CTICasesChronic Thromboembolic Pulmonary Hypertension (CTEPH) - Peripheral Disease
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Imaging findings
CT showed mosaic attenuation (ground glass and hyperlucent lung with corresponding vessel size variations), peripheral lobular ground-glass opacities, right heart enlargement, and an enlarged pulmonary artery. Only a few subtle intraluminal webs were identified in the pulmonary arteries. Ventilation-perfusion (V/Q) scan confirmed the diagnosis with multiple peripheral perfusion defects ("rat bites") and normal ventilation, highlighting diffuse peripheral disease not readily apparent on CT.
Key takeaways
V/Q scanning remains a crucial diagnostic tool for chronic thromboembolic pulmonary hypertension (CTEPH), especially in cases with predominantly peripheral disease or subtle CT findings. V/Q discordance (perfusion defects with normal ventilation) is highly specific for CTEPH, and a normal V/Q scan effectively excludes the diagnosis.
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