＜Editors' Choice＞ Pulmonary Hypertension: Diagnosis, Management, and Treatment

Abstract

Pulmonary hypertension (PH) is a hemodynamic state that is characterized by a resting mean pulmonary artery pressure ≧ 25 mmHg. The common forms 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 regarded as untreatable, the treatment of PAH has dramatically advanced since the introduction of the drug epoprostenol in 1999, with three-year survival rates improving from 30%-40% to over 85%. Drugs 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. In the past decade, management and treatment of CTEPH have also improved. While pulmonary endarterectomy used to be the only option for the treatment of CTEPH, newer treatments include a soluble guanylate cyclase stimulator, which has proven to be an efficacious targeted therapy. Other cases benefit from balloon pulmonary angioplasty.

Keywords: Pulmonary hypertension; chronic thromboembolic pulmonary hypertension (CTEPH); pulmonary arterial hypertension; pulmonary hypertension (PAH).

Fig. 1. Diagnostic algorithm for pulmonary arterial hypertension

Differential diagnosis of pulmonary arterial hypertension based on mean pulmonary arterial pressure, pulmonary arterial wedge pressure (PAWP), and pulmonary vascular resistance (PVR). CTEPH: chronic thromboembolic pulmonary hypertension. WU, Wood units