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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
Portal Hypertension01:22

Portal Hypertension

Portal hypertension is an increase in blood pressure within the portal venous system. Normally, this pressure is less than 5 mmHg. It is considered clinically significant when it rises above 10 mmHg. At this threshold, complications from altered blood flow and venous congestion emerge.EtiologyPortal hypertension arises from conditions that impede blood flow through the liver. The most common cause is cirrhosis, in which chronic liver injury leads to fibrotic scarring. This fibrosis narrows or...
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists01:23

Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists

Prostacyclin receptor agonists are a class of therapeutic agents integral to managing pulmonary arterial hypertension (PAH). These drugs operate by mimicking the action of prostaglandin I2, or PGI2, a naturally occurring compound in the body.
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

Oxygen therapy has emerged as a significant tool in enhancing the quality of life for patients suffering from pulmonary arterial hypertension (PAH). While this therapy has principally been studied on patients with significant hypoxemia, this therapeutic approach helps prevent potential organ damage and can be administered in the comfort of one's home.
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue, improving...
Pulmonary Embolism I: Introduction01:29

Pulmonary Embolism I: Introduction

Pulmonary embolism (PE) occurs when a thrombus, fat or air embolus, amniotic fluid, or tumor tissue blocks one or more pulmonary arteries. These blockages originate in the venous system or the right side of the heart.EtiologyPE primarily arises from deep vein thrombosis (DVT) and other hypercoagulable states, such as inherited thrombophilias. Additional etiological factors include venous stasis, commonly seen in obesity, and endothelial injury from surgery and trauma. Less common causes include...
Pulmonary Embolism I: Introduction01:19

Pulmonary Embolism I: Introduction

A blood clot, or thrombus, is a semi-solid mass composed of fibrin, platelets, and red blood cells. When it forms within a vessel, it can obstruct blood flow, known as thrombosis. If part of the clot detaches, it becomes an embolus that can travel and block distant vessels. When this occurs in the pulmonary arteries, it causes a condition known as pulmonary embolism (PE).Origin and ImpactMost often, the embolus originates from a thrombus in the deep veins of the lower limbs, a condition called...

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Newly diagnosed pulmonary arterial hypertension following liver transplantation: Insights from a multicenter case series.

Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·2026
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International Liver Transplantation Society practice guideline update on portopulmonary hypertension.

Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·2025
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The Utility of Follow-up Transthoracic Echocardiogram to Screen for Severe Portopulmonary Hypertension (POPH) in Patients Granted POPH Model for End-stage Liver Disease (MELD) Exceptions.

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Unmet needs in diagnosis and management of portopulmonary hypertension: How far have we reached?

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Smoking and outcomes in candidates for liver transplantation: Analysis of the Pulmonary Vascular Complications of Liver Disease 2 (PVCLD2).

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Echocardiography Screening of Consecutive Patients With Portal Hypertension Referred to Mayo Clinic for Liver Transplant Evaluation.

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Related Experiment Video

Updated: May 23, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
08:08

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets

Published on: May 11, 2015

Portopulmonary hypertension.

Michael J Krowka1

  • 1Department of Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA. krowka@mayo.edu

Seminars in Respiratory and Critical Care Medicine
|March 27, 2012
PubMed
Summary

Portopulmonary hypertension (POPH) is pulmonary arterial hypertension in patients with portal hypertension. POPH significantly impacts survival and liver transplant candidacy, with outcomes varying based on severity.

Area of Science:

  • Cardiology
  • Hepatology
  • Pulmonology

Background:

  • Portopulmonary hypertension (POPH) is defined as pulmonary arterial hypertension (PAH) in individuals with portal hypertension.
  • Multiple mechanisms contribute to pulmonary hypertension in liver disease, including hyperdynamic states, venous congestion, and pulmonary arterial obstruction.
  • Increased pulmonary vascular resistance (PVR) is a critical defining parameter for POPH.

Purpose of the Study:

  • To review the incidence, clinical implications, and management of portopulmonary hypertension.
  • To discuss the impact of POPH severity on outcomes, particularly in the context of liver transplantation.
  • To explore therapeutic responses and the need for further research.

Main Methods:

  • Literature review of studies on portopulmonary hypertension.

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The Left Pneumonectomy Combined with Monocrotaline or Sugen as a Model of Pulmonary Hypertension in Rats
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The Left Pneumonectomy Combined with Monocrotaline or Sugen as a Model of Pulmonary Hypertension in Rats
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  • Analysis of reported incidence rates and survival data.
  • Evaluation of treatment responses and liver transplantation outcomes.

    • Main Results:

    • The incidence of POPH in patients with portal hypertension ranges from 2% to 9%.
    • Long-term survival for POPH patients is generally poor.
    • Severe POPH with right ventricular failure is a contraindication for liver transplantation, while moderate POPH has unpredictable post-transplant outcomes.

    Conclusions:

    • POPH presents a significant challenge in patients with portal hypertension, affecting survival and treatment strategies.
    • Pulmonary vasodilator therapy shows promise, but robust clinical trials are needed.
    • Management of POPH requires careful consideration, especially regarding liver transplantation eligibility and post-transplant care.