Efficacy of surgical balloon valvuloplasty via right ventricular outflow tract for right ventricular decompression in the treatment of pulmonary atresia with intact ventricular septum_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

MA Kai , QI Lei , PANG Kunjing , ZHANG Benqing , RUI Lu , LIN Ye , LIU Rui , ZHANG Sen , WANG Guanxi , FENG Zicong ,  LI Shoujun

Pediatric Cardiac Surgery Center, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100037, P.R.China;

Corresponding author：

LI Shoujun, Email: drlishoujun@yahoo.com

Keywords：

Pulmonary atresia with intact ventricular septum; surgical balloon valvuloplasty; prognosis; surgery

DOI：

10.7507/1007-4848.202003084

Video：

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Objective To analyze the mid-long-term outcomes of surgical balloon valvuloplasty (SBV) for right ventricular decompression in the treatment of pulmonary atresia with intact ventricular septum (PA/IVS).Methods Clinical data of consecutive 91 patients who were diagnosed with PA/IVS and underwent SBV in our institution from January 2005 to December 2017 were retrospectively analyzed, including 52 (57.1%) males and 39 (42.9%) females. The median age was 3 months (1 d, 24 months) and the median weight was 4.1 (2.5, 12.0) kg.Results The SBV was performed in all patients, and 62 of whom received other simultaneous surgeries, including ligation of patent ductus arteriosus (PDA, 33 patients), ligation of PDA with modified Blalock-Taussig shunt (23 patients), ligation of PDA with bidirectional Glenn shunt (6 patients). There was no early postoperative death. The median follow-up time was 8.8 (2.5, 13.4) years, 4 patients were lost. There were 7 (8.0%) deaths and 1 (1.1%) patient with a re-SBV for pulmonary stenosis. The one and a half ventricular repair was performed in 5 (5.7%) patients and Fontan procedure in 2 (2.3%) patients. In addition, the mean Z-value of tricuspid valve annulus was −1.7±1.5, which was significant bigger than that before the operation (t=5.587, P<0.001).Conclusion SBV via right ventricular outflow tract for right ventricular decompression in the treatment of PA/IVS is safe and reliable. The majority of patients can receive biventricular repair instead of single ventricular palliation by SBV with individually customized shunt.

Citation： MA Kai, QI Lei, PANG Kunjing, ZHANG Benqing, RUI Lu, LIN Ye, LIU Rui, ZHANG Sen, WANG Guanxi, FENG Zicong, LI Shoujun. Efficacy of surgical balloon valvuloplasty via right ventricular outflow tract for right ventricular decompression in the treatment of pulmonary atresia with intact ventricular septum. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2020, 27(5): 498-502. doi: 10.7507/1007-4848.202003084 Copy

1.	Pradat P, Francannet C, Harris JA, et al. The epidemiology of cardiovascular defects, partⅠ: a study based on data from three large registries of congenital malformations. Pediatr Cardiol, 2003, 24(3): 195-221.
2.	Daubeney PE, Sharland GK, Cook AC, et al. Pulmonary atresia with intact ventricular septum: impact of fetal echocardiography on incidence at birth and postnatal outcome. UK and Eire collaborative study of pulmonary atresia with intact ventricular septum. Circulation, 1998, 98(6): 562-566.
3.	Leonard H, Derrick G, O'Sullivan J, et al. Natural and unnatural history of pulmonary atresia. Heart, 2000, 84(5): 499-503.
4.	Qureshi SA. Catheterization in neonates with pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv, 2006, 67(6): 924-931.
5.	Gittenberger-de Groot AC, Sauer U, Bindl L, et al. Competition of coronary arteries and ventriculo-coronary arterial communications in pulmonary atresia with intact ventricular septum. Int J Cardiol, 1988, 18(2): 243-258.
6.	Gorla SR, Singh AP. Pulmonary atresia with intact ventricular septum. StatPearls. Treasure Island (FL), 2020.
7.	Qureshi SA, Rosenthal E, Tynan M, et al. Transcatheter laser-assisted balloon pulmonary valve dilation in pulmonic valve atresia. Am J Cardiol, 1991, 67(5): 428-431.
8.	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.
9.	Marasini M, Gorrieri PF, Tuo G, et al. Long-term results of catheter-based treatment of pulmonary atresia and intact ventricular septum. Heart, 2009, 95(18): 1520-1524.
10.	Ashburn DA, Blackstone EH, Wells WJ, et al. Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg, 2004, 127(4): 1000-1007.
11.	Li S, Chen W, Zhang Y, et al. Hybrid therapy for pulmonary atresia with intact ventricular septum. Ann Thorac Surg, 2011, 91(5): 1467-1471.
12.	LaPar DJ, Bacha E. Pulmonary atresia with intact ventricular septum with borderline tricuspid valve: how small is too small. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2019, 22: 27-31.
13.	Chikkabyrappa SM, Loomba RS, Tretter JT. Pulmonary atresia with an intact ventricular septum: preoperative physiology, imaging, and management. Semin Cardiothorac Vasc Anesth, 2018, 22(3): 245-255.
14.	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.
15.	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.
16.	Lee ML, Tsao LY, Chiu HY, et al. Outcomes in neonates with pulmonary atresia and intact ventricular septum underwent pulmonary valvulotomy and valvuloplasty using a flexible 2-French radiofrequency catheter. Yonsei Med J, 2009, 50(2): 245-251.
17.	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.
18.	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.
19.	Alwi M. Management algorithm in pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv, 2006, 67(5): 679-686.
20.	Agnoletti G, Piechaud JF, Bonhoeffer P, et al. Perforation of the atretic pulmonary valve. Long-term follow-up. J Am Coll Cardiol, 2003, 41(8): 1399-1403.
21.	Sano S, Ishino K, Kawada M, et al. Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum. Ann Thorac Surg, 2000, 70(5): 1501-1506.
22.	何晓敏, 郑景浩, 罗凯, 等. 非体外循环下肺动脉瓣切开术初期治疗室间隔完整型肺动脉闭锁的单中心临床分析. 中国胸心血管外科临床杂志, 2020, 27(4): 411-414.

