Percutaneous left atrial appendage closure-An alternative strategy for anticoagulation in atrial fibrillation and hereditary hemorrhagic telangiectasia?

Many patients with hereditary hemorrhagic telangiectasia (HHT) are unable to sustain oral anticoagulation (OAC) because of severe epistaxis, gastrointestinal (GI) bleeding and the risk of life-threatening bleeding from cerebral arteriovenous malformations (CAVMs) or pulmonary arteriovenous malformations (PAVMs). In patients with atrial fibrillation (AF), most thromboembolic complications arise from the left atrial appendage (LAA) and percutaneous transcatheter LAA closure proved to be non-inferior to OAC at mid-term follow-up. We report our experience with LAA closure in HHT with a follow-up of 12 months. Percutaneous LAA closure was performed in five patients with both HHT and high thromboembolic risk AF (CHA2DS2-VASc score ≥2) without peri-procedural complications. At 3 months no thromboembolic event occurred. After 12 months one patient had a transient ischemic attack while another patient had recurrence of stroke, this latter patient had a significant stenosis of the carotid artery and an incomplete closure of the LAA without any signs of thrombus on echocardiogram. Both patients had a non-treatable pulmonary right-to-left shunt (RLS). Percutaneous closure of the LAA may provide an alternative strategy to long-term OAC therapy in HHT patients with AF induced high stroke risk and intolerance for OAC.