Related Experiment Video
Updated: Jan 2, 2026
Induction and Phenotyping of Acute Right Heart Failure in a Large Animal Model of Chronic Thromboembolic Pulmonary Hypertension
Published on: March 17, 2022
[Clinical characteristics of patients with chronic thromboembolic pulmonary hypertension]
1Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital; Institute of Respiratory Medicine, Chinese Academy of Medical Sciences; National Clinical Research Center for Respiratory Diseases, Beijing 100029, China.
Insights
Chronic thromboembolic pulmonary hypertension (CTEPH) commonly presents with dyspnea and edema. A history of venous thromboembolism (VTE) is frequent, with some patients exhibiting thrombophilia.
Area of Science:
- Cardiology
- Pulmonology
- Vascular Medicine
Background:
- Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe condition characterized by pulmonary hypertension due to unresolved pulmonary artery clots.
- Understanding the clinical spectrum of CTEPH is crucial for timely diagnosis and management.
- Pulmonary vascular resistance (PVR) is a key hemodynamic parameter in assessing CTEPH severity.
Purpose of the Study:
- To investigate the clinical characteristics of patients diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH).
- To compare clinical features between CTEPH patients with high versus low pulmonary vascular resistance (PVR).
- To identify common presenting symptoms, signs, and associated conditions in CTEPH.
Main Methods:
- Prospective data collection from 148 CTEPH patients admitted to China-Japan Friendship Hospital (September 2015 - June 2019).
- Analysis of medical histories, clinical manifestations, laboratory tests, imaging, and hemodynamic parameters.
- Patients categorized into high PVR (>1000 dyn·s·cm(-5)) and low PVR groups for comparative analysis.
Main Results:
- Dyspnea (99.3%), P2 accentuation (64.9%), and edema (43.9%) were the most frequent clinical findings.
- High PVR group showed significantly higher WHO functional class and jugular vein distention compared to the low PVR group (P<0.05).
- A history of venous thromboembolism (VTE) was present in 70.9% of patients, with a higher prevalence in the low PVR group (P<0.001). Thrombophilic disorders were identified in 14.2%.
Conclusions:
- Dyspnea, accentuated P2, and edema are hallmark clinical presentations of CTEPH.
- Higher PVR in CTEPH is associated with more severe functional limitation and specific clinical signs.
- A significant proportion of CTEPH patients have a history of VTE, and some have underlying thrombophilia.
Abstract:
Objective: To investigate the clinical characteristics of patients with chronic thromboembolic pulmonary hypertension (CTEPH). Methods: CTEPH cases consecutively admitted into China-Japan Friendship Hospital from September 2015 to June 2019 were enrolled with prospective data collection. The medical histories, clinical characteristics, laboratory tests, imaging manifestation and hemodynamic parameters were analyzed. Patients were divided into high pulmonary vascular resistance (PVR) group and low PVR group according to the PVR level>1 000 dyn·s·cm(-5) or not, and clinical characteristics were compared between these two groups. Results: In the 148 cases of CTEPH, right heart catheterization was performed in 103 cases with mPAP (45.1±11.0) mmHg and PVR of (992±430) dyn·s·cm(-5). At diagnosis, 88 (59.5%) cases were in WHO functional class Ⅲ and 27 (18.2%) in class Ⅳ. Most common presenting symptoms were dyspnea (147, 99.3%), chest tightness (68, 45.9%), hemoptysis (42, 28.3%), syncope (30, 20.3%), and most common signs were P2 accentuation (95, 64.9%), edema (65, 43.9%), cyanosis (47, 31.8%), systolic murmur (44, 29.7%) and jugular vein distention (35, 23.6%). In 103 cases with right heart catheterization, 52 were in the low PVR group and 51 in high PVR group. Compared to the low PVR group, high PVR group patients had higher WHO functional class and more jugular vein distention (both P<0.05). In all the 148 cases, previous venous thromboembolism (VTE) was confirmed in 105 (70.9%) patients, with a higher prevalence of previous VTE in low PVR group than that in high PVR group (P<0.001). 30 (20.3%) patients had varicose veins of the lower extremities, and 21 (14.2%) had other thrombophilic disorders including antiphospholipid syndrome, protein C and S deficiency and antithrombin Ⅲ deficiency. Conclusions: Dyspnea, P2 accentuation and edema are the most common clinical presentation of CTEPH. Previous history of VTE is common in CTEPH patients with thrombophilia in some cases.
Related Concept Videos
01:30Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
01:29Pulmonary Embolism I: Introduction
01:25Chronic Obstructive Pulmonary Disease-III: Symptoms and Complications.
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:
01:22Chronic Obstructive Pulmonary Disease
Smoking is a primary risk factor for COPD, with over 80% of patients having a history of it. Patients typically experience progressive dyspnea or labored breathing, frequent coughing, and recurrent pulmonary infections. Many eventually succumb to respiratory failure, characterized by...
01:20COPD: Pathogenesis and Clinical Features
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
01:27Pulmonary Embolism III: Nursing Management