Chinese expert consensus on surgical treatment of congenital heart disease (7): Double outlet right ventricle_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

ZHANG Benqing , MA Kai ,  LI Shoujun, Representatives of National Society of Congenital Heart Disease

Department of Pediatric Cardiac Surgery, Fuwai Hospital, Beijing Union Medical College, Chinese Academy of Medical Sciences, 100037, Beijing, P.R.China;

Corresponding author：

LI Shoujun, Representatives of National Society of Congenital Heart Disease, Email: drlishoujun@yahoo.com

Keywords：

Surgery; congenital heart disease; double outlet right ventricle; treatment; expert consensus

DOI：

10.7507/1007-4848.202004029

Video：

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Double outlet right ventricle (DORV) is a complex cardiac malformation with many anatomic variations and various approaches for surgical repair. DORV is mainly defined as the congenital heart disease with ventriculoarterial connection in which both pulmonary artery and aorta arising primarily (>50%) from the right ventricle, associated with continuity or discontinuity between the aorta and mitral valve. DORV can be subclassified by various ways. Now subclassification is usually performed according to the relationship between the ventricular septal defect (VSD) and the great arteries. Various approaches for surgical repair of DORV ranging from single ventricle palliation to biventricular repair are reported from many centers. However, the high-grade guideline of surgical management of DORV is still absent. Hence, we developed the Chinese expert consensus on DORV as the evidence for surgical strategies.

Citation： ZHANG Benqing, MA Kai, LI Shoujun, Representatives of National Society of Congenital Heart Disease. Chinese expert consensus on surgical treatment of congenital heart disease (7): Double outlet right ventricle. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2020, 27(8): 851-856. doi: 10.7507/1007-4848.202004029 Copy

