title: Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Portopulmonary hypertension.
abstract: Portopulmonary hypertension (POPH) is defined as pulmonary arterial hypertension (PAH) complicated by portal hypertension, with or without advanced hepatic disease. Significant percentage of patients with cirrhotic liver disease has high cardiac output and subsequently elevated pulmonary arterial pressures (PAP). However, patients with POPH develop a progressive increase in pulmonary vascular resistance (PVR), which is generally lower than that observed in other forms of PAH. The prognosis of untreated patients with POPH is very poor and the outcome of liver transplant (LT) in those patients is determined by the degree of severity of the associated pulmonary hemodynamics. In this narrative review, we describe the clinical presentation of POPH, the pathobiology, and the clinical implication of pulmonary hemodynamics. We also provide evidence-based recommendations for the diagnosic and management approaches of POPH.
authors: Department of Pulmonary Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
authors: Sarfraz
authors: S
authors: Saleemi
authors: Prince Sultan Medical Military City, Riyadh, Saudi Arabia.
authors: Majdy M
authors: MM
authors: Idrees
keywords: Liver transplant; Saudi Association for Pulmonary Hypertension Guidelines; portal hypertension; portopulmonary hypertension; pulmonary arterial hypertension; vasodilator therapy