A comparative study of three palliative surgical approaches for pulmonary atresia with ventricular septal defect_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

SONG Zhiying ,  ZHENG Jinghao , HE Xiaomin , LUO Kai , SUN Qi , CHEN Huiwen , ZHU Zhongqun , ZHANG Hao , XU Zhiwei , LIU Jinfen

Department of Cardiothoracic Surgery, Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, P. R. China;

Corresponding?author：

ZHENG Jinghao, Email: zhengjh210@163.com

Keywords：

Pulmonary atresia with ventricular septal defect; systemic-pulmonary shunt; right ventricle-pulmonary artery connection

DOI：

10.7507/1007-4848.202102069

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Objective To compare and investigate the efficacy and differences of modified B-T shunt, central shunt and right ventricle-pulmonary artery (RV-PA) connection in the treatment of pulmonary atresia with ventricular septal defect (PA/VSD).Methods A total of 124 children with PA/VSD underwent initial palliative repair in Shanghai Children's Medical Center from September 2014 to August 2019, including 63 males and 61 females, aged 7 days to 15 years. They were divided into in a modified B-T shunt group (55 patients), a central shunt group (22 patients) and a RV-PA connection group (47 patients). The clinical data of these children were retrospectively analyzed.Results There were 9 early deaths after palliation, with an early mortality rate of 7.3%. The mean follow-up time was 26.5±20.3 months, with 5 patients lost to follow-up, 5 deaths during the follow-up period, and 105 survivors. The 1-year and 5-year survival rates were both 89.7%. The monthly increased Nakata index was 5.2 (–0.2, 12.3) mm²/m², 9.2 (0.1, 23.6) mm²/m², 6.3 (1.8, 23.3) mm²/m² in the modified B-T shunt group, the central shunt group, and the RV-PA connection group, respectively, with no statistical difference among the three groups. The 1-year survival rate was 85.3%, 78.4%, 95.2%, and the 5-year (4-year in the central shunt group) survival rate was 85.3%, 58.8%, 95.2% in the three groups, respectively, with a statistical difference among them (P<0.05). The complete repair rate was 36.5%, 19.0% and 67.4% in the three groups, respectively, with a statistical difference among the three groups (P<0.001).Conclusion All these three palliative surgical approaches can effectively promote pulmonary vascular development. But compared with systemic-pulmonary shunt, RV-PA connection has a lower perioperative mortality rate and can achieve a higher complete repair rate at a later stage, which is beneficial for long-term prognosis.

Citation： SONG Zhiying, ZHENG Jinghao, HE Xiaomin, LUO Kai, SUN Qi, CHEN Huiwen, ZHU Zhongqun, ZHANG Hao, XU Zhiwei, LIU Jinfen. A comparative study of three palliative surgical approaches for pulmonary atresia with ventricular septal defect. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2023, 30(2): 266-272. doi: 10.7507/1007-4848.202102069 Copy

