A Rare Cause Of Polycythemia: Idiopathic Pulmonary Arterial Hypertension

Idiopathic pulmonary arterial hypertension (IPAH) with an incidence of 1-2/million per-year. IPAH is a term used to define a variety of progressive conditions that have in common, increased pulmonary vascular resistance leading to right heart failure and death. Pulmonary arterial hypertension is presented with cyanosis, polyctemia and right heart failure. Increased prevelance of mortality is due to progression and late diagnosis of the disease. We present this case to alert physicians to keep in mind rare but serious disease IPAH in the differential diagnosis of polyctemia.