Aitken William, Tsang Darren, Chaparro Sandra, Kir Devika
Department of Cardiology, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, Florida, USA.
Department of Cardiology, Jackson Memorial Hospital/University of Miami Miller School of Medicine, Miami, Florida, USA
BMJ Case Rep. 2021 Feb 9;14(2):e237530. doi: 10.1136/bcr-2020-237530.
A 67-year-old African-American woman with remote history of complete heart block (s/p pacemaker 3 years ago) and recent onset of ventricular tachycardia (VT) (s/p VT ablation and cardiac resynchronisation therapy defibrillator upgrade 3 months ago) presented to the hospital with VT storm. Workup showed newly reduced left ventricular ejection fraction with global hypokinesis (20%) and restrictive physiology. Positive technetium pyrophosphate scan was suspicious for TTR amyloid while serological workup revealed a monoclonal gammopathy. Cardiac MRI was contraindicated given remote brain aneurysm clip. Given clinical suspicion for cardiac sarcoidosis and divergent non-invasive workup, endomyocardial biopsy was performed which showed non-necrotising granulomas consistent with cardiac sarcoidosis. She was started on steroids with clinical improvement. Cardiac sarcoidosis is a challenging clinical diagnosis, particularly in patients without extracardiac manifestations. This case highlights the importance of a detailed and thorough workup of non-ischaemic cardiomyopathy and being cognizant of infiltrative disease as it can change patient management and outcomes.
一名67岁的非裔美国女性,有陈旧性完全性心脏传导阻滞病史(3年前植入起搏器),近期出现室性心动过速(VT)(3个月前接受VT消融及心脏再同步化治疗除颤器升级),因VT风暴入院。检查发现左心室射血分数新近降低,整体运动减弱(20%),且存在限制性生理学改变。焦磷酸锝扫描阳性,怀疑为转甲状腺素蛋白淀粉样变,而血清学检查显示单克隆丙种球蛋白病。鉴于有陈旧性脑动脉瘤夹,心脏磁共振成像检查属禁忌。鉴于临床怀疑心脏结节病且非侵入性检查结果不一致,遂进行心内膜心肌活检,结果显示为与心脏结节病相符的非坏死性肉芽肿。给予她类固醇治疗后临床症状改善。心脏结节病是一项具有挑战性的临床诊断,尤其是在没有心脏外表现的患者中。该病例凸显了对非缺血性心肌病进行详细全面检查以及认识到浸润性疾病的重要性,因为它会改变患者的治疗管理和预后。