Macitentan therapy for bosentan hepatic intolerance in Eisenmenger’s syndrome patient. (RCD code: II‑1A.4d)


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Abstract


Pulmonary arterial hypertension associated with congenital heart disease is included in clinical group 1 pulmonary arterial hypertension. Eisenmenger’s syndrome develops over time as a result of large intra- and extra-cardiac arterial and venous blood communication. The pulmonary vascular resistance (PVR) increases and thus the systemic-to pulmonary blood flow reverses direction, producing a pulmonary-to-systemic shunt (Eisenmenger’s syndrome). Bosentan, an oral endothelin receptor antagonist A and B, is recommended in monotherapy and drug combination therapy in this group of patients. Liver toxic reactions occur in about 10% of treated patients but severe hepatotoxicity is rare. We present a clinical case of a patient with Eisenmenger’s syndrome due to large ventricular septal defect. The patient was for many years successfully treated with bosentan and subsequently developed drug-induced hepatitis. JRCD 2016; 2 (8): 259–262


Keywords


congenital heart disease; pulmonary hypertension; hepatotoxicity; endothelin receptor

References


Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2016; 37: 67–119.

Lee YH, Song GG. Meta‑analysis of randomized controlled trials of bosentan for treatment of pulmonary arterial hypertension. Korean J Intern Med 2013; 28(6): 701–707.

McLaughlin VV, Sitbon O, Badesch DB, et al. Survival with first‑line bosentan in patients with primary pulmonary hypertension. Eur Respir J 2005; 25: 244–249.

Galiè N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double‑blind, randomized, placebo‑controlled study. Circulation 2006; 114: 48–54.

Galiè N, Rubin LJ, Hoeper M, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double‑blind, randomized controlled trial. Lancet 2008; 371: 2093–2100.

Humbert M, Segal ES, Kiely DG, et al. Results of European post‑marketing surveillance of bosentan in pulmonary hypertension. Eur Respir J 2007; 30: 338–344.

McLaughlin VV, Sitbon O, Badesch DB, et al. Survival with first‑line bosentan in patients with primary pulmonary hypertension. Eur Respir J 2005; 25: 244–249.

Eriksson C, Gustavsson A, Kronvall T, et al. Hepatotoxicity by Bosentan in a Patient with Portopulmonary Hypertension: a Case‑Report and Review of the Literature. Med Clin 2014; 142 (8): 333–342.

Pulido T, Adzerikho I, Channick RN. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369: 809–818.

Raghu G, Million‑Rousseau R, Morganti A, et al. Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial. Eur Respir J 2013; 42: 1622–1632.




DOI: http://dx.doi.org/10.20418%2Fjrcd.vol2no8.254

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