Right ventricular decompression for pulmonary atresia with intact ventricular septum_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

TI Yunxing , WANG Yuanxiang , LIU Huaipu , WANG Pengcheng , HUANG Junrong , MENG Baoying , ZHANG Qing ,  DING Yiqun

Department of Cardiovascular Surgery, Shenzhen Children's Hospital, Shenzhen, 518038, Shenzhen, P.R.China;

Corresponding?author：

DING Yiqun, Email: ikwan.ding@outlook.com

Keywords：

Pulmonary atresia; intact ventricular septum; right ventricle; decompression; hypoxemia

DOI：

10.7507/1007-4848.202002118

Video：

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Objective To summarize the experience and lessons of right ventricular decompression in children with pulmonary atresia and intact ventricular septum (PA/IVS) and to reflect on the strategies of right ventricular decompression.Methods The clinical data of 12 children with PA/IVS who underwent right ventricular decompression in our hospital from March 2015 to December 2019 were reviewed retrospectively. There were 10 males and 2 females with a median age at the time of surgery was 5 d (range, 1-627 d). Correlation analysis between the pulmonary valve transvalvular pressure gradient and changes in Z score of tricuspid valves after decompression was performed.Results One patient died of refractory hypoxemia due to circulatory shunt postoperatively and family members gave up treatment. There were 2 (16.67%) patients received postoperative intervention. The pulmonary transvalvular gradient after decompression was 31.95±21.75 mm Hg. Mild pulmonary regurgitation was found in 7 patients, moderate in 2 patients, and massive in 1 patient. The median time of mechanical ventilation was 30.50 h (range, 6.00-270.50 h), and the average duration of ICU stay was 164.06±87.74 h. The average postoperative follow-up time was 354.82±331.37 d. At the last follow-up, the average Z score of tricuspid valves was 1.32±0.71, the median pressure gradient between right ventricle and main pulmonary artery was 41.75 mm Hg (range, 21-146 mm Hg) and the average percutaneous oxygen saturation was 92.78%±3.73%. Two children underwent percutaneous balloon pulmonary valvoplasty at 6 and 10 months after surgery, respectively, with the rate of reintervention-free of 81.8%. There was no significant correlation between pulmonary transvalvular gradients after decompression and changes in Z score of tricuspid valves (r=–0.506, P=0.201).Conclusion For children with PA/IVS, the simple pursuit of adequate decompression during right ventricular decompression may lead to severe pulmonary dysfunction, increase the risk of ineffective circular shunt, and induce refractory hypoxemia. The staged decompression can ensure the safety and effectiveness for initial surgery and reduce the risk of postoperative death.

Citation： TI Yunxing, WANG Yuanxiang, LIU Huaipu, WANG Pengcheng, HUANG Junrong, MENG Baoying, ZHANG Qing, DING Yiqun. Right ventricular decompression for pulmonary atresia with intact ventricular septum. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2021, 28(1): 75-80. doi: 10.7507/1007-4848.202002118 Copy

