P.E. reveals no evidence of neurological sequela and multiple reddish purple discolorations of the buccal mucosa. Lateral chest x-ray demonstrates multiple pulmonary nodules, smooth interface with lung. Chest CT a. Note vessel prominence.
Pulmonary arteriogram reveals pulmonary multiple AVMs.
Note large feeding arteries and draining veins. This patient would be categorized as an HHT1. The pulmonary arteriovenous malformations are direct communications between the pulmonary artery and pulmonary vein, as a result of a defect in capillary structure. The feeding artery is dilated and there is aneurysmal dilatation of the proximal vein, which may be smooth or septated. Pathologically, these are hemangiomas of the cavernous type.