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Pulmonary Hypertension: Classification and Pathogenesis

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 Hypertension

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 Agonists

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 Failure

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,...

Pulmonary Embolism I: Introduction

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: Introduction

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: May 5, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets

Published on: May 11, 2015

Portopulmonary hypertension.

Nadine Al-Naamani¹, Kari E Roberts

¹Tufts Medical Center, 800 Washington Street, #257, Boston, MA 02111, USA.

Clinics in Chest Medicine

|November 26, 2013

Summary

This summary is machine-generated.

Diagnosing portopulmonary hypertension (PoPH) is complex. Early treatment with PAH-specific medications improves outcomes, but severe cases refractory to therapy should avoid transplantation due to high mortality.

Keywords:

Hypertension, portal Hypertension, pulmonary Liver transplantation

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Last Updated: May 5, 2026

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Published on: May 11, 2015

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

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Published on: March 8, 2019

Area of Science:

Cardiology
Pulmonology
Hepatology

Background:

Portopulmonary hypertension (PoPH) diagnosis is challenging due to co-occurring cardiac and pulmonary diseases in advanced liver disease.
PoPH's natural history is distinct, not solely driven by portal hypertension.

Purpose of the Study:

To review the pathophysiology and pathogenesis of PoPH.
To discuss current diagnostic and management strategies for PoPH.

Main Methods:

Review of existing clinical experience and literature on portopulmonary hypertension.
Analysis of diagnostic challenges and therapeutic benefits.

Main Results:

Patients with PoPH demonstrate benefit from PAH-specific medications.
Severe PoPH refractory to medical management carries a high perioperative mortality risk, contraindicating transplantation.

Conclusions:

Effective management of PoPH involves PAH-specific therapies.
Transplantation should be carefully considered and avoided in severe, medically refractory PoPH cases.