[Using nonselective endothelin receptor antagonist in idiopathic pulmonary hypertension].

ABSTRACT

Idiopathic (primary) pulmonary hypertension (IPH) is a rare disease of unknown etiology, which is characterized by elevated pulmonary artery pressure, increased total pulmonary vascular resistance, frequently a malignant course with evolving right ventricular decompensation, and a fatal outcome. The diagnosis of IPH is established on the increments in the mean resting and exercise pulmonary artery trunk pressure by more than 25 and more than 30 mm Hg at rest and during exercise, respectively, with a normal pulmonary artery wedge pressure. Endothelin receptor antagonists (ERA) are one of the effective classes of drugs for the treatment of patients with IPH. Bosentan is the first drug from the ERA class that blocks the receptors of both types and that has been recommended by the WHO to treat patients with functional class II-IV pulmonary hypertension. The described case demonstrates the possibility of concomitantly using bosentan in a female patient with IPH shortly after ineffective treatment with a calcium antagonist.