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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

645
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

605
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

467
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

509
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

619
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
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

534
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...
534

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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

Pulmonary Hypertension.

Brendan P Madden1

  • 1Cardiothoracic Unit, St Georges Hospital, London, UK.

European Cardiology
|October 13, 2018
PubMed
Summary
This summary is machine-generated.

Pulmonary arterial hypertension (PAH) involves elevated pressure in lung arteries, leading to right heart failure. Current treatments target vascular dysfunction, improving symptoms and survival, but no cure exists.

Keywords:
Pulmonary hypertension diagnosisclinical presentationinvestigation and therapeutic strategies

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Area of Science:

  • Cardiology
  • Pulmonology
  • Vascular Biology

Background:

  • Pulmonary hypertension (PH) is defined by elevated mean pulmonary arterial pressure.
  • Pulmonary arterial hypertension (PAH) involves specific arteriolar remodeling and progressive pulmonary vascular resistance.
  • Untreated PAH leads to right heart failure and high mortality, comparable to malignancies.

Purpose of the Study:

  • To review current understanding and treatment strategies for Pulmonary Arterial Hypertension (PAH).
  • To highlight the role of endothelial dysfunction in PAH pathogenesis.
  • To discuss the efficacy of existing and potential therapeutic approaches.

Main Methods:

  • Review of existing literature on Pulmonary Arterial Hypertension (PAH) and its treatments.
  • Analysis of pharmacological interventions targeting endothelial pathways.
  • Consideration of surgical and interventional options.

Main Results:

  • Endothelin receptor antagonists, PDE-5 inhibitors, and prostacyclin analogues improve morbidity and delay deterioration in PAH.
  • These agents also show benefit in chronic thromboembolic pulmonary hypertension (CTEPH).
  • Pulmonary thromboendarterectomy is an option for selected CTEPH patients, and lung transplantation for some PAH patients.

Conclusions:

  • Current PAH treatments focus on managing endothelial dysfunction, offering symptomatic relief and improved survival.
  • While effective, these therapies do not provide a cure for PAH.
  • Combined therapeutic strategies, including surgery and transplantation, are crucial for managing advanced disease.