Current therapeutics for pulmonary arterial hypertension
Journal
Acta Cardiologica Sinica
Journal Volume
28
Journal Issue
4
Pages
267-278
Date Issued
2012
Author(s)
Abstract
Pulmonary arterial hypertension (PAH) is characterized by a progressive rise in pulmonary vascular resistance resulting from vascular remodeling, vasoconstriction, and cellular proliferation. For patients diagnosed with PAH, the prognosis is poor, with an approximate 9-15% annual mortality rate.The goal of medical therapy is to improve survival and patients' quality of life. Although PAH is an incurable orphan disease with a high mortality rate, current treatment strategies have led to considerable gains, including exercise capacity, hemodynamic and time to clinical worsening. General disease management options include avoidance of salt in the diet, oxygen, appropriate vaccinations and routine health maintenance, and avoidance of pregnancy. Traditional treatments include warfarin, diuretics, and calcium channel blockers (if patients are deemed "responders" during cardiac catheterization). Currently, three classes of drugs are approved for the treatment of PAH based on results from clinical trials prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase type 5 inhibitors. Clinical practice patterns have shifted in favor of earlier diagnosis, aggressive front line treatment, and upfront versus stepwise combination therapy. Future drug development targeting other molecular pathways of pulmonary vascular disease is essential for advancing our understanding of this disease. The purpose of this review is to summarize recent guidelines for the management of pulmonary arterial hypertension, and highlight clinical topics for the primary care physician.
Subjects
Current therapy; Pulmonary artery hypertension
SDGs
Other Subjects
ambrisentan; anticoagulant agent; beraprost; bosentan; calcium channel blocking agent; digoxin; diuretic agent; endothelin receptor antagonist; iloprost; influenza vaccine; phosphodiesterase V inhibitor; Pneumococcus vaccine; prostacyclin; prostacyclin derivative; prostanoid; sildenafil; sitaxsentan; tadalafil; treprostinil; vasodilator agent; warfarin; altitude; anemia; anticoagulant therapy; cardiopulmonary hemodynamics; chronic thromboembolic pulmonary hypertension; clinical practice; congenital heart disease; connective tissue disease; contraception; diarrhea; disease classification; drug approval; dyspepsia; early diagnosis; echocardiography; epistaxis; exercise; faintness; flushing; general practitioner; headache; heart catheterization; heart index; heart right ventricle failure; human; hypoxia; immunization; injection site pain; jaw pain; leg edema; leg pain; liver toxicity; lung disease; lung vascular resistance; male infertility; mortality; nose obstruction; oxygen therapy; pericardial effusion; physical activity; pneumonia; practice guideline; prognosis; pulmonary artery occlusion pressure; pulmonary capillary hemangiomatosis; pulmonary hypertension; pulmonary veno-occlusive disease; quality of life; recommended drug dose; review; side effect; sodium restriction; survival; testis atrophy; vaccination
Type
review