1.	Stewart S. Double-outlet right ventricle. A collective review with a surgical viewpoint. J Thorac Cardiovasc Surg, 1976, 71(3): 355-365.
2.	Walters HL 3rd, Mavroudis C, Tchervenkov CI, et al. Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle. Ann Thorac Surg, 2000, 69(4 Suppl): S249-S263.
3.	Kirby ML, Waldo KL. Role of neural crest in congenital heart disease. Circulation, 1990, 82(2): 332-340.
4.	Loffredo CA. Epidemiology of cardiovascular malformations: prevalence and risk factors. Am J Med Genet, 2000, 97(4): 319-325.
5.	Ebadi A, Spicer DE, Backer CL, et al. Double-outlet right ventricle revisited. J Thorac Cardiovasc Surg, 2017, 154(2): 598-604.
6.	Van Praagh R. The importance of segmental situs in the diagnosis of congenital heart disease. Semin Roentgenol, 1985, 20(3): 254-271.
7.	Abdulla R. The sgmental approach to the diagnosis of congenital heart disease. Pediatr Cardiol, 2000, 21(2): 118.
8.	Van Praagh R. Normally and abnormally related great arteries: what have we learned? World J Pediatr Congenital Heart Surg, 2010, 1: 364-85.
9.	Lev M, Bharati S, Meng CC, et al. A concept of double-outlet right ventricle. J Thorac Cardiovasc Surg, 1972, 64(2): 271-281.
10.	Anderson RH, Becker AE, Wilcox BR, et al. Surgical anatomy of double-outlet right ventricle--a reappraisal. Am J Cardiol, 1983, 52(5): 555-559.
11.	Lacour-Gayet F, Maruszewski B, Mavroudis C, et al. Presentation of the International Nomenclature for Congenital Heart Surgery. The long way from nomenclature to collection of validated data at the EACTS. Eur J Cardiothorac Surg, 2000, 18(2): 128-135.
12.	Franklin RC, Anderson HR, Daniëls O, et al. Report of the Coding Committee of the Association for European Paediatric Cardiology. Cardiol Young, 1999, 9(6): 633-658.
13.	Kirklin JW, Pacifico AD, Blackstone EH, et al. Current risks and protocols for operations for double-outlet right ventricle. Derivation from an 18 year experience. J Thorac Cardiovasc Surg, 1986, 92(5): 913-930.
14.	Musumeci F, Shumway S, Lincoln C, et al. Surgical treatment for double-outlet right ventricle at the Brompton Hospital, 1973 to 1986. J Thorac Cardiovasc Surg, 1988, 96(2): 278-287.
15.	Lacour-Gayet F. Intracardiac repair of double outlet right ventricle. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2008: 39-43.
16.	Jonas RA, Chief editor. Comprehensive Surgical Management of Congenital Heart Disease. Boca Raton: CRC Press, 2004. 413-428.
17.	逄坤静, 孟红, 王浩, 等. 先天性右心室双出口的新分型方法及其对术式选择的指导作用. 中华心血管病杂志, 2015, 43(11): 969-974.
18.	逄坤静, 王浩. 超声心动图评价大动脉调转术患者冠状动脉解剖类型. 中华超声影像学杂志, 2009, 18(3): 189-193.
19.	吴健, 马延贺, 张洪. 双源 CT 在诊断右心室双出口中的应用. 放射学实践, 2015, 30(3): 245-249.
20.	花中东, 杨新令, 刘凯飏, 等. 应用 3D 打印技术改进远离型右心室双出口的外科治疗结果. 中国胸心血管外科临床杂志, 2016, 23(6): 532-536.
21.	Forte MNV, Hussain T, Roest A, et al. Living the heart in three dimensions: applications of 3D printing in CHD. Cardiol Young, 2019, 29(6): 733-743.
22.	Bogren HG, Bürsch JH. Digital angiography in the diagnosis of congenital heart disease. Cardiovasc IntervRadiol, 1984, 7(3-4): 180-191.
23.	Lacour-Gayet F, Belli E, Ghez O, Chief editors. Surgery of Conotruncal Anomalies. Berlin: Springer International Publishing, 2016. 269-282.
24.	高伟, 顾红, 胡大一, 等. 2015 年先天性心脏病相关性肺动脉高压诊治中国专家共识. 中国介入心脏病学杂志, 2015, 23(2): 61-69.
25.	黄智伟, 柳志红. 先天性心脏病相关性肺动脉高压的诊治现状和挑战. 心血管病学进展, 2014, 35(4): 427-432.
26.	Downing DF. Cardiac catheterization in congenital heart disease. J Am Med Assoc, 1959, 170(7): 770-772.
27.	Gearhart AS, Raymundo SA, Chang AC. Echocardiographic MRI: an innovative fusion of functional and anatomic assessment strategy for CHD. Cardiol Young, 2019, 29(1): 88-89.
28.	Oladunjoye O, Piekarski B, Baird C, et al. Repair of double outlet right ventricle: Midterm outcomes. J Thorac Cardiovasc Surg, 2019: S0022-5223(19)31694-0.
