Chinese expert consensus on surgical treatment of congenital heart disease (8): Series of procedures for univentricular heart_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

 HUA Zhongdong ,  LI Shoujun

Department of Pediatric Cardiac Surgery, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100037, P.R.China;

Corresponding author：

HUA Zhongdong, Email: richardhua@yahoo.com; LI Shoujun, Email: drlishoujun@yahoo.com

Keywords：

Univentricular; standard treatment; palliation operation; surgery; expert consensus

DOI：

10.7507/1007-4848.202005074

Video：

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In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have significantly improved survival rate. However, the resulting Fontan physiology is associated with high morbidity. Despite large improvements in outcomes of contemporary Fontan patients, a large burden of disease exists in this patient population. In face of the imbalance of medical resources in China and different understanding of Fontan treatment in various regions, there is no consensus on the current status of Fontan strategy, surgical technique and perioperative treatment. By reviewing large amounts of literature, assessing survival rate and risk factors for mortality and complications of the Fontan circulation, knowledge sharing from tens of experts, we achieved a consensus on many aspects of palliation of Fontan. We hope this consensus will help Chinese colleagues further continue their efforts to improve surgical outcomes of the univentricular heart patients.

Citation： HUA Zhongdong, LI Shoujun. Chinese expert consensus on surgical treatment of congenital heart disease (8): Series of procedures for univentricular heart. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2020, 27(9): 979-986. doi: 10.7507/1007-4848.202005074 Copy

