Bosentan Therapy in a Patient with Failed Fontan Procedure: A Case Report

  • Ting Lu Department of Cardiovascular Surgery, Second Xiangya Hospital of Central South University, Changsha, Hunan, China
  • Mi Tang Department of Cardiovascular Surgery, Second Xiangya Hospital of Central South University, Changsha, Hunan, China
  • Zhongshi Wu Department of Cardiovascular Surgery, Second Xiangya Hospital of Central South University, Changsha, Hunan, China
  • Can Huang Department of Cardiovascular Surgery, Second Xiangya Hospital of Central South University, Changsha, Hunan, China

Abstract

Background: Increased pulmonary vascular resistance index (PVR) leads to several complications in patients after a Fontan operation. This increase is mainly attributed to the overexpression of endothelin-1 for a long duration after the Fontan procedure. Here, we describe the case of a 3-year-old boy with a failed Fontan operation who was treated with bosentan, an endothelin-1 receptor blocker.

Case report: Cardiac catheterization was performed, which showed a main pulmonary artery pressure (MPAP) of 19 mmHg and PVRI of 5.6 woods/m2. Oral bosentan regimen at a dose of 31.25 mg was initiated twice a day. The treatment was continued as pleural effusion and ascites persisted. No adverse events were observed, and the treatment was well tolerated. Pleural effusion disappeared, and ascites decreased markedly after 4 weeks, whereas the MPAP was
15 mmHg and the PVRI was 4.3 woods/m2. After 3 months of bosentan therapy, the MPAP was 12 mmHg and the PVRI was 4.1 woods/m2.

Conclusion: We observed that bosentan reduces the PVRI and complications such as pleural effusion and ascites after a failed Fontan procedure.

References

Bowater SE, Weaver RA, Thorne SA, Clift PF. 2012. The safety and effects of bosentan in patients with a fontan circulation. Congenit Heart Dis 7:243-9.

Derk G, Houser L, Miner P, et al. 2015. Efficacy of endothelin blockade in adults with fontan physiology. Congenital heart disease. Congenit Heart Dis 10:E11-6.

Goldberg DJ, French B, McBride MG, et al. 2011. Impact of oral sildenafil on exercise performance in children and young adults after the fontan operation: a randomized, double-blind, placebo-controlled, crossover trial. Circulation 123:1185-93.

Hebert A, Mikkelsen UR, Thilen U, et al. 2014. Bosentan improves exercise capacity in adolescents and adults after fontan operation: The TEMPO (Treatment with Endothelin Receptor Antagonist in Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen Consumption) Study. Circulation 130:2021-30.

Hirono K, Yoshimura N, Taguchi M, et al. 2010. Bosentan induces clinical and hemodynamic improvement in candidates for right-sided heart bypass surgery. J Thorac Cardiovasc Surg 140:346-51.

Mitchell MB, Campbell DN, Ivy D, et al. 2004. Evidence of pulmonary vascular disease after heart transplantation for Fontan circulation failure. J Thorac Cardiovasc Surg 128:693-702.

Schuuring MJ, Vis JC, van Dijk AP, et al. 2013. Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial. Eur J Heart Fail 15:690-8.

Published
2019-05-08
How to Cite
Lu, T., Tang, M., Wu, Z., & Huang, C. (2019). Bosentan Therapy in a Patient with Failed Fontan Procedure: A Case Report. The Heart Surgery Forum, 22(3), E213-E214. https://doi.org/10.1532/hsf.2313
Section
Articles