A Lethal Side Effect of Treatment by Macitentan

Abstract

Pulmonary arterial hypertension is a progressive and ultimately fatal disease despite the availability of a number of new therapies, including endothelin receptor antagonists. Many side effects have been reported with the use of these drugs, such as hepatotoxicity, peripheral edema, anemia and other digestive reactions. Cardiac side effects have been rarely mentioned. We report the case of a 17-year-old girl with Eisenmenger syndrome secondary to double outlet right ventricle and sub-pulmonary ventricular septal defect, Macitentan treatment has been started, three days later, she presented a ST segment elevation in septo-apico- lateral territory which shortly complicated with cardiogenic shock and death. A coronary arteritis or acute myocarditis of toxic origin was the two evoked diagnoses. This is the first human case in the medical literature describing a relationship between initiation of Macitentan and the onset of this event. This case highlights a lethal side effect of Macitentan, which should prompt patients to notify their doctors of any symptoms suggestive of coronary or myocardial injury (chest pain, dyspnea, heart failure symptoms), and implementation of electrocardiogram in combination with a control in any patient who develops chest pain on Macitentan.