Portopulmonary hypertension

Rodrigo Cartin-Ceba, Michael Joseph Krowka

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Portopulmonary hypertension is a serious complication of portal hypertension that is associated with significant mortality. It refers to the presence of pulmonary arterial hypertension in patients with portal hypertension. The severity of portopulmonary hypertension is unrelated to the severity of portal hypertension or the etiology or severe of liver disease. Pulmonary hypertension in patients with liver disease or portal hypertension can be due to multiple mechanisms: hyperdynamic (high-flow) state, increased pulmonary venous congestion (pulmonary venous hypertension), and vascular constriction or obstruction of the pulmonary arterial bed. Vascular obstruction to pulmonary arterial flow, reflected by increased pulmonary vascular resistance, is an important parameter that defines portopulmonary hypertension. Among patients with portal hypertension, reported incidence rates of portopulmonary hypertension range from 2% to 9%. Long-term survival in cases of portopulmonary hypertension is poor. Favorable responses to pulmonary artery hypertension-specific therapy have been observed, but prospective, randomized trials are lacking. Severe portopulmonary hypertension with right ventricular failure despite vasodilator therapy is associated with adverse outcomes in the setting of liver transplantation and is therefore considered a contraindication to liver transplantation unless pulmonary artery hypertension-specific therapy is able to lower the pulmonary vascular resistance to safe levels. The post-liver transplant course of patients with moderate portopulmonary hypertension is unpredictable, but most patients can be weaned from pulmonary artery hypertension-specific therapy over time.

Original languageEnglish (US)
Title of host publicationCirrhosis: A Practical Guide to Management
PublisherWiley Blackwell
Pages212-224
Number of pages13
ISBN (Electronic)9781118412640
ISBN (Print)9781118274828
DOIs
StatePublished - Jan 30 2015

Fingerprint

Pulmonary Hypertension
Portal Hypertension
Hypertension
Pulmonary Artery
Vascular Resistance
Liver Transplantation
Lung
Blood Vessels
Liver Diseases
Hyperemia
Therapeutics
Vasodilator Agents
Constriction
Transplants
Survival
Mortality
Liver
Incidence

Keywords

  • Cirrhosis
  • Liver transplant
  • Portal hypertension
  • Portopulmonary hypertension
  • Pulmonary hypertension

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Cartin-Ceba, R., & Krowka, M. J. (2015). Portopulmonary hypertension. In Cirrhosis: A Practical Guide to Management (pp. 212-224). Wiley Blackwell. https://doi.org/10.1002/9781118412640.ch19

Portopulmonary hypertension. / Cartin-Ceba, Rodrigo; Krowka, Michael Joseph.

Cirrhosis: A Practical Guide to Management. Wiley Blackwell, 2015. p. 212-224.

Research output: Chapter in Book/Report/Conference proceedingChapter

Cartin-Ceba, R & Krowka, MJ 2015, Portopulmonary hypertension. in Cirrhosis: A Practical Guide to Management. Wiley Blackwell, pp. 212-224. https://doi.org/10.1002/9781118412640.ch19
Cartin-Ceba R, Krowka MJ. Portopulmonary hypertension. In Cirrhosis: A Practical Guide to Management. Wiley Blackwell. 2015. p. 212-224 https://doi.org/10.1002/9781118412640.ch19
Cartin-Ceba, Rodrigo ; Krowka, Michael Joseph. / Portopulmonary hypertension. Cirrhosis: A Practical Guide to Management. Wiley Blackwell, 2015. pp. 212-224
@inbook{5d6d58135ba64c00b2703dd8b805a64f,
title = "Portopulmonary hypertension",
abstract = "Portopulmonary hypertension is a serious complication of portal hypertension that is associated with significant mortality. It refers to the presence of pulmonary arterial hypertension in patients with portal hypertension. The severity of portopulmonary hypertension is unrelated to the severity of portal hypertension or the etiology or severe of liver disease. Pulmonary hypertension in patients with liver disease or portal hypertension can be due to multiple mechanisms: hyperdynamic (high-flow) state, increased pulmonary venous congestion (pulmonary venous hypertension), and vascular constriction or obstruction of the pulmonary arterial bed. Vascular obstruction to pulmonary arterial flow, reflected by increased pulmonary vascular resistance, is an important parameter that defines portopulmonary hypertension. Among patients with portal hypertension, reported incidence rates of portopulmonary hypertension range from 2{\%} to 9{\%}. Long-term survival in cases of portopulmonary hypertension is poor. Favorable responses to pulmonary artery hypertension-specific therapy have been observed, but prospective, randomized trials are lacking. Severe portopulmonary hypertension with right ventricular failure despite vasodilator therapy is associated with adverse outcomes in the setting of liver transplantation and is therefore considered a contraindication to liver transplantation unless pulmonary artery hypertension-specific therapy is able to lower the pulmonary vascular resistance to safe levels. The post-liver transplant course of patients with moderate portopulmonary hypertension is unpredictable, but most patients can be weaned from pulmonary artery hypertension-specific therapy over time.",
keywords = "Cirrhosis, Liver transplant, Portal hypertension, Portopulmonary hypertension, Pulmonary hypertension",
author = "Rodrigo Cartin-Ceba and Krowka, {Michael Joseph}",
year = "2015",
month = "1",
day = "30",
doi = "10.1002/9781118412640.ch19",
language = "English (US)",
isbn = "9781118274828",
pages = "212--224",
booktitle = "Cirrhosis: A Practical Guide to Management",
publisher = "Wiley Blackwell",

