Strategy of surgical management for pulmonary atresia with intact ventricular septum and mid-term follow-up_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

HE Xiaomin ,  ZHENG Jinghao , LUO Kai , DU Xinwei , SUN Qi , ZHU Zhongqun , 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 intact ventricular septum; initial surgery; Hybrid therapy; one and half ventricular repair; single ventricle operation

DOI：

10.7507/1007-4848.202001020

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Objective To evaluate the surgical strategy and follow-up for pulmonary atresia with intact ventricular septum (PA/IVS) in our heart center.Methods From January 2008 to December 2018, 151 patients with PA/IVS were divided into two groups: a one-stage surgery group (26 patients), including 17 males and 9 females at an average age of 14.7±13.2 months, and a staged surgery group (125 patients) including 72 males and 53 females at an average age of 6.4±6.3 months. The clinical effectiveness of the two groups were analyzed.Results All patients were followed up for 1-11 years. Eighteen patients died and 19 patients were lost to follow-up. The 1-year, 5-year and 10-year survival rate was 90.2%, 87.0%, and 85.2%, respectively. Two patients died in the one-stage surgery group. Twelve patients died after initial surgery, and 4 patients died after final operation in the staged surgery group. The Z value of tricuspid valve (P=0.013) and severe right ventricular dysplasia (P=0.025) were the risk factors of postoperative death in the patients with PA/IVS. Furthermore, 58 patients completed final operation, and the total number of the final operation (including one-stage radical surgery) accounted for 55.6% (84/151). Five patients accepted the re-operation intervention in the medium-term follow-up. The rest of the patients recovered well. Only 2 patients were classified as grade Ⅲ in cardiac function, and the rest patients were classified as gradeⅠ-Ⅱ.Conclusion According to the degree of right ventricular hypoplasia, the age at operation and the presence or absence of coronary artery malformation, the individualized surgical strategy could significantly improve the success rate of PA/IVS, and early completion of right ventricular decompression operation is conducive to improve the chance for biventricular repair.

Citation： HE Xiaomin, ZHENG Jinghao, LUO Kai, DU Xinwei, SUN Qi, ZHU Zhongqun, XU Zhiwei, LIU Jinfen. Strategy of surgical management for pulmonary atresia with intact ventricular septum and mid-term follow-up. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2020, 27(5): 503-509. doi: 10.7507/1007-4848.202001020 Copy

1.	He X, Zhang X, Jing H, et al. Rare copy number variations might not be involved in the molecular pathogenesis of PA-IVS in an unselected Chinese cohort. Pediatr Cardiol, 2019, 40(4): 762-767.
2.	Bull C, de Leval MR, Mercanti C, et al. Pulmonary atresia and intact ventricular septum: a revised classification. Circulation, 1982, 66(2): 266-272.
3.	祝忠群, 刘锦纷, 苏肇伉, 等. 室间隔完整型肺动脉闭锁的个体化手术治疗. 中华医学杂志, 2008, 88(11): 738-741.
4.	Schneider AW, Blom NA, Bruggemans EF, et al. More than 25 years of experience in managing pulmonary atresia with intact ventricular septum. Ann Thorac Surg, 2014, 98(5): 1680-1686.
5.	Wright LK, Knight JH, Thomas AS, et al. Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum. Heart, 2019, 105(13): 1007-1013.
6.	Liava'a M, Brooks P, Konstantinov I, et al. Changing trends in the management of pulmonary atresia with intact ventricular septum: the Melbourne experience. Eur J Cardiothorac Surg, 2011, 40(6): 1406-1411.
7.	Kotani Y, Kasahara S, Fujii Y, et al. A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum. Eur J Cardiothorac Surg, 2016, 50(2): 298-303.
8.	Lin ZQ, Chen Q, Cao H, et al. Transthoracic balloon pulmonary valvuloplasty for treatment of congenial pulmonary atresia patients with intact ventricular septum. Med Sci Monit, 2017, 23: 4874.
9.	王宁, 刘大为, 刘宇航, 等. 新生儿室间隔完整型肺动脉闭锁Hybrid手术预后分析. 中国胸心血管外科临床杂志, 2018, 25(10): 910-913.
10.	Yoshimura N, Yamaguchi M. Surgical strategy for pulmonary atresia with intact ventricular septum: initial management and definitive surgery. Gen Thorac Cardiovasc Surg, 2009, 57(7): 338-346.
11.	Do N, Hill KD, Wallace AS, et al. Shunt failure-risk factors and outcomes: An analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database. Ann Thorac Surg, 2018, 105(3): 857-864.
12.	Hanley FL, Sade RM, Blackstone EH, et al. Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study. J Thorac Cardiovasc Surg, 1993, 105(3): 406-427.
13.	Sasikumar N, Hermuzi A, Fan CS, et al. Outcomes of Blalock-Taussig shunts in current era: A single center experience. Congenit Heart Dis, 2017, 12(6): 808-814.
14.	Glatz AC, Petit CJ, Goldstein BH, et al. Comparison between patent ductus arteriosus stent and modified blalock-taussig shunt as palliation for infants with ductal-dependent pulmonary blood flow: insights from the congenital catheterization research collaborative. Circulation, 2018, 137(6): 589-601.
15.	Daubeney PE, Wang D, Delany DJ, et al. Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study. J Thorac Cardiovasc Surg, 2005, 130(4): 1071.
16.	Grant S, Faraoni D, DiNardo J, et al. Predictors of mortality in children with pulmonary atresia with intact ventricular septum. Pediatr Cardiol, 2017, 38(8): 1627-1632.
17.	Elias P, Poh CL, du Plessis K, et al. Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricular septum: Fontan survivors remain at risk of late myocardial ischaemia and death. Eur J Cardiothorac Surg, 2018, 53(6): 1230-1236.
18.	Karamlou T, Poynter JA, Walters HL 3rd, et al. Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: a Congenital Heart Surgeons Society study. J Thorac Cardiovasc Surg, 2013, 145(4): 1018-1027.

