Corrado D, Nava A, Buja G, Martini B, Fasoli G, Oselladore L, Turrini P, Thiene G
Department of Pathology, University of Padua Medical School, Italy.
J Am Coll Cardiol. 1996 Feb;27(2):443-8. doi: 10.1016/0735-1097(95)00485-8.
We sought to assess whether structural heart disease underlies the syndrome of right bundle branch block, persistent ST segment elevation and sudden death.
Ventricular fibrillation and sudden death may occur in patients with a distinctive electrocardiographic (ECG) pattern of right bundle branch block and persistent ST segment elevation in the right precordial leads.
Sixteen members of a family affected by this syndrome underwent noninvasive cardiac evaluation, including electrocardiography, Holter ambulatory ECG monitoring, stress testing, echocardiography and signal-averaged electrocardiography; two patients had electrophysiologic and angiographic study. Endomyocardial biopsy was performed in one living patient, and postmortem examination, including study of the specialized conduction system, was performed in one victim of sudden death.
Five years before a fatal cardiac arrest, the proband had been resuscitated from sudden cardiac arrest due to recorded ventricular fibrillation. Serial ECGs showed a prolonged PR interval, right bundle branch block, left-axis deviation and persistent ST segment elevation in the right precordial leads, in the absence of clinical heart disease. Postmortem investigation disclosed right ventricular dilation and myocardial atrophy with adipose replacement of the right ventricular free wall as well as sclerotic interruption of the right bundle branch. A variable degree of right bundle branch block and upsloping right precordial ST segment was observed in seven family members; four of the seven had structural right ventricular abnormalities on echocardiography and late potentials on signal-averaged electrocardiography. A sib of the proband also had a prolonged HV interval, inducible ventricular tachycardia and fibrofatty replacement on endomyocardial biopsy.
An autosomal dominant familial cardiomyopathy, mainly involving the right ventricle and the conduction system, accounted for the ECG changes and the electrical instability of the syndrome.
我们试图评估结构性心脏病是否是右束支传导阻滞、持续性ST段抬高和猝死综合征的基础。
右束支传导阻滞和右胸前导联持续性ST段抬高的独特心电图（ECG）模式的患者可能发生心室颤动和猝死。
16名受该综合征影响的家庭成员接受了无创心脏评估，包括心电图、动态心电图监测、负荷试验、超声心动图和信号平均心电图；两名患者进行了电生理和血管造影研究。对一名在世患者进行了心内膜活检，对一名猝死受害者进行了尸检，包括对特殊传导系统的研究。
在致命性心脏骤停前5年，先证者因记录到的心室颤动而从心脏骤停中复苏。系列心电图显示PR间期延长、右束支传导阻滞、左轴偏移和右胸前导联持续性ST段抬高，无临床心脏病。尸检发现右心室扩张和心肌萎缩，右心室游离壁有脂肪替代，右束支有硬化中断。7名家庭成员观察到不同程度的右束支传导阻滞和右胸前ST段上斜；其中4名在超声心动图上有结构性右心室异常，在信号平均心电图上有晚电位。先证者的一名同胞也有HV间期延长、可诱导的室性心动过速和心内膜活检显示纤维脂肪替代。
一种主要累及右心室和传导系统的常染色体显性遗传性心肌病可解释该综合征的心电图变化和电不稳定。
