CLINICAL CASE OF COMBINATION THERAPY INCLUDING AMBRIZENTAN IN THE PATIENT WITH SEVERE IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION
https://doi.org/10.38109/2225-1685-2017-2-52-57
Abstract
Idiopathic pulmonary hypertension (IPAH) is a rare cardiovascular disease of unknown etiology, but the most common form of pulmonary arterial hypertension, defined as mean pulmonary arterial pressure >25 mmHg and pulmonary vascular resistance >3 Wood Units. Endothelial dysfunction seems to play an integral role in the pathogenesis of IPAH: the activation of endothelin-1 causes the hypertrophy of smooth muscle cells and endothelial cells, fibrotic changes, immune inflammation and vasoconstriction which leads to small pulmonary arteries and arterioles remodeling. The endothelial system activation is the reason for using endothelin receptor antagonists (ERA) to block the endothelin receptors type A (ETA) or both types (ETA, ETB). ERA, as the main class of PAH-specific therapy, is presented by bosentan and macitentan, dual endothelin receptor antagonists, and ambrisentan, non-sulfonamide, ETA-selective ERA. This clinical case demonstrates the successful treatment with ambrisentan as the part of combination therapy in the patient with severe IPAH.
About the Authors
G. A. Movsisyan
Russian Cardiology Research and Production Complex of the Ministry of Health
Russian Federation
Russian Federation
Resident of the department of pulmonary hypertension and heart disease
121552, Moscow, 3rd Street Cherepkovskaya, Building 15 a
O. A. Arkhipova
Russian Cardiology Research and Production Complex of the Ministry of Health
Russian Federation
Russian Federation
Researcher of the department of pulmonary hypertension and heart disease
121552, Moscow, 3rd Street Cherepkovskaya, Building 15 a
I. Z. Korobkova
Russian Cardiology Research and Production Complex of the Ministry of Health
Russian Federation
Russian Federation
PhD, board certified cardiologist of radiology department
121552, Moscow, 3rd Street Cherepkovskaya, Building 15 a
Yu. G. Matchin
Russian Cardiology Research and Production Complex of the Ministry of Health
Russian Federation
Russian Federation
Doctor of science, head of laboratory of endovascular treatment ambulance patient
121552, Moscow, 3rd Street Cherepkovskaya, Building 15 a
M. A. Saidova
Russian Cardiology Research and Production Complex of the Ministry of Health
Russian Federation
Russian Federation
MD, Professor, Head of the Laboratory of ultrasound methods of research department of new research methods
121552, Moscow, 3rd Street Cherepkovskaya, Building 15 a
T. V. Martynyuk
Russian Cardiology Research and Production Complex of the Ministry of Health
Russian Federation
Russian Federation
MD, leading researcher, head of the department of pulmonary hypertension and heart disease
21552, Moscow, 3rd Street Cherepkovskaya, Building 15 a
I. Ye. Chazova
Russian Cardiology Research and Production Complex of the Ministry of Health
Russian Federation
Russian Federation
Academician of Russian Academy of Science, MD, Director
121552, Moscow, 3rd Street Cherepkovskaya, Building 15 a
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Review
For citations:
Movsisyan G.A., Arkhipova O.A., Korobkova I.Z., Matchin Yu.G., Saidova M.A., Martynyuk T.V., Chazova I.Ye. CLINICAL CASE OF COMBINATION THERAPY INCLUDING AMBRIZENTAN IN THE PATIENT WITH SEVERE IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION. Eurasian heart journal. 2017;(2):52-57. (In Russ.) https://doi.org/10.38109/2225-1685-2017-2-52-57
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