CTICasesSevere Pulmonary Arterial Hypertension with In Situ Thrombosis and Tortuous Vessels
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Imaging findings
Serial chest radiographs show progressive, severe enlargement of the main pulmonary arteries and right-sided cardiomegaly over an 11-year period. High-resolution chest computed tomography and maximum intensity projection reconstructions show highly tortuous, corkscrew-like peripheral pulmonary vessels without a classic mosaic attenuation pattern. CT angiography reveals atherosclerotic calcification of the pulmonary arterial walls, sluggish flow, and eccentric intraluminal soft tissue consistent with in situ thrombosis, while a ventilation-perfusion scan shows no segmental perfusion defects.
Key takeaways
Severe, long-standing pulmonary arterial hypertension can cause dramatic tortuosity of peripheral pulmonary vessels and wall calcification. Atherosclerotic calcification of the pulmonary artery walls is a sign of chronic, high-pressure shunting or long-standing severe PAH. In situ thrombosis is a common secondary phenomenon in dilated, high-pressure vessels with sluggish flow and does not necessarily indicate chronic thromboembolic pulmonary hypertension or eligibility for pulmonary endarterectomy.
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