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Related Concept Videos

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...
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 (ECE). Of...
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...
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...
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers01:26

Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers

Receptor tyrosine kinase inhibitors (TKIs) and calcium channel blockers (CCBs) are two critical categories of drugs employed in the treatment of pulmonary artery hypertension (PAH). PAH is a disease that causes high blood pressure in the pulmonary arteries, resulting in chest pain, fatigue, and shortness of breath.
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...

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Related Experiment Video

Updated: May 24, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
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Published on: May 11, 2015

Improving outcomes for pulmonary vascular disease.

Ivan M Robbins1, Timothy M Moore, Carol J Blaisdell

  • 1Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA.

American Journal of Respiratory and Critical Care Medicine
|February 17, 2012
PubMed
Summary

This workshop focused on improving pulmonary vascular diseases (PVDs) research by identifying knowledge gaps and prioritizing future directions for better diagnosis and treatment in adults and children.

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Area of Science:

  • Pulmonary Medicine
  • Translational Research
  • Clinical Trials

Background:

  • Pulmonary vascular diseases (PVDs) require improved research for better lung health.
  • Multidisciplinary experts convened to address PVDs in adults and children.

Purpose of the Study:

  • Review current scientific knowledge on PVD treatment.
  • Identify research gaps, barriers, and opportunities in translational PVD research.
  • Prioritize research directions to impact PVD clinical course and inform NHLBI recommendations.

Main Methods:

  • Workshop convened multidisciplinary experts.
  • Reviewed PVD clinical trials and research networks for other lung disorders.
  • Discussed bioinformatics and health IT applications in clinical studies.
  • Considered improved phenotyping, new clinical trial endpoints, and research priorities for PVD subsets.

Main Results:

  • Identified key areas for advancing PVD research.
  • Highlighted the need for improved phenotyping and endpoints in clinical studies.
  • Established research priorities for pediatric and adult PVD care.

Conclusions:

  • Recommendations made to the NHLBI to fill identified gaps in adult and pediatric PVD clinical research.
  • Emphasized the need for enhanced clinical research to improve PVD diagnosis and treatment.