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

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 pr...

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Main Authors: Sarfraz Saleemi, Majdy M. Idrees
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2014-01-01
Series:Annals of Thoracic Medicine
Subjects:
Online Access:http://www.thoracicmedicine.org/article.asp?issn=1817-1737;year=2014;volume=9;issue=5;spage=42;epage=46;aulast=Saleemi
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author Sarfraz Saleemi
Majdy M. Idrees
author_facet Sarfraz Saleemi
Majdy M. Idrees
author_sort Sarfraz Saleemi
collection DOAJ
description 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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spelling doaj.art-cddb0985745e4a59becdf824d7a87e5b2022-12-21T18:40:22ZengWolters Kluwer Medknow PublicationsAnnals of Thoracic Medicine1817-17371998-35572014-01-0195424610.4103/1817-1737.134021Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Portopulmonary hypertensionSarfraz SaleemiMajdy M. IdreesPortopulmonary 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.http://www.thoracicmedicine.org/article.asp?issn=1817-1737;year=2014;volume=9;issue=5;spage=42;epage=46;aulast=SaleemiLiver transplantportal hypertensionportopulmonary hypertensionpulmonary arterial hypertensionvasodilator therapySaudi Association for Pulmonary Hypertension Guidelines
spellingShingle Sarfraz Saleemi
Majdy M. Idrees
Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Portopulmonary hypertension
Annals of Thoracic Medicine
Liver transplant
portal hypertension
portopulmonary hypertension
pulmonary arterial hypertension
vasodilator therapy
Saudi Association for Pulmonary Hypertension Guidelines
title Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Portopulmonary hypertension
title_full Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Portopulmonary hypertension
title_fullStr Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Portopulmonary hypertension
title_full_unstemmed Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Portopulmonary hypertension
title_short Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Portopulmonary hypertension
title_sort saudi guidelines on the diagnosis and treatment of pulmonary hypertension portopulmonary hypertension
topic Liver transplant
portal hypertension
portopulmonary hypertension
pulmonary arterial hypertension
vasodilator therapy
Saudi Association for Pulmonary Hypertension Guidelines
url http://www.thoracicmedicine.org/article.asp?issn=1817-1737;year=2014;volume=9;issue=5;spage=42;epage=46;aulast=Saleemi
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AT majdymidrees saudiguidelinesonthediagnosisandtreatmentofpulmonaryhypertensionportopulmonaryhypertension