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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

820
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...
820
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

723
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,...
723
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors

704
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...
704
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

631
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...
631
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

541
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...
541
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists01:23

Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists

591
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...
591

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Related Experiment Video

Updated: Mar 23, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
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[Pulmonary (Arterial) Hypertension].

G Kwapiszewska1, J Hoffmann2, G Kovacs2

  • 1Universitätsklinik für Anästhesiologie und Intensivmedizin, Medizinische Universität Graz.

Pneumologie (Stuttgart, Germany)
|April 7, 2016
PubMed
Summary
This summary is machine-generated.

Pulmonary arterial hypertension (PAH) is a rare lung disease causing narrowed arteries. Recent genetic and epigenetic research offers new diagnostic, prognostic, and therapeutic strategies for PAH.

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Area of Science:

  • Cardiology
  • Pulmonology
  • Genetics

Background:

  • Pulmonary arterial hypertension (PAH) involves vascular remodeling of small lung arteries, leading to lumen decrease and occlusion.
  • Current PAH guidelines define it by pulmonary arterial pressure ≥25 mmHg, wedge pressure ≤15 mmHg, and elevated pulmonary vascular resistance (>3 WU).
  • Pathophysiology includes extracellular matrix disturbances, inflammation, and genetic mutations (BMPR2, KCNK3).

Purpose of the Study:

  • To review recent findings in genetic and epigenetic research relevant to PAH diagnosis, prognosis, and therapy.
  • To highlight the potential for developing individualized therapy strategies for PAH.
  • To provide an overview of ongoing clinical trials for novel PAH treatments.

Main Methods:

  • Review of current literature on PAH pathophysiology, genetics, and epigenetics.
  • Analysis of recent diagnostic and therapeutic guidelines for PAH.
  • Summary of findings from ongoing Phase I and Phase II clinical studies.

Main Results:

  • Recent epigenetic and genetic investigations have yielded significant new insights into PAH.
  • These discoveries are crucial for improving PAH diagnosis, predicting patient outcomes, and tailoring treatments.
  • Several promising new substances are currently being evaluated in Phase I and Phase II clinical studies.

Conclusions:

  • Advances in understanding PAH genetics and epigenetics are paving the way for personalized medicine.
  • New therapeutic strategies are emerging, with several promising drug candidates in clinical development.
  • Continued research is essential for further improving the management and outcomes of patients with pulmonary arterial hypertension.