1.	Van Praagh R, Ongley PA, Swam HJC. Anatomic types of single or common ventricle in man. Morphologic and geometric aspects of 60 necropsied cases. Am J Cardiol, 1964, 13: 367.
2.	白凯, 苏肇伉, 金彪, 等. MRI评估对于不同手术阶段功能性单心室的功能. 中华胸心血管外科杂志, 2006, 22(6): 21-24.
3.	Jonas RA. Fontan or septation: when I abandon septation in complex lesions with two ventricles. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2009, 12(1): 94-98.
4.	吴清玉, 唐秀杰, 李洪银, 等. 分隔手术治疗单心室. 中华外科杂志, 2008, 46(6): 469-470.
5.	Bacha EA. Individualized approach in the management of patients with hypoplastic left heart syndrome (HLHS). Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2013, 16(1): 3-6.
6.	Trusler GA, Mustard WT. A method of banding the pulmonary artery for large isolated ventricular septal defect with and without transposition of the great arteries. Ann Thorac Surg, 1972, 13(4): 351.
7.	Albus RA, Trusler GA, Izukawa, et al. Pulmonary artery banding. J Thorac Cardiovasc Surg, 1984, 88: 645.
8.	刘承虎, 苏俊武, 李志强, 等. 肺动脉环缩术治疗不同年龄伴肺动脉高压单心室. 中华胸心血管外科杂志, 2012, 28(4): 219-222.
9.	Nuland SB, Glenn WWL, Guifoil PH. Circulatory bypass of the right heart. Ⅲ. Some observations on long-term survivors. Surgery, 1958, 43(2): 184-201.
10.	Patino JF, Glenn WWL, Guifoil PH, et al. Circulatory bypass of the right heart. Further observations on vena caval pulmonary artery shunts. Surg Forum, 1957, 6: 189.
11.	Glenn WWL, Patino JF. Circulatory bypass of the right heart. Preliminary observations on the direct delivery of vena caval blood into the pulmonary arterial circulation. Azygous vein pulmonary artery shunt. Yale J Biol Med, 1954, 27: 14.
12.	Gaynor JW, Collins MH, Rychik J, et al. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovsc Surg, 1999, 117(3): 506-514.
13.	Alsoufi B, Gillespie S, Kogon B, et al. Results of palliation with an initial modified Blalock-Taussig shunt in neonates with single ventricle anomalies associated with restrictive pulmonary blood flow. Ann Thorac Surg, 2015, 99(5): 1639-1647.
14.	Friedman KG, Salvin JW, Wypij D, et al. Risk factors for failed staged palliation after bidirectional Glenn in infants who have undergone stage one palliation. Eur J Cardiaothorac Surg, 2011, 40(14): 1000-1006.
15.	肖雅琼, 董念国, 刘金平, 等. 功能性单心室外科治疗及近期疗效. 中华小儿外科杂志, 2011, 32(4): 248-251.
16.	Bridges ND, Jonas RA, Mayer JE, et al. Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates. Early results. Circulation, 1990, 82(5 Suppl): 200-176.
17.	陈伟丹, 陈欣欣, 王武军. 单心室合并完全性肺静脉异位引流的外科治疗. 中国循环杂志, 2017, 32(10): 77-80.
18.	欧阳文斌, 潘湘斌, 张浩, 等. 单心室合并完全性肺静脉异位引流的外科治疗. 中国循环杂志, 2012, 27(10): 208-211.
19.	Choussat A, Fontan F, Besse P, et al. Selection criteria for Fontan procedure. In: Anderson RH, Shinebourne EA, editors. Pediatric cardiology. Edinburgh: Churchill Livingstone, 1978. 559-599.
20.	Fontan F, Deville C, Quaegebeur J, et al. Repair of tricuspid atresia in 100 patients. J Thorac Cardiovasc Surg, 1983, 85(5): 647.
21.	Vargas FJ, Mayer JE, Jonas RA, et al. Anomalous systemic and pulmonary venous connections in conjunction with atriopulmonary anastomosis (Fontan-Kreutzer). Technical considerations. J Thorac Cardiovasc Surg, 1987, 93(4): 523-532.
22.	Zhang T, Shi Y, Wu K, et al. Uncontrolled antegrade pulmonary blood flow and delayed Fontan completion after the bidirectional glenn procedure: real-world outcomes in China. Ann Thorac Surg, 2016, 101(4): 1530-1538.
23.	Yan T, Tong G, Zhang B, et al. The effect of antegrade pulmonary blood flow following a late bidirectional Glenn procedure. Interact Cardiovasc Thorac Surg, 2018, 26(3): 454-459.
24.	deLeval MR, Kilner P, Gewillig M, et al. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg, 1988, 96(5): 682-695.
25.	Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax, 1971, 26(3): 240-248.
26.	Ross DN. Replacement of aortic and mitral valve with a pulmonary autograft. Lancet, 1967, 2(7523): 956.
27.	Gentles TL, Mayer Jr JE, Gauvreau K, et al. Fontan operation in five hundred consecutive patients: factors influencing early and late outcome. J Thorac Cardiovasc Surg, 1997, 114(3): 376-391.
28.	Driscoll DJ, Offord KP, Feldt RH, et al. Five-to-fifteen-year follow-up after Fontan operation. Circulation, 1992, 85(2): 469-496.
29.	Fontan F, Kirklin JW, Fernandez G, et al. Outcome after a ‘perfect’ Fontan operation. Circulation, 1990, 81(5): 1520-1536.
30.	Kawashima Y, Kitamura S, Matsuda H, et al. Total cavopulmonary shunt operation in complex cardiac anomalies. J Thorac Cardiovasc Surg, 1984, 87(1): 74-81.
31.	Marcelletti C, Corno A, Giannico S, et al. Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg, 1990, 100(2): 228-232.
32.	Lin Z, Ge H, Xue J, et al. Comparison of extracardiac conduit and lateral tunnel for functional single-ventricle patients: A meta-analysis. Congenit Heart Dis, 2017, 12(6): 711-720.
33.	Zhu ZQ, Hong HF, Chen HW, et al. Intraatrial conduit Fontan procedure: indications, operative techniques, and clinical outcomes. Ann Thorac Surg, 2015, 99(1): 156-161.
34.	Croti UA, Braile DM, Godoy MFD, et al. Alternative to an extracardiac Fontan-type operation: direct anastomosis between pulmonary trunk and inferior vena cava. Braz J Cardiov Surg, 2008, 23(23): 439-441.
35.	Liu X, Yuan H, Chen J, et al. Outcomes following modified extracardiac Fontan procedure of direct total cavopulmonary connection with autologous vessels: a single-centre 10-year experience. Eur J Cardiothorac Surg, 2020, 57(4): 628-634.
36.	Bartz PJ, Driscoll DJ, Dearani JA, et al. Early and late results of the modified fontan operation for heterotaxy syndrome: 30 years of experience in 142 patients. J Am Coll Cardiol, 2006, 48(11): 2301-2305.
37.	Yi T, Fan G, Xing Y, et al. Impact of time interval between Glenn and Fontan procedures on Fontan operative and long-term follow-up results. Pediatr Cardiol, 2019, 40(4): 705-712.
38.	黄继红, 苏肇伉, 王亮君, 等. 单心室手术治疗内脏异位综合症早期死亡危险因素分析. 上海交通大学学报(医学版), 2011, 31(9): 1269-1271.
39.	Ramakrishnan K, Alfares FA, Hammond-Jack K, et al. Optimal timing of pulmonary banding for newborns with single ventricle physiology and unrestricted pulmonary blood flow. Pediatr Cardiol, 2016, 37(3): 606-609.
40.	Chen Q, Li S, Hua Z, et al. Anatomical repair conversion after bidirectional cavopulmonary shunt for complex cardiac anomalies: Palliation is not a one-way path. Pediatr Cardiol, 2018, 39(3): 604-609.