1.	鐘穎, 葉艷瓊, 趙明一, 等. 小兒室間隔完整型肺動脈閉鎖治療進展. 中華胸心血管外科雜志, 2018, 34(9): 568-572.
2.	Maskatia SA, Petit CJ, Travers CD, et al. Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study. Congenit Heart Dis, 2018, 13(6): 892-902.
3.	Vall Camell M, Rodríguez-Fanjul J, Bautista Rodríguez C, et al. Percutaneous management of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis. An Pediatr (Barc), 2019, 91(5): 336-343.
4.	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.
5.	Cleuziou J, Schreiber C, Eicken A, et al. Predictors for biventricular repair in pulmonary atresia with intact ventricular septum. Thorac Cardiovasc Surg, 2010, 58(6): 339-344.
6.	El Shedoudy S, El-Doklah E. Transcatheter perforation of atretic pulmonary valve by the stiff end of a coronary wire in neonates with pulmonary atresia with intact ventricular septum: a solution in developing countries. J Saudi Heart Assoc, 2018, 30(3): 222-232.
7.	Huang SC, Ishino K, Kasahara S, et al. The potential of disproportionate growth of tricuspid valve after decompression of the right ventricle in patients with pulmonary atresia and intact ventricular septa. J Thorac Cardiovasc Surg, 2009, 138(5): 1160-1166.
8.	Lefort B, Saint-Etienne C, Soulé N, et al. Perforation of the atretic pulmonary valve using chronic total occlusion (CTO) wire and coronary microcatheter. Congenit Heart Dis, 2019, 14(5): 814-818.
9.	El Saiedi SA, Attia WA, Abd El-Aziz OM, et al. A perforation procedure for pulmonary atresia with intact ventricular septum: Egyptian experience and adaptations. Herz, 2018, 43(7): 633-641.
10.	Rathgeber S, Auld B, Duncombe S, et al. Outcomes of radiofrequency perforation for pulmonary atresia and intact ventricular septum: a single-centre experience. Pediatr Cardiol, 2017, 38(1): 170-175.
11.	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: 4874-4879.
12.	Gupta SK, Juneja R, Saxena A. Central perforation of atretic pulmonary valve using coronary microcatheter. Ann Pediatr Cardiol, 2017, 10(3): 304-305.
13.	Kim YH. Pulmonary valvotomy with echocardiographic guidance in neonates with pulmonary atresia and intact ventricular septum. Catheter Cardiovasc Interv, 2015, 85(4): E123-E128.
14.	Zampi JD, Hirsch-Romano JC, Goldstein BH, et al. Hybrid approach for pulmonary atresia with intact ventricular septum: early single center results and comparison to the standard surgical approach. Catheter Cardiovasc Interv, 2014, 83(5): 753-761.
15.	Chen RHS, Chau AKT, Chow PC, et al. Achieving biventricular circulation in patients with moderate hypoplastic right ventricle in pulmonary atresia intact ventricular septum after transcatheter pulmonary valve perforation. Congenit Heart Dis, 2018, 13(6): 884-891.
16.	Petit CJ, Qureshi AM, Glatz AC, et al. Technical factors are associated with complications and repeat intervention in neonates undergoing transcatheter right ventricular decompression for pulmonary atresia and intact ventricular septum: results from the congenital catheterisation research collaborative. Cardiol Young, 2018, 28(8): 1042-1049.
17.	Bakhru S, Marathe S, Saxena M, et al. Transcatheter pulmonary valve perforation using chronic total occlusion wire in pulmonary atresia with intact ventricular septum. Ann Pediatr Cardiol, 2017, 10(1): 5-10.
18.	Hasan BS, Bautista-Hernandez V, McElhinney DB, et al. Outcomes of transcatheter approach for initial treatment of pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv, 2013, 81(1): 111-118.
19.	Patil NC, Saxena A, Gupta SK, et al. Perforating the atretic pulmonary valve with CTO hardware: technical aspects. Catheter Cardiovasc Interv, 2016, 88(5): E145-E150.
20.	王寧, 劉大為, 劉宇航, 等. 新生兒室間隔完整型肺動脈閉鎖 Hybrid 手術預后分析. 中國胸心血管外科臨床雜志, 2018, 25(10): 910-913.
21.	Cho MJ, Ban KH, Kim MJ, et al. Catheter-based treatment in patients with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum: a single institute experience with comparison between patients with and without additional procedure for pulmonary flow. Congenit Heart Dis, 2013, 8(5): 440-449.
22.	吳松, 劉迎龍, 萬峰, 等. 室間隔完整的肺動脈閉鎖外科治療. 臨床心血管病雜志, 2012, 28(11): 854-857.
23.	陳綱, 吳琳, 劉芳, 等. 經右室肺動脈瓣成形術治療室間隔完整型肺動脈閉鎖的中期隨訪. 中華小兒外科雜志, 2016, 37(2): 85-90.
24.	齊建川, 張澤偉, 李建華, 等. 室間隔完整型肺動脈閉鎖分期治療策略的療效分析. 中華外科雜志, 2018, 56(6): 427-431.
25.	Petit CJ, Glatz AC, Qureshi AM, et al. Outcomes after decompression of the right ventricle in infants with pulmonary atresia with intact ventricular septum are associated with degree of tricuspid regurgitation: results from the congenital catheterization research collaborative. Circ Cardiovasc Interv, 2017, 10(5): e004428.

