Captur Gabriella, Manisty Charlotte H, Raman Betty, Marchi Alberto, Wong Timothy C, Ariga Rina, Bhuva Anish, Ormondroyd Elizabeth, Lobascio Ilaria, Camaioni Claudia, Loizos Savvas, Bonsu-Ofori Jenade, Turer Aslan, Zaha Vlad G, Augutsto João B, Davies Rhodri H, Taylor Andrew J, Nasis Arthur, Al-Mallah Mouaz H, Valentin Sinitsyn, Perez de Arenaza Diego, Patel Vimal, Westwood Mark, Petersen Steffen E, Li Chunming, Tang Lijun, Nakamori Shiro, Nezafat Reza, Kwong Raymond Y, Ho Carolyn Y, Fraser Alan G, Watkins Hugh, Elliott Perry M, Neubauer Stefan, Lloyd Guy, Olivotto Iacopo, Nihoyannopoulos Petros, Moon James C
UCL MRC Unit for Lifelong Health and Ageing, University College London, London, United Kingdom; The Royal Free Hospital, Centre for Inherited Heart Muscle Conditions, Cardiology Department, London, United Kingdom; UCL Institute of Cardiovascular Science, University College London, London, United Kingdom.
UCL Institute of Cardiovascular Science, University College London, London, United Kingdom; Barts Heart Center, St. Bartholomew's Hospital, West Smithfield, London, United Kingdom.
JACC Cardiovasc Imaging. 2021 Nov;14(11):2123-2134. doi: 10.1016/j.jcmg.2021.03.032. Epub 2021 Jun 16.
The aim of this study was to define the variability of maximal wall thickness (MWT) measurements across modalities and predict its impact on care in patients with hypertrophic cardiomyopathy (HCM).
Left ventricular MWT measured by echocardiography or cardiovascular magnetic resonance (CMR) contributes to the diagnosis of HCM, stratifies risk, and guides key decisions, including whether to place an implantable cardioverter-defibrillator (ICD).
A 20-center global network provided paired echocardiographic and CMR data sets from patients with HCM, from which 17 paired data sets of the highest quality were selected. These were presented as 7 randomly ordered pairs (at 6 cardiac conferences) to experienced readers who report HCM imaging in their daily practice, and their MWT caliper measurements were captured. The impact of measurement variability on ICD insertion decisions was estimated in 769 separately recruited multicenter patients with HCM using the European Society of Cardiology algorithm for 5-year risk for sudden cardiac death.
MWT analysis was completed by 70 readers (from 6 continents; 91% with >5 years' experience). Seventy-nine percent and 68% scored echocardiographic and CMR image quality as excellent. For both modalities (echocardiographic and then CMR results), intramodality inter-reader MWT percentage variability was large (range -59% to 117% [SD ±20%] and -61% to 52% [SD ±11%], respectively). Agreement between modalities was low (SE of measurement 4.8 mm; 95% CI 4.3 mm-5.2 mm; r = 0.56 [modest correlation]). In the multicenter HCM cohort, this estimated echocardiographic MWT percentage variability (±20%) applied to the European Society of Cardiology algorithm reclassified risk in 19.5% of patients, which would have led to inappropriate ICD decision making in 1 in 7 patients with HCM (8.7% would have had ICD placement recommended despite potential low risk, and 6.8% would not have had ICD placement recommended despite intermediate or high risk).
Using the best available images and experienced readers, MWT as a biomarker in HCM has a high degree of inter-reader variability and should be applied with caution as part of decision making for ICD insertion. Better standardization efforts in HCM recommendations by current governing societies are needed to improve clinical decision making in patients with HCM.
本研究旨在确定肥厚型心肌病（HCM）患者最大壁厚度（MWT）测量在不同检查方式之间的变异性，并预测其对治疗的影响。
通过超声心动图或心血管磁共振成像（CMR）测量的左心室MWT有助于HCM的诊断、风险分层并指导关键决策，包括是否植入植入式心脏复律除颤器（ICD）。
一个20中心的全球网络提供了HCM患者的配对超声心动图和CMR数据集，从中选取了17对质量最高的数据集。这些数据集以7对随机排序的形式（在6次心脏会议上）呈现给在日常工作中报告HCM成像的经验丰富的读者，并记录他们的MWT卡尺测量值。使用欧洲心脏病学会的心脏性猝死5年风险算法，在另外招募的769例多中心HCM患者中评估测量变异性对ICD植入决策的影响。
70位读者（来自6个大洲；91%有超过5年经验）完成了MWT分析。79%和68%的读者将超声心动图和CMR图像质量评为优秀。对于两种检查方式（先超声心动图结果，后CMR结果），读者间MWT百分比变异性在检查方式内都很大（范围分别为-59%至117%[标准差±20%]和-61%至52%[标准差±11%]）。两种检查方式之间的一致性较低（测量标准误为4.8毫米；95%置信区间为4.3毫米至5.2毫米；r = 0.56[中度相关]）。在多中心HCM队列中，这种估计的超声心动图MWT百分比变异性（±20%）应用于欧洲心脏病学会算法时，使19.5%的患者风险重新分类，这将导致每7例HCM患者中有1例ICD决策不当（8.7%的患者尽管潜在风险低但会被建议植入ICD，6.8%的患者尽管风险为中度或高度但不会被建议植入ICD）。
使用可得的最佳图像和经验丰富的读者，MWT作为HCM中的生物标志物，读者间变异性很高，作为ICD植入决策的一部分应谨慎应用。当前管理学会需要在HCM建议方面做出更好的标准化努力，以改善HCM患者的临床决策。
