Peter Munk Cardiac Centre, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada.
Division of Cardiology, Hypertrophic Cardiomyopathy Institute, Tufts Medical Center, Boston, Massachusetts.
JACC Cardiovasc Imaging. 2021 May;14(5):947-958. doi: 10.1016/j.jcmg.2020.09.037. Epub 2020 Nov 25.
This study examined fibrosis progression in hypertrophic cardiomyopathy (HCM) patients, as well as its relationship to patient characteristics, clinical outcomes, and its effect on clinical decision making.
Myocardial fibrosis, as quantified by late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR), provides valuable prognostic information in patients with HCM.
A total of 157 patients with HCM were enrolled in this study, with 2 sequential CMR scans separated by an interval of 4.7 ± 1.9 years.
At the first CMR session (CMR-1), 70% of patients had LGE compared with 85% at CMR-2 (p = 0.001). The extent of LGE extent increased between the 2 CMR procedures, from 4.0 ± 5.6% to 6.3 ± 7.4% (p < 0.0001), with an average LGE progression rate of 0.5 ± 1.0%/year. LGE mass progression was correlated with higher LGE mass and extent on CMR-1 (p = 0.0017 and p = 0.007, respectively), greater indexed left ventricular (LV) mass (p < 0.0001), greater LV maximal wall thickness (p < 0.0001), apical aneurysm at CMR-1 (p < 0.0001), and lower LV ejection fraction (EF) (p = 0.029). Patients who were more likely to have a higher rate of LGE progression presented with more severe disease at baseline, characterized by LGE extent >8% of LV mass, indexed LV mass >100 g/m, maximal wall thickness ≥20 mm, LVEF ≤60%, and apical aneurysm. There was a significant correlation between the magnitude of LGE progression and future implantation of insertable cardioverter-defibrillators (p = 0.004), EF deterioration to ≤50% (p < 0.0001), and admission for heart failure (p = 0.0006).
Myocardial fibrosis in patients with HCM is a slowly progressive process. Progression of LGE is significantly correlated with a number of clinical outcomes such as progression to EF ≤50% and heart failure admission. Judicious use of serial CMR with LGE can provide valuable information to help patient management.
本研究旨在探讨肥厚型心肌病（HCM）患者的纤维化进展及其与患者特征、临床结局的关系，以及对临床决策的影响。
心脏磁共振（CMR）中的晚期钆增强（LGE）可量化心肌纤维化，为 HCM 患者提供有价值的预后信息。
共纳入 157 例 HCM 患者，两次 CMR 检查间隔 4.7±1.9 年。
在第一次 CMR 检查（CMR-1）时，70%的患者有 LGE，而在第二次 CMR 检查（CMR-2）时为 85%（p=0.001）。两次 CMR 检查之间，LGE 程度从 4.0%±5.6%增加到 6.3%±7.4%（p<0.0001），平均 LGE 进展率为 0.5%±1.0%/年。LGE 质量的进展与 CMR-1 上更高的 LGE 质量和程度（p=0.0017 和 p=0.007）、更大的左心室（LV）质量指数（p<0.0001）、更大的 LV 最大壁厚度（p<0.0001）、CMR-1 上的顶瘤（p<0.0001）和更低的 LV 射血分数（EF）（p=0.029）相关。更有可能出现较高 LGE 进展率的患者在基线时表现出更严重的疾病，特征为 LGE 程度>8%的 LV 质量、指数化 LV 质量>100 g/m、最大壁厚度≥20 mm、LVEF≤60%和顶瘤。LGE 进展程度与未来植入埋藏式心脏复律除颤器（ICD）（p=0.004）、EF 恶化至≤50%（p<0.0001）和因心力衰竭入院（p=0.0006）之间存在显著相关性。
肥厚型心肌病患者的心肌纤维化是一个缓慢进展的过程。LGE 的进展与 EF≤50%和心力衰竭入院等多个临床结局显著相关。明智地使用 LGE 序列 CMR 可以提供有价值的信息，以帮助患者管理。
