Portopulmonary hypertension: A consequence of portal hypertension

Michael Joseph Krowka, Roberto Rodriguez-Roisin

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Portopulmonary hypertension (POPH) is an uncommon pulmonary vascular consequence of portal hypertension. It presents as exertional dyspnoea andmay lead to right heart failure and death if untreated. There is no relationship between the existence and degree of POPH and the severity of advanced liver disease. Due to the spectrum of pulmonary haemodynamic changes associated with hepatic dysfunction, screening by transthoracic echocardiography and confirmation by right heart catheterisation (RHC) is necessary for accurate diagnosis and therapeutic considerations. Despite the lack of controlled studies, pulmonary vasomodulation therapies in POPH can significantly improve pulmonary haemodynamics and right ventricular (RV) function. The potential to "cure" POPH, at least haemodynamically, with a combination of pulmonary vasomodulation therapy and liver transplantation appears to be an attainable goal in a cohort of POPH patients yet to be optimally characterised. Controlled, multicentre studies and long-term follow-up post-liver transplantation are needed.

Original languageEnglish (US)
Pages (from-to)58-70
Number of pages13
JournalEuropean Respiratory Monograph
Volume57
DOIs
StatePublished - 2012

Fingerprint

Portal Hypertension
Hypertension
Lung
Liver Transplantation
Hemodynamics
Right Ventricular Function
Cardiac Catheterization
Dyspnea
Multicenter Studies
Blood Vessels
Echocardiography
Liver Diseases
Therapeutics
Heart Failure
Liver

Keywords

  • Echocardiography
  • Liver transplant
  • Right heart catheterisation

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Portopulmonary hypertension : A consequence of portal hypertension. / Krowka, Michael Joseph; Rodriguez-Roisin, Roberto.

In: European Respiratory Monograph, Vol. 57, 2012, p. 58-70.

Research output: Contribution to journalArticle

@article{20e7d7e9cca54f0cbd2e460870e36d22,
title = "Portopulmonary hypertension: A consequence of portal hypertension",
abstract = "Portopulmonary hypertension (POPH) is an uncommon pulmonary vascular consequence of portal hypertension. It presents as exertional dyspnoea andmay lead to right heart failure and death if untreated. There is no relationship between the existence and degree of POPH and the severity of advanced liver disease. Due to the spectrum of pulmonary haemodynamic changes associated with hepatic dysfunction, screening by transthoracic echocardiography and confirmation by right heart catheterisation (RHC) is necessary for accurate diagnosis and therapeutic considerations. Despite the lack of controlled studies, pulmonary vasomodulation therapies in POPH can significantly improve pulmonary haemodynamics and right ventricular (RV) function. The potential to {"}cure{"} POPH, at least haemodynamically, with a combination of pulmonary vasomodulation therapy and liver transplantation appears to be an attainable goal in a cohort of POPH patients yet to be optimally characterised. Controlled, multicentre studies and long-term follow-up post-liver transplantation are needed.",
keywords = "Echocardiography, Liver transplant, Right heart catheterisation",
author = "Krowka, {Michael Joseph} and Roberto Rodriguez-Roisin",
year = "2012",
doi = "10.1183/1025448x.10018811",
language = "English (US)",
volume = "57",
pages = "58--70",
journal = "European Respiratory Monograph",
issn = "1025-448X",
publisher = "European Respiratory Society",

}

TY - JOUR

T1 - Portopulmonary hypertension

T2 - A consequence of portal hypertension

AU - Krowka, Michael Joseph

AU - Rodriguez-Roisin, Roberto

PY - 2012

Y1 - 2012

N2 - Portopulmonary hypertension (POPH) is an uncommon pulmonary vascular consequence of portal hypertension. It presents as exertional dyspnoea andmay lead to right heart failure and death if untreated. There is no relationship between the existence and degree of POPH and the severity of advanced liver disease. Due to the spectrum of pulmonary haemodynamic changes associated with hepatic dysfunction, screening by transthoracic echocardiography and confirmation by right heart catheterisation (RHC) is necessary for accurate diagnosis and therapeutic considerations. Despite the lack of controlled studies, pulmonary vasomodulation therapies in POPH can significantly improve pulmonary haemodynamics and right ventricular (RV) function. The potential to "cure" POPH, at least haemodynamically, with a combination of pulmonary vasomodulation therapy and liver transplantation appears to be an attainable goal in a cohort of POPH patients yet to be optimally characterised. Controlled, multicentre studies and long-term follow-up post-liver transplantation are needed.

AB - Portopulmonary hypertension (POPH) is an uncommon pulmonary vascular consequence of portal hypertension. It presents as exertional dyspnoea andmay lead to right heart failure and death if untreated. There is no relationship between the existence and degree of POPH and the severity of advanced liver disease. Due to the spectrum of pulmonary haemodynamic changes associated with hepatic dysfunction, screening by transthoracic echocardiography and confirmation by right heart catheterisation (RHC) is necessary for accurate diagnosis and therapeutic considerations. Despite the lack of controlled studies, pulmonary vasomodulation therapies in POPH can significantly improve pulmonary haemodynamics and right ventricular (RV) function. The potential to "cure" POPH, at least haemodynamically, with a combination of pulmonary vasomodulation therapy and liver transplantation appears to be an attainable goal in a cohort of POPH patients yet to be optimally characterised. Controlled, multicentre studies and long-term follow-up post-liver transplantation are needed.

KW - Echocardiography

KW - Liver transplant

KW - Right heart catheterisation

UR - http://www.scopus.com/inward/record.url?scp=84865604774&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84865604774&partnerID=8YFLogxK

U2 - 10.1183/1025448x.10018811

DO - 10.1183/1025448x.10018811

M3 - Article

AN - SCOPUS:84865604774

VL - 57

SP - 58

EP - 70

JO - European Respiratory Monograph

JF - European Respiratory Monograph

SN - 1025-448X

ER -