CTICasesChronic Thromboembolic Pulmonary Hypertension (CTEPH) with Peripheral Arterial Disease
Imaging findings
Chest CT demonstrates findings of severe pulmonary hypertension, including right ventricular hypertrophy, right ventricular enlargement, and a dilated main pulmonary artery. Contrast-enhanced CT shows subsegmental arterial webs and stenosis without large central thrombi, accompanied by a prominent regional mosaic perfusion pattern. A ventilation-perfusion (V/Q) scan is highly striking, demonstrating multiple peripheral, wedge-shaped perfusion defects ("rat bites") with completely normal, homogeneous ventilation.
Key takeaways
Chronic thromboembolic pulmonary hypertension (CTEPH) can present with primarily peripheral, microvascular, and subsegmental disease rather than large, surgically accessible central pulmonary artery thrombi. While CT pulmonary angiography is excellent for visualizing central webs and stenoses, a V/Q scan remains the gold standard screening tool because it is highly sensitive for demonstrating multiple mismatched peripheral perfusion defects (classic "rat bites").
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