Primary pulmonary hypertension with fatal outcome in a young woman and review of the literature

: A 32-year-old female is described, who was admitted with symptoms of severe right heart failure. The most likely diagnosis of pulmonary embolism was excluded. Echocardiography and left-right catheterisation confirmed the diagnosis of primary pulmonary hypertension. A possible mediator in the process of PPH could be the appetite suppressants she had taken for some months after her second pregnancy. Before further pharmacologic tests could be performed the patient died in circulatory collapse. Postmortem pathological examination confirmed the diagnosis of PPH by the presence of narrowed pulmonary arterioles, media hypertrophy, thrombotic lesions and normal surrounding pulmonary parenchyma. The literature on primary pulmonary hypertension is revised with special emphasis on diagnosis and treatment algorithms.