Pulmonary Hypertension: Mechanisms, Investigations and Management

Background: Pulmonary hypertension (PH) is a hemodynamic condition characterized by a mean-pulmonary arterial pressure of ≥ 25 mmHg. The most common types of PH are pulmonary arterial hypertension (PAH), chronic thromboembolic pulmonary hypertension (CTEPH), PH caused by left heart disease, and PH due to lung disease. Previously considered incurable, PAH treatment has improved significantly since the introduction of the drug epoprostenol in 1999, with a 3-year survival rate of 30% - 40% to more than 85%. Medications available for the specific treatment of PAH include endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogs, and prostacyclin receptor, agonists. Over the last decade, the management and treatment of CTEPH have improved. Although pulmonary endarterectomy is the only treatment option for CTEPH, newer treatments include the stimulant soluble guanylate cyclase, which is an effective targeted therapy.

Conclusion: The diagnosis and treatment of severe forms of PH, in particular, pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension, is complex and best done with close collaboration between local physicians and specialized institutions.