1. He X, Zhang X, Jing H, et al. Rare copy number variations might not be involved in the molecular pathogenesis of PA-IVS in an unselected Chinese cohort. Pediatr Cardiol, 2019, 40(4): 762-767.
2. Bull C, de Leval MR, Mercanti C, et al. Pulmonary atresia and intact ventricular septum: a revised classification. Circulation, 1982, 66(2): 266-272.
3. 祝忠群, 刘锦纷, 苏肇伉, 等. 室间隔完整型肺动脉闭锁的个体化手术治疗. 中华医学杂志, 2008, 88(11): 738-741.
4. Schneider AW, Blom NA, Bruggemans EF, et al. More than 25 years of experience in managing pulmonary atresia with intact ventricular septum. Ann Thorac Surg, 2014, 98(5): 1680-1686.
5. Wright LK, Knight JH, Thomas AS, et al. Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum. Heart, 2019, 105(13): 1007-1013.
6. Liava'a M, Brooks P, Konstantinov I, et al. Changing trends in the management of pulmonary atresia with intact ventricular septum: the Melbourne experience. Eur J Cardiothorac Surg, 2011, 40(6): 1406-1411.
7. Kotani Y, Kasahara S, Fujii Y, et al. A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum. Eur J Cardiothorac Surg, 2016, 50(2): 298-303.
8. Lin ZQ, Chen Q, Cao H, et al. Transthoracic balloon pulmonary valvuloplasty for treatment of congenial pulmonary atresia patients with intact ventricular septum. Med Sci Monit, 2017, 23: 4874.
9. 王宁, 刘大为, 刘宇航, 等. 新生儿室间隔完整型肺动脉闭锁Hybrid手术预后分析. 中国胸心血管外科临床杂志, 2018, 25(10): 910-913.
10. Yoshimura N, Yamaguchi M. Surgical strategy for pulmonary atresia with intact ventricular septum: initial management and definitive surgery. Gen Thorac Cardiovasc Surg, 2009, 57(7): 338-346.
11. Do N, Hill KD, Wallace AS, et al. Shunt failure-risk factors and outcomes: An analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database. Ann Thorac Surg, 2018, 105(3): 857-864.
12. Hanley FL, Sade RM, Blackstone EH, et al. Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study. J Thorac Cardiovasc Surg, 1993, 105(3): 406-427.
13. Sasikumar N, Hermuzi A, Fan CS, et al. Outcomes of Blalock-Taussig shunts in current era: A single center experience. Congenit Heart Dis, 2017, 12(6): 808-814.
14. Glatz AC, Petit CJ, Goldstein BH, et al. Comparison between patent ductus arteriosus stent and modified blalock-taussig shunt as palliation for infants with ductal-dependent pulmonary blood flow: insights from the congenital catheterization research collaborative. Circulation, 2018, 137(6): 589-601.
15. Daubeney PE, Wang D, Delany DJ, et al. Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study. J Thorac Cardiovasc Surg, 2005, 130(4): 1071.
16. Grant S, Faraoni D, DiNardo J, et al. Predictors of mortality in children with pulmonary atresia with intact ventricular septum. Pediatr Cardiol, 2017, 38(8): 1627-1632.
17. Elias P, Poh CL, du Plessis K, et al. Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricular septum: Fontan survivors remain at risk of late myocardial ischaemia and death. Eur J Cardiothorac Surg, 2018, 53(6): 1230-1236.
18. Karamlou T, Poynter JA, Walters HL 3rd, et al. Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: a Congenital Heart Surgeons Society study. J Thorac Cardiovasc Surg, 2013, 145(4): 1018-1027.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Strategy of surgical management for pulmonary atresia with intact ventricular septum and mid-term follow-up

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