Lung parenchymal and cardiac appearances on computed tomography pulmonary angiography impact survival in chronic thromboembolic pulmonary hypertension: results from the ASPIRE Registry

TL;DR

This study shows that lung and heart features seen on CT scans of CTEPH patients affect their survival, especially when lung disease is present.

Contribution

The study identifies how lung and cardiac CT findings impact survival in CTEPH patients, particularly the masking effect of lung disease on mosaic perfusion.

Findings

Co-existing lung disease is associated with worse survival in CTEPH patients not undergoing surgery.

Mosaic perfusion is less common in patients with parenchymal lung disease.

Increased right/left ventricular ratio and aortic diameter predict worse outcomes.

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is commonly evaluated using computed tomography pulmonary angiography (CTPA). We evaluated the frequency and impact of parenchymal and cardiac abnormalities on survival in CTEPH.

Patients were identified from the ASPIRE (Assessing the Spectrum of Pulmonary Hypertension Identified at a Referral Centre) Registry. Kaplan–Meier analysis was used to assess survival.

290 patients (55% female, mean±sd age 65±14 years) with CTEPH were included. Mosaic perfusion was noted in 83%, lung infarction in 73% and parenchymal lung disease in 28%. The severity of mosaic perfusion and lung infarction correlated with markers of disease severity (p<0.001). Whereas the presence of mosaic perfusion was associated with improved survival in all patients (p=0.03), it did not predict outcome in those undergoing pulmonary endarterectomy (PEA) (p=0.6) and those not undergoing PEA (p=0.22). The presence of lung infarction had no impact on mortality. The presence of co-existing lung disease was associated with a worse survival (p<0.008) in patients not undergoing PEA. Mosaic perfusion was less common in patients with parenchymal lung disease (65%) compared to those without parenchymal lung disease (90%), p<0.001. An increased right/left ventricular ratio and aortic diameter predicted a worse outcome (p<0.002).

Lung parenchymal and cardiac changes on CTPA predict outcome in CTEPH. Co-existing parenchymal lung disease is not uncommon and when present may mask the presence of mosaic perfusion. This study highlights the importance of systematically evaluating the lung parenchyma and cardiac changes in patients with CTEPH.

This study highlights the value of systematically evaluating vessels, cardiac chambers and the lung parenchyma in patients with CTEPH and the adverse impact of parenchymal lung disease on survival
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