Pulmonary Vein Sign on Computed Tomography Pulmonary Angiography in Proximal and Distal Chronic Thromboembolic Pulmonary Hypertension With Hemodynamic Correlation

Pulmonary vein sign (PVS) indicates abnormal pulmonary venous flow on computed tomography pulmonary angiography (CTPA) is a frequent finding in proximal chronic thromboembolic pulmonary hypertension (CTEPH). PVS’s occurrence in distal CTEPH and correlation to disease severity is unknown. Using right heart catheterization data, we evaluated the relationship between PVS and CTEPH disease distribution and severity. MATERIALS AND METHOD: A total of 93 consecutive CTEPH cases with both CTPA and right heart catheterization were identified in this retrospective multi-institutional study. After excluding 17 cases with suboptimal CTPA, there were 52 proximal and 24 distal CTEPH cases. Blood flow in the major pulmonary veins was graded qualitatively. Subgroup analysis of PVS was performed in 38 proximal CTEPH cases before and after pulmonary endarterectomy. RESULTS: PVS was more frequent in proximal (79%) than distal CTEPH (29%) (P<0.001). No significant difference was noted in invasive mean pulmonary artery pressure (46±11 and 41±12 mm Hg) or pulmonary vascular resistance (9.4±4.5 and 8.4±4.8 WU) between the 2 groups. In the subgroup analysis, PVS was present in 29/38 patients (76%) before surgery. Postoperatively, 33/38 cases (87%, P<0.001) had normal venous flow (mean pulmonary artery pressure 46±11 and 25; pulmonary vascular resistance 9.2±4.3 and 2.6 WU preop and postop, respectively). CONCLUSION: PVS is a common feature in proximal but infrequent findings in distal CTEPH. PVS does not correlate with hemodynamic severity. PVS resolution was seen in the majority of patients following successful endarterectomy.