- HPS is a term used when pulmonary hypertension is present with liver disease but the absence of portal HTN.
- PPHTN is a term used exclusively when portal hypertension is present with or without the hepatic disease.
Clinical exam can also provide this distinction at bedside as patients with HPS will have platypnea and orthodeoxia.
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4. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015; 46:903.