ABSTRACT
Pulmonary hypertension is a complex disorder, defined as mean pulmonary artery pressure ≥20mmHg. The disease may encompass various clinical conditions and diverse pathophysiologic mechanisms, all sharing the same denominator: the increase of the pulmonary vascular resistance. The chronically increased right ventricular pressure overload leads to right ventricular remodeling and finally to right heart failure, characterized by impaired right ventricular function, insufficient blood flow to the pulmonary circulation and/or elevated filling pressures at rest or during physiologically demanding conditions. The symptoms remain nonspecific and include exertional dyspnea, fatigue, weakness, chest pain, syncope, etc. At later stages, symptoms of right heart failure may be present, mainly congestion. The diagnosis of PH requires clinical suspicion and a comprehensive set of diagnostic tests to confirm that hemodynamic criteria for pulmonary hypertension are met and to describe the etiology and the severity of the disease. Despite advancements in the pharmacotherapy of pulmonary arterial hypertension (PAH) in the last decades, it remains non-curable with severe prognosis. Advanced treatments targeting the three pathways that contribute to the pathogenesis of PAH (endothelin, nitric oxide, and prostacyclin pathways) include endothelin receptor antagonists, phosphodiesterase 5 inhibitors, soluble guanylate cyclase stimulator, and prostanoids, and have changed the natural course of PAH.
