Why is pulmonary hypertension so frustrating?

Pulmonary hypertension (PH) is a relatively uncommon but on the other hand all too frequently fatal disorder of the pulmonary arteries with diverse etiology. Vascular remodeling leads to increased pulmonary vascular resistance and this again to right ventricular overload and failure. Discussed are the current classification, pathogenesis and particularly treatment possibilities and contemporary trends, as well as the impact on the natural history of this disorder. The effect of conventional PH management of the past decades was insufficient. Understanding the pathophysiological mechanisms of the disease development was the greatest progress made in the last years. Targeting the pathogenic pathways of endothelial function is now being utilized in the so called specific therapy, which to some point improves patients' clinical course and life expectancy. The therapy of PH today seems to have the potential to slow down the progression of the disease and with some new promising molecules on the horizon there might be hope even to reverse the entire disease process. On the whole though, PH treatment remains frustrating because the majority of up-to-date managed and treated patients have still low exercise tolerance, unacceptable hemodynamics, suboptimal life quality and their mortality stays high. Despite all the advances, still the most important goal remains a wide-spread early detection of PH patients and associated conditions leading to PH (e.g. congenital heart defects and connective tissue diseases) and a centralized management of the disease in highly specialized expert centers (Tab. 1, Fig. 4, Ref. 36).