Hostname: page-component-54dcc4c588-rz4zl Total loading time: 0 Render date: 2025-10-05T15:22:15.056Z Has data issue: false hasContentIssue false
  • English
  • Français

Revived role of reverse Potts shunt in management of paediatric severe pulmonary hypertension

Published online by Cambridge University Press:  27 August 2025

Zhangwei Wang Open the ORCID record for Zhangwei Wang [Opens in a new window] ,
Honghao Fu  and
Shoujun Li
Zhangwei Wang
Affiliation:
Department of Cardiovascular Surgery, Chinese Academy of Medical Sciences and Peking Union Medical College, National Center for Cardiovascular Diseases, Fuwai Hospital, Beijing, China
Honghao Fu
Affiliation:
Department of Cardiovascular Surgery, Chinese Academy of Medical Sciences and Peking Union Medical College, National Center for Cardiovascular Diseases, Fuwai Hospital, Beijing, China
Shoujun Li*
Affiliation:
Department of Cardiovascular Surgery, Chinese Academy of Medical Sciences and Peking Union Medical College, National Center for Cardiovascular Diseases, Fuwai Hospital, Beijing, China
*
Corresponding author: Shoujun Li; Email: beijing348@qq.com
Get access Rights & Permissions [Opens in a new window]

Abstract

Pulmonary artery hypertension in children is a progressive, fatal disease secondary to a variety of causes leading to right ventricular dysfunction. Traditional treatments include lung transplantation (LTx) and targeted therapy. However, due to the shortage of donor sources, heavy medical economic burden, immune rejection, and other problems, many patients are discouraged. In the past 20 years, the Potts palliative shunt, which was initially used for the treatment of cyanotic heart disease, has been gradually revived. Studies have shown that reverse Potts shunt can improve postoperative cardiac function, delay lung transplantation, and improve long-term quality of life in children with severe pulmonary arterial hypertension, which is a feasible and effective alternative to LTx. The surgical procedure is relatively less complex, so it is worth promoting in resource-poor countries, and limited medical expertise. In the future, with refinements of the reverse Potts shunt (unidirectional-valved shunt and transcatheter Potts shunt) and formulation of guidelines (shunt sizes, involvement of multidisciplinary team), there is expected to be further expansion of indications. This article reviews the physiological mechanisms, past and present status of reverse Potts shunt indications and contraindications, surgical procedures, benefits, future application prospects, and key unsolved problems of reverse Potts shunt in the treatment of severe pulmonary arterial hypertension in children.

Keywords

pulmonary artery hypertensionreverse Potts shuntright heart failureprognosis

Information

Type
Review
Information
Cardiology in the Young , Volume 35 , Issue 8 , August 2025 , pp. 1567 - 1576
Copyright
© The Author(s), 2025. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Article purchase

Temporarily unavailable

Footnotes

*

Zhangwei Wang and Honghao Fu contributed equally to this work.