29.	Li S, Ma K, Hu S, et al. Surgical outcomes of 380 patients with double outlet right ventricle who underwent biventricular repair. J Thorac Cardiovasc Surg, 2014, 148(3): 817-824.
30.	Soszyn N, Fricke TA, Wheaton GR, et al. Outcomes of the arterial switch operation in patients with Taussig-Bing anomaly. Ann Thorac Surg, 2011, 92(2): 673-679.
31.	Takeuchi K, McGowan FX Jr, Moran AM, et al. Surgical outcome of double-outlet right ventricle with subpulmonary VSD. Ann Thorac Surg, 2001, 71(1): 49-52.
32.	Wetter J, Sinzobahamvya N, Blaschczok HC, et al. Results of arterial switch operation for primary total correction of the Taussig-Bing anomaly. Ann Thorac Surg, 2004, 77(1): 41-46.
33.	Hu S, Xie Y, Li S, et al. Double-root translocation for double-outlet right ventricle with noncommitted ventricular septal defect or double-outlet right ventricle with subpulmonary ventricular septal defect associated with pulmonary stenosis: an optimized solution. Ann Thorac Surg, 2010, 89(5): 1360-1365.
34.	宋杰, 张达雄. 改良 Nikaidoh 术治疗大动脉转位或右心室双出口合并室间隔缺损、肺动脉狭窄的早中期结果. 国际心血管病杂志, 2018, 45(2): 94-97.
35.	李守军, 马凯, 花中东, 等. 远离型右心室双出口双心室矫治中期结果分析. 中国循环杂志, 2014, 24(z1): 121.
36.	Li S, Ma K, Hu S, et al. Biventricular repair for double outlet right ventricle with non-committed ventricular septal defect. Eur J Cardiothorac Surg, 2015, 48(4): 580-587.
37.	Lu T, Li J, Hu J, et al. Biventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect using intraventricular conduit. J Thorac Cardiovasc Surg, 2019: S0022-5223(19)31637-X.
38.	Belli E, Serraf A, Lacour-Gayet F, et al. Double-outlet right ventricle with non-committed ventricular septal defect. Eur J Cardiothorac Surg, 1999, 15(6): 747-752.
39.	Barbero-Marcial M, Tanamati C, Atik E, et al. Intraventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect: advantages of multiple patches. J Thorac Cardiovasc Surg, 1999, 118(6): 1056-1067.
40.	张惠丽, 李守军, 胡盛寿, 等. 室间隔缺损远离两大动脉开口右心室双出口的中期随访结果. 中国循环杂志, 2014, 29(z1): 125-126.
41.	Brauner R, Laks H, Drinkwater DC Jr, et al. Benefits of early surgical repair in fixed subaortic stenosis. J Am Coll Cardiol, 1997, 30(7): 1835-1842.
42.	Backer CL. Commentary: Double-outlet right ventricle revisited. J Thorac Cardiovasc Surg, 2019: S0022-5223(19)31720-9.
43.	Hörer J, Schreiber C, Dworak E, et al. Long-term results after the Rastelli repair for transposition of the great arteries. Ann Thorac Surg, 2007, 83(6): 2169-2175.
44.	Wu Q, Yu Q, Yang X. Modified Rastelli procedure for double outlet right ventricle with left-malposition of the great arteries: report of 9 cases. Ann Thorac Surg, 2003, 75(1): 138-142.
45.	Alwi M. ECMO in children post cardiac surgery-opportunity for redress. Anatol J Cardiol, 2017, 18(6): 431-432.
46.	Villemain O, Belli E, Ladouceur M, et al. Impact of anatomic characteristics and initial biventricular surgical strategy on outcomes in various forms of double-outlet right ventricle. J Thorac Cardiovasc Surg, 2016, 152(3): 698-706.
47.	Fraser CD Jr, Chacon-Portillo MA, Well A, et al. Twenty-three-year experience with the arterial switch operation: expectations and long-term outcomes. Semin Thorac Cardiovasc Surg, 2020: S1043-0679(20)30004-6.
48.	Brown JW, Ruzmetov M, Okada Y, et al. Surgical results in patients with double outlet right ventricle: a 20-year experience. Ann Thorac Surg, 2001, 72(5): 1630-1635.
49.	Artrip JH, Sauer H, Campbell DN, et al. Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification. Eur J Cardiothorac Surg, 2006, 29(4): 545-550.
50.	Villemain O, Bonnet D, Houyel L, et al. Double-outlet right ventricle with noncommitted ventricular septal defect and 2 adequate ventricles: Is anatomical repair advantageous? Semin Thorac Cardiovasc Surg, 2016, 28(1): 69-77.

