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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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Related Experiment Video

Updated: Jun 1, 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.

Eugenia Hopps1, Amelia Valenti, Gregorio Caimi

  • 1Department of Internal Medicine, Cardiovascular Disease and Nephrology, Policlinico Universitario di Palermo, Palermo, Italy. euhopps@libero.it

Clinical and Investigative Medicine. Medecine Clinique Et Experimentale
|June 3, 2011
PubMed
Summary

Portopulmonary hypertension (PPHT) is a severe respiratory complication in liver cirrhosis patients. Diagnosis requires right heart catheterization, and treatment involves vasomodulating agents, but prognosis remains poor with a 15-month survival rate.

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Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
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The Left Pneumonectomy Combined with Monocrotaline or Sugen as a Model of Pulmonary Hypertension in Rats
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Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
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The Left Pneumonectomy Combined with Monocrotaline or Sugen as a Model of Pulmonary Hypertension in Rats
07:29

The Left Pneumonectomy Combined with Monocrotaline or Sugen as a Model of Pulmonary Hypertension in Rats

Published on: March 8, 2019

Area of Science:

  • Cardiology
  • Pulmonology
  • Hepatology

Background:

  • Portopulmonary hypertension (PPHT) is a serious complication of portal hypertension, frequently seen in liver cirrhosis patients.
  • It is characterized by elevated pulmonary artery pressure (PAP) and pulmonary vascular resistance with normal pulmonary capillary wedge pressure.
  • Pathological changes include vascular remodeling with endothelial and smooth-muscle cell proliferation and fibrosis.

Purpose of the Study:

  • To define portopulmonary hypertension (PPHT), its diagnostic criteria, and clinical presentation.
  • To outline the histopathological features and potential pathogenetic mechanisms of PPHT.
  • To discuss current treatment strategies and the prognosis associated with PPHT.

Main Methods:

  • Diagnosis is established via right heart catheterization, measuring mean pulmonary artery pressure (PAP).
  • Classification of PPHT severity is based on PAP levels: mild (<35 mmHg), moderate (35-45 mmHg), and severe (≥45 mmHg).
  • Histopathological analysis reveals vascular changes in pulmonary resistance arteries.

Main Results:

  • PPHT is defined by PAP > 25 mmHg, pulmonary vascular resistance > 240 dyn.s/cm(-5), and normal pulmonary capillary wedge pressure (<15 mmHg).
  • Common symptoms include exertional dyspnea, fatigue, chest pain, and syncope in advanced stages.
  • Signs of right ventricular failure and decompensated cirrhosis are often present.

Conclusions:

  • Medical management of PPHT aligns with other forms of pulmonary arterial hypertension, utilizing vasomodulating agents.
  • Liver transplantation carries a high mortality risk due to potential right ventricular failure.
  • The overall prognosis for PPHT is poor, with a median survival of approximately 15 months.