References

Jiawang, X, Qiguang, W, Xianyang, Z. Research progress of potts shunt in the treatment of end-stage pulmonary hypertension. Chin J Intervent Cardiol 2020; 28: 634–637.Google Scholar
Humbert, M, Sitbon, O, Guignabert, C, et al. Treatment of pulmonary arterial hypertension: recent progress and a look to the future. Lancet Respir Med 2023; 11: 804–819. DOI: 10.1016/S2213-2600(23)00264-3.CrossRefGoogle Scholar
D’Alonzo, GE, Barst, RJ, Ayres, SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991; 115: 343–349. DOI: 10.7326/0003-4819-115-5-343.CrossRefGoogle ScholarPubMed
Moledina, S, Hislop, AA, Foster, H, Schulze-Neick, I, Haworth, SG. Childhood idiopathic pulmonary arterial hypertension: a national cohort study. Heart 2010; 96: 1401–1406. DOI: 10.1136/hrt.2009.182378.CrossRefGoogle ScholarPubMed
Barst, RJ, McGoon, MD, Elliott, CG, Foreman, AJ, Miller, DP, Ivy, DD. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation 2012; 125: 113–122. DOI: 10.1161/CIRCULATIONAHA.111.026591.CrossRefGoogle ScholarPubMed
Jiang, W, Wu, Z, Huang, C, et al. Reverse potts shunt in severe pulmonary hypertension: a 20-year retrospective review. Int J Cardiol 2025; 428: 133129. DOI: 10.1016/j.ijcard.2025.133129.CrossRefGoogle ScholarPubMed
Gali`e, N, Humbert, M, Vachiery, JL, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European society of cardiology (ESC) and the European respiratory society (ERS): endorsed by: association for European paediatric and congenital cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur. Heart J 2016; 37: : 67–119.CrossRefGoogle Scholar
van Loon, RL, Roofthooft, MT, Delhaas, T, et al. Outcome of pediatric patients with pulmonary arterial hypertension in the era of new medical therapies. Am J Cardiol 2010; 106: 117–124. DOI: 10.1016/j.amjcard.2010.02.023.CrossRefGoogle ScholarPubMed
Ploegstra, MJ, Douwes, JM, Roofthooft, MT, Zijlstra, WM, Hillege, HL, Berger, RM. Identification of treatment goals in paediatric pulmonary arterial hypertension. Eur Respir J 2014; 44: 1616–1626. DOI: 10.1183/09031936.00030414.CrossRefGoogle ScholarPubMed
Qian, Y, Quan, R, Chen, X, et al. Characteristics, long-term survival, and risk assessment of pediatric pulmonary arterial hypertension in China: insights from a national multicenter prospective registry. Chest 2023; 163: 1531–1542. DOI: 10.1016/j.chest.2022.11.038.CrossRefGoogle Scholar
Ahya, VN, Diamond, JM. Lung transplantation. Med Clin North Am 2019; 103: 425–433. DOI: 10.1016/j.mcna.2018.12.003.CrossRefGoogle ScholarPubMed
de Perrot, M, Granton, JT, Mcrae, K, et al. Outcome of patients with pulmonary arterial hypertension referred for lung transplantation: a 14-year single-center experience. J Thorac Cardiovasc Surg 2012; 143: 910–918. DOI: 10.1016/j.jtcvs.2011.08.055.CrossRefGoogle ScholarPubMed
Potts, EJ, Smith, S, Gibson, S. Anastomosis of the aorta to a pulmonary artery. JAMA 1946; 192: 627–631. DOI: 10.1001/jama.1946.02870460017005.CrossRefGoogle Scholar
Blanc, J, Vouhe, P, Bonnet, D. Potts shunt in patients with pulmonary hypertension. N Engl J Med 2004; 350: 623. DOI: 10.1056/NEJM200402053500623.CrossRefGoogle ScholarPubMed
Hansmann, G, Koestenberger, M, Alastalo, TP, et al. Updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: the european pediatric pulmonary vascular disease network (eppvdn), endorsed by aepc, espr and ishlt. J Heart Lung Transplant 2019; 38: 879–901. DOI: 10.1016/j.healun.2019.06.022.CrossRefGoogle ScholarPubMed
Baruteau, AE, Belli, E, Boudjemline, Y, et al. Palliative potts shunt for the treatment of children with drug-refractory pulmonary arterial hypertension: updated data from the first 24 patients. Eur J Cardiothorac Surg 2015; 47: e105–e110. DOI: 10.1093/ejcts/ezu445.CrossRefGoogle ScholarPubMed
Micheletti, A, Hislop, AA, Lammers, A, et al. Role of atrial septostomy in the treatment of children with pulmonary arterial hypertension. Heart 2006; 92: 969–972. DOI: 10.1136/hrt.2005.077669.CrossRefGoogle ScholarPubMed
Diller, GP, Dimopoulos, K, Broberg, CS, et al. Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study. Eur Heart J 2006; 27: 1737–1742. DOI: 10.1093/eurheartj/ehl116.CrossRefGoogle ScholarPubMed
