Department of Pediatrics/Division of Pediatric Cardiology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
Eur Heart J. 2011 May;32(9):1114-20. doi: 10.1093/eurheartj/ehr021. Epub 2011 Feb 22.
Congenital or acquired QT prolongation is a risk factor for life-threatening arrhythmias. In patients with hypertrophic cardiomyopathy (HCM), the QT interval may be intrinsically prolonged. However, the prevalence, cause, and significance of QT prolongation among patients with HCM are unknown.
After exclusion of patients on QT-prolonging drugs, a blinded, retrospective analysis of electrocardiograms, echocardiograms, and genotype status in 479 unrelated patients with HCM [201 females, age at diagnosis 41 ± 18 years, maximal left ventricular wall thickness (MLVWT) 22 ± 6 mm] from two independent centres was performed. The mean QTc was 440 ± 28 ms. The QTc exceeded 480 ms in 13% of patients. Age, gender, family history of HCM or sudden cardiac arrest, and genotype status had no association with QTc. Patients with a QTc over 480 ms were more symptomatic at diagnosis (P < 0.001), had a higher MLVWT (P = 0.03), were more obstructive (P < 0.001), and were more likely to have undergone septal reduction therapy (P = 0.02). There was a weak but significant direct linear relationship between QTc and peak outflow gradient (r(2) = 0.05, P < 0.0001).
Compared with <1 in 200 otherwise healthy adults, QT prolongation (QTc > 480 ms) was present in 1 out of 8 patients with HCM. The QTc was partly reflective of the degree of cardiac hypertrophy and left ventricular outflow tract obstruction. Because of its pro-arrhythmic potential and its potential relevance to management and risk stratification, routine QTc assessment should be performed in patients with HCM, particularly when concomitant use of QT-prolonging medications is considered.
先天性或获得性 QT 间期延长是发生危及生命的心律失常的一个危险因素。在肥厚型心肌病(HCM)患者中,QT 间期可能本身就延长。然而,HCM 患者中 QT 间期延长的发生率、原因和意义尚不清楚。
排除正在使用延长 QT 间期药物的患者后,对来自两个独立中心的 479 例无关 HCM 患者(201 例女性,诊断时年龄为 41±18 岁,最大左心室壁厚度[MLVWT]为 22±6mm)的心电图、超声心动图和基因型状态进行了盲法、回顾性分析。平均 QTc 为 440±28ms。13%的患者 QTc 超过 480ms。年龄、性别、HCM 或心源性猝死的家族史以及基因型状态与 QTc 无关。QTc 超过 480ms 的患者在诊断时更有症状(P<0.001),MLVWT 更高(P=0.03),梗阻程度更严重(P<0.001),更有可能接受室间隔切除术(P=0.02)。QTc 与峰值流出梯度之间存在微弱但显著的直接线性关系(r²=0.05,P<0.0001)。
与<1/200 例其他健康成年人相比,1/8 的 HCM 患者存在 QT 间期延长(QTc>480ms)。QTc 部分反映了心脏肥厚和左心室流出道梗阻的程度。由于其致心律失常的潜在风险以及与管理和风险分层的潜在相关性,应在 HCM 患者中常规评估 QTc,尤其是当考虑同时使用延长 QT 间期的药物时。