Paratz Elizabeth D, Khav Nancy, Burns Andrew T
Department of Cardiology, St Vincent's Hospital Melbourne, Melbourne, Vic, Australia.
Department of Cardiology, St Vincent's Hospital Melbourne, Melbourne, Vic, Australia.
Heart Lung Circ. 2017 Aug;26(8):772-778. doi: 10.1016/j.hlc.2016.12.009. Epub 2017 Feb 15.
A 72-year-old male reported a long-standing history of unexplained syncope. Stress echocardiography demonstrated inducible anterior hypokinesis, and he proceeded to percutaneous coronary intervention for an 80% stenosis of the left anterior descending artery. Thirty minutes post-procedure, he experienced a pulseless electrical activity (PEA) cardiac arrest. Urgent repeat angiography demonstrated profound coronary artery spasm consistent with Kounis syndrome. Three days later, a second PEA arrest occurred. Systemic mastocytosis was ultimately diagnosed as the cause of his recurrent syncopal episodes and cardiac arrests. Our patient was discharged 56days after his cardiac arrest on appropriate immunotherapy, and has made an excellent event-free recovery. Systemic mastocytosis is the pathological accumulation of mast cells in organs, and it may cause life-threatening syncope and cardiac arrests. It is estimated to affect up to 1 in 10,000 people, however is often underdiagnosed. No previous reviews have examined cardiac manifestations of systemic mastocytosis. We undertook a structured systematic review of cardiac presentations of systemic mastocytosis in adults, screening 619 publications. Twenty-three cases met inclusion criteria; our review suggests that short-term mortality is high (22%), and patients with cardiac presentations are predominantly male (83%). Unexplained cardiac arrest (26%) may be the first presentation of this haematological disorder. From our review of the literature, we have also derived suggested management approaches for cardiologists encountering or suspecting systemic mastocytosis in a variety of clinical scenarios.
一名72岁男性有长期不明原因晕厥病史。负荷超声心动图显示可诱导的前壁运动减弱，随后他因左前降支动脉80%狭窄接受了经皮冠状动脉介入治疗。术后30分钟，他发生了无脉电活动（PEA）心脏骤停。紧急重复血管造影显示严重冠状动脉痉挛，符合库尼斯综合征。三天后，再次发生PEA心脏骤停。最终诊断全身性肥大细胞增多症为其反复晕厥发作和心脏骤停的原因。我们的患者在心脏骤停56天后接受适当免疫治疗出院，并且恢复良好，未再出现相关事件。全身性肥大细胞增多症是肥大细胞在器官中的病理性积聚，可能导致危及生命的晕厥和心脏骤停。据估计，每10000人中就有1人受其影响，但该病常常被漏诊。此前尚无综述研究全身性肥大细胞增多症的心脏表现。我们对成人全身性肥大细胞增多症的心脏表现进行了结构化系统综述，筛选了619篇出版物。23例符合纳入标准；我们的综述表明，短期死亡率很高（22%），有心脏表现的患者以男性为主（83%）。不明原因的心脏骤停（26%）可能是这种血液系统疾病的首发表现。通过对文献的综述，我们还得出了针对心脏病专家在各种临床情况下遇到或怀疑全身性肥大细胞增多症时的建议管理方法。
