Hostname: page-component-8448b6f56d-cfpbc Total loading time: 0 Render date: 2024-04-16T05:54:42.802Z Has data issue: false hasContentIssue false

Clinical impact of subcutaneous treprostinil in trisomy 21 patient with pulmonary arterial hypertension associated with CHD

Published online by Cambridge University Press:  10 January 2022

Ryusuke Numata*
Affiliation:
Department of Pediatric Cardiology, Nagano Children’s Hospital, Nagano, Japan
Kiyohiro Takigiku
Affiliation:
Department of Pediatric Cardiology, Nagano Children’s Hospital, Nagano, Japan
Kouta Takei
Affiliation:
Department of Pediatric Cardiology, Nagano Children’s Hospital, Nagano, Japan
*
Author for correspondence: R. Numata, Department of Pediatric Cardiology, Nagano Children’s Hospital, Azumino, Nagano 8288, Japan. Tel: +81-263-73-6700; Fax: +81-263-73-555. E-mail: k054eb@gmail.com

Abstract

Subcutaneous treprostinil is commonly used to improve idiopathic pulmonary arterial hypertension in children. However, its effectiveness has not been reported in trisomy 21. We report the case of 9-year-old boy in trisomy 21 with CHD-pulmonary artery hypertension after surgical correction of CHD. Haemodynamics and exercise capacity dramatically improved with a transition from oral selexipag to subcutaneous treprostinil.

Type
Brief Report
Copyright
© The Author(s), 2022. 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.)

References

van der Velde, ET, Vriend, JW, Mannens, MM, Uiterwaal, CS, Brand, R, Mulder, BJ. CONCOR, an initiative towards a national registry and DNA-bank of patients with congenital heart disease in the Netherlands: rationale, design, and first results. Eur J Epidemiol 2005; 20: 549557.CrossRefGoogle Scholar
Pascall, E, Tulloh, RM. Pulmonary hypertension in congenital heart disease. Future Cardiol 2018; 14: 343353.CrossRefGoogle ScholarPubMed
Yamaki, S, Horiuchi, T, Takahashi, T. Pulmonary changes in congenital heart disease with Down’s syndrome: their significance as a cause of postoperative respiratory failure. Thorax 1985; 40: 380386.CrossRefGoogle ScholarPubMed
Galie, 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: 67119.CrossRefGoogle Scholar
Barst, RJ, Rubin, LJ, Long, WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996; 334: 296301.CrossRefGoogle ScholarPubMed
Levy, M, Del Cerro, MJ, Nadaud, S, et al. Safety, efficacy and management of subcutaneous treprostinil infusions in the treatment of severe pediatric pulmonary hypertension. Int J Cardiol 2018; 264: 153157.CrossRefGoogle ScholarPubMed
Zuckerman, WA, Leaderer, D, Rowan, CA, Mituniewicz, JD, Rosenzweig, EB. Ambrisentan for pulmonary arterial hypertension due to congenital heart disease. Am J Cardiol 2011; 107: 13811385.CrossRefGoogle ScholarPubMed
Rosenzweig, EB, Abman, SH, Adatia, I, et al. Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. Eur Respir J 2019; 53: 1801916.CrossRefGoogle Scholar
Rosenzweig, EB, Kerstein, D, Barst, RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999; 99: 18581865.CrossRefGoogle ScholarPubMed
Skoro-Sajer, N, Gerges, C, Balint, OH, et al. Subcutaneous treprostinil in congenital heart disease-related pulmonary arterial hypertension. Heart 2018; 104: 11951199.CrossRefGoogle ScholarPubMed