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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

297
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...
297
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

327
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,...
327
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists01:23

Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists

260
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...
260
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

228
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...
228
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors

256
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...
256
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

263
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...
263

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Related Experiment Video

Updated: Sep 18, 2025

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

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The PAH patient's perspective.

Daniel A Jeffery1, Wendy Gin-Sing2, Radwa Bedair3

  • 1Morriston Cardiac Centre, Swansea Bay University Healthboard, Swansea, SA6 6NL, UK.

International Journal of Cardiology. Congenital Heart Disease
|June 23, 2025
PubMed
Summary
This summary is machine-generated.

Pulmonary Arterial Hypertension associated with Congenital Heart Disease (PAH-CHD) presents significant challenges. Patients experience physical, emotional, and social impacts, affecting daily life and relationships.

Keywords:
Adult congenital heart diseasePulmonary arterial hypertensionQuality of life

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Area of Science:

  • Cardiology
  • Pulmonology
  • Patient Advocacy

Background:

  • Pulmonary Hypertension (PH) is a serious condition in adults with congenital heart disease (CHD).
  • Advances in CHD care allow patients to live longer, increasing the prevalence of PH-associated conditions.
  • Pulmonary Arterial Hypertension associated with Congenital Heart Disease (PAH-CHD) presents unique challenges.

Purpose of the Study:

  • To explore the multifaceted issues faced by patients living with PAH-CHD.
  • To highlight the physical, emotional, social, and financial impacts of PAH-CHD.
  • To provide patient perspectives on living with this complex condition.

Main Methods:

  • Qualitative study involving interviews with two patients diagnosed with PAH-CHD.
  • Exploration of patient experiences related to diagnosis, physical limitations, mental health, relationships, finances, and family planning.

Main Results:

  • Patients with PAH-CHD encounter significant physical limitations and psychological distress, including depression and anxiety.
  • The condition impacts social interactions, family dynamics, financial stability, and reproductive choices.
  • Living with PAH-CHD necessitates comprehensive management strategies addressing diverse patient needs.

Conclusions:

  • PAH-CHD imposes a substantial burden on patients' quality of life.
  • Addressing the holistic needs of PAH-CHD patients is crucial for improving outcomes.
  • Further research and patient support are essential for managing PAH-CHD effectively.