Right ventricular-pulmonary artery connection for palliative treatment of pulmonary atresia with ventricular septal defect in children: A single-center retrospective study_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

ZHANG Shuai ^1,2 , MA Jianrui ^1,2 , QIU Hailong ^1,2 , YAN Xinjian ^1,2 , XIE Wen ^1,2 , REN Qiushi ^1,2 , YU Juemin ^1,2 , CHEN Tianyu ^1,2 , ZHANG Yong ^1,2 , LI Xiaohua ^1,2 , LIU Furong ^1,2 , WEN Shusheng ^1,2 , ZHUANG Jian ^1,2 ,  GAO Qiang ^1,2 ,  CEN Jianzheng ^1,2

1. Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People’s Hospital, Guangdong Academy of Medical Sciences, Guangzhou, 510080, P. R. China;
2. Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangzhou, 510080, P. R. China;

Corresponding author：

GAO Qiang, Email: gaoqiang_89@163.com; CEN Jianzheng, Email: jamesofnebulae@163.com

Keywords：

Pulmonary atresia; ventricular septal defect; congenital heart disease; right ventricular-pulmonary artery connection; patch expansion; pericardial tube

DOI：

10.7507/1007-4848.202303044

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Objective To compare the benefits and drawbacks of primary patch expansion versus pericardial tube right ventricular-pulmonary artery connection in patients diagnosed with pulmonary atresia with ventricular septal defect (PA/VSD). Methods A retrospective study was conducted on patients diagnosed with PA/VSD who underwent primary right ventricular-pulmonary artery connection surgery at our center between 2010 and 2020. Patients were categorized into two groups based on the type of right ventricular-pulmonary artery connection: a pericardial tube group and a patch expansion group. Clinical data and imaging findings were compared between the two groups. Results A total of 51 patients were included in the study, comprising 31 males and 20 females, with a median age of 12.57 (4.57, 49.67) months. The pericardial tube group included 19 patients with a median age of 17.17 (7.33, 49.67) months, while the patch expansion group consisted of 32 patients with a median age of 8.58 (3.57, 52.72) months. In both groups, the diameter of pulmonary artery, McGoon index, and Nakata index significantly increased after treatment (P<0.001). However, the pericardial tube group exhibited a longer extracorporeal circulation time (P<0.001). The reoperation rate was notably high, with 74.51% of patients requiring further surgical intervention, including 26 (81.25%) patients in the patch expansion group and 12 (63.16%) patients in the pericardial tube group. No statistical differences were observed in long-term cure rates or mortality between the two groups (P＞0.005). Conclusion In patients with PA/VSD, both patch expansion and pericardial tube right ventricular-pulmonary artery connection serve as effective initial palliative treatment strategies that promote pulmonary vessel development and provide a favorable foundation for subsequent radical operations. However, compared to the pericardial tube approach, the patch expansion technique is simpler to perform and preserves some intrinsic potential for pulmonary artery development, making it the preferred procedure.

Citation： ZHANG Shuai, MA Jianrui, QIU Hailong, YAN Xinjian, XIE Wen, REN Qiushi, YU Juemin, CHEN Tianyu, ZHANG Yong, LI Xiaohua, LIU Furong, WEN Shusheng, ZHUANG Jian, GAO Qiang, CEN Jianzheng. Right ventricular-pulmonary artery connection for palliative treatment of pulmonary atresia with ventricular septal defect in children: A single-center retrospective study. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2025, 32(3): 366-371. doi: 10.7507/1007-4848.202303044 Copy

1.	Soquet J, Barron DJ, d'Udekem Y. A review of the management of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Ann Thorac Surg, 2019, 108(2): 601-612.
2.	宋治莹, 郑景浩. 肺动脉闭锁伴室间隔缺损的外科手术策略进展. 上海交通大学学报 (医学版), 2021, 41(10): 1389-1393.Song ZY, Zheng JH. Progress in surgical strategies of pulmonary atresia with ventricular septal defect. J Shanghai Jiaotong Univ (Med Sci), 2021, 41(10): 1389-1393.
3.	Lenoir M, Pontailler M, Gaudin R, et al. Outcomes of palliative right ventricle to pulmonary artery connection for pulmonary atresia with ventricular septal defect. Eur J Cardiothorac Surg, 2017, 52(3): 590-598.
4.	Zou MH, Ma L, Cui YQ, et al. Outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: A tailored approach in a developing setting. Front Cardiovasc Med, 2021, 8: 665038.
5.	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.
6.	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): 278-288.
7.	刘晓冰, 陈寄梅, 岑坚正, 等. 肺动脉后壁扩大法矫治合并左肺动脉狭窄的法洛四联症. 中国胸心血管外科临床杂志, 2016, 23(8): 800-803.Liu XB, Chen JM, Cen JZ, et al. Widening pulmonary arterial posterior wall for tetralogy of Fallot repair patients complicated with left pulmonary artery stenosis. Chin J Clin Thorac Cardiovasc Surg, 2016, 23(8): 800-803.
8.	Malhotra SP, Hanley FL. Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: A protocol-based approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2009, 12(1): 145-151.

1. Soquet J, Barron DJ, d'Udekem Y. A review of the management of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Ann Thorac Surg, 2019, 108(2): 601-612.
2. 宋治莹, 郑景浩. 肺动脉闭锁伴室间隔缺损的外科手术策略进展. 上海交通大学学报 (医学版), 2021, 41(10): 1389-1393.Song ZY, Zheng JH. Progress in surgical strategies of pulmonary atresia with ventricular septal defect. J Shanghai Jiaotong Univ (Med Sci), 2021, 41(10): 1389-1393.
3. Lenoir M, Pontailler M, Gaudin R, et al. Outcomes of palliative right ventricle to pulmonary artery connection for pulmonary atresia with ventricular septal defect. Eur J Cardiothorac Surg, 2017, 52(3): 590-598.
4. Zou MH, Ma L, Cui YQ, et al. Outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: A tailored approach in a developing setting. Front Cardiovasc Med, 2021, 8: 665038.
5. 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.
6. 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): 278-288.
7. 刘晓冰, 陈寄梅, 岑坚正, 等. 肺动脉后壁扩大法矫治合并左肺动脉狭窄的法洛四联症. 中国胸心血管外科临床杂志, 2016, 23(8): 800-803.Liu XB, Chen JM, Cen JZ, et al. Widening pulmonary arterial posterior wall for tetralogy of Fallot repair patients complicated with left pulmonary artery stenosis. Chin J Clin Thorac Cardiovasc Surg, 2016, 23(8): 800-803.
8. Malhotra SP, Hanley FL. Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: A protocol-based approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2009, 12(1): 145-151.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Right ventricular-pulmonary artery connection for palliative treatment of pulmonary atresia with ventricular septal defect in children: A single-center retrospective study

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