Department of Cardiology-Electrophysiology, University Heart Center, University Hospital, Hamburg-Eppendorf, Hamburg, Germany.
Int J Cardiol. 2013 Sep 10;167(6):2539-45. doi: 10.1016/j.ijcard.2012.06.036. Epub 2012 Jun 26.
Marfan syndrome (MFS) is a variable, autosomal-dominant disorder of the connective tissue. In MFS serious ventricular arrhythmias and sudden cardiac death (SCD) can occur. The aim of this prospective study was to reveal underlying risk factors and to prospectively investigate the association between MFS and SCD in a long-term follow-up.
77 patients with MFS were included. At baseline serum N-terminal pro-brain natriuretic peptide (NT-proBNP), transthoracic echocardiogram, 12-lead resting ECG, signal-averaged ECG (SAECG) and a 24-h Holter ECG with time- and frequency domain analyses were performed. The primary composite endpoint was defined as SCD, ventricular tachycardia (VT), ventricular fibrillation (VF) or arrhythmogenic syncope.
The median follow-up (FU) time was 868 days. Among all risk stratification parameters, NT-proBNP remained the exclusive predictor (hazard ratio [HR]: 2.34, 95% confidence interval [CI]: 1.1 to 4.62, p=0.01) for the composite endpoint. With an optimal cut-off point at 214.3 pg/ml NT-proBNP predicted the composite primary endpoint accurately (AUC 0.936, p=0.00046, sensitivity 100%, specificity 79.0%). During FU, seven patients of Group 2 (NT-proBNP ≥ 214.3 pg/ml) reached the composite endpoint and 2 of these patients died due to SCD. In five patients, sustained VT was documented. All patients with a NT-proBNP<214.3 pg/ml (Group 1) experienced no events. Group 2 patients had a significantly higher risk of experiencing the composite endpoint (logrank-test, p<0.001).
In contrast to non-invasive electrocardiographic parameter, NT-proBNP independently predicts adverse arrhythmogenic events in patients with MFS.
马凡综合征（MFS）是一种可变的、常染色体显性遗传的结缔组织疾病。在 MFS 中，严重的室性心律失常和心脏性猝死（SCD）可能发生。本前瞻性研究的目的是揭示潜在的危险因素，并在长期随访中前瞻性地研究 MFS 与 SCD 之间的关联。
纳入 77 例 MFS 患者。在基线时，检测血清 N 末端脑利钠肽前体（NT-proBNP）、经胸超声心动图、12 导联静息心电图、信号平均心电图（SAECG）和 24 小时动态心电图，同时进行时域和频域分析。主要复合终点定义为 SCD、室性心动过速（VT）、心室颤动（VF）或心律失常性晕厥。
中位随访（FU）时间为 868 天。在所有危险分层参数中，NT-proBNP 仍然是唯一的预测因子（危险比[HR]：2.34，95%置信区间[CI]：1.1 至 4.62，p=0.01）。当 NT-proBNP 的最佳截断值为 214.3 pg/ml 时，NT-proBNP 准确预测了复合主要终点（AUC 0.936，p=0.00046，敏感性 100%，特异性 79.0%）。在 FU 期间，2 组（NT-proBNP≥214.3 pg/ml）的 7 例患者达到了复合终点，其中 2 例患者因 SCD 死亡。在 5 例患者中，持续性 VT 被记录下来。所有 NT-proBNP<214.3 pg/ml（1 组）的患者均未发生事件。2 组患者发生复合终点的风险显著增加（对数秩检验，p<0.001）。
与非侵入性心电图参数相比，NT-proBNP 独立预测 MFS 患者的不良致心律失常事件。
