Primary pulmonary hypertension associated with portal hypertension in a cirrhotic female patient

: We present the case of a 28-year-old woman with hepatic cirrhosis, portal hypertension and hypersplenism associated with primary oligosymptomatic pulmonary hypertension, who had a survival somewhat longer than usual (10 years since she was diagnosed as pulmonary hypertension). The patient died in hepatic coma after upper gastrointestinal bleeding. The association between portal and pulmonary hypertension is rare, its true prevalence not being known and its pathogenesis being unclear. We remark the diagnostic tools and therapeutic possibilities.