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Chronic Pulmonary Thromboembolic Hypertension (CTEPH)

Chronic Pulmonary Thromboembolic Hypertension (CTEPH)▶ Watch on YouTube — age-restricted, cannot embed here

Imaging findings

Chest radiographs over several years showed progression to pulmonary arterial hypertension, with dilated pulmonary arteries, non-dilated pulmonary veins, and an enlarged right atrium. CT revealed extensive clot in the right pulmonary artery, extending into the left lower lobe, with overall diminutive pulmonary artery calibers in the right lower lobe. Lungs showed mosaic attenuation and a cavitary pulmonary infarction. Follow-up CT after anticoagulation showed diminished clot burden and some resolution, particularly in areas amenable to endarterectomy. The left side had less central clot but extensive peripheral occlusion.

Key takeaways

CTEPH presents with progressive pulmonary hypertension, leading to dilated pulmonary arteries and right heart enlargement. CT shows extensive chronic thromboemboli, mosaic attenuation, and often pulmonary infarction. Anticoagulation can lead to some resolution of clot. Surgical endarterectomy is a treatment option, with varying success depending on the location and nature of the thrombi (e.g., easier to form a plane on the right than the left). Heparin-induced thrombocytopenia can complicate management.

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