Bennett Sadie, Phan Thanh, Patwala Ashish, Thamman Ritu, Kwok Chun Shing
Royal Stoke University Hospital, Stoke-on-Trent, UK.
University of Pittsburgh, Pittsburgh, Pennsylvania.
Echocardiography. 2019 Jul;36(7):1405-1408. doi: 10.1111/echo.14411. Epub 2019 Jul 9.
A 38-year-old man with no previous medical history presented to hospital after having an out-of-hospital cardiac arrest. He was found to have a ventricular fibrillation and was successfully resuscitated after receiving cardiopulmonary resuscitation and three shocks. Extensive investigations were performed which included an electrocardiogram that showed no significant abnormality, coronary angiogram which showed unobstructed arteries, and a flecainide challenge test which was negative for Brugada syndrome. A resting echocardiogram showed a myxomatous mitral valve with mild bi-leaflet bowing, trivial mitral regurgitation, normal left ventricular systolic function, and no other structural abnormalities. A cardiac magnetic resonance imaging showed no significant late gadolinium enhancement to suggest infarct or myocardial scarring. He was subsequently diagnosed with idiopathic ventricular fibrillation and treated with a subcutaneous internal cardioverter-defibrillator for secondary prevention. A follow-up echocardiogram was performed which revealed the presence of mitral annular disjunction which has been recently shown to be associated with significant life-threatening arrhythmias and sudden cardiac death. This case highlights the importance of improving awareness of mitral annular disjunction which is not often considered as a cause for adverse patient outcomes.
一名38岁无既往病史的男性在院外心脏骤停后被送往医院。他被发现患有室颤，在接受心肺复苏和三次电击后成功复苏。进行了广泛的检查，包括心电图显示无明显异常、冠状动脉造影显示动脉通畅以及氟卡尼激发试验对 Brugada 综合征呈阴性。静息超声心动图显示二尖瓣黏液瘤样改变，轻度双叶瓣叶弯曲，轻度二尖瓣反流，左心室收缩功能正常，无其他结构异常。心脏磁共振成像显示无明显延迟钆增强，提示无梗死或心肌瘢痕形成。他随后被诊断为特发性室颤，并接受皮下植入式心脏复律除颤器进行二级预防。随后进行的随访超声心动图显示存在二尖瓣环分离，最近已证明其与严重的危及生命的心律失常和心源性猝死有关。该病例强调了提高对二尖瓣环分离的认识的重要性，二尖瓣环分离通常不被认为是导致患者不良结局的原因。