1.	d'Udekem Y, Alphonso N, N?rgaard MA, 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(6): 1496-1502.
2.	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.
3.	Hobbes B, d'Udekem Y, Zannino D, et al. Determinants of adverse outcomes after systemic-to-pulmonary shunts in biventricular circulation. Ann Thorac Surg, 2017, 104(4): 1365-1370.
4.	Fan F, Peng B, Liu Z, et al. Systemic-to-pulmonary shunt vs right ventricle to pulmonary artery connection in the treatment of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. J Card Surg, 2020, 35(2): 345-351.
5.	張永輝, 花中東, 王旭, 等. 不同姑息性手術對肺動脈閉鎖合并室間隔缺損患者肺血管發育促進作用的比較. 中國循環雜志, 2018, 33(11): 1108-1112.
6.	Fiore AC, Tobin C, Jureidini S, et al. A comparison of the modified Blalock-Taussig shunt with the right ventricle-to-pulmonary artery conduit. Ann Thorac Surg, 2011, 91(5): 1479-1484.
7.	祝忠群, 劉錦紛, 鄭景浩, 等. 肺動脈閉鎖伴室間隔缺損的分期手術治療. 中華醫學雜志, 2010, 90(13): 898-901.
8.	鄧喜成, 沈向東, 郭少先, 等. 姑息手術在肺動脈閉鎖合并室間隔缺損分期矯治中的應用. 中國胸心血管外科臨床雜志, 2011, 18(2): 104-108.
9.	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.
10.	Mainwaring RD, Patrick WL, Roth SJ, et al. Surgical algorithm and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg, 2018, 156(3): 1194-1204.
11.	陳欣欣, 李守軍. 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. 中國胸心血管外科臨床雜志, 2020, 27(4): 401-407.
12.	Gates RN, Laks H, Johnson K. Side-to-side aorto-Gore-Tex central shunt. Ann Thorac Surg, 1998, 65(2): 515-516.
13.	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.
14.	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.
15.	Chowdhury UK, Sathia S, Ray R, et al. Histopathology of the right ventricular outflow tract and its relationship to clinical outcomes and arrhythmias in patients with tetralogy of Fallot. J Thorac Cardiovasc Surg, 2006, 132(2): 270-277.
16.	Hibino N, He D, Yuan F, et al. Growth of diminutive central pulmonary arteries after right ventricle to pulmonary artery homograft implantation. Ann Thorac Surg, 2014, 97(6): 2129-2133.
17.	Zhang Y, Hua Z, Yang K, 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, 46(2): 297-303.

1. d'Udekem Y, Alphonso N, N?rgaard MA, 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(6): 1496-1502.
2. 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.
3. Hobbes B, d'Udekem Y, Zannino D, et al. Determinants of adverse outcomes after systemic-to-pulmonary shunts in biventricular circulation. Ann Thorac Surg, 2017, 104(4): 1365-1370.
4. Fan F, Peng B, Liu Z, et al. Systemic-to-pulmonary shunt vs right ventricle to pulmonary artery connection in the treatment of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. J Card Surg, 2020, 35(2): 345-351.
5. 張永輝, 花中東, 王旭, 等. 不同姑息性手術對肺動脈閉鎖合并室間隔缺損患者肺血管發育促進作用的比較. 中國循環雜志, 2018, 33(11): 1108-1112.
6. Fiore AC, Tobin C, Jureidini S, et al. A comparison of the modified Blalock-Taussig shunt with the right ventricle-to-pulmonary artery conduit. Ann Thorac Surg, 2011, 91(5): 1479-1484.
7. 祝忠群, 劉錦紛, 鄭景浩, 等. 肺動脈閉鎖伴室間隔缺損的分期手術治療. 中華醫學雜志, 2010, 90(13): 898-901.
8. 鄧喜成, 沈向東, 郭少先, 等. 姑息手術在肺動脈閉鎖合并室間隔缺損分期矯治中的應用. 中國胸心血管外科臨床雜志, 2011, 18(2): 104-108.
9. 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.
10. Mainwaring RD, Patrick WL, Roth SJ, et al. Surgical algorithm and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg, 2018, 156(3): 1194-1204.
11. 陳欣欣, 李守軍. 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. 中國胸心血管外科臨床雜志, 2020, 27(4): 401-407.
12. Gates RN, Laks H, Johnson K. Side-to-side aorto-Gore-Tex central shunt. Ann Thorac Surg, 1998, 65(2): 515-516.
13. 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.
14. 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.
15. Chowdhury UK, Sathia S, Ray R, et al. Histopathology of the right ventricular outflow tract and its relationship to clinical outcomes and arrhythmias in patients with tetralogy of Fallot. J Thorac Cardiovasc Surg, 2006, 132(2): 270-277.
16. Hibino N, He D, Yuan F, et al. Growth of diminutive central pulmonary arteries after right ventricle to pulmonary artery homograft implantation. Ann Thorac Surg, 2014, 97(6): 2129-2133.
17. Zhang Y, Hua Z, Yang K, 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, 46(2): 297-303.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

A comparative study of three palliative surgical approaches for pulmonary atresia with ventricular septal defect

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