The clinical profile, genetic basis and survival of childhood cardiomyopathy: a single-center retrospective study
The clinical profile, genetic basis and survival of childhood cardiomyopathy: a single-center retrospective study
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DOI：
10.1007/s00431-023-05358-6
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发表时间：
2024
期刊：
影响因子：
--
通讯作者：
Junjun Quan
中科院分区：
文献类型：
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作者：
Wenjing Yuan;Zhongli Jia;Jiajin Li;Lingjuan Liu;Jie Tian;Xupei Huang;Junjun Quan
Cardiomyopathy (CM) is a heterogeneous group of myocardial diseases in children. This study aimed to identify demographic features, clinical presentation and prognosis of children with CM. Clinical characteristics and prognostic factors associated with mortality were evaluated by Cox proportional hazards regression analyses. Genetic testing was also conducted on a portion of patients. Among the 317 patients, 40.1%, 25.2%, 24.6% and 10.1% were diagnosed with dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), left ventricular noncompaction cardiomyopathy (LVNC) and restrictive cardiomyopathy (RCM), respectively. The most common symptom observed was dyspnea (84.2%). Except for HCM, the majority of patients were classified as NYHA/Ross class III or IV. The five-year survival rates were 75.5%, 67.3%, 74.1% and 51.1% in DCM, HCM, LVNC and RCM, respectively. The ten-year survival rates were 60.1%, 56.1%, 57.2% and 41.3% in DCM, HCM, LVNC and RCM, respectively. Survival was inversely related to NYHA/Ross class III or IV in patients with DCM, HCM and RCM. Out of 42 patients, 32 were reported to carry gene mutations.
