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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: 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...
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...
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: 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...

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Related Experiment Video

Updated: Jun 29, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
08:08

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets

Published on: May 11, 2015

Treprostinil for pulmonary hypertension.

Nika Skoro-Sajer1, Irene Lang, Robert Naeije

  • 1Division of Cardiology, Department of Internal Medicione II, Vienna General Hospital, Medical University of Vienna Austria.

Vascular Health and Risk Management
|October 2, 2008
PubMed
Summary

Subcutaneous treprostinil improves exercise capacity and quality of life in pulmonary arterial hypertension patients. While side effects occur, long-term survival is comparable to other treatments, with ongoing research into new formulations.

Keywords:
HIV infectionanorexigenscongenital heart diseaseconnective tissue diseaseepoprostenolportal hypertensionprostacyclinpulmonary hypertensiontreprostinil

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

Last Updated: Jun 29, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
08:08

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets

Published on: May 11, 2015

The Left Pneumonectomy Combined with Monocrotaline or Sugen as a Model of Pulmonary Hypertension in Rats
07:29

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

Published on: March 8, 2019

Area of Science:

  • Pharmacology
  • Cardiovascular Medicine
  • Pulmonology

Background:

  • Pulmonary arterial hypertension (PAH) is a rare disease with a poor prognosis.
  • Prostacyclin analogues are a key treatment modality for PAH.
  • Treprostinil offers a stable, long-acting prostacyclin analogue option.

Purpose of the Study:

  • To evaluate the efficacy and safety of subcutaneous treprostinil in PAH patients.
  • To compare long-term survival with other PAH treatments.
  • To explore treprostinil's potential in other forms of pulmonary hypertension and different administration routes.

Main Methods:

  • A large, multicenter, randomized controlled trial (RCT) assessed subcutaneous treprostinil.
  • Survival data was compared to intravenous epoprostenol.
  • Uncontrolled data and preliminary RCT results for other formulations (intravenous, inhaled, oral) were reviewed.

Main Results:

  • Subcutaneous treprostinil significantly improved exercise capacity, clinical status, functional class, hemodynamics, and quality of life.
  • Common side effects include flushing, headache, and infusion site pain, manageable with dose adjustments.
  • Long-term survival with subcutaneous treprostinil is similar to intravenous epoprostenol.

Conclusions:

  • Subcutaneous treprostinil is an effective treatment for PAH, improving key clinical outcomes.
  • While side effects are manageable, infusion site pain can limit treatment in some patients.
  • Treprostinil shows promise across various administration routes and in other pulmonary hypertension types, with oral formulations under investigation.