Anticoagulation for pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) often affects young patients for whom life-long anticoagulation can represent an incisive decision. Therefore, it is important to analyze the sometimes conflicting clinical and scientific results for this therapy. PAH is often accompanied by thrombosis and thrombo-embolisms in the pulmonary circulation, in particular the small pulmonary arterioles with prothrombotic and reduced antifibrinolytic activity due to endothelial dysfunction. Measurable alterations of coagulation factors are of prognostic significance. Thrombocyte function is also disrupted. Furthermore, most patients with PAH have a right-sided cardiac insufficiency with an enlarged right ventricle and reduced circulation. In addition to these pathophysiologic findings four non-randomized studies, which allow the assumption of improved prognosis by phenprocoumon therapy, have confirmed the rational of anticoagulation for PAH and the recommendations of national and international guidelines. Clinical studies on alternatives, such as the administration of acetylsalicylic acid have not yet been carried out. Efforts should be made to diagnose the disease as early as possible in order to correct alterations in coagulation and thrombocyte function by an early therapy. © 2009 Springer Medizin Verlag.