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Related Concept Videos

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: 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...
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: 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...
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: 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...

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

Published on: May 11, 2015

Pulmonary arterial hypertension.

David Montani, Sven Günther, Peter Dorfmüller

    Orphanet Journal of Rare Diseases
    |July 9, 2013
    PubMed
    Summary
    This summary is machine-generated.

    Pulmonary arterial hypertension (PAH) is a progressive disease requiring accurate diagnosis and classification. Current therapies target symptoms and specific pathways, but a cure remains elusive.

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    Area of Science:

    • Cardiology and Respiratory Medicine
    • Genetics and Molecular Biology
    • Clinical Diagnostics

    Background:

    • Pulmonary arterial hypertension (PAH) is a severe, progressive condition leading to right heart failure and death.
    • Accurate classification of pulmonary hypertension (PH) is crucial for diagnosis and treatment, with the current system dividing it into five main groups.
    • Genetic factors, such as BMPR2 gene mutations, play a role in familial PAH.

    Purpose of the Study:

    • To review the current classification of pulmonary hypertension (PH).
    • To discuss diagnostic approaches, including invasive hemodynamics and echocardiography.
    • To summarize existing and emerging therapies for PAH.

    Main Methods:

    • Review of current literature on PH classification, diagnosis, and treatment.
    • Analysis of diagnostic criteria including right heart catheterization and echocardiography.
    • Summary of pharmacologic agents used in PAH management.

    Main Results:

    • PH is classified into five groups based on underlying causes and mechanisms.
    • Right heart catheterization is essential for diagnosis (mPAP ≥ 25 mmHg), with PCWP distinguishing pre- and post-capillary PH.
    • Echocardiography aids in screening and estimating PH presence.
    • Current PAH therapies include non-specific drugs (diuretics, anticoagulation) and specific agents (prostanoids, ERAs, PDE5 inhibitors).

    Conclusions:

    • The current PH classification provides a framework for understanding and managing diverse forms of the disease.
    • Accurate diagnosis relies on a combination of clinical assessment, invasive hemodynamics, and imaging.
    • While significant therapeutic advancements have been made, PAH remains a challenging condition without a definitive cure.