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Pulmonary arterial hypertension


Authors: P. Jansa;  D. Ambrož;  T. Paleček;  P. Poláček;  J. Marešová;  L. Jelínková;  M. Aschermann;  A. Linhart
Published in: Kardiol Rev Int Med 2007, 9(3): 145-153

Overview

Pulmonary arterial hypertension (PAH) is a potentially fatal disease of pulmonary arterioles. Genetic predisposition plays a role in the pathophysiology of the disease, as well as a number of exogenous factors which cause endothelial dysfunction and subsequent vasoconstriction, vascular remodelling and thrombosis primarily in the region of small pulmonary arteries. The symptomatology of PAH is non–specific, hence the high rate of late-diagnosed cases of the disease. Patients with higher risk of pulmonary hypertension (i.e. systemic sclerodermia and HIV patients, patients with portal hypertension preceding liver transplantation and first grade relatives of PAH patients) should have preventative echocardiography examinations on a regular basis. The treatment of pulmonary hypertension is extremely complex and expensive, and therefore it is confined to specialised centres. The choice of PAH pharmacotherapy depends on the outcome of the acute pulmonary vasodilator test. Only patients with a positive outcome (11 % of patients) are indicated for the treatment by high doses of calcium channel blockers. This is what we call „conventional“ treatment, together with anticoagulation treatment and the treatment of heart failure. In case of a negative outcome of the test, „specific“ pharmacotherapy (prostanoids, endothelin receptor antagonists, phosphodiesterase 5 inhibitors) with both vasodilator and antiproliferative and antiaggregation effect are indicated in addition to chronic anticoagulation treatment. In patients in NYHA functional class (II) and III, treatment is initiated with bosentan, alternatively with sildenafil. Prostacyclin is the basic therapy in NYHA functional class IV. In a number of cases, monotherapy does not provide optimum response. Combination therapy is a rational alternative in such case, using several specific drugs. Once the possibilities of pharmacotherapy have been exhausted, atrial septostomy and lung transplantation can be considered. In addition to research for better therapeutic procedures for PAH, a major challenge in PAH treatment today is timely and correct diagnosing based on a correctly formulated suspicion. Still toady, great part of PAH patients is referred to specialised centres in advanced stages of the disease.

Keywords:
pulmonary arterial hypertension – conventional treatment – specific treatment – calcium channel blockers – prostanoids – endothelin receptor antagonists – phosphodiesterase 5 inhibitors


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