Early Postoperative Triple Targeted Therapy in Patients With CHD-PAH in the "Grey Zone"

Summary

The purpose of this study is to observe the efficacy of early application of triple targeted drug therapy, including trilostane, in patients with congenital heart disease who are in the "gray zone" of preoperative evaluation, have surgical repair indications after evaluation, and have undergone defect repair.

Timeline

Start

2022-08-25

Primary completion

2025-07-30

Completion

2025-08-31

Publications

• Background Baumgartner H, De Backer J, Babu-Narayan SV, Budts W, Chessa M, Diller GP, Lung B, Kluin J, Lang IM, Meijboom F, Moons P, Mulder BJM, Oechslin E, Roos-Hesselink JW, Schwerzmann M, Sondergaard L, Zeppenfeld K; ESC Scientific Document Group. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021 Feb 11;42(6):563-645. doi: 10.1093/eurheartj/ehaa554. No abstract available.
• Background Skoro-Sajer N, Gerges C, Balint OH, Kohalmi D, Kaldararova M, Simkova I, Jakowitsch J, Gabriel H, Baumgartner H, Gerges M, Sadushi-Kolici R, Celermajer DS, Lang IM. Subcutaneous treprostinil in congenital heart disease-related pulmonary arterial hypertension. Heart. 2018 Jul;104(14):1195-1199. doi: 10.1136/heartjnl-2017-312143. Epub 2018 Feb 7.
• Background Thomas IC, Glassner-Kolmin C, Gomberg-Maitland M. Long-term effects of continuous prostacyclin therapy in adults with pulmonary hypertension associated with congenital heart disease. Int J Cardiol. 2013 Oct 9;168(4):4117-21. doi: 10.1016/j.ijcard.2013.07.072. Epub 2013 Jul 25.
• Background Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation. 1999 Apr 13;99(14):1858-65. doi: 10.1161/01.cir.99.14.1858.
• Background Kozlik-Feldmann R, Hansmann G, Bonnet D, Schranz D, Apitz C, Michel-Behnke I. Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016 May;102 Suppl 2:ii42-8. doi: 10.1136/heartjnl-2015-308378.
• Background Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019 Apr 2;139(14):e637-e697. doi: 10.1161/CIR.0000000000000602. No abstract available.

Drugs

Indications