}

TY - CHAP

T1 - Portopulmonary hypertension

AU - Cartin-Ceba, Rodrigo

AU - Krowka, Michael Joseph

PY - 2015/1/30

Y1 - 2015/1/30

N2 - Portopulmonary hypertension is a serious complication of portal hypertension that is associated with significant mortality. It refers to the presence of pulmonary arterial hypertension in patients with portal hypertension. The severity of portopulmonary hypertension is unrelated to the severity of portal hypertension or the etiology or severe of liver disease. Pulmonary hypertension in patients with liver disease or portal hypertension can be due to multiple mechanisms: hyperdynamic (high-flow) state, increased pulmonary venous congestion (pulmonary venous hypertension), and vascular constriction or obstruction of the pulmonary arterial bed. Vascular obstruction to pulmonary arterial flow, reflected by increased pulmonary vascular resistance, is an important parameter that defines portopulmonary hypertension. Among patients with portal hypertension, reported incidence rates of portopulmonary hypertension range from 2% to 9%. Long-term survival in cases of portopulmonary hypertension is poor. Favorable responses to pulmonary artery hypertension-specific therapy have been observed, but prospective, randomized trials are lacking. Severe portopulmonary hypertension with right ventricular failure despite vasodilator therapy is associated with adverse outcomes in the setting of liver transplantation and is therefore considered a contraindication to liver transplantation unless pulmonary artery hypertension-specific therapy is able to lower the pulmonary vascular resistance to safe levels. The post-liver transplant course of patients with moderate portopulmonary hypertension is unpredictable, but most patients can be weaned from pulmonary artery hypertension-specific therapy over time.

AB - Portopulmonary hypertension is a serious complication of portal hypertension that is associated with significant mortality. It refers to the presence of pulmonary arterial hypertension in patients with portal hypertension. The severity of portopulmonary hypertension is unrelated to the severity of portal hypertension or the etiology or severe of liver disease. Pulmonary hypertension in patients with liver disease or portal hypertension can be due to multiple mechanisms: hyperdynamic (high-flow) state, increased pulmonary venous congestion (pulmonary venous hypertension), and vascular constriction or obstruction of the pulmonary arterial bed. Vascular obstruction to pulmonary arterial flow, reflected by increased pulmonary vascular resistance, is an important parameter that defines portopulmonary hypertension. Among patients with portal hypertension, reported incidence rates of portopulmonary hypertension range from 2% to 9%. Long-term survival in cases of portopulmonary hypertension is poor. Favorable responses to pulmonary artery hypertension-specific therapy have been observed, but prospective, randomized trials are lacking. Severe portopulmonary hypertension with right ventricular failure despite vasodilator therapy is associated with adverse outcomes in the setting of liver transplantation and is therefore considered a contraindication to liver transplantation unless pulmonary artery hypertension-specific therapy is able to lower the pulmonary vascular resistance to safe levels. The post-liver transplant course of patients with moderate portopulmonary hypertension is unpredictable, but most patients can be weaned from pulmonary artery hypertension-specific therapy over time.

KW - Cirrhosis

KW - Liver transplant

KW - Portal hypertension

KW - Portopulmonary hypertension

KW - Pulmonary hypertension

UR - http://www.scopus.com/inward/record.url?scp=84976552424&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84976552424&partnerID=8YFLogxK

U2 - 10.1002/9781118412640.ch19

DO - 10.1002/9781118412640.ch19

M3 - Chapter

SN - 9781118274828

SP - 212

EP - 224

BT - Cirrhosis: A Practical Guide to Management

PB - Wiley Blackwell

ER -