Bokhari Syed W, Movahed M Reza, Longhurst John C
Section of Cardiology, University of Chicago-Pritzker School of Medicine, Chicago, Illinois, USA.
Rev Cardiovasc Med. 2004 Winter;5(1):58-64.
A 37-year-old man, brought in following a syncopal episode, was found to be in cardiogenic shock with a complete infranodal heart block. A temporary transvenous pacemaker and an intra-aortic balloon pump were inserted emergently. Cardiac catheterization revealed a high left ventricular end-diastolic pressure but normal coronary vasculature. An echocardiogram demonstrated a hyperdynamic left ventricle, severe hypokinesis of the septum, and asymmetric septal hypertrophy. An endomyocardial biopsy showed myofibril hypertrophy and disarray. The patient required implantation of a permanent pacemaker for full recovery. Although arrhythmias are common in asymmetric septal hypertrophy, complete atrioventricular block is rare but can cause syncope and cardiogenic shock. This is the first case, reported in the literature, of asymmetric septal hypertrophy in which the patient presented with cardiogenic shock and complete heart block secondary to a septal infarction, despite normal coronaries, and in whom a myocardial biopsy was performed. The case report is followed by a review of the literature on hypertrophic cardiomyopathy associated with complete heart block.
一名37岁男性,在一次晕厥发作后被送来就诊,被发现处于心源性休克状态,伴有完全性结下心脏传导阻滞。紧急插入了临时经静脉起搏器和主动脉内球囊泵。心脏导管检查显示左心室舒张末期压力升高,但冠状动脉血管正常。超声心动图显示左心室动力增强、室间隔严重运动减弱以及不对称性室间隔肥厚。心内膜心肌活检显示肌原纤维肥大和排列紊乱。患者需要植入永久性起搏器才能完全康复。虽然心律失常在不对称性室间隔肥厚中很常见,但完全性房室传导阻滞很少见,但可导致晕厥和心源性休克。这是文献报道的首例不对称性室间隔肥厚病例,该患者尽管冠状动脉正常,但因室间隔梗死继发心源性休克和完全性心脏传导阻滞,并进行了心肌活检。病例报告之后是对与完全性心脏传导阻滞相关的肥厚型心肌病的文献综述。