Abstract
BACKGROUND: pulmonary arterial hypertension (PAH) is a rare complication of hepatic diseases with portal hypertension that, however, has a significant influence on prognosis. We present a mini-review of how to diagnose and treat it based on a clinical case. CASE PRESENTATION: in early childhood, a patient had portal hypertension associated with cavernous transformation of the portal vein. It was successfully treated by reno-splenic surgery. At the age of 20 years, this patient experienced increased dyspnea at minimal physical activity after the hepatic biopsy due to a hepatocellular adenoma. The examination in the specialized unit showed PAH, which was evaluated as associated with portal hypertension (PAH-PoH). The specific two-drug combination therapy was started with prominent improvement in patient's state. Successful surgical tumor treatment was provided some months later. The practical and clinical approaches to the diagnosis and treatment of PAH-PoH are discussed. It was emphasized that not all patients with portal hypertension have pulmonary hypertension, which needs to be treated. A lot of evidence gaps exist in management of these patients. CONCLUSION: all patients, even with past history of portal hypertension, should be monitored closely and screened for PAH earlier, for better results of treatment.