This is a rare presentation of Takayasu arteritis in a 30-year-old Canadian First Nations woman with cardiac and aortic root-predominant disease, which manifested in complete heart block. She had a past medical history significant for substance misuse. At presentation, cardiac magnetic resonance imaging identified diffuse thickening of the left atrium and ventricular outflow tract with left ventricular cavity dilation and preserved systolic function. A pacemaker was inserted at this time. Nine months later, the patient died following an out-of-hospital cardiac arrest in the context of cocaine intoxication. At autopsy, the cardiac thickening was also found to involve the proximal aortic root, which on microscopy demonstrated non-infectious aortitis and myocarditis with a granulomatous inflammatory pattern and dense fibrosis indicative of Takayasu arteritis. Important clinical clues to the diagnosis include age, sex, and Pacific Islands, American indigenous and Asian ethnicity. The case also underscores the need to rule out secondary causes of complete heart block, including systemic vasculitides, for all patients regardless of substance use history.

这是一位 30 岁的加拿大原住民女性患有心脏和主动脉根部为主的疾病，表现为完全性心脏传导阻滞，这是一种罕见的 Takayasu 动脉炎表现。她有严重滥用药物的既往病史。就诊时，心脏磁共振成像发现左心房和心室流出道弥漫性增厚，左心室腔扩张并保留收缩功能。这时候植入了心脏起搏器。九个月后，患者在可卡因中毒的情况下在院外心脏骤停后死亡。在尸检中，还发现心脏增厚涉及近端主动脉根部，显微镜下显示非感染性主动脉炎和心肌炎，伴有肉芽肿性炎症和致密纤维化，提示高安动脉炎。诊断的重要临床线索包括年龄、性别和太平洋岛屿、美国土著和亚洲种族。该病例还强调需要排除所有患者的完全性心脏传导阻滞的继发性原因，包括全身性血管炎，无论物质使用史如何。