Objectives To quantify myocardial fibrosis in Kawasaki disease (KD) patients with coronary artery aneurysms (CAAs) by MRI-derived extracellular volume fraction (ECV). Materials and methods This prospective study included KD with CAAs and healthy volunteers with cardiac MRI. The image analyses included CAA size and thrombus, late gadolinium enhancement (LGE), global and regional (basal, middle, apex) ECV values. ECV values of the control and KD subgroups were compared, and the independent predictive factors of ECV were explored. Results The analysis included 49 patients with large CAAs (mean age, 5.87 ± 2.18 [SD]; 40 (81.6%) males), 40 patients with medium (n = 20) and small (n = 20) CAAs (mean age, 5.34 ± 1.96 [SD]; 33 (82.5%) males), and 20 healthy volunteers (mean age, 6.22 ± 1.77 [SD]; 15 (75.0%) males). Patients with large CAAs are more prone to thrombosis than those with medium and small CAAs (p = 0.033). The global ECV value in patients with large CAAs, medium and small CAAs, and controls has a significant difference (34.37% ± 3.33 vs 33.64% ± 3.27 vs 32.23% ± 2.68, p = 0.032, p = 0.038, p = 0.006, respectively). The regional ECV value showed the same trend. There were significant differences between patients with and without coronary thrombosis and LGE (all p < 0.050). Multivariate analysis revealed that coronary artery thrombosis and LGE were independently correlated with the global ECV. Conclusion KD patients with CAAs have diffuse myocardial fibrosis, and the degree of myocardial fibrosis is more severe in patients with large CAAs and coronary thrombosis and LGE. Key Points <Emphasis Type="BoldItalic">Question</Emphasis> Highlights the unmet need for non-invasive fibrosis quantification in KD, as prior methods (e.g., LGE) lack sensitivity for diffuse fibrosis. <Emphasis Type="BoldItalic">Findings</Emphasis> ECV elevation (size-dependent) in CAAs, along with thrombosis and LGE, serves as an independent predictor of ECV. <Emphasis Type="BoldItalic">Clinical relevance</Emphasis> Emphasizes ECV’s role in risk stratification and guiding therapy (e.g., thromboprophylaxis), directly linking to patient outcomes. Graphical Abstract

Quantitative evaluation of myocardial fibrosis in pediatric patients with Kawasaki disease and coronary artery aneurysms by MRI-derived extracellular volume fraction

期刊论文
发表日期：

2025年11月17日

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Zhiming Yang,
Yanan Chen,
Quanli Shen,
Yan Zheng,
Guanke Cai,
Chenxi Hu,
Junpu Hu,
Kai Lu,
Yuanzheng Zheng,
Ai Shang,
Fei Shan,
Qiong Yao,
Xihong Hu

摘要

Objectives

To quantify myocardial fibrosis in Kawasaki disease (KD) patients with coronary artery aneurysms (CAAs) by MRI-derived extracellular volume fraction (ECV).

Materials and methods

This prospective study included KD with CAAs and healthy volunteers with cardiac MRI. The image analyses included CAA size and thrombus, late gadolinium enhancement (LGE), global and regional (basal, middle, apex) ECV values. ECV values of the control and KD subgroups were compared, and the independent predictive factors of ECV were explored.

Results

The analysis included 49 patients with large CAAs (mean age, 5.87 ± 2.18 [SD]; 40 (81.6%) males), 40 patients with medium (n = 20) and small (n = 20) CAAs (mean age, 5.34 ± 1.96 [SD]; 33 (82.5%) males), and 20 healthy volunteers (mean age, 6.22 ± 1.77 [SD]; 15 (75.0%) males). Patients with large CAAs are more prone to thrombosis than those with medium and small CAAs (p = 0.033). The global ECV value in patients with large CAAs, medium and small CAAs, and controls has a significant difference (34.37% ± 3.33 vs 33.64% ± 3.27 vs 32.23% ± 2.68, p = 0.032, p = 0.038, p = 0.006, respectively). The regional ECV value showed the same trend. There were significant differences between patients with and without coronary thrombosis and LGE (all p < 0.050). Multivariate analysis revealed that coronary artery thrombosis and LGE were independently correlated with the global ECV.

Conclusion

KD patients with CAAs have diffuse myocardial fibrosis, and the degree of myocardial fibrosis is more severe in patients with large CAAs and coronary thrombosis and LGE.

Key Points

Question Highlights the unmet need for non-invasive fibrosis quantification in KD, as prior methods (e.g., LGE) lack sensitivity for diffuse fibrosis.

Findings ECV elevation (size-dependent) in CAAs, along with thrombosis and LGE, serves as an independent predictor of ECV.

Clinical relevance Emphasizes ECV’s role in risk stratification and guiding therapy (e.g., thromboprophylaxis), directly linking to patient outcomes.

Graphical Abstract