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CT scoring system to defined thrombus distribution in chronic thromboembolic pulmonary hypertension
Lojain L Abdulaal1,2, Michael M J Sharkey1, Ahmed A Maiter3,4
1Division of Cardiovascular Medicine, University of Sheffield, Sheffield, S10 2TN, United Kingdom.
Insights
A new scoring system for chronic thromboembolic pulmonary hypertension (CTEPH) on CT pulmonary angiography (CTPA) identifies disease location as a predictor of mortality. Central CTEPH is linked to worse survival, especially in patients not undergoing pulmonary endarterectomy.
Area of Science:
- Cardiology
- Radiology
- Pulmonary Medicine
Background:
- Characterizing thrombus is crucial for managing chronic thromboembolic pulmonary hypertension (CTEPH).
- Current assessment methods may not fully capture the prognostic impact of disease distribution.
Purpose of the Study:
- To develop and validate a novel scoring system for visual assessment of CTEPH on CT pulmonary angiography (CTPA).
- To evaluate the impact of thrombus location and extent on survival outcomes in CTEPH patients.
Main Methods:
- Retrospective analysis of 208 CTEPH patients from the ASPIRE registry.
- Development of a scoring system based on predominant disease location: central, segmental, and distal.
- Survival analysis using Cox-regression and Kaplan-Meier curves.
Main Results:
- Patients with central and distal CTEPH exhibited more severe pulmonary hemodynamics and lower gas transfer (TLCO) compared to segmental disease.
- Central disease was independently associated with increased mortality in patients not undergoing pulmonary endarterectomy (HR 1.9, p<0.01).
- The novel scoring system demonstrated excellent interobserver agreement.
Conclusions:
- Thromboembolic disease location is a significant predictor of mortality in CTEPH.
- Central CTEPH location is independently linked to shorter survival, particularly in patients not eligible for pulmonary endarterectomy.
- This scoring system offers a new framework for stratifying CTEPH patients and guiding clinical decisions.
Objectives:
Characterization of thrombus is important for guiding treatment in chronic thromboembolic pulmonary hypertension (CTEPH). This study presents a novel scoring system for visual assessment of CTEPH on CT pulmonary angiography (CTPA), incorporating both disease location and extent to determine the impact on survival outcomes.
Methods:
Patients with CTEPH were identified retrospectively from the Assessing the Spectrum of Pulmonary Hypertension Identified at a Referral Centre registry. The scoring system emphasizes disease based on their predominant location as central, segmental, and distal disease. Survival analysis was conducted using Cox-regression and Kaplan-Meier survival curves.
Results:
A total of 208 patients with CTEPH were included (mean age 66 ± 13.6 years, 52.4% female). Mosaic perfusion and infarction were commonly seen in patients with distal disease (92% and 88%). Patients with central and distal disease had more severe pulmonary hemodynamics and lower gas transfer (TLCO) than patients with segmental disease. Central and distal disease showed similar survival, whereas survival was worse in central compared to segmental disease for all patients (P < .001), including those undergoing (P < .04) and not undergoing endarterectomy (P < .001). Central disease was an independent predictor of mortality in those not undergoing endarterectomy (hazard ratio 1.9, P < .01).
Conclusions:
Our scoring system showed excellent interobserver agreement. Thromboembolic disease location was shown to be a predictor of mortality, with central disease independently associated with shorter survival in patients not undergoing pulmonary endarterectomy.
Advances In Knowledge:
This is a novel scoring system for characterizing CTEPH on CTPA, considering disease location and extent. It provides disease location as a predictor of survival, introducing a new framework for patient stratification and clinical decision-making.
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