The current treatment of pulmonary hypertension.

ABSTRACT

Pulmonary vascular hypertension in general is a progressive, nearly always fatal condition that until recently has had very few treatment options. Our understanding of the pulmonary vascular disease process has opened the window to earlier screening techniques, diagnosis, and treatment options. However, all current treatment options are complex and expensive and therefore require clinical support strategies often necessitating specialized pulmonary hypertension treatment centers. Whether idiopathic or secondary, pulmonary arterial hypertension is characterized by the deregulated proliferation of pulmonary artery endothelial cells and intimal smooth muscle cells, both resistant to cellular apoptosis. Early recognition of such disregulation may lead to earlier diagnosis and treatment and thus alteration in the disease process. Screening of high-risk populations such as those with connective tissue disorders, HIV disease, congenital heart disease, portal hypertension, and those exposed to certain drugs and toxins such as methamphetamines and the diet drugs Dexfenfluramine and Fenfluramine is of utmost importance. Similarly, early symptom recognition in these high-risk groups is essential to earlier diagnosis and treatment.