1. Stewart S. Double-outlet right ventricle. A collective review with a surgical viewpoint. J Thorac Cardiovasc Surg, 1976, 71(3): 355-365.
2. Walters HL 3rd, Mavroudis C, Tchervenkov CI, et al. Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle. Ann Thorac Surg, 2000, 69(4 Suppl): S249-S263.
3. Kirby ML, Waldo KL. Role of neural crest in congenital heart disease. Circulation, 1990, 82(2): 332-340.
4. Loffredo CA. Epidemiology of cardiovascular malformations: prevalence and risk factors. Am J Med Genet, 2000, 97(4): 319-325.
5. Ebadi A, Spicer DE, Backer CL, et al. Double-outlet right ventricle revisited. J Thorac Cardiovasc Surg, 2017, 154(2): 598-604.
6. Van Praagh R. The importance of segmental situs in the diagnosis of congenital heart disease. Semin Roentgenol, 1985, 20(3): 254-271.
7. Abdulla R. The sgmental approach to the diagnosis of congenital heart disease. Pediatr Cardiol, 2000, 21(2): 118.
8. Van Praagh R. Normally and abnormally related great arteries: what have we learned? World J Pediatr Congenital Heart Surg, 2010, 1: 364-85.
9. Lev M, Bharati S, Meng CC, et al. A concept of double-outlet right ventricle. J Thorac Cardiovasc Surg, 1972, 64(2): 271-281.
10. Anderson RH, Becker AE, Wilcox BR, et al. Surgical anatomy of double-outlet right ventricle--a reappraisal. Am J Cardiol, 1983, 52(5): 555-559.
11. Lacour-Gayet F, Maruszewski B, Mavroudis C, et al. Presentation of the International Nomenclature for Congenital Heart Surgery. The long way from nomenclature to collection of validated data at the EACTS. Eur J Cardiothorac Surg, 2000, 18(2): 128-135.
12. Franklin RC, Anderson HR, Daniëls O, et al. Report of the Coding Committee of the Association for European Paediatric Cardiology. Cardiol Young, 1999, 9(6): 633-658.
13. Kirklin JW, Pacifico AD, Blackstone EH, et al. Current risks and protocols for operations for double-outlet right ventricle. Derivation from an 18 year experience. J Thorac Cardiovasc Surg, 1986, 92(5): 913-930.
14. Musumeci F, Shumway S, Lincoln C, et al. Surgical treatment for double-outlet right ventricle at the Brompton Hospital, 1973 to 1986. J Thorac Cardiovasc Surg, 1988, 96(2): 278-287.
15. Lacour-Gayet F. Intracardiac repair of double outlet right ventricle. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2008: 39-43.
16. Jonas RA, Chief editor. Comprehensive Surgical Management of Congenital Heart Disease. Boca Raton: CRC Press, 2004. 413-428.
17. 逄坤静, 孟红, 王浩, 等. 先天性右心室双出口的新分型方法及其对术式选择的指导作用. 中华心血管病杂志, 2015, 43(11): 969-974.
18. 逄坤静, 王浩. 超声心动图评价大动脉调转术患者冠状动脉解剖类型. 中华超声影像学杂志, 2009, 18(3): 189-193.
19. 吴健, 马延贺, 张洪. 双源 CT 在诊断右心室双出口中的应用. 放射学实践, 2015, 30(3): 245-249.
20. 花中东, 杨新令, 刘凯飏, 等. 应用 3D 打印技术改进远离型右心室双出口的外科治疗结果. 中国胸心血管外科临床杂志, 2016, 23(6): 532-536.
21. Forte MNV, Hussain T, Roest A, et al. Living the heart in three dimensions: applications of 3D printing in CHD. Cardiol Young, 2019, 29(6): 733-743.
22. Bogren HG, Bürsch JH. Digital angiography in the diagnosis of congenital heart disease. Cardiovasc IntervRadiol, 1984, 7(3-4): 180-191.
23. Lacour-Gayet F, Belli E, Ghez O, Chief editors. Surgery of Conotruncal Anomalies. Berlin: Springer International Publishing, 2016. 269-282.
24. 高伟, 顾红, 胡大一, 等. 2015 年先天性心脏病相关性肺动脉高压诊治中国专家共识. 中国介入心脏病学杂志, 2015, 23(2): 61-69.
25. 黄智伟, 柳志红. 先天性心脏病相关性肺动脉高压的诊治现状和挑战. 心血管病学进展, 2014, 35(4): 427-432.
26. Downing DF. Cardiac catheterization in congenital heart disease. J Am Med Assoc, 1959, 170(7): 770-772.
27. Gearhart AS, Raymundo SA, Chang AC. Echocardiographic MRI: an innovative fusion of functional and anatomic assessment strategy for CHD. Cardiol Young, 2019, 29(1): 88-89.
28. Oladunjoye O, Piekarski B, Baird C, et al. Repair of double outlet right ventricle: Midterm outcomes. J Thorac Cardiovasc Surg, 2019: S0022-5223(19)31694-0.
29. Li S, Ma K, Hu S, et al. Surgical outcomes of 380 patients with double outlet right ventricle who underwent biventricular repair. J Thorac Cardiovasc Surg, 2014, 148(3): 817-824.