1. Pradat P, Francannet C, Harris JA, et al. The epidemiology of cardiovascular defects, partⅠ: a study based on data from three large registries of congenital malformations. Pediatr Cardiol, 2003, 24(3): 195-221.
2. Daubeney PE, Sharland GK, Cook AC, et al. Pulmonary atresia with intact ventricular septum: impact of fetal echocardiography on incidence at birth and postnatal outcome. UK and Eire collaborative study of pulmonary atresia with intact ventricular septum. Circulation, 1998, 98(6): 562-566.
3. Leonard H, Derrick G, O'Sullivan J, et al. Natural and unnatural history of pulmonary atresia. Heart, 2000, 84(5): 499-503.
4. Qureshi SA. Catheterization in neonates with pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv, 2006, 67(6): 924-931.
5. Gittenberger-de Groot AC, Sauer U, Bindl L, et al. Competition of coronary arteries and ventriculo-coronary arterial communications in pulmonary atresia with intact ventricular septum. Int J Cardiol, 1988, 18(2): 243-258.
6. Gorla SR, Singh AP. Pulmonary atresia with intact ventricular septum. StatPearls. Treasure Island (FL), 2020.
7. Qureshi SA, Rosenthal E, Tynan M, et al. Transcatheter laser-assisted balloon pulmonary valve dilation in pulmonic valve atresia. Am J Cardiol, 1991, 67(5): 428-431.
8. 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.
9. Marasini M, Gorrieri PF, Tuo G, et al. Long-term results of catheter-based treatment of pulmonary atresia and intact ventricular septum. Heart, 2009, 95(18): 1520-1524.
10. Ashburn DA, Blackstone EH, Wells WJ, et al. Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg, 2004, 127(4): 1000-1007.
11. Li S, Chen W, Zhang Y, et al. Hybrid therapy for pulmonary atresia with intact ventricular septum. Ann Thorac Surg, 2011, 91(5): 1467-1471.
12. LaPar DJ, Bacha E. Pulmonary atresia with intact ventricular septum with borderline tricuspid valve: how small is too small. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2019, 22: 27-31.
13. Chikkabyrappa SM, Loomba RS, Tretter JT. Pulmonary atresia with an intact ventricular septum: preoperative physiology, imaging, and management. Semin Cardiothorac Vasc Anesth, 2018, 22(3): 245-255.
14. 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.
15. 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.
16. Lee ML, Tsao LY, Chiu HY, et al. Outcomes in neonates with pulmonary atresia and intact ventricular septum underwent pulmonary valvulotomy and valvuloplasty using a flexible 2-French radiofrequency catheter. Yonsei Med J, 2009, 50(2): 245-251.
17. 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.
18. 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.
19. Alwi M. Management algorithm in pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv, 2006, 67(5): 679-686.
20. Agnoletti G, Piechaud JF, Bonhoeffer P, et al. Perforation of the atretic pulmonary valve. Long-term follow-up. J Am Coll Cardiol, 2003, 41(8): 1399-1403.
21. Sano S, Ishino K, Kawada M, et al. Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum. Ann Thorac Surg, 2000, 70(5): 1501-1506.
22. 何晓敏, 郑景浩, 罗凯, 等. 非体外循环下肺动脉瓣切开术初期治疗室间隔完整型肺动脉闭锁的单中心临床分析. 中国胸心血管外科临床杂志, 2020, 27(4): 411-414.

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Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Efficacy of surgical balloon valvuloplasty via right ventricular outflow tract for right ventricular decompression in the treatment of pulmonary atresia with intact ventricular septum

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