肥厚型心肌病伴收缩功能保留患者的晚期钆增强。

Late Gadolinium Enhancement in Patients With Hypertrophic Cardiomyopathy and Preserved Systolic Function.

机构信息

Hypertrophic Cardiomyopathy Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.

出版信息

J Am Coll Cardiol. 2018 Aug 21;72(8):857-870. doi: 10.1016/j.jacc.2018.05.060.

DOI:10.1016/j.jacc.2018.05.060

PMID:30115224

Abstract

BACKGROUND

A high proportion of patients with hypertrophic cardiomyopathy (HCM) have evidence of late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR).

OBJECTIVES

This study sought to assess the incremental prognostic utility of LGE in patients with HCM.

METHODS

We studied 1,423 consecutive low-/intermediate-risk patients with HCM (age ≥18 years) with preserved left ventricular (LV) ejection fraction (mean age 66 ± 14 years, 60% men) who underwent transthoracic echocardiography (TTE) (including dimensions and LV outflow tract gradients) and CMR (including LGE as a % of LV mass) at our center between January 2008 and December 2015. The primary composite endpoint was sudden cardiac death (SCD) and appropriate implantable cardioverter-defibrillator discharge. The percent 5-year SCD risk score was calculated.

RESULTS

The mean 5-year SCD risk score was 2.3 ± 2.0. Mean maximal LV outflow tract gradient (TTE) was 70 ± 55 mm Hg (median 74 mm Hg [interquartile range (IQR): 10 to 67 mm Hg]); indexed LV mass and LGE (both on CMR) were 91 ± 10 g/m and 8.4 ± 12% (IQR: 0% to 19%); 50% had LGE on CMR. Of these, 458 were nonobstructive and 965 were obstructive (of which 686 were underwent myectomy). At 4.7 ± 2.0 years of follow-up, 60 (4%) met the composite endpoint. On quadratic spline analysis, LGE ≥15% was associated with increased risk of composite events. In the obstructive subgroup, on competing risk regression analysis, ≥15% LGE (subhazard ratio: 3.04 [95% confidence interval: 1.48 to 6.10]) was associated with a higher rate and myectomy (subhazard ratio: 0.44 [95% confidence interval: 0.20 to 0.76]) was associated with a lower rate of composite endpoints (both p < 0.01). Similarly, sequential addition of LGE ≥15% and myectomy to % 5-year SCD risk score improved the log likelihood ratios from -227.85 to -219.14 (chi-square 17) and to -215.14 (chi-square 8; both p < 0.01). Association of %LGE with composite events was similar even in myectomy and nonobstructive subgroups.

CONCLUSIONS

In low-/intermediate-risk adult patients with HCM (obstructive, myectomy, and nonobstructive subgroups) with preserved systolic function, %LGE was significantly associated with a higher rate of composite endpoint, providing incremental prognostic utility.

摘要

背景

肥厚型心肌病（HCM）患者中有很大一部分在心脏磁共振（CMR）上有晚期钆增强（LGE）的证据。

目的

本研究旨在评估 LGE 在 HCM 患者中的预后预测价值。

方法

我们研究了 1423 例连续的低/中危 HCM 患者（年龄≥18 岁，左心室射血分数保留[LV ejection fraction，LVEF]（平均年龄 66±14 岁，60%为男性），在我们中心接受了经胸超声心动图（TTE）（包括心脏大小和左心室流出道梯度）和 CMR（包括 LGE 作为 LV 质量的百分比）检查。主要复合终点为心脏性猝死（sudden cardiac death，SCD）和适当的植入式心脏复律除颤器（implantable cardioverter-defibrillator，ICD）放电。计算 5 年 SCD 风险评分。

结果

平均 5 年 SCD 风险评分为 2.3±2.0。最大 LV 流出道梯度（TTE）的平均中位数为 74mmHg（四分位距[interquartile range，IQR]：10 至 67mmHg）；LV 质量和 LGE（均为 CMR 上的参数）的平均中位数为 91g/m（IQR：71 至 137g/m）和 8.4%（IQR：0%至 19%）；50%的患者 CMR 上有 LGE。其中，458 例为非梗阻性，965 例为梗阻性（其中 686 例进行了心肌切除术）。在 4.7±2.0 年的随访中，60 例（4%）达到了复合终点。在二次样条分析中，LGE≥15%与复合事件的风险增加相关。在梗阻性亚组中，竞争风险回归分析显示，LGE≥15%（亚危险比：3.04[95%置信区间：1.48 至 6.10]）与更高的发生率相关，而心肌切除术（亚危险比：0.44[95%置信区间：0.20 至 0.76]）与复合终点发生率降低相关（均 p<0.01）。同样，将 LGE≥15%和心肌切除术依次添加到 5 年 SCD 风险评分中，对数似然比从-227.85 降至-219.14（卡方 17），再降至-215.14（卡方 8；均 p<0.01）。即使在心肌切除术和非梗阻性亚组中，LGE%与复合事件的相关性也相似。