30. Soszyn N, Fricke TA, Wheaton GR, et al. Outcomes of the arterial switch operation in patients with Taussig-Bing anomaly. Ann Thorac Surg, 2011, 92(2): 673-679.
31. Takeuchi K, McGowan FX Jr, Moran AM, et al. Surgical outcome of double-outlet right ventricle with subpulmonary VSD. Ann Thorac Surg, 2001, 71(1): 49-52.
32. Wetter J, Sinzobahamvya N, Blaschczok HC, et al. Results of arterial switch operation for primary total correction of the Taussig-Bing anomaly. Ann Thorac Surg, 2004, 77(1): 41-46.
33. Hu S, Xie Y, Li S, et al. Double-root translocation for double-outlet right ventricle with noncommitted ventricular septal defect or double-outlet right ventricle with subpulmonary ventricular septal defect associated with pulmonary stenosis: an optimized solution. Ann Thorac Surg, 2010, 89(5): 1360-1365.
34. 宋杰, 张达雄. 改良 Nikaidoh 术治疗大动脉转位或右心室双出口合并室间隔缺损、肺动脉狭窄的早中期结果. 国际心血管病杂志, 2018, 45(2): 94-97.
35. 李守军, 马凯, 花中东, 等. 远离型右心室双出口双心室矫治中期结果分析. 中国循环杂志, 2014, 24(z1): 121.
36. Li S, Ma K, Hu S, et al. Biventricular repair for double outlet right ventricle with non-committed ventricular septal defect. Eur J Cardiothorac Surg, 2015, 48(4): 580-587.
37. Lu T, Li J, Hu J, et al. Biventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect using intraventricular conduit. J Thorac Cardiovasc Surg, 2019: S0022-5223(19)31637-X.
38. Belli E, Serraf A, Lacour-Gayet F, et al. Double-outlet right ventricle with non-committed ventricular septal defect. Eur J Cardiothorac Surg, 1999, 15(6): 747-752.
39. Barbero-Marcial M, Tanamati C, Atik E, et al. Intraventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect: advantages of multiple patches. J Thorac Cardiovasc Surg, 1999, 118(6): 1056-1067.
40. 张惠丽, 李守军, 胡盛寿, 等. 室间隔缺损远离两大动脉开口右心室双出口的中期随访结果. 中国循环杂志, 2014, 29(z1): 125-126.
41. Brauner R, Laks H, Drinkwater DC Jr, et al. Benefits of early surgical repair in fixed subaortic stenosis. J Am Coll Cardiol, 1997, 30(7): 1835-1842.
42. Backer CL. Commentary: Double-outlet right ventricle revisited. J Thorac Cardiovasc Surg, 2019: S0022-5223(19)31720-9.
43. Hörer J, Schreiber C, Dworak E, et al. Long-term results after the Rastelli repair for transposition of the great arteries. Ann Thorac Surg, 2007, 83(6): 2169-2175.
44. Wu Q, Yu Q, Yang X. Modified Rastelli procedure for double outlet right ventricle with left-malposition of the great arteries: report of 9 cases. Ann Thorac Surg, 2003, 75(1): 138-142.
45. Alwi M. ECMO in children post cardiac surgery-opportunity for redress. Anatol J Cardiol, 2017, 18(6): 431-432.
46. Villemain O, Belli E, Ladouceur M, et al. Impact of anatomic characteristics and initial biventricular surgical strategy on outcomes in various forms of double-outlet right ventricle. J Thorac Cardiovasc Surg, 2016, 152(3): 698-706.
47. Fraser CD Jr, Chacon-Portillo MA, Well A, et al. Twenty-three-year experience with the arterial switch operation: expectations and long-term outcomes. Semin Thorac Cardiovasc Surg, 2020: S1043-0679(20)30004-6.
48. Brown JW, Ruzmetov M, Okada Y, et al. Surgical results in patients with double outlet right ventricle: a 20-year experience. Ann Thorac Surg, 2001, 72(5): 1630-1635.
49. Artrip JH, Sauer H, Campbell DN, et al. Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification. Eur J Cardiothorac Surg, 2006, 29(4): 545-550.
50. Villemain O, Bonnet D, Houyel L, et al. Double-outlet right ventricle with noncommitted ventricular septal defect and 2 adequate ventricles: Is anatomical repair advantageous? Semin Thorac Cardiovasc Surg, 2016, 28(1): 69-77.

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Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Chinese expert consensus on surgical treatment of congenital heart disease (7): Double outlet right ventricle

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