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Severe CTEPH in a Young Adult with Systemic-to-Pulmonary Collaterals

Imaging findings

CT angiography in a 25-year-old man shows massive bilateral central pulmonary artery thrombus with circumferential mural thickening, near-absent left atrial filling (underfilled LV), right ventricular hypertrophy, mosaic pulmonary perfusion, and recruited systemic-to-pulmonary collateral vessels arising from the left subclavian artery to the right lung apex.

Key takeaways

Severe, long-standing CTEPH can recruit systemic-to-pulmonary arterial collaterals in an attempt to maintain perfusion to the chronically obstructed lung segments, similar to what is seen in congenital heart disease with diminished pulmonary blood flow. These tortuous, enlarged collateral vessels originating from subclavian or intercostal arteries are identifiable on CT and indicate chronicity. Pulmonary endarterectomy offers the best chance of cure in operable central disease.

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