RESUMEN
Primary pulmonary arterial hypertension, so called idiopathic pulmonary arterial hypertension (IPAH), is a rare and progressive disease with poor prognosis. Pregnancy in patients with this condition is hazardous and makes the prognosis significantly worse. According to WHO, IPAH is a contraindication to pregnancy because of high risk of maternal death and WHO advises to discuss termination in the event of pregnancy Below we describe a case of a young woman at 16 weeks pregnancy with severe decompensated primary pulmonary hypertension. The patient was admitted to our department because of increasing dyspnoea and swollen legs occurring from 14th week of pregnancy. In the past the patient had been diagnosed with pulmonary hypertension, which had been defined during differential diagnostics as primary pulmonary hypertension. Echocardiographic examination over the last 4 years revealed stable mean pulmonary artery pressure (PAP) of about 50 mmHg. The patient was treated efficiently with sildenafil for the last 2 years, but the therapy was discontinued after finding pregnancy. On admission it was established that pregnancy should be terminated. Other reasons of circulatory decompensation, such as pulmonary embolism, cardiac tamponade or pulmonary diseases, were excluded.