Kurzyna, M, Dabrowski, M, Bielecki, D, et al. Atrial septostomy in treatment of end-stage right heart failure in patients with pulmonary hypertension. Chest 2007; 131: 977–983. DOI: 10.1378/chest.06-1227.CrossRefGoogle ScholarPubMed
Kula, S, Atasayan, V. Surgical and transcatheter management alternatives in refractory pulmonary hypertension: potts shunt. Anatol J Cardiol 2015; 15: 843–847. DOI: 10.5152/AnatolJCardiol.2015.6447.CrossRefGoogle ScholarPubMed
Lammers, AE, Haworth, SG, Diller, GP. Atrial septostomy in patients with pulmonary hypertension: should it be recommended? Expert Rev Respir Med 2011; 5: 363–376. DOI: 10.1586/ers.11.25.CrossRefGoogle ScholarPubMed
Kempny, A, Sorensen, HC, Li, W, et al. Response by, kempny, etal, to letter regarding article, predictors of death in contemporary adult patients with Eisenmenger syndrome: a multicenter study. Circulation 2017; 136: 1078–1079. DOI: 10.1161/CIRCULATIONAHA.117.029768.CrossRefGoogle Scholar
Baruteau, AE, Serraf, A, Levy, M, et al. Potts shunt in children with idiopathic pulmonary arterial hypertension: long-term results. Ann Thorac Surg 2012; 94: 817–824. DOI: 10.1016/j.athoracsur.2012.03.099.CrossRefGoogle ScholarPubMed
Grady, RM, Eghtesady, P. Potts shunt and pediatric pulmonary hypertension: what we have learned. Ann Thorac Surg 2016; 101: 1539–1543. DOI: 10.1016/j.athoracsur.2015.08.068.CrossRefGoogle ScholarPubMed
Gorbachevsky, SV, Shmalts, AA, Barishnikova, IY, Zaets, SB. Potts shunt in children with pulmonary arterial hypertension: institutional experience. Interact Cardiovasc Thorac Surg 2017; 25: 595–599. DOI: 10.1093/icvts/ivx209.CrossRefGoogle ScholarPubMed
Aggarwal, M, Grady, RM, Choudhry, S, Anwar, S, Eghtesady, P, Singh, GK. Potts shunt improves right ventricular function and coupling with pulmonary circulation in children with suprasystemic pulmonary arterial hypertension. Circ Cardiovasc Imaging 2018; 11: e7964. DOI: 10.1161/CIRCIMAGING.118.007964.CrossRefGoogle ScholarPubMed
Hao, Zhang, Yongxuan, Peng, Yanjun, Sun, Zhuoming, Xu, Jie, Bai. Efficacy of emergent systemic-pulmonary arterial anastomosis (Potts shunt) in a child with severe idiopathic pulmonary hypertension. Chin J Cardiol 2021; 49: 621–623. DOI: 10.3760/cma.j.cn112148-20200803-00612.Google Scholar
Bobhate, P, Mohanty, SR, Tailor, K, et al. Potts shunt as an effective palliation for patients with end stage pulmonary arterial hypertension. Indian Heart J 2021; 73: 196–204. DOI: 10.1016/j.ihj.2021.01.007.CrossRefGoogle Scholar
Grady, RM, Canter, MW, Wan, F, et al. Pulmonary-to-systemic arterial shunt to treat children with severe pulmonary hypertension. J Am Coll Cardiol 2021; 78: 468–477. DOI: 10.1016/j.jacc.2021.05.039.CrossRefGoogle ScholarPubMed
Xiafeng, Yu, Yanjun, Sun, Lijun, Fu, et al. Reversed systemic-to-pulmonary shunt for severe pulmonary hypertension in children: a single-center experience. Chin J Pediatr Surg 2024; 45: 481–486.Google Scholar
Lancaster, TS, Shahanavaz, S, Balzer, DT, Sweet, SC, Grady, RM, Eghtesady, P. Midterm outcomes of the potts shunt for pediatric pulmonary hypertension, with comparison to lung transplant. J Thorac Cardiovasc Surg 2021; 161: 1139–1148. DOI: 10.1016/j.jtcvs.2020.10.163.CrossRefGoogle ScholarPubMed
Valdeolmillos, E, Le Pavec, J, Audie, M, et al. Thirty years of surgical management of pediatric pulmonary hypertension: mid-term outcomes following reverse potts shunt and transplantation. J Thorac Cardiovasc Surg 2024; 168: 943–954. DOI: 10.1016/j.jtcvs.2023.11.045.CrossRefGoogle ScholarPubMed
Bui, MT, Grollmus, O, Ly, M, et al. Surgical palliation of primary pulmonary arterial hypertension by a unidirectional valved potts anastomosis in an animal model. J Thorac Cardiovasc Surg 2011; 142: 1223–1228. DOI: 10.1016/j.jtcvs.2010.10.060.CrossRefGoogle ScholarPubMed
Rosenzweig, EB, Ankola, A, Krishnan, U, Middlesworth, W, Bacha, E, Bacchetta, M. A novel unidirectional-valved shunt approach for end-stage pulmonary arterial hypertension: early experience in adolescents and adults. J Thorac Cardiovasc Surg 2021; 161: 1438–1446. DOI: 10.1016/j.jtcvs.2019.10.149.CrossRefGoogle ScholarPubMed
