[Pulmonary hypertension--clinical aspects, pathophysiology, diagnostic and therapy]

Pulmonary hypertension (PH) is a disease which is characterised by an increase in the mean pulmonary arterial pressure (mPAP) in the lung circulation of over 25 mmHg in rest and over 30 mmHg in movement. Due to the chronic overload of the right ventricle, the heart is always affected by a PH and often develops a so-called cor pulmonale chronicum which can lead to right-heart failure. There are five groups in the clinical WHO Venice classification which are arranged according to pathogenetical, clinical and therapeutical criteria. In addition, an adjusted NYHA classification helps to grade the significance of the disease stages. Principally, one classifies a mostly isolated form of the pulmonary arterial hypertension (PAH) and other secondary forms of the PH which develop on the grounds of existing problems such as left-heart diseases, hypoxic lung diseases, pulmonary embolism and infections. The pathophysiological reasons for a PH are just as various as the different manifestations. Yet there are generally four main alterations in the walls of the pulmonary vessels. This includes vasoconstriction, rarefaction of vessels, vascular remodelling and the occlusion of vascular lumen by a thrombus with subsequent structural remodelling of the vascular and mounted extracellular matrix. The diagnostic procedure should be algorithm-oriented and includes anamnesis, physical examination, electrocardiogram (ECG), thoracic x-ray and echocardiography. To confirm the diagnosis and for a better measuring of the prognosis, an examination with a right-heart flow-directed balloon-tipped catheter is favourable. Because of the change in the pathophysiological concepts of the PH from a vasoconstrictive to a vasoproliterative genesis, additional pharmacological targets are developed for therapeutic treatment. Today the former regime of therapy with high-dosed calcium-channel blockers such as vasodilatators only finds application after pharmacological testing at so-called responders. The current scheme of therapy is focused on the synergic effects of different drugs, such as prostacyclines, endothelial-receptor blockers and phosphodiesterase-5 inhibitors. After the failure of pharmacological treatments, the endarteriectomy remains as the last therapy option, although it is accompanied by poor survival rates.