RECURRENT ISCHEMIC STROKE SECONDARY TO PULMONARY ARTERIOVENOUS MALFORMATIONS

Abstract

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder that has a variety of clinical manifestations and can affect many organ systems. Prevalence rates of HHT are estimated to be between 1:5000 and 1:8000. Knowledge of the risk of developing pulmonary arteriovenous malformations associated with HHT is essential to prevent potentially catastrophic complications, including stroke. CASE PRESENTATION: A 42 year-old female, non-smoker with hereditary hemorrhagic telangiectasia, headaches, epistaxis, and multiple strokes presented with an hour of dizziness, slurred speech, and right sided weakness. Her symptoms completely resolved within three hours of arrival. Family history was significant for three family members with HHT. On physical exam she had dried blood in her nares and multiple blanching telangiectasias on her lips and arms. Her lungs were clear without adventitious sounds on room air and she had no neurologic deficits. CT scan of the head did not demonstrate acute hemorrhage. She had a normal ejection fraction with a positive bubble study on contrast enhanced echocardiogram. She coughed up a quarter-sized bright red blood clot after arrival. She denied a history of massive hemoptysis. CT chest demonstrated a two centimeter right pulmonary arteriovenous malformation (AVM) in the right lower lobe, an eight millimeter left lower lobe AVM, and multiple smaller AVMs throughout both lungs. She underwent coil embolization of the largest AVMs by interventional radiology, and post-embolization angiogram showed no further flow in the arterial branches feeding the AVMs. Within two months, her headaches had improved and she was no longer developing transient slurred speech or unilateral weakness. DISCUSSION: Pulmonary AVMs in patients with HHT are under recognized and undertreated as patients are often asymptomatic, and patients with HHT can have catastrophic strokes before their AVMs are identified. The incidence of pulmonary AVMs in patients with HHT exceeds 50%. In this case, the patient had two strokes secondary to paradoxical emboli before her pulmonary AVMs were identified. The British Thoracic Society recommends that all patients over age 16 with known or suspected HHT should be offered screening for pulmonary AVMs, and that patients with pulmonary AVMs should be considered for referral for embolization. CONCLUSIONS: Screening for asymptomatic pulmonary AVMs and subsequent embolization of pulmonary AVMs in hereditary hemorrhagic telangiectasia is critical to decreasing the morbidity and mortality associated with cerebral abscess and cerebral ischemia from paradoxical emboli, hemoptysis, and hemothorax associated with pulmonary AVMs secondary to HHT. Reference #1: Dupuis-Girod S, Cottin V, Shovlin C, L. The Lung in Hereditary Hemorrhagic Telangiectasia. Respiration. 2017; 94:315-330. Reference #2: Shovlin CL, Condliffe R, Donaldson JW on behalf of the British Thoracic Society, et al. British Thoracic Society Clinical Statement on Pulmonary Arteriovenous Malformations. Thorax. 2017; 72:1154-1163. DISCLOSURES: No relevant relationships by Daniel Hershberger, source=Web Response No relevant relationships by Maureen McElligott, source=Web Response