Cardiac MRI-Derived Right Ventricular Strain for Risk Stratification in Pediatric and Adolescent Arrhythmogenic Cardiomyopathy

IF 19 1区医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS

JACC. Cardiovascular imaging Pub Date : 2026-10-01 Epub Date: 2026-07-18 DOI:10.1016/j.jcmg.2026.05.018

Wenqing Xu MD, Fen Sa PhD, Yanyan Song MD, Shujuan Yang PhD, Zhixiang Dong MD, Xingrui Chen PhD, Yun Tang PhD, Kaisaierjiang Aisikaier PhD, Xuan Ma MD, Xi Jia MD, Pengyu Zhou MD, Bo Li MD, Yujie Liu PhD, Kankan Zhao PhD, Kai Yang MD, Xiuyu Chen PhD, Lei Xu MD, Yun Peng MD, Sanjay K. Prasad MD, Hui Liu MD, Shihua Zhao MD, PhD

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Abstract

Background

Pediatric and adolescent arrhythmogenic cardiomyopathy (ACM) is a clinically severe phenotype that remains underrepresented in cardiac magnetic resonance (CMR) research. The prognostic value of CMR-derived right ventricular deformation analysis in this population is unclear.

Objectives

This study sought to describe the burden of severe cardiac outcomes in pediatric and adolescent ACM, and to assess the prognostic and incremental value of CMR-derived right ventricular global longitudinal strain (RVGLS) for risk stratification when incorporated into conventional clinical and CMR markers.

Methods

This multicenter study included consecutive pediatric and adolescent patients (≤21 years of age) with ACM who underwent baseline CMR evaluation. We assessed clinical, electrocardiographic, and CMR parameters according to the 2010 Task Force Criteria, along with biventricular strain parameters. Severe cardiac outcomes were defined as a composite of malignant ventricular arrhythmias (MVAs), heart transplantation, and heart failure–related death.

Results

The cohort included 102 patients (16.3 ± 3.4 years; 72% male) and 95 controls (mean age: 15.6 ± 3.3 years; 70% male). During a median of 58-month follow-up, 51 (50%) patients experienced severe cardiac outcomes and 28 (27.5%) MVA outcomes. Reduced RVGLS was independently associated with severe cardiac outcomes (HR: 1.14; P = 0.004) and MVA outcomes (subdistribution HR: 1.15; P < 0.001) after adjustment for clinical and conventional CMR parameters. Incorporation of RVGLS significantly improved prediction of severe cardiac outcomes beyond both right ventricular–based and left ventricular–based multivariable models, with significant improvement in discrimination and reclassification. Kaplan-Meier analysis further showed that RVGLS ≥−12.2% identified patients at higher risk of severe cardiac outcomes and MVA outcomes (both log-rank P < 0.001).

Conclusions

Pediatric and adolescent ACM manifests a malignant phenotype with high burden of heart failure and arrhythmia. RVGLS provides independent and incremental prognostic value beyond conventional CMR markers and could improve risk stratification in this high-risk population.

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心脏mri衍生的右心室应变对儿童和青少年心律失常性心肌病的风险分层：一项多中心队列研究。

背景：儿童和青少年心律失常性心肌病（ACM）是一种临床上严重的表型，在心脏磁共振（CMR）研究中仍未得到充分代表。cmr衍生的右心室变形分析在这一人群中的预后价值尚不清楚。目的：本研究旨在描述儿童和青少年ACM严重心脏结局的负担，并评估CMR衍生的右心室整体纵向应变（RVGLS）在纳入常规临床和CMR标志物时的预后和风险分层的增量价值。方法：该多中心研究纳入了连续的儿童和青少年（年龄≤21岁）ACM患者，他们接受了基线CMR评估。我们根据2010年工作组标准评估临床、心电图和CMR参数，以及双心室应变参数。严重心脏结局被定义为恶性室性心律失常（MVAs）、心脏移植和心力衰竭相关死亡的综合结果。结果纳入102例患者（16.3±3.4岁，男性72%）和95例对照组（平均年龄15.6±3.3岁，男性70%）。在中位58个月的随访期间，51例（50%）患者出现严重心脏结局，28例（27.5%）出现MVA结局。在调整临床和常规CMR参数后，RVGLS降低与严重心脏结局（HR: 1.14; P = 0.004）和MVA结局（亚分布HR: 1.15; P < 0.001）独立相关。RVGLS的结合显著提高了基于右心室和左心室的多变量模型对严重心脏结局的预测，在区分和重新分类方面有显著改善。Kaplan-Meier分析进一步显示，RVGLS≥-12.2%识别出严重心脏结局和MVA结局风险较高的患者（log-rank P均< 0.001）。结论儿童和青少年ACM表现为恶性表型，心衰和心律失常负担高。RVGLS在传统CMR标记物之外提供了独立和递增的预后价值，可以改善高危人群的风险分层。

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

JACC. Cardiovascular imaging CARDIAC & CARDIOVASCULAR SYSTEMS-RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING

CiteScore

24.90

自引率

5.70%

发文量

330

审稿时长

4-8 weeks

期刊介绍： JACC: Cardiovascular Imaging, part of the prestigious Journal of the American College of Cardiology (JACC) family, offers readers a comprehensive perspective on all aspects of cardiovascular imaging. This specialist journal covers original clinical research on both non-invasive and invasive imaging techniques, including echocardiography, CT, CMR, nuclear, optical imaging, and cine-angiography. JACC. Cardiovascular imaging highlights advances in basic science and molecular imaging that are expected to significantly impact clinical practice in the next decade. This influence encompasses improvements in diagnostic performance, enhanced understanding of the pathogenetic basis of diseases, and advancements in therapy. In addition to cutting-edge research,the content of JACC: Cardiovascular Imaging emphasizes practical aspects for the practicing cardiologist, including advocacy and practice management.The journal also features state-of-the-art reviews, ensuring a well-rounded and insightful resource for professionals in the field of cardiovascular imaging.