Giaj Levra Alessandro, Jamie Gulrays, Cozzi Ottavia
Department of Biomedical Sciences, Humanitas University, Milan, ITA.
Cardio Center, Humanitas Research Hospital, Scientific Institute for Research, Hospitalization and Healthcare (IRCCS), Milan, ITA.
Cureus. 2024 Jul 6;16(7):e63936. doi: 10.7759/cureus.63936. eCollection 2024 Jul.
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 (CHA2DS2-VASC) 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 nine-month 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.
左心耳封堵术（LAAC）可用于预防无法耐受口服抗凝剂的房颤患者发生栓塞事件。获得性血管性血友病综合征患者尚未进行过LAAC。一名74岁患有血管性血友病的男性因心悸就诊于急诊科。诊断为房颤合并充血性心力衰竭、高血压、年龄≥75岁、糖尿病、中风、血管疾病、年龄在65 - 74岁之间以及女性（CHA2DS2-VASC评分为4分）。尽管对潜在的出血素质进行了治疗，但由于有重大出血事件的既往病史，未给予口服抗凝治疗。因此，考虑进行LAAC以预防中风。然而，由于凝血级联水平异常，该手术被推迟。由于治疗无效且凝血因子VIII和血管性血友病因子（vWF）水平持续较低，血管性血友病的假设被摒弃，促使对出血性疾病做出新的诊断。凝血因子VIII和vWF的快速清除、对静脉注射免疫球蛋白的良好反应以及意义未明的单克隆丙种球蛋白病的存在，提示诊断为获得性血管性血友病综合征。给予免疫球蛋白后，凝血因子VIII和vWF水平恢复正常，随后进行了LAAC。患者出院时服用小剂量阿司匹林。在9个月的随访中，患者未发生出血或栓塞事件。房颤且出血风险增加的患者预防中风需要口服抗凝治疗的替代方法。LAAC可在获得性血管性血友病综合征合并房颤的患者中安全进行。
