[Primary pulmonary hypertension: new treatment options]

Primary pulmonary hypertension (PPH) is a rare, progressive and life-threatening pulmonary vascular disease of unknown aetiology, which results in elevated pulmonary vascular resistance, pulmonary hypertension and eventually right ventricle failure. PPH occurs in all age groups, but most frequently in women aged 20-30 years old. The mean survival after the onset of symptoms is 2-3 years. Although the disease remains incurable at present, major advances in treatment options have been made during the last 10 years. This had led to a considerable improvement in extended survival and the quality of life in a substantial number of PPH patients. At present the most important treatment options include anticoagulants, calcium channel antagonists, chronic prostacyclin therapy (cost of this only reimbursed by health insurers in the Netherlands since 1999) and if needs be lung or heart-lung transplantation. Chronic continuous intravenous administration of epoprostenol is burdensome for the patient and requires adequate patient selection and a well-developed intramural and extramural infrastructure.