Related Experiment Video

Updated: Feb 4, 2026

Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension
10:03

Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension

Published on: June 27, 2025

786

Pathobiology, pathology and genetics of pulmonary hypertension: Update from the Cologne Consensus Conference 2018

Andrea Olschewski1, Eva M Berghausen2, Christina A Eichstaedt3

  • 1Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria; Institute of Physiology, Medical University of Graz, Austria.

International Journal of Cardiology
|October 14, 2018
PubMed

Insights

This review details the pathobiology of pulmonary hypertension (PH), exploring vascular inflammation, genetics, and metabolic dysfunction. It highlights novel concepts and therapeutic targets for improving treatment options in pulmonary arterial hypertension (PAH).

Area of Science:

  • Cardiovascular Medicine
  • Pulmonary Medicine
  • Pathobiology

Background:

  • European guidelines offer limited insight into the pathophysiological mechanisms of pulmonary hypertension (PH).
  • A deeper understanding of PH pathobiology is crucial for advancing treatment strategies.

Purpose of the Study:

  • To provide a detailed review of the pathobiology, pathology, and genetics of PH.
  • To highlight novel concepts and potential therapeutic targets for PH, particularly pulmonary arterial hypertension (PAH).

Main Methods:

  • Comprehensive review of existing literature on PH pathobiology.
  • Focus on specific mechanisms including vascular inflammation, transcription factors, ion channels, hypoxic pulmonary vasoconstriction, genetics/epigenetics, and metabolic dysfunction.
  • Consideration of the future role of histopathology in PH therapy.

Main Results:

  • Detailed examination of mechanisms contributing to PH, such as vascular inflammation and metabolic dysfunction.
  • Exploration of the roles of genetics, epigenetics, and ion channels in PH development.
  • Identification of potential new therapeutic avenues based on current pathobiological insights.

Conclusions:

  • Understanding the intricate pathobiology of PH is essential for developing more effective therapies.
  • Novel therapeutic targets identified in this review hold promise for improving outcomes in PAH patients.
  • Further research into histopathology may offer new diagnostic and therapeutic insights.

Keywords:
And the potential future role of histopathologyGenetics/epigeneticsHypoxic pulmonary vasoconstrictionInflammationIon channels/ion channel diseasesMetabolic dysfunctionPulmonary hypertensionTranscription factors

More Related Videos

Establishment and Validation of a Rat Model of Pulmonary Arterial Hypertension Associated with Pulmonary Fibrosis07:11

Establishment and Validation of a Rat Model of Pulmonary Arterial Hypertension Associated with Pulmonary Fibrosis

Published on: May 23, 2025

939
Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat08:34

Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat

Published on: November 18, 2018

7.7K

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...
645
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors

Phosphodiesterase 5 (PDE5) inhibitors are potent enzymes that function to hydrolyze cyclic nucleotides to their corresponding 5' monophosphates. Their unique biochemical properties have been applied in treating Pulmonary Arterial Hypertension (PAH).
Among the PDE5 inhibitors, sildenafil (Revatio) stands out as a competitive and selective inhibitor. It operates by elevating cellular levels of cGMP and augmenting signaling through the cGMP-PKG pathway, promoting vasodilation. Upon oral...
605
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
467
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...
509
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,...
619
Types of Genetic Transfer Between Organisms02:18

Types of Genetic Transfer Between Organisms

Genetic transfer occurs when genetic information is passed from one organism to another. It occurs via two mechanisms: vertical gene transfer and horizontal gene transfer. Vertical gene transfer occurs when genetic information is transferred from one generation to the next, which happens much more frequently than horizontal gene transfer. Both sexual and asexual reproduction are forms of vertical gene transfer, where one or more organisms pass some or all of their genome onto their progeny.
30.7K