Annals of Thoracic Medicine Official publication of the Saudi Thoracic Society, affiliated to King Saud University
 
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GUIDELINES
Year : 2014  |  Volume : 9  |  Issue : 5  |  Page : 42-46

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Portopulmonary hypertension


1 Department of Pulmonary Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
2 Prince Sultan Medical Military City, Riyadh, Saudi Arabia

Correspondence Address:
Sarfraz Saleemi
FCCP, Division of Pulmonary Medicine, Department of Medicine, King Faisal Specialist Hospital and Research Center, PO Box 3354, Riyadh 11211
Saudi Arabia
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DOI: 10.4103/1817-1737.134021

PMID: 25076996

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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.


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