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Chronic Thromboembolic Pulmonary Hypertension

Imaging findings

Chest radiograph shows pulmonary arterial hypertension with an enlarged main pulmonary artery, enlarged right cardiac chambers, and distended azygos vein and SVC. Analysis of the pulmonary vessels reveals a subtle difference in caliber, with dilated vessels in some zones and unusually small pulmonary arteries in others. CTPA confirms chronic intraluminal thromboembolic material with regionally diminished perfusion corresponding to the small vessels, and the patient underwent surgical pulmonary endarterectomy with a measured drop in pulmonary artery pressure.

Key takeaways

In pulmonary hypertension, a discrepancy in the caliber of pulmonary arteries between lung regions, with segmentally small vessels, should prompt consideration of chronic thromboembolic pulmonary hypertension. Beyond simply diagnosing pulmonary arterial hypertension, careful analysis of pulmonary vascular patterns (flow inversion, chronic venous hypertension, balanced left-to-right shunt physiology, and regional oligemia) refines the diagnosis, and CTEPH is surgically treatable by endarterectomy.

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