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Updated: Nov 8, 2025
Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
Risk prediction in medically treated chronic thromboembolic pulmonary hypertension
Ruilin Quan1, Yuanhua Yang2, Zhenwen Yang3
1Department of Pulmonary Vascular Disease, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167, Beilishi Road, Xicheng District, Beijing, 100037, China.
Insights
A new risk prediction model and score were developed for medically treated chronic thromboembolic pulmonary hypertension (CTEPH) patients. This tool improves prognostic accuracy and aids in guiding patient management strategies.
Area of Science:
- Cardiology
- Pulmonology
- Medical Statistics
Background:
- No comprehensive prognostic model exists for medically treated chronic thromboembolic pulmonary hypertension (CTEPH) patients.
- Accurate risk stratification is crucial for optimizing management and improving outcomes in CTEPH.
Purpose of the Study:
- To develop and validate a novel prognostic risk prediction model and a simplified risk score for medically treated CTEPH patients.
- To compare the performance of the new model against the existing Swedish/COMPERA risk stratification method.
Main Methods:
- A national multicenter prospective registry study enrolled 432 medically treated CTEPH patients.
- A multivariable Cox proportional hazards model was used to derive the prognostic model and a risk score.
- Model performance was assessed for discrimination and calibration, with internal and external validation.
Main Results:
- The final model incorporated Swedish/COMPERA risk stratum, pulmonary vascular resistance (PVR), total bilirubin (TBIL), and chronic kidney disease (CKD).
- The derived model and risk score demonstrated improved discriminatory power compared to the Swedish/COMPERA method alone (C-index > 0.715).
- Validation in an external cohort confirmed the model's robust performance (C-index = 0.707 for model, 0.721 for score).
Conclusions:
- A novel, validated risk stratification strategy can effectively predict prognosis in medically treated CTEPH patients.
- This tool can assist clinicians in making informed decisions regarding patient management and treatment strategies.
Background:
At present, there is no generally accepted comprehensive prognostic risk prediction model for medically treated chronic thromboembolic pulmonary hypertension (CTEPH) patients.
Methods:
Consecutive medically treated CTEPH patients were enrolled in a national multicenter prospective registry study from August 2009 to July 2018. A multivariable Cox proportional hazards model was utilized to derive the prognostic model, and a simplified risk score was created thereafter. Model performance was evaluated in terms of discrimination and calibration, and compared to the Swedish/COMPERA risk stratification method. Internal and external validation were conducted to validate the model performance.
Results:
A total of 432 patients were enrolled. During a median follow-up time of 38.73 months (IQR: 20.79, 66.10), 94 patients (21.8%) died. The 1-, 3-, and 5-year survival estimates were 95.5%, 83.7%, and 70.9%, respectively. The final model included the following variables: the Swedish/COMPERA risk stratum (low-, intermediate- or high-risk stratum), pulmonary vascular resistance (PVR, ≤ or > 1600 dyn·s/cm5), total bilirubin (TBIL, ≤ or > 38 µmol/L) and chronic kidney disease (CKD, no or yes). Compared with the Swedish/COMPERA risk stratification method alone, both the derived model [C-index: 0.715; net reclassification improvement (NRI): 0.300; integrated discriminatory index (IDI): 0.095] and the risk score (C-index: 0.713; NRI: 0.300; IDI: 0.093) showed improved discriminatory power. The performance was validated in a validation cohort of 84 patients (C-index = 0.707 for the model and 0.721 for the risk score).
Conclusions:
A novel risk stratification strategy can serve as a useful tool for determining prognosis and guide management for medically treated CTEPH patients.
Trial Registration:
ClinicalTrials.gov (Identifier: NCT01417338).
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