): 2567-2576.
39.	N?rgaard MA, Alphonso N, Cochrane AD, et al. Major aorto-pulmonary collateral arteries of patients with pulmonary atresia and ventricular septal defect are dilated bronchial arteries. Eur J Cardiothorac Surg, 2006, 29(5): 653-658.
40.	Carrillo SA, Mainwaring RD, Patrick WL, et al. Surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals with absent intrapericardial pulmonary arteries. Ann Thorac Surg, 2015, 100(2): 606-614.
41.	Davidson J, Tong S, Hancock H, et al. Prospective validation of the vasoactive-inotropic score and correlation to short-term outcomes in neonates and infants after cardiothoracic surgery. Intensive Care Med, 2012, 38(7): 1184-1190.
42.	Gaies MG, Jeffries HE, Niebler RA, et al. Vasoactive-inotropic score is associated with outcome after infant cardiac surgery: an analysis from the Pediatric Cardiac Critical Care Consortium and Virtual PICU System Registries. Pediatr Crit Care Med, 2014, 15(6): 529-537.
43.	Honjo O, Al-Radi OO, MacDonald C, 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(11 Suppl): S46-S52.
44.	Chetaille P, Fraisse A, Ghez O, et al. Rehabilitation of hypoplastic pulmonary arteries and anatomic correction of pulmonary atresia with interventricular communication. Arch Mal Coeur Vaiss, 2001, 94(5): 446-451.
45.	Schouvey S, Dragulescu A, Ghez O, et al. Rehabilitation of hypoplastic pulmonary arteries in pulmonary atresia with ventricular septal defect. Medium term results. Arch Mal Coeur Vaiss, 2007, 100(5): 422-427.

