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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: 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: 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...
Portal Hypertension01:22

Portal Hypertension

Portal hypertension is an increase in blood pressure within the portal venous system. Normally, this pressure is less than 5 mmHg. It is considered clinically significant when it rises above 10 mmHg. At this threshold, complications from altered blood flow and venous congestion emerge.EtiologyPortal hypertension arises from conditions that impede blood flow through the liver. The most common cause is cirrhosis, in which chronic liver injury leads to fibrotic scarring. This fibrosis narrows or...
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...
Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...

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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
04:44

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Systemic sclerosis-associated pulmonary arterial hypertension.

Neal F Chaisson1, Paul M Hassoun1

  • 1Division of Pulmonary and Critical Care, Johns Hopkins University School of Medicine, Baltimore, MD.

Chest
|October 2, 2013
PubMed
Summary

Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) has a higher mortality rate than idiopathic PAH. Early detection and tailored treatments are crucial for improving outcomes in SSc-PAH patients.

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Area of Science:

  • Cardiology
  • Rheumatology
  • Pulmonology

Background:

  • Pulmonary arterial hypertension (PAH) is a primary cause of mortality in systemic sclerosis (SSc).
  • SSc-associated PAH (SSc-PAH) presents unique challenges, including insidious onset, comorbidities, and poorer treatment response compared to idiopathic PAH (IPAH).
  • Late diagnosis and poorly understood pathogenic mechanisms contribute to high mortality rates in SSc-PAH.

Purpose of the Study:

  • To review the distinct features of SSc-PAH compared to IPAH.
  • To discuss current screening and longitudinal assessment methods for SSc-PAH.
  • To explore novel treatment strategies for SSc-PAH.

Main Methods:

  • Literature review focusing on SSc-PAH pathogenesis, diagnosis, and treatment.
  • Comparative analysis of SSc-PAH and IPAH characteristics.
  • Evaluation of current screening tools (6-min walk test, echocardiography, MRI) and their limitations.

Main Results:

  • SSc-PAH patients face a threefold increased mortality risk compared to IPAH.
  • Conventional PAH therapies show limited efficacy in SSc-PAH, necessitating further research into underlying mechanisms.
  • Existing screening and monitoring tools have significant advantages and disadvantages.

Conclusions:

  • SSc-PAH requires specialized management distinct from IPAH due to differing pathophysiology and treatment responses.
  • Improved screening protocols and early detection are vital for timely intervention.
  • Development of targeted therapies and refined longitudinal assessment strategies are essential for improving SSc-PAH patient outcomes.