Spirito Paolo, Autore Camillo, Rapezzi Claudio, Bernabò Paola, Badagliacca Roberto, Maron Martin S, Bongioanni Sergio, Coccolo Fabio, Estes N A Mark, Barillà Caterina S, Biagini Elena, Quarta Giovanni, Conte Maria Rosa, Bruzzi Paolo, Maron Barry J
Divisione di Cardiologia, Ente Ospedaliero Ospedali Galliera, Via Volta 8, Genova 16128, Italy.
Circulation. 2009 Apr 7;119(13):1703-10. doi: 10.1161/CIRCULATIONAHA.108.798314. Epub 2009 Mar 23.
The prognostic significance of syncope has not been investigated systematically in hypertrophic cardiomyopathy, and treatment strategies have been based largely on intuition and experience.
We assessed the relationship between syncope and sudden death in 1511 consecutive patients with hypertrophic cardiomyopathy. Unexplained (n=153) or neurally mediated (n=52) syncope occurred in 205 patients (14%). Over a 5.6+/-5.2-year follow-up, 74 patients died suddenly. Relative risk of sudden death was 1.78 (95% confidence interval 0.88 to 3.51, P=0.08) in patients with unexplained syncope and 0.91 (95% confidence interval 0.00 to 3.83, P=1.0) in those with neurally mediated syncope compared with patients without syncope. In multivariable analysis, the temporal proximity of unexplained syncope to initial patient evaluation was independently associated with risk of sudden death (P=0.006). Patients with unexplained syncope within 6 months before the initial evaluation showed a 5-fold increase in risk compared with patients without syncope (adjusted hazard ratio 4.89, 95% confidence interval 2.19 to 10.94), a relationship that was maintained throughout all age groups (<18, 18 to 39, and > or =40 years). Older patients (> or =40 years of age) with remote episodes of syncope (>5 years before initial evaluation) did not show an increased risk of sudden death (adjusted hazard ratio 0.38, 95% confidence interval 0.05 to 2.74).
In the present large cohort of patients with hypertrophic cardiomyopathy, unexplained syncope was a risk factor for sudden death. Patients with syncopal events that occurred in close temporal proximity to the initial evaluation showed a substantially higher risk of sudden death than patients without syncope. Older patients with remote syncopal events did not show an increased risk.
晕厥在肥厚型心肌病中的预后意义尚未得到系统研究，治疗策略很大程度上基于直觉和经验。
我们评估了1511例连续性肥厚型心肌病患者中晕厥与猝死之间的关系。205例患者（14%）发生了不明原因（n = 153）或神经介导性（n = 52）晕厥。在5.6±5.2年的随访中，74例患者猝死。与无晕厥患者相比，不明原因晕厥患者的猝死相对风险为1.78（95%置信区间0.88至3.51，P = 0.08），神经介导性晕厥患者为0.91（95%置信区间0.00至3.83，P = 1.0）。在多变量分析中，不明原因晕厥与初始患者评估的时间接近程度与猝死风险独立相关（P = 0.006）。初始评估前6个月内发生不明原因晕厥的患者与无晕厥患者相比，风险增加了5倍（校正风险比4.89，95%置信区间2.19至10.94），这种关系在所有年龄组（<18岁、18至39岁和≥40岁）中均存在。初始评估前>5年有晕厥远期发作史的老年患者（≥40岁）猝死风险未增加（校正风险比0.38，95%置信区间0.05至2.74）。
在目前这个大型肥厚型心肌病患者队列中，不明原因晕厥是猝死的危险因素。初始评估时近期发生晕厥事件的患者比无晕厥患者的猝死风险显著更高。有晕厥远期发作史的老年患者猝死风险未增加。
