Key result
NYHA or Ross class III or IV linked to ~277% higher mortality in pediatric dilated cardiomyopathy.
Why the study?
Cardiomyopathy is a heterogeneous group of myocardial diseases in children, prompting this evaluation of their demographic features, clinical presentation, and prognosis.
Cohort (n=317)
No
Childhood cardiomyopathy, particularly restrictive cardiomyopathy, is associated with high mortality, and NYHA/Ross class III or IV is a strong predictor of adverse outcomes.
Answers are grounded in this paper and cite their sources.
May inform risk stratification in pediatric cardiomyopathy; hypothesis-generating for targeted interventions in restrictive subtypes.
Background: 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. Methods: 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. Results: 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- and ten- year survival rates were 75.5% and 60.1%, 67.3% and 56.1%, 74.1% and 57.2%, and 51.1% 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. Conclusions: This study demonstrates that CM, especially RCM, is related to a high incidence of death. NYHA/Ross class III or IV is a predictor of mortality in the patients and gene mutations may be a common cause.
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Yuan et al. (2023) conducted a cohort in Childhood cardiomyopathy (DCM, HCM, LVNC, RCM) (n=317). NYHA/Ross class III or IV vs. NYHA/Ross class I or II was evaluated on Mortality in dilated cardiomyopathy (HR 3.77, 95% CI 1.3-10.9, p=0.014). NYHA/Ross class III or IV was a significant predictor of mortality in children with dilated cardiomyopathy (HR 3.77) and restrictive cardiomyopathy (HR 8.65), which had the highest overall mortality.
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