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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

664
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...
664
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors

618
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...
618
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

478
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...
478
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists01:23

Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists

513
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...
513
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
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

551
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...
551

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Related Experiment Video

Updated: Feb 9, 2026

Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension
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Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension

Published on: June 27, 2025

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Evaluating suspected pulmonary hypertension: A structured approach.

Akshay Bhatnagar1, Jonathan Wiesen2,3, Raed Dweik4,5

  • 1Department of Internal Medicine, Cleveland Clinic, Cleveland, OH, USA.

Cleveland Clinic Journal of Medicine
|June 9, 2018
PubMed
Summary
This summary is machine-generated.

Pulmonary arterial hypertension (PAH) requires careful evaluation, as other conditions can mimic its symptoms. This review guides clinicians through assessing patients with suspected PAH for accurate diagnosis and treatment.

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Area of Science:

  • Cardiology
  • Pulmonology
  • Internal Medicine

Background:

  • Pulmonary arterial hypertension (PAH) is a diagnostic consideration for unexplained cardiopulmonary disease.
  • Clinical guidelines exist for PAH diagnosis and management.
  • Differential diagnosis is essential as multiple conditions can mimic PAH.

Purpose of the Study:

  • To provide a case-based approach for evaluating patients with suspected Pulmonary Arterial Hypertension (PAH).
  • To emphasize the importance of comprehensive clinical assessment for accurate PAH diagnosis.
  • To guide clinicians in the etiologic definition of PAH for appropriate therapeutic selection.

Main Methods:

  • Review of clinical cases presenting with signs of cardiopulmonary disease.
  • Discussion of diagnostic criteria and differential diagnoses for PAH.
  • Emphasis on integrated clinical evaluation and etiological workup.

Main Results:

  • Suspected PAH necessitates a thorough evaluation beyond initial symptoms.
  • Recognizing conditions that mimic PAH is critical for correct diagnosis.
  • A systematic approach ensures proper patient categorization and treatment planning.

Conclusions:

  • Accurate diagnosis of Pulmonary Arterial Hypertension (PAH) relies on comprehensive patient evaluation.
  • Differentiating PAH from mimicking conditions is key to effective management.
  • This case-based review aids clinicians in the diagnostic process for suspected PAH.