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Related Concept Videos

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors

615
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...
615
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

477
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...
477
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists01:23

Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists

512
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...
512
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

633
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,...
633
Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

662
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...
662
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

550
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: Feb 8, 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

795

Pulmonary Arterial Hypertension.

Mark W Dodson1, Lynette M Brown2, Charles Gregory Elliott2

  • 1Department of Medicine, Intermountain Medical Center, 5121 South Cottonwood Street, Building 2, Suite 307, Murray, UT 84107, USA.

Heart Failure Clinics
|July 4, 2018
PubMed
Summary
This summary is machine-generated.

Pulmonary arterial hypertension (PAH) has evolved from initial recognition to current diagnostic categories. Modern PAH management emphasizes serial risk assessment and combination pharmacotherapy for improved outcomes.

Keywords:
BMPR2Endothelin receptor antagonistsPhosphodiesterase-5 inhibitorsProstacyclinPulmonary arterial hypertensionPulmonary hypertensionREVEAL Registry

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Area of Science:

  • Cardiology
  • Pulmonology
  • Medical Research

Background:

  • Pulmonary hypertension (PH) was first pathologically recognized over a century ago.
  • Pulmonary arterial hypertension (PAH) represents a specific subset of PH with distinct characteristics.
  • Understanding the historical context is crucial for appreciating current diagnostic and therapeutic advancements.

Purpose of the Study:

  • To provide a comprehensive overview of Pulmonary Arterial Hypertension (PAH).
  • To detail the progression of PAH recognition, diagnosis, and treatment strategies.
  • To highlight recent advancements in PAH management.

Main Methods:

  • Literature review of historical and contemporary studies on PAH.
  • Analysis of epidemiological data and pathophysiological mechanisms.
  • Synthesis of information on genetic factors and current treatment modalities.

Main Results:

  • PAH has a complex pathophysiology and diverse genetic underpinnings.
  • Current diagnostic criteria for PAH have been refined over time.
  • Recent studies underscore the significance of updated management strategies.

Conclusions:

  • Modern PAH management involves serial risk assessment.
  • Combination pharmacotherapy is a key component of current treatment protocols.
  • Continued research is vital for further improving PAH patient outcomes.