[Primary pulmonary hypertension]

In 1% of the patients with cor pulmonale the cause of the high pulmonary artery pressure remains unclear. The underlying severe and mostly progressive pulmonary vascular disease with unknown aetiology is defined as primary pulmonary hypertension (PPH) with three different pathomorphological subtypes, plexogenic pulmonary arteriopathy (PPA), thrombotic pulmonary arteriopathy (TPA) and pulmonary venoocclusive disease (PVOD). The endemic occurrence of PPH after the ingestion of anorexigenic drugs (aminorex fumarate) and toxic rapeseed oil lead to the hypothesis that PPH is a pulmonary vascular reaction to exogenous toxic agents on the base of a genetic disposition. The initial response could be an endothelial cell dysfunction leading to pathological proliferation of vascular smooth muscle cells, vasospasm and local disturbances of haemostasis. The derived therapeutic concepts with vasodilators (high dose calcium channel-blocking therapy, prostacyclin) and with anticoagulant drugs show some encouraging results. The lung and heart-lung transplantation have become real therapeutic options for the patients with PPH considering the mostly still very unfavourable prognosis of PPH.