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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

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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...
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Pulmonary embolism (PE) occurs when a thrombus, fat or air embolus, amniotic fluid, or tumor tissue blocks one or more pulmonary arteries. These blockages originate in the venous system or the right side of the heart.EtiologyPE primarily arises from deep vein thrombosis (DVT) and other hypercoagulable states, such as inherited thrombophilias. Additional etiological factors include venous stasis, commonly seen in obesity, and endothelial injury from surgery and trauma. Less common causes include...
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Chronic Obstructive Pulmonary Disease01:24

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COPD is defined as a heterogeneous lung condition marked by persistent respiratory symptoms such as dyspnea, cough, and sputum production, caused by abnormalities in the airways that cause airflow obstruction.
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Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers01:26

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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...
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Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care01:29

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Diagnosing Pulmonary EmbolismDiagnosing pulmonary embolism (PE) involves clinical assessment and advanced imaging tests. The preferred diagnostic tool is the spiral (helical) CT scan or CT angiography (CTA), which uses intravenous contrast media to visualize the pulmonary vasculature and identify emboli.A ventilation-perfusion (V/Q) scan is an alternative for patients unable to receive contrast media. This scan includes both perfusion and ventilation scanning. Perfusion scanning involves...
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Chronic Obstructive Pulmonary Disease-I: Introduction01:20

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Chronic Obstructive Pulmonary Disease (COPD) is a long-lasting respiratory condition requiring continuous attention and care. It is a progressive lung disease that leads to breathing challenges due to airflow obstruction. It manifests as persistent respiratory symptoms and restricted airflow resulting from abnormalities in the airways and alveoli, usually due to long-term exposure to harmful particles or gases. COPD mainly consists of two primary conditions: emphysema and chronic bronchitis.
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Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
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Chronic thromboembolic pulmonary hypertension.

Caroline O'Connell1, David Montani1, Laurent Savale1

  • 1Université Paris-Saclay, faculté de médecine, 94270 Le Kremlin-Bicêtre, France; AP-HP, hôpital Bicêtre, service de pneumologie, 94270 Le Kremlin-Bicêtre, France; Centre chirurgical Marie-Lannelongue, Inserm UMR_S 999, 92060 Le Plessis-Robinson, France.

Presse Medicale (Paris, France : 1983)
|November 21, 2015
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Summary

Chronic thromboembolic pulmonary hypertension (CTEPH) is a WHO Group IV pulmonary hypertension caused by persistent blood clots. Early diagnosis and specialized treatment, including surgery or medication, are vital for managing this condition.

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Area of Science:

  • Cardiology
  • Pulmonology
  • Vascular Medicine

Background:

  • Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe form of pulmonary hypertension (PH) characterized by organized thromboemboli in pulmonary arteries.
  • It leads to increased pulmonary vascular resistance and right heart failure, classified as WHO Group IV PH.
  • Diagnosis requires specific hemodynamic and imaging criteria, even without a clear history of pulmonary embolism (PE).

Purpose of the Study:

  • To define CTEPH, its diagnostic criteria, and associated risk factors.
  • To outline the diagnostic pathway and treatment options for CTEPH.
  • To emphasize the importance of expert centers for management.

Main Methods:

  • Diagnostic criteria include precapillary PH (mPAP ≥ 25 mmHg, PCWP ≤ 15 mmHg), perfusion defects on V/Q scans, and evidence of chronic thromboembolic disease.
  • Ventilation-perfusion (V/Q) scans are key for identifying the thromboembolic origin of PH.
  • Referral to expert centers is crucial for pulmonary angiography, right heart catheterization, and CT scans to confirm diagnosis and assess operability.

Main Results:

  • Pulmonary endarterectomy (PEA) is the gold standard surgical treatment for eligible patients with accessible thrombi.
  • For inoperable patients, riociguat is recommended, with potential consideration for off-label drug use and pulmonary angioplasty in expert centers.
  • CTEPH can develop without a prior symptomatic pulmonary embolism, highlighting the need for vigilance in unexplained PH cases.

Conclusions:

  • CTEPH is a distinct entity within pulmonary hypertension requiring a high index of suspicion, especially in unexplained PH.
  • Timely diagnosis and management at specialized centers are critical for improving outcomes.
  • Treatment strategies are tailored to individual patient operability, ranging from surgical intervention to medical therapy and angioplasty.