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A 50-year-old woman presented with mild exercise-induced shortness of breath; she reported being otherwise healthy. Her family history was notable for her mother's having the Osler–Weber–Rendu syndrome, also called hereditary hemorrhagic telangiectasia. Physical examination revealed a reduced arterial oxygen saturation level (80%) and telangiectasias of the lips and the tongue (Panel A). Chest radiography showed a tubular opacity in the right lower pulmonary lobe (Panel B, left side, white box, and right side [magnified view], arrows). A gadolinium-enhanced four-dimensional magnetic resonance angiogram revealed a complex vascular structure in the right lower lobe immediately posterior to the left atrium. The posterior view (Panel C) revealed an arteriovenous fistula with two feeding arteries (arrows) and one draining vein (arrowhead). Selective pulmonary angiography confirmed this diagnosis, and the feeding arteries were occluded. Subsequently, the patient's arterial oxygen saturation level returned to normal (98%). The diagnosis of hereditary hemorrhagic telangiectasia was later confirmed by means of genetic examination.
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