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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

184
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...
184
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

216
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,...
216
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors

158
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...
158
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

168
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...
168
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

166
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...
166
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists01:23

Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists

183
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...
183

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

Ana Mocumbi1,2, Marc Humbert3,4, Anita Saxena5

  • 1Faculdade de Medicina, Universidade Eduardo Mondlane, Maputo, Moçambique. ana.mocumbi@uem.ac.mz.

Nature Reviews. Disease Primers
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Summary

Pulmonary hypertension (PH) involves high blood pressure in lung arteries, affecting 1% of people globally. Early diagnosis and management are crucial to prevent right ventricle failure, the primary cause of death.

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Area of Science:

  • Cardiology
  • Pulmonology
  • Vascular Medicine

Background:

  • Pulmonary hypertension (PH) is a group of conditions characterized by elevated pulmonary artery pressures (>20 mmHg mean).
  • It affects at least 1% of the global population, with significant disparities in low- and middle-income countries.
  • PH involves vascular remodeling, leading to obstruction, stiffening, and vasoconstriction, ultimately causing right ventricular failure.

Purpose of the Study:

  • To provide a comprehensive overview of the five recognized groups of pulmonary hypertension.
  • To highlight the pathophysiology, clinical presentation, diagnostic approaches, and current management strategies for PH.
  • To identify knowledge gaps and emphasize the need for further research, particularly in vulnerable populations.

Main Methods:

  • Classification of PH into five groups based on etiology and diagnostic criteria.
  • Review of the epidemiology, pathophysiology, and clinical manifestations of PH.
  • Summary of diagnostic pathways, including right heart catheterization.
  • Overview of approved and emerging treatment strategies targeting underlying causes.

Main Results:

  • Five distinct groups of PH are identified, with varying prevalence (e.g., PH with left-sided heart disease is very common, PH arterial hypertension is rare).
  • Dyspnea is a common symptom, especially in older individuals.
  • Right ventricular hypertrophy and failure are the main causes of mortality.
  • Effective treatments exist for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension, with ongoing research for other forms.

Conclusions:

  • Pulmonary hypertension is a serious condition with diverse causes and significant mortality, primarily due to right ventricular failure.
  • Current management focuses on treating the underlying cause, with established therapies for specific PH types.
  • Substantial research gaps remain, necessitating further investigation to improve characterization, detection, and treatment across all PH forms, especially in underserved populations.