Role of late gadolinium enhancement in the risk stratification of pediatric hypertrophic cardiomyopathy: based on a Chinese cohort

IF 8.1 1区医学 Q1 HEALTH CARE SCIENCES & SERVICES

The Lancet Regional Health: Western Pacific Pub Date : 2025-05-01 DOI:10.1016/j.lanwpc.2025.101573

Xingrui Chen , Wei Xiangli , Xuan Ma , Yun Tang , Jiaxin Wang , Zhixiang Dong , Kankan Zhao , Zhuxin Wei , Xi Jia , Pengyu Zhou , Yujie Liu , Yanyan Song , Chen Cui , Minjie Lu , Kai Yang , Xiuyu Chen , Shujuan Yang , Shihua Zhao

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Abstract

Background

Recently, late gadolinium enhancement (LGE) has been identified as an important risk factor in pediatric hypertrophic cardiomyopathy (HCM). However, its prognostic significance in pediatric HCM remains to be fully validated, particularly in Asian population. This study aims to assess the prognostic value of LGE and explore its incremental utility in predicting sudden cardiac death (SCD) in pediatric HCM using data from a Chinese cohort.

Methods

231 primary HCM patients ≤18 years of age with cardiac magnetic resonance (CMR) were retrospectively and consecutively enrolled in a single center. The composite outcomes included SCD or equivalent events and heart failure-related events.

Findings

Of 231 patients (median age 15, IQR: 12–16), LGE was present in 195 (84.4%) with a median LGE extent of 4.7% (IQR: 2.0%–9.2%). During a median follow-up of 62 months (IQR: 39–85), 26 (11.3%) patients reached composite outcomes, and 13 (5.6%) patients experienced SCD events. Kaplan–Meier analysis showed a significantly increased risk of composite outcomes (log-rank P < 0.001) and SCD (log-rank P < 0.001) in the group with LGE extent ≥5%. In multivariable Cox analysis adjusted by clinical and imaging factors, LGE extent was independently associated with composite outcomes (adjusted HR: 1.15; P < 0.001) and SCD (adjusted HR: 1.11; P = 0.009). For SCD events, the addition of LGE extent could improve the model performance of HCM Risk-Kids model (C-statistics: 0.65 versus 0.79, P = 0.015) and PRIMaCY model (C-statistics: 0.62 versus 0.82, P = 0.002), respectively.

Interpretation

In Chinese pediatric HCM, LGE serves as a risk factor in predicting adverse outcomes and may enhance SCD risk stratification strategies.

Funding

This study was funded by the National Key Research and Development Program of China (2021YFF0501400 and 2021YFF0501404), the Key Project of the National Natural Science Foundation of China (82430066), and the Yunnan Province Science and Technology Platform and Talent Project (202305AF150033).

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晚期钆增强在儿童肥厚性心肌病风险分层中的作用：基于中国队列研究

最近，晚期钆增强（LGE）已被确定为儿童肥厚性心肌病（HCM）的重要危险因素。然而，其在儿童HCM中的预后意义仍有待充分验证，特别是在亚洲人群中。本研究旨在利用中国队列数据评估LGE的预后价值，并探讨其在预测儿童HCM的心源性猝死（SCD）中的增量效用。方法对231例18岁以下经心脏磁共振（CMR）检查的原发性HCM患者进行回顾性和连续研究。综合结果包括SCD或同等事件和心力衰竭相关事件。231例患者（中位年龄15岁，IQR: 12-16）中，有195例（84.4%）存在LGE，中位LGE程度为4.7% （IQR: 2.0%-9.2%）。在62个月的中位随访期间（IQR: 39-85）， 26例（11.3%）患者达到复合结局，13例（5.6%）患者出现SCD事件。Kaplan-Meier分析显示复合结局的风险显著增加(log-rank P <；0.001)和SCD (log-rank P <；0.001)， LGE程度≥5%组。在经临床和影像学因素校正的多变量Cox分析中，LGE程度与综合结果独立相关(校正HR: 1.15；P & lt;0.001)和SCD(调整后HR: 1.11；P = 0.009)。对于SCD事件，LGE程度的增加可以提高HCM Risk-Kids模型（C-statistics: 0.65 vs . 0.79, P = 0.015）和PRIMaCY模型（C-statistics: 0.62 vs . 0.82, P = 0.002）的模型性能。在中国儿童HCM中，LGE可作为预测不良结局的危险因素，并可增强SCD风险分层策略。本研究由国家重点研发计划项目（2021YFF0501400和2021YFF0501404）、国家自然科学基金重点项目（82430066）和云南省科技平台与人才项目（202305AF150033）资助。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

The Lancet Regional Health: Western Pacific Medicine-Pediatrics, Perinatology and Child Health

CiteScore

8.80

自引率

2.80%

发文量

305

审稿时长

11 weeks

期刊介绍： The Lancet Regional Health – Western Pacific, a gold open access journal, is an integral part of The Lancet's global initiative advocating for healthcare quality and access worldwide. It aims to advance clinical practice and health policy in the Western Pacific region, contributing to enhanced health outcomes. The journal publishes high-quality original research shedding light on clinical practice and health policy in the region. It also includes reviews, commentaries, and opinion pieces covering diverse regional health topics, such as infectious diseases, non-communicable diseases, child and adolescent health, maternal and reproductive health, aging health, mental health, the health workforce and systems, and health policy.