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肥厚型心肌病患者左心室小梁化模式

Patterns of left ventricular trabeculation in hypertrophic cardiomyopathy.

作者信息

Zhang Yu, Zhou Di, Zhu Yuming, Huang Qiya, Wang Jizheng, Zhang Channa, Kang Lianming, Lu Minjie, Song Lei

机构信息

State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, 167 Beilishilu, Xicheng District, 100037, Beijing, China.

Department of Magnetic Resonance Imaging, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, 167 Beilishilu, Xicheng District, 100037, Beijing, China.

出版信息

BMC Med. 2025 May 30;23(1):318. doi: 10.1186/s12916-025-04142-7.

Abstract

BACKGROUND

Left ventricular (LV) trabeculation are increasingly observed in patients with hypertrophic cardiomyopathy (HCM), but its clinical significance remains controversial. This study aims to clarify the characteristics of LV trabeculation and evaluate its prognostic value in HCM.

METHODS

We evaluated 1028 patients with HCM undergoing cardiac magnetic resonance. For each patient, thickness of compacted and trabeculated myocardium was measured at 16 segments of LV. The extent of LV trabeculation was expressed as the maximal trabeculation/compaction (T/C) ratio in any of 16 segments. The primary endpoint was major adverse cardiovascular events (MACEs). The secondary endpoints were heart failure, thromboembolic events, and ventricular arrhythmias. There were 689 patients undergoing whole-exome sequencing.

RESULTS

LV trabeculation predominantly located in midventricular-to-apical area, anterior, and lateral free walls in HCM. A greater extent of LV trabeculation was correlated with a higher prevalence of female, lower LV ejection fraction, and higher prevalence of extensive late gadolinium enhancement. During a median 4.8-year follow-up, a greater extent of LV trabeculation was associated with an increased risk of MACEs (adjusted hazard ratio [HR] 1.214, P = 0.005), heart failure (adjusted HR 1.372, P = 0.006), thromboembolic events (adjusted HR 1.242, P = 0.032), and ventricular arrhythmias (adjusted HR 1.240, P = 0.047). No gene was significantly associated with trabeculation at genome-wide level.

CONCLUSIONS

The distribution pattern of LV trabeculation was inhomogeneous and asymmetric in HCM. A greater extent of LV trabeculation was associated with poor prognosis. The progression of LV trabeculation might be the natural course of HCM.

摘要

背景

肥厚型心肌病(HCM)患者中左心室(LV)小梁形成越来越常见,但其临床意义仍存在争议。本研究旨在阐明LV小梁形成的特征并评估其在HCM中的预后价值。

方法

我们评估了1028例接受心脏磁共振成像的HCM患者。对于每位患者,在LV的16个节段测量致密心肌和小梁化心肌的厚度。LV小梁形成的程度用16个节段中任一节段的最大小梁化/致密化(T/C)比值表示。主要终点是主要不良心血管事件(MACE)。次要终点是心力衰竭、血栓栓塞事件和室性心律失常。有689例患者接受了全外显子组测序。

结果

HCM中LV小梁形成主要位于心室中部至心尖区域、前壁和外侧游离壁。LV小梁形成程度越高,女性患病率越高、LV射血分数越低、广泛延迟钆增强的患病率越高。在中位4.8年的随访期间,LV小梁形成程度越高,发生MACE的风险增加(校正风险比[HR]1.214,P = 0.005)、心力衰竭(校正HR 1.372,P = 0.006)、血栓栓塞事件(校正HR 1.242,P = 0.032)和室性心律失常(校正HR 1.240,P = 0.047)的风险增加。在全基因组水平上,没有基因与小梁形成显著相关。

结论

HCM中LV小梁形成的分布模式不均匀且不对称。LV小梁形成程度越高,预后越差。LV小梁形成的进展可能是HCM的自然病程。

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