1. Chan KC, Fyfe DA, McKay CA, et al. Right ventricular outflow reconstruction with cryopreserved homografts in pediatric patients: intermediate-term follow-up with serial echocardio-graphic assessment. J Am Coll Cardiol, 1994, 24(2): 483-489.
2. Amark KM, Karamlou T, O'Carroll A, 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(7): 1448-1456.
3. Zheng S, Yang K, Li K, et al. Establishment of right ventricle-pulmonary artery continuity as the first-stage palliation in older infants with pulmonary atresia with ventricular septal defect may be preferable to use of an arterial shunt. Interact Cardiovasc Thorac Surg, 2014, 19(1): 88-94.
4. Meinel FG, Huda W, Schoepf UJ, et al. Diagnostic accuracy of CT angiography in infants with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Cardiovasc Comput Tomogr, 2013, 7(6): 367-375.
5. Mackie AS, Gauvreau K, Perry SB, et al. Echocardiographic predictors of aortopulmonary collaterals in infants with tetralogy of Fallot and pulmonary atresia. J Am Coll Cardiol, 2003, 41(5): 852-857.
6. Asija R, Roth SJ, Hanley FL, et al. Reperfusion pulmonary edema in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries undergoing unifocalization procedures: A pilot study examining potential pathophysiologic mechanisms and clinical significance. J Thorac Cardiovasc Surg, 2014, 148(4): 1560-1565.
7. Tchervenkov CI, Roy N. Congenital Heart Surgery Nomenclature and Database Project: pulmonary atresia--ventricular septal defect. Ann Thorac Surg, 2000, 69(4 Suppl): S97-S105.
8. Barbero-Marcial M, Jatene AD. Surgical management of the anomalies of the pulmonary arteries in the tetralogy of Fallot with pulmonary atresia. Semin Thorac Cardiovasc Surg, 1990, 2(1): 93-107.
9. Smyllie JH, Sutherland GR, Keeton BR. The value of Doppler color flow mapping in determining pulmonary blood supply in infants with pulmonary atresia with ventricular septal defect. J Am Coll Cardiol, 1989, 14(7): 1759-1765.
10. McGoon DC, Baird DK, Davis GD. Surgical management of large bronchial collateral arteries with pulmonary stenosis or atresia. Circulation, 1975, 52(1): 109-118.
11. Faletra F, Giardina A, Petroni R, et al. Evaluation of pulmonary atresia with 64-slice multidetector computed tomography (MDCT). Echocardiography, 2007, 24(9): 998-999.
12. Ryan JR, Moe TG, Richardson R, et al. A novel approach to neonatal management of tetralogy of Fallot, with pulmonary atresia, and multiple aortopulmonary collaterals. JACC Cardiovasc Imaging, 2015, 8(1): 103-104.
13. Mainwaring RD, Margetson TD, McCarthy P, et al. Measurement of residual collateral flow in pulmonary atresia with major aortopulmonary collaterals. Ann Thorac Surg, 2019, 108(1): 154-159.
14. Reddy VM, Petrossian E, McElhinney DB, et al. One-stage complete unifocalization in infants: when should the ventricular septal defect be closed. J Thorac Cardiovasc Surg, 1997, 113(5): 858-868.
15. Geva T, Greil GF, Marshall AC, et al. Gadolinium-enhanced 3-dimensional magnetic resonance angiography of pulmonary blood supply in patients with complex pulmonary stenosis or atresia: comparison with x-ray angiography. Circulation, 2002, 106(4): 473-8.
16. Kersting-Sommerhoff BA, Sechtem UP, Higgins CB. Evaluation of pulmonary blood supply by nuclear magnetic resonance imaging in patients with pulmonary atresia. J Am Coll Cardiol, 1988, 11(1): 166-171.
17. Baque J, Paul JF. Evaluation of pulmonary atresia with magnetic resonance imaging. Heart, 2002, 87(2): 159.
18. Hofbeck M, Rauch A, Buheitel G, et al. Monosomy 22q11 in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Heart, 1998, 79(2): 180-185.
19. Momma K, Kondo C, Matsuoka R. Tetralogy of Fallot with pulmonary atresia associated with chromosome 22q11 deletion. J Am Coll Cardiol, 1996, 27(1): 198-202.
20. Bauser-Heaton H, Borquez A, Han B, et al. Programmatic approach to management of tetralogy of Fallot with major aortopulmonary collateral arteries: a 15-year experience with 458 patients. Circ Cardiovasc Interv, 2017, 10(4): e004952.
21. Gerelli S, van Steenberghe M, Murtuza B, et al. Neonatal right ventricle to pulmonary connection as a palliative procedure for pulmonary atresia with ventricular septal defect or severe tetralogy of Fallot. Eur J Cardiothorac Surg, 2014, 45(2): 278-88.
22. Kostolny M, Schreiber C, Hess J, et al. Successful primary correction of tetralogy of Fallot with pulmonary atresia and aortopulmonary window in a 2, 220-g neonate with a valved bovine jugular vein conduit. Her, 2004, 29(7): 710-712.
23. Di Donato RM, Jonas RA, Lang P, et al. Neonatal repair of tetralogy of Fallot with and without pulmonary atresia. J Thorac Cardiovasc Surg, 1991, 101(1): 126-137.
24. Watanabe N, Mainwaring RD, Reddy VM, et al. Early complete repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. Ann Thorac Surg, 2014, 97(3): 909-915.
25. Mainwaring RD, Reddy VM, Perry SB, et al. Late outcomes in patients undergoing aortopulmonary window for pulmonary atresia/stenosis and major aortopulmonary collaterals. Ann Thorac Surg, 2012, 94(3): 842-848.
26. Zhu J, Meza J, Kato A, et al. Pulmonary flow study predicts survival in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg, 2016, 152(6): 1494-1503.
27. Kim H, Sung SC, Choi KH, et al. A central shunt to rehabilitate diminutive pulmonary arteries in patients with pulmonary atresia with ventricular septal defect. J Thorac Cardiovasc Surg, 2015, 149(2): 515-520.
28. Soquet J, Liava'a M, Eastaugh L, et al. Achievements and limitations of a strategy of rehabilitation of native pulmonary vessels in pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Ann Thorac Surg, 2017, 103(5): 1519-1526.
29. Reddy VM, McElhinney DB, Amin Z, et al. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. Circulation, 2000, 101(15): 1826-1832.
30. Metras D, Chetaille P, Kreitmann B, et al. Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals. Eur J Cardiothorac Surg, 2001, 20(3): 590-597.
31. Zahorec M, Hrubsova Z, Skrak P, et al. A comparison of Blalock-Taussig shunts with and without closure of the ductus arteriosus in neonates with pulmonary atresia. Ann Thorac Surg, 2011, 92(2): 653-658.
32. 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: 139-44.
33. Mumtaz MA, Rosenthal G, Qureshi A, 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(6): 2079-2084.
34. Duncan BW, Mee RB, Prieto LR, et al. Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg, 2003, 126(3): 694-702.
35. Barozzi L, Brizard CP, Galati JC, et al. Side-to-side aorto-GoreTex central shunt warrants central shunt patency and pulmonary arteries growth. Ann Thorac Surg, 2011, 92(4): 1476-1482.
36. Fouilloux V, Bonello B, Kammache I, et al. 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(12): 666-675.
37. Yuan SM, Mishaly D, Shinfeld A, et al. Right ventricular outflow tract reconstruction: valved conduit of choice and clinical outcomes. J Cardiovasc Med (Hagerstown), 2008, 9(4): 327-337.
38. Miyazaki T, Yamagishi M, Maeda Y, et al. Long-term outcomes of expanded polytetrafluoroethylene conduits with bulging sinuses and a fan-shaped valve in right ventricular outflow tract reconstruction. J Thorac Cardiovasc Surg, 2018, 155(6): 2567-2576.
39. N?rgaard MA, Alphonso N, Cochrane AD, et al. Major aorto-pulmonary collateral arteries of patients with pulmonary atresia and ventricular septal defect are dilated bronchial arteries. Eur J Cardiothorac Surg, 2006, 29(5): 653-658.
40. Carrillo SA, Mainwaring RD, Patrick WL, et al. Surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals with absent intrapericardial pulmonary arteries. Ann Thorac Surg, 2015, 100(2): 606-614.
41. Davidson J, Tong S, Hancock H, et al. Prospective validation of the vasoactive-inotropic score and correlation to short-term outcomes in neonates and infants after cardiothoracic surgery. Intensive Care Med, 2012, 38(7): 1184-1190.
42. Gaies MG, Jeffries HE, Niebler RA, et al. Vasoactive-inotropic score is associated with outcome after infant cardiac surgery: an analysis from the Pediatric Cardiac Critical Care Consortium and Virtual PICU System Registries. Pediatr Crit Care Med, 2014, 15(6): 529-537.
43. Honjo O, Al-Radi OO, MacDonald C, 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(11 Suppl): S46-S52.
44. Chetaille P, Fraisse A, Ghez O, et al. Rehabilitation of hypoplastic pulmonary arteries and anatomic correction of pulmonary atresia with interventricular communication. Arch Mal Coeur Vaiss, 2001, 94(5): 446-451.
45. Schouvey S, Dragulescu A, Ghez O, et al. Rehabilitation of hypoplastic pulmonary arteries in pulmonary atresia with ventricular septal defect. Medium term results. Arch Mal Coeur Vaiss, 2007, 100(5): 422-427.

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先天性心臟病外科治療中國專家共識（三）：肺動脈閉鎖合并室間隔缺損

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