Isolated cardiac sarcoidosis associated with coronary vasomotion abnormalities: a case report.
Isolated cardiac sarcoidosis associated with coronary vasomotion abnormalities: a case report.
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DOI：
10.1093/ehjcr/ytac083
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发表时间：
2022-03
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心脏结节病是一种慢性炎症性疾病，可影响心脏，经常导致心力衰竭和致命的心律失常。不经心内膜活检的多模态成像方法可诊断孤立性心脏结节病。冠状动脉血管舒张异常在各种心血管和炎症疾病中非常普遍。目前尚不清楚心肌结节病引起的活动性心肌炎症是否与冠状动脉血管舒张异常有关。68岁男性，无既往病史，院外心脏骤停，因心室颤动，成功复苏，无神经系统后遗症。冠状动脉造影显示冠状动脉正常；然而，冠状动脉内乙酰胆碱激发试验显示心外膜冠状动脉和冠状微血管痉挛。诊断为孤立性心脏结节病，符合日本循环学会2016年诊断指南提出的诊断标准，包括致死性室性心律失常、局灶性左室壁无能性、正电子发射断层扫描心肌氟氧葡萄糖摄取增加、心脏磁共振晚期钆增强。他接受了钙通道阻滞剂治疗冠状动脉痉挛，强的松龙治疗心脏血管病，并植入了植入式心律转复除颤器进行二级预防。治疗后，冠状动脉痉挛的严重程度随心肌炎症的消退而减轻。孤立性心脏结节病心外膜冠状动脉和冠状微血管痉挛可伴有心肌炎症活动，皮质类固醇和钙通道阻滞剂治疗可有效缓解与疾病心肌炎症消退相关的冠状动脉痉挛的严重程度。
Cardiac sarcoidosis is a chronic, inflammatory disease that can affect the heart and often results in heart failure and lethal arrhythmias. A multimodality imaging approach without endomyocardial biopsy allows for the diagnosis of isolated cardiac sarcoidosis. Coronary vasomotion abnormalities are highly prevalent in various cardiovascular and inflammatory diseases. It remains unknown whether active myocardial inflammation due to cardiac sarcoidosis is associated with coronary vasomotion abnormalities. A 68-year-old man without a past medical history experienced an out-of-hospital cardiac arrest due to ventricular fibrillation and was successfully resuscitated without neurologic sequelae. Coronary angiography showed normal coronary arteries; however, intracoronary acetylcholine provocation testing demonstrated both epicardial coronary and coronary microvascular spasm. He was diagnosed with isolated cardiac sarcoidosis by fulfilling the diagnostic criteria proposed by the Japanese Circulation Society 2016 diagnostic guidelines, including fatal ventricular arrhythmia, focal left ventricular wall asynergy, increased myocardial fluorodeoxyglucose uptake by positron emission tomography, and late gadolinium enhancement by cardiac magnetic resonance in the heart. He was treated with calcium-channel blocker for coronary artery spasm and prednisolone for cardiac sarcoidosis and underwent implantation of an implantable cardioverter-defibrillator for secondary prevention. Following the treatment, the severity of coronary artery spasm was reduced along with regression of the myocardial inflammation. Epicardial coronary artery and coronary microvascular spasm can be accompanied by active myocardial inflammation of isolated cardiac sarcoidosis, and the treatment with corticosteroid and calcium-channel blocker may be effective for relieving the severity of coronary artery spasm in association with regression of myocardial inflammation of the disease.