Abstract Introduction Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by recurrent epistaxis, mucocutaneous telangiectasias, and visceral arteriovenous malformations (AVMs). Pulmonary AVMs, present in about one-third of patients, can lead to paradoxical embolization and stroke, though this remains underrecognized. Ischemic stroke occurs in approximately 10-12.5% of cases. We present a 70-year-old woman with previously undiagnosed pulmonary AVMs who presented with transient neurological symptoms and was found to have multifocal ischemic strokes. Case Presentation The patient, with a family history of genetically confirmed HHT and personal history of recurrent epistaxis, presented with acute left-hand numbness and difficulty writing lasting 20 minutes. Head CT demonstrated chronic bilateral cerebellar lacunar infarcts. MRI brain revealed acute multifocal infarcts in the right frontal-parietal lobe along with chronic bilateral cerebellar infarcts. CTA head and neck showed no intracranial or extracranial stenosis. She was started on aspirin 81 mg and atorvastatin 80 mg daily.Further evaluation for embolic sources was pursued. Transthoracic echocardiography with bubble study demonstrated late passage of bubbles, suggestive of an intrapulmonary shunt. CTA of the chest and abdomen identified segmental and subsegmental pulmonary emboli in the right lower lobe without right ventricular strain, as well as two pulmonary AVMs. A subsequent CT chest angiogram detailed multiple AVMs: a right upper lobe lesion with a 4 mm feeding artery, a right lower lobe anterior basal lesion with a 2 mm feeder, and another right lower lobe posterior basal lesion with a 1.5 mm feeder. Bilateral lower extremity Doppler ultrasound was negative for deep venous thrombosis.Given her recent travel, pulmonary embolism was considered provoked, and she was initially managed with intravenous heparin, later transitioned to apixaban. Considering paradoxical embolization leading to cerebral infarcts, embolization of her pulmonary AVMs was pursued. Interventional radiology successfully occluded three feeding vessels using an Amplatzer vascular ring, MVP plug, and platinum coil. Post-embolization, the patient has been doing well. Discussion This case highlights ischemic stroke due to paradoxical embolism across pulmonary AVMs as an important manifestation of HHT. The patient’s stroke led to the discovery of multiple pulmonary AVMs, despite her suggestive history of epistaxis and family history of HHT. This emphasizes the importance of early screening for pulmonary AVMs in suspected or confirmed HHT, as timely embolization can reduce recurrent neurologic events. This abstract is funded by: None
Khalid et al. (Fri,) studied this question.