Left atrial appendage closure in patients with hereditary hemorrhagic telangiectasia and atrial fibrillation: a prospective study and systematic review.

Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant vascular disease with a high risk of iron deficiency anemia secondary to epistaxis and gastrointestinal bleeding. Atrial fibrillation (AF) is associated with a high risk of ischemic stroke and oral anticoagulation therapy is indicated for its prevention. Percutaneous left atrial appendage closure (LAAC) is recommended to prevent stroke in patients with AF and high risk of bleeding such as HHT patients. However, double antiplatelet therapy at first, followed by long-term single antiplatelet therapy, is also recommended after the procedure to prevent device-related thrombus (DRT), which most HHT patients cannot tolerate. This prospective observational study describes 36 HHT patients with AF, 23 of whom underwent LAAC. To prevent DRT, simple antiplatelet therapy was indicated for 3 months if tolerated. In addition, a systematic review on LAAC in HHT patients was performed to compare our results with what has been previously reported. A total of 57 HHT patients with AF and LAAC were analysed. Although only 12.3% tolerated long-term simple antiplatelet therapy, none presented DRT during follow-up. Three ischemic events were reported but only one of them appears to be partially related to AF in a patient with significant leakage peri-device. In conclusion, percutaneous LAAC is a safe and potentially effective strategy for preventing ischemic stroke in HHT patients with AF. A simplified and personalized antiplatelet regimen was not associated with DRT or ischemic events.