Clinical characteristics and mortality risk factors in pediatric hypertrophic, restrictive, and rapidly progressive hypertrophic cardiomyopathy: a retrospective cohort study with follow-up

Asad Nawaz, Zixian Sheng, Muhammad Junaid Akram, Jianjin Li, Lingjuan Liu, Yuxing Yuan, Jie Tian
National Clinical Research Center for Child Health and Disorders and Children’s Hospital of Chongqing Medical University.
China

Frontiers in Cardiovascular Medicine
Front Cardiovasc Med 2025; 15:
DOI: 10.3389/fcvm.2025.1541651

Abstract
Background: Pediatric cardiomyopathies are rare but life-threatening conditions with high mortality. Limited data exists on their clinical features and risk factors, especially in Asian populations, highlighting the need for further research in this area.
Methods: This retrospective cohort study analyzed data from 212 pediatric patients diagnosed with hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy (RCM), or restrictive phenotype hypertrophic cardiomyopathy (RP-HCM) at a single center in China from October 2012 to October 2023, with follow-up until October 31, 2024. Demographic, clinical, and diagnostic data, as well as follow-up outcomes, were reviewed. Logistic and Cox regression models identified risk factors for in-hospital and long-term mortality.
Results: Among the 212 patients, 79.72% (169/212) had HCM, 16.98% (36/212) had RCM, and 3.30% (7/212) had RP-HCM. Infection (75.47%, 160/212) and heart failure (51.42%, 109/212) were common comorbidities. In-hospital mortality was 5.19% (11/212), with follow-up mortality of 20.28% (43/212). The independent risk factors for mortality included left ventricular ejection fraction (LVEF), pulmonary hypertension, and low-density lipoprotein (LDL) levels (P < 0.05). Patients with RP-HCM showed the poorest outcomes, with a follow-up mortality rate of 42.86%. Only 10.4% (22/212) of patients underwent genetic testing, yet the positive detection rate was 63.7% (14/22).
Conclusions: This study underscores the importance of early diagnosis, genetic testing, and integrated management in pediatric cardiomyopathies. LVEF, pulmonary hypertension, and LDL levels are critical prognostic factors, offering insights for risk assessment and management in affected children.

Category
Class II. Pulmonary Hypertension Associated with Left Ventricular Systolic or Diastolic Dysfunction

Age Focus: Pediatric Pulmonary Vascular Disease

Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication

Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes

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