Percutaneous Left Atrial Appendage Closure in a Patient With Acquired Von Willebrand Disease and Atrial Fibrillation

Left atrial appendage closure (LAAC) can be used to prevent embolic events in patients with atrial fibrillation who cannot tolerate oral anticoagulants. LAAC has not yet been performed in patients with acquired von Willebrand syndrome. A 74-year-old male with von Willebrand disease presents to the emergency department because of palpitations. Atrial fibrillation with congestive heart failure, hypertension, age >= 75, diabetes, stroke, vascular disease, age between 65-74, and female sex (CHA2DS2VASC) of 4 was diagnosed. Oral anticoagulation was withheld because of a past medical history of major bleeding events despite treatment of the underlying bleeding diathesis. Therefore, LAAC was considered for stroke prevention. However, the procedure was delayed due to abnormal coagulation cascade levels. Because of the ineffectiveness of treatment and persistently low levels of factor VIII and von Willebrand factor (vWF), the von Willebrand disease hypothesis was abandoned, prompting a new diagnosis for the bleeding disorder. Rapid clearance of factor VIII and vWF, the good response to intravenous immunoglobulins, and the presence of monoclonal gammopathy of undetermined significance allowed the diagnosis of acquired von Willebrand syndrome. After administration of immunoglobulins, factor VIII and vWF levels were normalized, and the LAAC was performed. The patient was discharged on low-dose aspirin. At the ninemonth follow-up, the patient did not experience bleeding or embolic events. Stroke prevention in patients with atrial fibrillation and increased bleeding risk requires alternatives to oral anticoagulation. LAAC can be safely performed in patients with acquired von Willebrand syndrome and atrial fibrillation.