Portopulmonary Hypertension: From Bench to Bedside
Portopulmonary hypertension (PoPH) is defined as pulmonary arterial hypertension (PAH) associated with portal hypertension and is a subset of Group 1 pulmonary hypertension (PH). PoPH is a cause of significant morbidity and mortality in patients with portal hypertension with or without liver disease...
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2020-11-01
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doaj-2a01f3b3ba6347edb61456f8ffda58552020-11-25T04:06:14ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2020-11-01710.3389/fmed.2020.569413569413Portopulmonary Hypertension: From Bench to BedsideChristopher Thomas0Vladimir Glinskii1Vinicio de Jesus Perez2Sandeep Sahay3Division of Pulmonary, Allergy & Critical Care Medicine, Stanford University School of Medicine, Stanford, CA, United StatesDivision of Pulmonary, Allergy & Critical Care Medicine, Stanford University School of Medicine, Stanford, CA, United StatesDivision of Pulmonary, Allergy & Critical Care Medicine, Stanford University School of Medicine, Stanford, CA, United StatesHouston Methodist Hospital Lung Center, Houston Methodist Hospital, Houston, TX, United StatesPortopulmonary hypertension (PoPH) is defined as pulmonary arterial hypertension (PAH) associated with portal hypertension and is a subset of Group 1 pulmonary hypertension (PH). PoPH is a cause of significant morbidity and mortality in patients with portal hypertension with or without liver disease. Significant strides in elucidating the pathogenesis, effective screening algorithms, accurate diagnoses, and treatment options have been made in past 20 years. Survival of PoPH has remained poor compared to IPAH and other forms of PAH. Recently, the first randomized controlled trial was done in this patient population and showed promising results with PAH specific therapy. Despite positive effects on hemodynamics and functional outcomes, it is unclear whether PAH specific therapy has a beneficial effect on long term survival or transplant outcomes. In this review, we will discuss the epidemiology, pathophysiology, clinical and hemodynamic characteristics of PoPH. Additionally, this review will highlight the lacunae in our current management strategy, challenges faced and will provide direction to potentially useful futuristic management strategies.https://www.frontiersin.org/articles/10.3389/fmed.2020.569413/fullportopulmonary hypertensionpulmonary arterial hypertensionportal hypertensionliver transplantMELD exception |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Christopher Thomas Vladimir Glinskii Vinicio de Jesus Perez Sandeep Sahay |
spellingShingle |
Christopher Thomas Vladimir Glinskii Vinicio de Jesus Perez Sandeep Sahay Portopulmonary Hypertension: From Bench to Bedside Frontiers in Medicine portopulmonary hypertension pulmonary arterial hypertension portal hypertension liver transplant MELD exception |
author_facet |
Christopher Thomas Vladimir Glinskii Vinicio de Jesus Perez Sandeep Sahay |
author_sort |
Christopher Thomas |
title |
Portopulmonary Hypertension: From Bench to Bedside |
title_short |
Portopulmonary Hypertension: From Bench to Bedside |
title_full |
Portopulmonary Hypertension: From Bench to Bedside |
title_fullStr |
Portopulmonary Hypertension: From Bench to Bedside |
title_full_unstemmed |
Portopulmonary Hypertension: From Bench to Bedside |
title_sort |
portopulmonary hypertension: from bench to bedside |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Medicine |
issn |
2296-858X |
publishDate |
2020-11-01 |
description |
Portopulmonary hypertension (PoPH) is defined as pulmonary arterial hypertension (PAH) associated with portal hypertension and is a subset of Group 1 pulmonary hypertension (PH). PoPH is a cause of significant morbidity and mortality in patients with portal hypertension with or without liver disease. Significant strides in elucidating the pathogenesis, effective screening algorithms, accurate diagnoses, and treatment options have been made in past 20 years. Survival of PoPH has remained poor compared to IPAH and other forms of PAH. Recently, the first randomized controlled trial was done in this patient population and showed promising results with PAH specific therapy. Despite positive effects on hemodynamics and functional outcomes, it is unclear whether PAH specific therapy has a beneficial effect on long term survival or transplant outcomes. In this review, we will discuss the epidemiology, pathophysiology, clinical and hemodynamic characteristics of PoPH. Additionally, this review will highlight the lacunae in our current management strategy, challenges faced and will provide direction to potentially useful futuristic management strategies. |
topic |
portopulmonary hypertension pulmonary arterial hypertension portal hypertension liver transplant MELD exception |
url |
https://www.frontiersin.org/articles/10.3389/fmed.2020.569413/full |
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