Role of late gadolinium enhancement in the risk stratification of pediatric hypertrophic cardiomyopathy: based on a Chinese cohortResearch in context
Summary: 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...
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Elsevier
2025-05-01
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| Series: | The Lancet Regional Health. Western Pacific |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2666606525001105 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Xingrui Chen |
| collection | DOAJ |
| description | Summary: 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). |
| format | Article |
| id | doaj-art-99a1cb2fbd084072ac7dee8a37e448b5 |
| institution | Kabale University |
| issn | 2666-6065 |
| language | English |
| publishDate | 2025-05-01 |
| publisher | Elsevier |
| record_format | Article |
| series | The Lancet Regional Health. Western Pacific |
| spelling | doaj-art-99a1cb2fbd084072ac7dee8a37e448b52025-08-20T03:49:41ZengElsevierThe Lancet Regional Health. Western Pacific2666-60652025-05-015810157310.1016/j.lanwpc.2025.101573Role of late gadolinium enhancement in the risk stratification of pediatric hypertrophic cardiomyopathy: based on a Chinese cohortResearch in contextXingrui Chen0Wei Xiangli1Xuan Ma2Yun Tang3Jiaxin Wang4Zhixiang Dong5Kankan Zhao6Zhuxin Wei7Xi Jia8Pengyu Zhou9Yujie Liu10Yanyan Song11Chen Cui12Minjie Lu13Kai Yang14Xiuyu Chen15Shujuan Yang16Shihua Zhao17MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, ChinaMR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China; Department of Cardiovascular Surgery, The Second Affiliated Hospital of Xi’an Jiaotong University, Xi’an, ChinaMR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, ChinaMR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, ChinaDepartment of Radiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, ChinaMR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, ChinaSchool of Biomedical Engineering and Informatics, Nanjing Medical University, Nanjing, ChinaMR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, ChinaMR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, ChinaMR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, ChinaMR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, ChinaMR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, ChinaMR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, ChinaMR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, ChinaMR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, ChinaMR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, ChinaDepartment of Radiology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China; Corresponding author. Department of Radiology, Beijing Anzhen Hospital, Capital Medical University, No. 2 Anzhen Road, Chaoyang District, Beijing 100029, China.MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/National Center for Cardiovascular Diseases, Beijing, China; Corresponding author. MR Center, Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College/ National Center for Cardiovascular Diseases, Beilishi Road 167, Xicheng District, Beijing 100037, China.Summary: 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).http://www.sciencedirect.com/science/article/pii/S2666606525001105Hypertrophic cardiomyopathyPediatricsCardiac magnetic resonanceLate gadolinium enhancementPrognosis |
| spellingShingle | 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 Role of late gadolinium enhancement in the risk stratification of pediatric hypertrophic cardiomyopathy: based on a Chinese cohortResearch in context The Lancet Regional Health. Western Pacific Hypertrophic cardiomyopathy Pediatrics Cardiac magnetic resonance Late gadolinium enhancement Prognosis |
| title | Role of late gadolinium enhancement in the risk stratification of pediatric hypertrophic cardiomyopathy: based on a Chinese cohortResearch in context |
| title_full | Role of late gadolinium enhancement in the risk stratification of pediatric hypertrophic cardiomyopathy: based on a Chinese cohortResearch in context |
| title_fullStr | Role of late gadolinium enhancement in the risk stratification of pediatric hypertrophic cardiomyopathy: based on a Chinese cohortResearch in context |
| title_full_unstemmed | Role of late gadolinium enhancement in the risk stratification of pediatric hypertrophic cardiomyopathy: based on a Chinese cohortResearch in context |
| title_short | Role of late gadolinium enhancement in the risk stratification of pediatric hypertrophic cardiomyopathy: based on a Chinese cohortResearch in context |
| title_sort | role of late gadolinium enhancement in the risk stratification of pediatric hypertrophic cardiomyopathy based on a chinese cohortresearch in context |
| topic | Hypertrophic cardiomyopathy Pediatrics Cardiac magnetic resonance Late gadolinium enhancement Prognosis |
| url | http://www.sciencedirect.com/science/article/pii/S2666606525001105 |
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