Abstract
Pulmonary hypertension (PH) is a syndrome characterized by elevated mean arterial pulmonary pressure. PH occurs in a variety of clinical situations (pulmonary arterial hypertension, cardiovascular and lung diseases, hypoxic states including high altitude sojourn, thromboembolic disease) and differs in etiology, prognosis and therapy.
PAH (pulmonary arterial hypertension) is a serious and progressive dissease leading to right ventricular failure. Regarding treatment, causal therapy does not exist, conventional therapy prevails and several specific drugs are under clinical trial.
The common feature of all groups of PH is the structural remodeling of peripheral pulmonary arteries. Recent studies confirm important role of activated mast cells in pathogenesis of pulmonary vessel wall remodeling.
It was described in a variety of animal models, which allow testing of new pathways and drugs including mast cell targeted therapy.