Related Experiment Video

Updated: Jul 18, 2026

In Vitro Microfluidic Disease Model to Study Whole Blood-Endothelial Interactions and Blood Clot Dynamics in Real-Time
09:19

In Vitro Microfluidic Disease Model to Study Whole Blood-Endothelial Interactions and Blood Clot Dynamics in Real-Time

Published on: May 24, 2020

Molecular biology of chronic thromboembolic pulmonary hypertension

Richard S Sacks1, Carmelle V Remillard, Negin Agange

  • 1Department of Medicine, University of California, San Diego, La Jolla 92093-0725, USA.

Seminars in Thoracic and Cardiovascular Surgery
|December 23, 2006
PubMed

Insights

Mechanisms of chronic thromboembolic pulmonary hypertension (CTEPH) remain unclear, despite advances in pulmonary arterial hypertension research. This review explores the cellular and molecular factors contributing to CTEPH development and progression.

Area of Science:

  • Cardiovascular Medicine
  • Pulmonary Medicine
  • Molecular Biology

Background:

  • Pulmonary arterial hypertension (PAH) research has advanced, but chronic thromboembolic pulmonary hypertension (CTEPH) is often excluded.
  • While clinical features of CTEPH are known, its cellular, molecular, and genetic underpinnings are poorly understood.
  • Prior venous thromboembolism is a risk factor, but predisposition to CTEPH remains unclear.

Purpose of the Study:

  • To review potential molecular and cellular mechanisms in chronic thromboembolic pulmonary hypertension (CTEPH) pathogenesis.
  • To highlight the knowledge gap in CTEPH compared to other forms of pulmonary hypertension.
  • To discuss the dual pathogenic components of CTEPH: thrombotic obstruction and vascular remodeling.

Main Methods:

  • Literature review focusing on cellular and molecular mechanisms.
  • Synthesis of existing knowledge on CTEPH pathogenesis.
  • Comparative analysis with idiopathic pulmonary arterial hypertension.

Main Results:

  • CTEPH involves both pulmonary artery obstruction by thrombi and severe pulmonary vascular remodeling.
  • Molecular and cellular pathways contributing to vascular remodeling in CTEPH are under investigation.
  • Genetic and cellular factors influencing predisposition to CTEPH require further elucidation.

Conclusions:

  • Understanding the molecular and cellular basis of CTEPH is crucial for developing targeted therapies.
  • Further research is needed to identify specific cellular and molecular drivers of CTEPH.
  • Addressing the knowledge gap in CTEPH mechanisms will improve patient outcomes.

More Related Videos

Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet09:22

Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet

Published on: November 4, 2015

Right Ventricular Systolic Pressure Measurements in Combination with Harvest of Lung and Immune Tissue Samples in Mice10:20

Right Ventricular Systolic Pressure Measurements in Combination with Harvest of Lung and Immune Tissue Samples in Mice

Published on: January 16, 2013

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...
Pulmonary Embolism I: Introduction01:19

Pulmonary Embolism I: Introduction

A blood clot, or thrombus, is a semi-solid mass composed of fibrin, platelets, and red blood cells. When it forms within a vessel, it can obstruct blood flow, known as thrombosis. If part of the clot detaches, it becomes an embolus that can travel and block distant vessels. When this occurs in the pulmonary arteries, it causes a condition known as pulmonary embolism (PE).Origin and ImpactMost often, the embolus originates from a thrombus in the deep veins of the lower limbs, a condition called...
Pulmonary Embolism I: Introduction01:29

Pulmonary Embolism I: Introduction

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

Chronic Obstructive Pulmonary Disease-II: Pathophysiology

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
Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care01:29

Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care

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...