1. Van Praagh R, Ongley PA, Swam HJC. Anatomic types of single or common ventricle in man. Morphologic and geometric aspects of 60 necropsied cases. Am J Cardiol, 1964, 13: 367.
2. 白凯, 苏肇伉, 金彪, 等. MRI评估对于不同手术阶段功能性单心室的功能. 中华胸心血管外科杂志, 2006, 22(6): 21-24.
3. Jonas RA. Fontan or septation: when I abandon septation in complex lesions with two ventricles. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2009, 12(1): 94-98.
4. 吴清玉, 唐秀杰, 李洪银, 等. 分隔手术治疗单心室. 中华外科杂志, 2008, 46(6): 469-470.
5. Bacha EA. Individualized approach in the management of patients with hypoplastic left heart syndrome (HLHS). Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2013, 16(1): 3-6.
6. Trusler GA, Mustard WT. A method of banding the pulmonary artery for large isolated ventricular septal defect with and without transposition of the great arteries. Ann Thorac Surg, 1972, 13(4): 351.
7. Albus RA, Trusler GA, Izukawa, et al. Pulmonary artery banding. J Thorac Cardiovasc Surg, 1984, 88: 645.
8. 刘承虎, 苏俊武, 李志强, 等. 肺动脉环缩术治疗不同年龄伴肺动脉高压单心室. 中华胸心血管外科杂志, 2012, 28(4): 219-222.
9. Nuland SB, Glenn WWL, Guifoil PH. Circulatory bypass of the right heart. Ⅲ. Some observations on long-term survivors. Surgery, 1958, 43(2): 184-201.
10. Patino JF, Glenn WWL, Guifoil PH, et al. Circulatory bypass of the right heart. Further observations on vena caval pulmonary artery shunts. Surg Forum, 1957, 6: 189.
11. Glenn WWL, Patino JF. Circulatory bypass of the right heart. Preliminary observations on the direct delivery of vena caval blood into the pulmonary arterial circulation. Azygous vein pulmonary artery shunt. Yale J Biol Med, 1954, 27: 14.
12. Gaynor JW, Collins MH, Rychik J, et al. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovsc Surg, 1999, 117(3): 506-514.
13. Alsoufi B, Gillespie S, Kogon B, et al. Results of palliation with an initial modified Blalock-Taussig shunt in neonates with single ventricle anomalies associated with restrictive pulmonary blood flow. Ann Thorac Surg, 2015, 99(5): 1639-1647.
14. Friedman KG, Salvin JW, Wypij D, et al. Risk factors for failed staged palliation after bidirectional Glenn in infants who have undergone stage one palliation. Eur J Cardiaothorac Surg, 2011, 40(14): 1000-1006.
15. 肖雅琼, 董念国, 刘金平, 等. 功能性单心室外科治疗及近期疗效. 中华小儿外科杂志, 2011, 32(4): 248-251.
16. Bridges ND, Jonas RA, Mayer JE, et al. Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates. Early results. Circulation, 1990, 82(5 Suppl): 200-176.
17. 陈伟丹, 陈欣欣, 王武军. 单心室合并完全性肺静脉异位引流的外科治疗. 中国循环杂志, 2017, 32(10): 77-80.
18. 欧阳文斌, 潘湘斌, 张浩, 等. 单心室合并完全性肺静脉异位引流的外科治疗. 中国循环杂志, 2012, 27(10): 208-211.
19. Choussat A, Fontan F, Besse P, et al. Selection criteria for Fontan procedure. In: Anderson RH, Shinebourne EA, editors. Pediatric cardiology. Edinburgh: Churchill Livingstone, 1978. 559-599.
20. Fontan F, Deville C, Quaegebeur J, et al. Repair of tricuspid atresia in 100 patients. J Thorac Cardiovasc Surg, 1983, 85(5): 647.
21. Vargas FJ, Mayer JE, Jonas RA, et al. Anomalous systemic and pulmonary venous connections in conjunction with atriopulmonary anastomosis (Fontan-Kreutzer). Technical considerations. J Thorac Cardiovasc Surg, 1987, 93(4): 523-532.
22. Zhang T, Shi Y, Wu K, et al. Uncontrolled antegrade pulmonary blood flow and delayed Fontan completion after the bidirectional glenn procedure: real-world outcomes in China. Ann Thorac Surg, 2016, 101(4): 1530-1538.
