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Individualised interventional strategies and clinical outcomes in paediatric patients with pulmonary arterial hypertension
Published online by Cambridge University Press: 23 February 2026
Abstract
Objective:
This study evaluated the safety and efficacy of individualised interventional strategies in paediatric pulmonary arterial hypertension, focusing on the outcomes of atrial septostomy, reverse Potts shunt, and partial ASD closure in patients unresponsive to medical therapy or with rapid right ventricular deterioration.
Methods:
A retrospective analysis included 12 paediatric patients who underwent 18 interventional procedures between 2019 and 2024. Pulmonary arterial hypertension was confirmed by right heart catheterisation, and pre- and post-procedural clinical, echocardiographic, and haemodynamic data were compared.
Results:
Interventions included graded balloon atrial septostomy (n = 10), partial ASD closure (n = 2), and reverse Potts shunt via ductal stenting (n = 1). All surviving patients showed clinical improvement with significant reductions in heart rate (p < 0.01), NT-proBNP (p = 0.008), mean right atrial pressure (p < 0.001), and RV/LV end-diastolic diameter ratio (p = 0.014), along with improvements in six-minute walk distance (p = 0.002), WHO functional class (p < 0.001), and TAPSE (p = 0.028). One patient (8.3%) died within days following atrial septostomy due to nonadherence to medical therapy. Three patients required repeat atrial septostomy because of shunt restriction or spontaneous narrowing.
Conclusion:
Individualised interventional strategies guided by comprehensive haemodynamic assessment can slow disease progression and improve quality of life in paediatric pulmonary arterial hypertension. Partial ASD closure, as a novel approach in this cohort, reduces excessive left-to-right volume load while preserving a controlled right-to-left shunt, thereby enhancing haemodynamic stability and optimising outcomes. Timely implementation of interventional strategies before disease progression may help reduce mortality.
Keywords
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- © The Author(s), 2026. Published by Cambridge University Press
References
Humbert, M, Kovacs, G, Hoeper, MM, et al. ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension developed by the task force for the diagnosis and treatment of (ESC) and the European Respiratory Society (ERS). Eur Heart J 2022; 43: 3618–3731.10.1093/eurheartj/ehac237CrossRefGoogle Scholar
Haworth, SG, Hislop, AA. Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001 2006. Heart 2009; 95: 312–317.10.1136/hrt.2008.150086CrossRefGoogle ScholarPubMed
Ivy, D, Rosenzweig, EB, Abman, SH, et al. Embracing the challenges of neonatal and paediatric pulmonary hypertension. Eur Respir J 2024; 64: 2401345.10.1183/13993003.01345-2024CrossRefGoogle ScholarPubMed
Hansmann, G, Koestenberger, M, Alastalo, TP, et al. 2019 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 Transpl 2019; 38: 879–901.10.1016/j.healun.2019.06.022CrossRefGoogle ScholarPubMed
Doyle, RL, McCrory, D, Channick, RN, Conte, J. Surgical treatments/interventions for pulmonary arterial hypertension. Chest. 2004;126(1 Suppl):63S–71S.10.1378/chest.126.1_suppl.63SCrossRefGoogle ScholarPubMed
Boshoff, DE, Butera, G, Gewillig, M. Creating an interatrial communication. In: Butera, G, Chessa, M, Eicken, A, Thomson, J (eds.) Cardiac Catheterization for Congenital Heart Disease: From Fetal Life to Adulthood. 2nd ed. Springer, Cham, 2020, pp 687–705.Google Scholar
Barst, RJ. Role of atrial septostomy in the treatment of pulmonary vascular disease. Thorax 2000; 55: 95–96.10.1136/thorax.55.2.95CrossRefGoogle ScholarPubMed
Kothari, SS 1, Yusuf, A, Juneja, R, Yadav, R, Naik, N. Graded balloon atrial septostomy in severe pulmonary hypertension. Indian Heart J 2002; 54: 164–169.Google 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: 969–972.10.1136/hrt.2005.077669CrossRefGoogle ScholarPubMed
Rothman, A, Sklansky, MS, Lucas, VW, et al. Atrial septostomy as a bridge to lung transplantation in patients with severe pulmonary hypertension. Am J Cardiol 1999; 84: 682–686.10.1016/S0002-9149(99)00416-6CrossRefGoogle ScholarPubMed
Sandoval, J. Interventional therapies in pulmonary hypertension. Revista Española de Cardiología (English Edition) 2018; 71: 565–574.10.1016/j.rec.2018.02.002CrossRefGoogle ScholarPubMed
Sivakumar, K, Rohitraj, GR, Rajendran, M, Thivianathan, N. Study of the effect of occlutech atrial flow regulator on symptoms, hemodynamics, and echocardiographic parameters in advanced pulmonary arterial hypertension. Pulm Circ 2021; 11: 1–10.10.1177/2045894021989966CrossRefGoogle ScholarPubMed
Butera, G, Piccinelli, E, Kolesnik, A, et al. Implantation of atrial flow regulator devices in patients with congenital heart disease and children with severe pulmonary hypertension or cardiomyopathy—an international multicenter case series. Front Cardiovasc Med 2023; 10: 1332395.10.3389/fcvm.2023.1332395CrossRefGoogle ScholarPubMed
D’Alto, M, Romeo, E, Argiento, P, et al. Pulmonary arterial hypertension with left to right shunts: when to treat and/or close? Int J Cardiol Congenit Heart Dis 2024; 17: 100526.Google ScholarPubMed
Baruteau, AE, Serraf, A, Lévy, M, et al. Potts shunt in children with idiopathic pulmonary arterial hypertension: Long-term results. Ann Thorac Surg 2012; 94: 817–824.10.1016/j.athoracsur.2012.03.099CrossRefGoogle ScholarPubMed
Latus, H, Apitz, C, Moysich, A, et al. Creation of a functional Potts shunt by stenting the persistent arterial duct in newborns and infants with suprasystemic pulmonary hypertension of various etiologies. J Heart Lung Transplant 2014; 33: 542–546.10.1016/j.healun.2014.01.860CrossRefGoogle ScholarPubMed
Kovacs, G, Bartolome, S, Denton, CP, et al. Definition, classification and diagnosis of pulmonary hypertension. Eur Respir J 2024; 64: 2401324.10.1183/13993003.01324-2024CrossRefGoogle ScholarPubMed
Bruch, L, Winkelmann, A, Sonntag, S, et al. Fenestrated occluders for treatment of ASD in elderly patients with pulmonary hypertension and/or right heart failure. J Interv Cardiol 2008; 21: 44–49.10.1111/j.1540-8183.2007.00324.xCrossRefGoogle ScholarPubMed
Abdelkarim, A, Levi, DS, Tran, B, Ghobrial, J, Aboulhosn, J. Fenestrated transcatheter ASD closure in adults with diastolic dysfunction and/or pulmonary hypertension: case series and review of the literature. Congenit Heart Dis 2016; 11: 663–671.10.1111/chd.12367CrossRefGoogle ScholarPubMed
Chiu, JS, Zuckerman, WA, Turner, ME, et al. Balloon atrial septostomy in pulmonary arterial hypertension: effect on survival and associated outcomes. J Heart Lung Transplant 2015; 34: 376–380.10.1016/j.healun.2015.01.004CrossRefGoogle ScholarPubMed
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