Evolving dilemmas and management of portopulmonary hypertension

Research output: Contribution to journalArticle

40 Citations (Scopus)

Abstract

Portopulmonary hypertension (POPH) is an uncommon but serious pulmonary vascular consequence of portal hypertension. Untreated, POPH results in right heart failure and death, regardless of the severity of portal hypertension. The existence of moderate to severe POPH has posed higher risk for orthotopic liver transplantation (OLT). New and evolving medical approaches may change the natural history of this disorder. Such approaches include administration of prostacyclins, endothelin receptor antagonists, and phosphodiesterase inhibitors. Administration may involve 24-hour continuous infusions, periodic inhaled, and oral routes. Liver transplantation in patients with moderate to severe POPH is problematic. Aside from patients being denied OLT specifically because of POPH, intraoperative death has occurred and unacceptable mortality during the transplant hospitalization has been reported. Finally, guidelines are evolving concerning which patients will benefit from the combination of evolving medical therapies and OLT. Whether the natural history of POPH can be dramatically changed remains an optimistic conjecture.

Original languageEnglish (US)
Pages (from-to)265-272
Number of pages8
JournalSeminars in Liver Disease
Volume26
Issue number3
DOIs
StatePublished - Aug 2006

Fingerprint

Hypertension
Liver Transplantation
Portal Hypertension
Natural History
Epoprostenol Receptors
Phosphodiesterase Inhibitors
Blood Vessels
Hospitalization
Heart Failure
Guidelines
Transplants
Lung
Mortality
Therapeutics

Keywords

  • Echocardiography
  • Liver transplantation
  • Pulmonary hypertension

ASJC Scopus subject areas

  • Hepatology

Cite this

Evolving dilemmas and management of portopulmonary hypertension. / Krowka, Michael Joseph.

In: Seminars in Liver Disease, Vol. 26, No. 3, 08.2006, p. 265-272.

Research output: Contribution to journalArticle

@article{521f93a78e194937987cfdd77c8bdd7b,
title = "Evolving dilemmas and management of portopulmonary hypertension",
abstract = "Portopulmonary hypertension (POPH) is an uncommon but serious pulmonary vascular consequence of portal hypertension. Untreated, POPH results in right heart failure and death, regardless of the severity of portal hypertension. The existence of moderate to severe POPH has posed higher risk for orthotopic liver transplantation (OLT). New and evolving medical approaches may change the natural history of this disorder. Such approaches include administration of prostacyclins, endothelin receptor antagonists, and phosphodiesterase inhibitors. Administration may involve 24-hour continuous infusions, periodic inhaled, and oral routes. Liver transplantation in patients with moderate to severe POPH is problematic. Aside from patients being denied OLT specifically because of POPH, intraoperative death has occurred and unacceptable mortality during the transplant hospitalization has been reported. Finally, guidelines are evolving concerning which patients will benefit from the combination of evolving medical therapies and OLT. Whether the natural history of POPH can be dramatically changed remains an optimistic conjecture.",
keywords = "Echocardiography, Liver transplantation, Pulmonary hypertension",
author = "Krowka, {Michael Joseph}",
year = "2006",
month = "8",
doi = "10.1055/s-2006-947294",
language = "English (US)",
volume = "26",
pages = "265--272",
journal = "Seminars in Liver Disease",
issn = "0272-8087",
publisher = "Thieme Medical Publishers",
number = "3",

}

TY - JOUR

T1 - Evolving dilemmas and management of portopulmonary hypertension

AU - Krowka, Michael Joseph

PY - 2006/8

Y1 - 2006/8

N2 - Portopulmonary hypertension (POPH) is an uncommon but serious pulmonary vascular consequence of portal hypertension. Untreated, POPH results in right heart failure and death, regardless of the severity of portal hypertension. The existence of moderate to severe POPH has posed higher risk for orthotopic liver transplantation (OLT). New and evolving medical approaches may change the natural history of this disorder. Such approaches include administration of prostacyclins, endothelin receptor antagonists, and phosphodiesterase inhibitors. Administration may involve 24-hour continuous infusions, periodic inhaled, and oral routes. Liver transplantation in patients with moderate to severe POPH is problematic. Aside from patients being denied OLT specifically because of POPH, intraoperative death has occurred and unacceptable mortality during the transplant hospitalization has been reported. Finally, guidelines are evolving concerning which patients will benefit from the combination of evolving medical therapies and OLT. Whether the natural history of POPH can be dramatically changed remains an optimistic conjecture.

AB - Portopulmonary hypertension (POPH) is an uncommon but serious pulmonary vascular consequence of portal hypertension. Untreated, POPH results in right heart failure and death, regardless of the severity of portal hypertension. The existence of moderate to severe POPH has posed higher risk for orthotopic liver transplantation (OLT). New and evolving medical approaches may change the natural history of this disorder. Such approaches include administration of prostacyclins, endothelin receptor antagonists, and phosphodiesterase inhibitors. Administration may involve 24-hour continuous infusions, periodic inhaled, and oral routes. Liver transplantation in patients with moderate to severe POPH is problematic. Aside from patients being denied OLT specifically because of POPH, intraoperative death has occurred and unacceptable mortality during the transplant hospitalization has been reported. Finally, guidelines are evolving concerning which patients will benefit from the combination of evolving medical therapies and OLT. Whether the natural history of POPH can be dramatically changed remains an optimistic conjecture.

KW - Echocardiography

KW - Liver transplantation

KW - Pulmonary hypertension

UR - http://www.scopus.com/inward/record.url?scp=33746569076&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=33746569076&partnerID=8YFLogxK

U2 - 10.1055/s-2006-947294

DO - 10.1055/s-2006-947294

M3 - Article

VL - 26

SP - 265

EP - 272

JO - Seminars in Liver Disease

JF - Seminars in Liver Disease

SN - 0272-8087

IS - 3

ER -