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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

175
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...
175
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors

153
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...
153
Chronic Obstructive Pulmonary Disease-III: Symptoms and Complications.01:25

Chronic Obstructive Pulmonary Disease-III: Symptoms and Complications.

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Understanding the variety of primary symptoms and systemic complications that characterize chronic obstructive pulmonary disease (COPD) is crucial for healthcare professionals.
Symptoms of COPD can be classified as primary or systemic. Primary symptoms relate to reduced airflow, while systemic or extrapulmonary symptoms relate to COPD's broader impact on the body.
Primary Symptoms of COPD:
2.7K
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

206
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,...
206
Chronic Obstructive Pulmonary Disease-II: Pathophysiology01:20

Chronic Obstructive Pulmonary Disease-II: Pathophysiology

2.8K
Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
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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

162
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...
162

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Related Experiment Video

Updated: Jun 29, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Pulmonary Hypertension in Systemic Sclerosis.

Sarah Cullivan1, Eleanor Cronin1, Sean Gaine1

  • 1Department of Respiratory Medicine and Pulmonary Hypertension, National Pulmonary Hypertension Unit, Mater Misericordiae University Hospital, Dublin, Ireland.

Seminars in Respiratory and Critical Care Medicine
|March 26, 2024
PubMed
Summary

Systemic sclerosis can cause pulmonary hypertension (PH), particularly pulmonary arterial hypertension (PAH). Early screening and multidisciplinary treatment are crucial for managing this serious complication and improving patient survival.

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Area of Science:

  • Rheumatology
  • Pulmonology
  • Cardiology

Background:

  • Systemic sclerosis (SSc) is a multisystem connective tissue disease with significant morbidity and mortality.
  • Visceral organ involvement, including pulmonary hypertension (PH), is common in SSc and independent of skin manifestations.
  • Pulmonary arterial hypertension (PAH) is a prevalent and serious complication of SSc.

Purpose of the Study:

  • To provide an overview of PH in SSc.
  • To focus on group 1 PAH associated with connective tissue disease in SSc.
  • To discuss screening, classification, and treatment strategies for PH in SSc.

Main Methods:

  • Review of current literature and guidelines on PH in SSc.
  • Discussion of classification systems for PH.
  • Overview of screening algorithms (e.g., DETECT, ASIG) and treatment approaches.

Main Results:

  • SSc patients can present with various forms of PH, including group 1 PAH, group 2 PH (left heart disease), and group 3 PH (interstitial lung disease).
  • Annual risk assessment for PAH is recommended for early detection and improved outcomes.
  • Multidisciplinary care involving PH specialists and rheumatologists is essential for optimizing treatment.

Conclusions:

  • Early recognition and management of PH in SSc are critical for improving patient survival and reducing hemodynamic impairment.
  • Novel biomarkers and therapies, such as sotatercept, show promise for future PAH treatment in SSc.
  • A comprehensive, multidisciplinary approach is necessary for effective management of PH in systemic sclerosis.