Cardiovascular Sciences Research Centre, St. George's University of London, London, UK.
J Am Coll Cardiol. 2011 Jun 7;57(23):2340-5. doi: 10.1016/j.jacc.2010.11.067.
The objective of this study was to determine the prevalence of conventional risk factors in sudden arrhythmic death syndrome (SADS) probands with Brugada syndrome (BrS).
Patients with BrS and previous aborted sudden cardiac death (SCD) are at high risk of recurrent events. Other universally accepted clinical features associated with higher risk include unheralded syncope and the presence of a spontaneous type 1 electrocardiogram (ECG).
We analyzed reported symptoms and reviewed ECGs from SADS probands with familial diagnoses of BrS, established by cardiological evaluation, including ECG, 2-dimensional echocardiography, Holter monitoring, exercise tolerance testing, and ajmaline provocation. These cases underwent familial evaluation between 2003 and 2010.
A total of 49 consecutive families with a confirmed SADS death and a diagnosis of BrS were evaluated, comprising assessment of 202 family members in total. One family had 2 members with SADS, resulting in a total of 50 probands included. Mean age of death of probands was 29.1 ± 10.6 years, with 41 males (82%) (p < 0.05). Antemortem ECGs were available for 5 SADS probands, 1 of which demonstrated a spontaneous type 1 pattern. In 45 probands, symptoms before death were reported reliably by family members. Of these, 9 (20%) had experienced at least 1 syncopal episode before the fatal event. Importantly, 68% of probands would not have fulfilled any current criteria for consideration of implantable cardioverter-defibrillator.
The "low-risk" asymptomatic BrS group comprises the majority of SCD in this cohort. Current risk stratification would appear to be inadequate, and new markers of risk are vital.
本研究旨在确定布鲁加达综合征（BrS）先证者中心律失常性猝死综合征（SADS）的常见危险因素的流行率。
患有 BrS 和先前心搏骤停（SCD）的患者复发风险较高。其他普遍公认的与更高风险相关的临床特征包括无症状晕厥和自发性 1 型心电图（ECG）。
我们分析了报告的症状，并回顾了通过心血管评估（包括心电图、二维超声心动图、动态心电图监测、运动耐量测试和喷他脒激发试验）确诊为家族性 BrS 的 SADS 先证者的 ECG。这些病例在 2003 年至 2010 年期间接受了家族评估。
共评估了 49 个连续的家族性 SADS 死亡和 BrS 诊断家族，共评估了 202 名家族成员。一个家族有 2 名成员患有 SADS，总共有 50 名先证者纳入研究。先证者死亡的平均年龄为 29.1±10.6 岁，其中 41 名男性（82%）（p<0.05）。5 名 SADS 先证者的死前心电图可用，其中 1 名显示自发性 1 型模式。在 45 名先证者中，家庭成员可靠地报告了死前的症状。其中，9 名（20%）在致命事件前至少经历过 1 次晕厥发作。重要的是，68%的先证者不符合当前考虑植入式心脏除颤器的任何标准。
在这个队列中，无症状 BrS 组构成了大多数 SCD。当前的风险分层似乎不足，新的风险标志物至关重要。
