CT scoring system to defined thrombus distribution in chronic thromboembolic pulmonary hypertension

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.