Open AccessSaudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Portopulmonary hypertension
Author(s) -
Sarfraz Saleemi,
Majdy Idrees
Publication year - 2014
Publication title -
annals of thoracic medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.639
H-Index - 33
eISSN - 1817-1737
pISSN - 1998-3557
DOI - 10.4103/1817-1737.134021
Subject(s) - portopulmonary hypertension , medicine , portal hypertension , pulmonary hypertension , cardiology , hemodynamics , vascular resistance , liver disease , cirrhosis
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.