1. 鐘穎, 葉艷瓊, 趙明一, 等. 小兒室間隔完整型肺動脈閉鎖治療進展. 中華胸心血管外科雜志, 2018, 34(9): 568-572.
2. Maskatia SA, Petit CJ, Travers CD, et al. Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study. Congenit Heart Dis, 2018, 13(6): 892-902.
3. Vall Camell M, Rodríguez-Fanjul J, Bautista Rodríguez C, et al. Percutaneous management of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis. An Pediatr (Barc), 2019, 91(5): 336-343.
4. 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.
5. Cleuziou J, Schreiber C, Eicken A, et al. Predictors for biventricular repair in pulmonary atresia with intact ventricular septum. Thorac Cardiovasc Surg, 2010, 58(6): 339-344.
6. El Shedoudy S, El-Doklah E. Transcatheter perforation of atretic pulmonary valve by the stiff end of a coronary wire in neonates with pulmonary atresia with intact ventricular septum: a solution in developing countries. J Saudi Heart Assoc, 2018, 30(3): 222-232.
7. Huang SC, Ishino K, Kasahara S, et al. The potential of disproportionate growth of tricuspid valve after decompression of the right ventricle in patients with pulmonary atresia and intact ventricular septa. J Thorac Cardiovasc Surg, 2009, 138(5): 1160-1166.
8. Lefort B, Saint-Etienne C, Soulé N, et al. Perforation of the atretic pulmonary valve using chronic total occlusion (CTO) wire and coronary microcatheter. Congenit Heart Dis, 2019, 14(5): 814-818.
9. El Saiedi SA, Attia WA, Abd El-Aziz OM, et al. A perforation procedure for pulmonary atresia with intact ventricular septum: Egyptian experience and adaptations. Herz, 2018, 43(7): 633-641.
10. Rathgeber S, Auld B, Duncombe S, et al. Outcomes of radiofrequency perforation for pulmonary atresia and intact ventricular septum: a single-centre experience. Pediatr Cardiol, 2017, 38(1): 170-175.
11. 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: 4874-4879.
12. Gupta SK, Juneja R, Saxena A. Central perforation of atretic pulmonary valve using coronary microcatheter. Ann Pediatr Cardiol, 2017, 10(3): 304-305.
13. Kim YH. Pulmonary valvotomy with echocardiographic guidance in neonates with pulmonary atresia and intact ventricular septum. Catheter Cardiovasc Interv, 2015, 85(4): E123-E128.
14. Zampi JD, Hirsch-Romano JC, Goldstein BH, et al. Hybrid approach for pulmonary atresia with intact ventricular septum: early single center results and comparison to the standard surgical approach. Catheter Cardiovasc Interv, 2014, 83(5): 753-761.
15. Chen RHS, Chau AKT, Chow PC, et al. Achieving biventricular circulation in patients with moderate hypoplastic right ventricle in pulmonary atresia intact ventricular septum after transcatheter pulmonary valve perforation. Congenit Heart Dis, 2018, 13(6): 884-891.
16. Petit CJ, Qureshi AM, Glatz AC, et al. Technical factors are associated with complications and repeat intervention in neonates undergoing transcatheter right ventricular decompression for pulmonary atresia and intact ventricular septum: results from the congenital catheterisation research collaborative. Cardiol Young, 2018, 28(8): 1042-1049.
17. Bakhru S, Marathe S, Saxena M, et al. Transcatheter pulmonary valve perforation using chronic total occlusion wire in pulmonary atresia with intact ventricular septum. Ann Pediatr Cardiol, 2017, 10(1): 5-10.
18. Hasan BS, Bautista-Hernandez V, McElhinney DB, et al. Outcomes of transcatheter approach for initial treatment of pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv, 2013, 81(1): 111-118.
19. Patil NC, Saxena A, Gupta SK, et al. Perforating the atretic pulmonary valve with CTO hardware: technical aspects. Catheter Cardiovasc Interv, 2016, 88(5): E145-E150.
20. 王寧, 劉大為, 劉宇航, 等. 新生兒室間隔完整型肺動脈閉鎖 Hybrid 手術預后分析. 中國胸心血管外科臨床雜志, 2018, 25(10): 910-913.
21. Cho MJ, Ban KH, Kim MJ, et al. Catheter-based treatment in patients with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum: a single institute experience with comparison between patients with and without additional procedure for pulmonary flow. Congenit Heart Dis, 2013, 8(5): 440-449.
22. 吳松, 劉迎龍, 萬峰, 等. 室間隔完整的肺動脈閉鎖外科治療. 臨床心血管病雜志, 2012, 28(11): 854-857.
23. 陳綱, 吳琳, 劉芳, 等. 經右室肺動脈瓣成形術治療室間隔完整型肺動脈閉鎖的中期隨訪. 中華小兒外科雜志, 2016, 37(2): 85-90.
24. 齊建川, 張澤偉, 李建華, 等. 室間隔完整型肺動脈閉鎖分期治療策略的療效分析. 中華外科雜志, 2018, 56(6): 427-431.
25. Petit CJ, Glatz AC, Qureshi AM, et al. Outcomes after decompression of the right ventricle in infants with pulmonary atresia with intact ventricular septum are associated with degree of tricuspid regurgitation: results from the congenital catheterization research collaborative. Circ Cardiovasc Interv, 2017, 10(5): e004428.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Right ventricular decompression for pulmonary atresia with intact ventricular septum

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