23. Yan T, Tong G, Zhang B, et al. The effect of antegrade pulmonary blood flow following a late bidirectional Glenn procedure. Interact Cardiovasc Thorac Surg, 2018, 26(3): 454-459.
24. deLeval MR, Kilner P, Gewillig M, et al. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg, 1988, 96(5): 682-695.
25. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax, 1971, 26(3): 240-248.
26. Ross DN. Replacement of aortic and mitral valve with a pulmonary autograft. Lancet, 1967, 2(7523): 956.
27. Gentles TL, Mayer Jr JE, Gauvreau K, et al. Fontan operation in five hundred consecutive patients: factors influencing early and late outcome. J Thorac Cardiovasc Surg, 1997, 114(3): 376-391.
28. Driscoll DJ, Offord KP, Feldt RH, et al. Five-to-fifteen-year follow-up after Fontan operation. Circulation, 1992, 85(2): 469-496.
29. Fontan F, Kirklin JW, Fernandez G, et al. Outcome after a ‘perfect’ Fontan operation. Circulation, 1990, 81(5): 1520-1536.
30. Kawashima Y, Kitamura S, Matsuda H, et al. Total cavopulmonary shunt operation in complex cardiac anomalies. J Thorac Cardiovasc Surg, 1984, 87(1): 74-81.
31. Marcelletti C, Corno A, Giannico S, et al. Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg, 1990, 100(2): 228-232.
32. Lin Z, Ge H, Xue J, et al. Comparison of extracardiac conduit and lateral tunnel for functional single-ventricle patients: A meta-analysis. Congenit Heart Dis, 2017, 12(6): 711-720.
33. Zhu ZQ, Hong HF, Chen HW, et al. Intraatrial conduit Fontan procedure: indications, operative techniques, and clinical outcomes. Ann Thorac Surg, 2015, 99(1): 156-161.
34. Croti UA, Braile DM, Godoy MFD, et al. Alternative to an extracardiac Fontan-type operation: direct anastomosis between pulmonary trunk and inferior vena cava. Braz J Cardiov Surg, 2008, 23(23): 439-441.
35. Liu X, Yuan H, Chen J, et al. Outcomes following modified extracardiac Fontan procedure of direct total cavopulmonary connection with autologous vessels: a single-centre 10-year experience. Eur J Cardiothorac Surg, 2020, 57(4): 628-634.
36. Bartz PJ, Driscoll DJ, Dearani JA, et al. Early and late results of the modified fontan operation for heterotaxy syndrome: 30 years of experience in 142 patients. J Am Coll Cardiol, 2006, 48(11): 2301-2305.
37. Yi T, Fan G, Xing Y, et al. Impact of time interval between Glenn and Fontan procedures on Fontan operative and long-term follow-up results. Pediatr Cardiol, 2019, 40(4): 705-712.
38. 黄继红, 苏肇伉, 王亮君, 等. 单心室手术治疗内脏异位综合症早期死亡危险因素分析. 上海交通大学学报(医学版), 2011, 31(9): 1269-1271.
39. Ramakrishnan K, Alfares FA, Hammond-Jack K, et al. Optimal timing of pulmonary banding for newborns with single ventricle physiology and unrestricted pulmonary blood flow. Pediatr Cardiol, 2016, 37(3): 606-609.
40. Chen Q, Li S, Hua Z, et al. Anatomical repair conversion after bidirectional cavopulmonary shunt for complex cardiac anomalies: Palliation is not a one-way path. Pediatr Cardiol, 2018, 39(3): 604-609.

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Chinese expert consensus on surgical treatment of congenital heart disease (8): Series of procedures for univentricular heart

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