Aggarwal, M, Grady, RM. Treatment of pediatric pulmonary hypertension. Curr Treat Options Cardiovasc Med 2018; 20: 8. DOI: 10.1007/s11936-018-0597-z.CrossRefGoogle ScholarPubMed
Castro, MF, Oliveira, EC, Nunes, M, Oliveira, C, Almeida, M, Barbosa, J. Reverse potts for the treatment of severe idiopathic pulmonary hypertension in children. Braz J Cardiovasc Surg 2023; 38: e20220320. DOI: 10.21470/1678-9741-2022-0320.CrossRefGoogle ScholarPubMed
Pasupneti, S, Dhillon, G, Reitz, B, Khush, K. Combined heart lung transplantation: an updated review of the current literature. Transplantation 2017; 101: 2297–2302. DOI: 10.1097/TP.0000000000001820.CrossRefGoogle ScholarPubMed
Latus, H, Apitz, C, Schmidt, D, et al. Potts shunt and atrial septostomy in pulmonary hypertension caused by left ventricular disease. Ann Thorac Surg 2013; 96: 317–319. DOI: 10.1016/j.athoracsur.2012.10.069.CrossRefGoogle ScholarPubMed
Schafer, M, Frank, BS, Grady, RM, et al. Monitoring and evaluation of the surgical potts shunt physiology using 4-dimensional flow magnetic resonance imaging. J Thorac Cardiovasc Surg 2022; 164: 331–341. DOI: 10.1016/j.jtcvs.2021.11.025.CrossRefGoogle ScholarPubMed
Garekar, S, Meeran, T, Dhake, S, Malankar, D. Valved reverse potts shunt in a case of pulmonary hypertension due to pulmonary veno-occlusive disease. Indian J Thorac Cardiovasc Surg 2021; 37: 89–92. DOI: 10.1007/s12055-020-00993-2.CrossRefGoogle Scholar
Sabi, TM, Schmitt, B, Sigler, M, et al. Transcatheter creation of an aortopulmonary shunt in an animal model. Catheter Cardiovasc Interv 2010; 75 : 563–569. DOI: 10.1002/ccd.22150.Google ScholarPubMed
Boudjemline, Y, Sizarov, A, Malekzadeh-Milani, S, et al. Safety and feasibility of the transcatheter approach to create a reverse potts shunt in children with idiopathic pulmonary arterial hypertension. Can J Cardiol 2017; 33: 1188–1196. DOI: 10.1016/j.cjca.2017.06.004.Google ScholarPubMed
Haddad, RN, Levy, M, Szezepanski, I, Malekzadeh-Milani, S, Bonnet, D. Long-term outcomes of transcatheter potts shunt in children with suprasystemic pulmonary arterial hypertension. Front Cardiovasc Med 2022; 9: 1028304. DOI: 10.3389/fcvm.2022.1028304.CrossRefGoogle ScholarPubMed
Esch, JJ, Shah, PB, Cockrill, BA, et al. Transcatheter potts shunt creation in patients with severe pulmonary arterial hypertension: initial clinical experience. J Heart Lung Transplant 2013; 32: 381–387. DOI: 10.1016/j.healun.2013.01.1049.CrossRefGoogle ScholarPubMed
Levi, DS, Danon, S, Gordon, B, et al. Creation of transcatheter aortopulmonary and cavopulmonary shunts using magnetic catheters: feasibility study in swine. Pediatr Cardiol 2009; 30: 397–403. DOI: 10.1007/s00246-009-9422-5.CrossRefGoogle ScholarPubMed
Boudjemline, Y, Patel, M, Malekzadeh-Milani, S, Szezepanski, I, Levy, M, Bonnet, D. Patent ductus arteriosus stenting (transcatheter potts shunt) for palliation of suprasystemic pulmonary arterial hypertension: a case series. Circ Cardiovasc Interv 2013; 6: e18–e20. DOI: 10.1161/CIRCINTERVENTIONS.112.000091.CrossRefGoogle ScholarPubMed
Tianliu, Zhan, Yiwei, Chen, Jinjin, Wu, Fen, Li, Hao, Zhang, Lijun, Fu. A case of severe pulmonary hypertension in children treated by transcatheter potts shunt. Chin J Pediatr 2023; 61: 169–171. DOI: 10.3760/cma.j.cn112140-20220717-00654.Google Scholar
Mirabile, C, Malekzadeh-Milani, S, Bojan, M, et al. A case series of transcatheter potts shunt creation in a pediatric population affected with refractory pulmonary artery hypertension: focus on the role of ecmo. Perfusion 2021; 36: 415–420. DOI: 10.1177/0267659120954169.CrossRefGoogle Scholar
Schranz, D. Reverse potts shunt for pulmonary hypertension: back to a “fetal” circulation in part? J Am Coll Cardiol 2021; 78: 478–480. DOI: 10.1016/j.jacc.2021.05.038.CrossRefGoogle ScholarPubMed
Liu, Y, Zhu, Y, Jiang, C, et al. An electrochemical nitric oxide generator for in-home inhalation therapy in pulmonary artery hypertension. BMC Med 2022; 20: 481. DOI: 10.1186/s12916-022-02686-6.CrossRefGoogle ScholarPubMed