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. 2022 Apr 28:10:833434.
doi: 10.3389/fped.2022.833434. eCollection 2022.

Clinical Profile and Risk Factors for Cardiac Death in Pediatric Patients With Primary Dilated Cardiomyopathy at a Tertiary Medical Center in China

Yan Wang  1 , Bo Han  1 , Youfei Fan  1 , Yingchun Yi  1 , Jianli Lv  1 , Jing Wang  1 , Xiaofei Yang  1 , Diandong Jiang  1 , Lijian Zhao  1 , Jianjun Zhang  1 , Hui Yuan  1
Affiliations

Clinical Profile and Risk Factors for Cardiac Death in Pediatric Patients With Primary Dilated Cardiomyopathy at a Tertiary Medical Center in China

Yan Wang et al. Front Pediatr. .

Abstract

Aim: We sought to identify the clinical characteristics and risk factors for cardiac mortality in pediatric patients with primary dilated cardiomyopathy (DCM) in China.

Methods: A total of 138 pediatric patients who were consecutively diagnosed with primary DCM from January 2011 to December 2020 were included. We assessed patients' clinical symptoms and performed laboratory examinations, electrocardiography, and echocardiography.

Results: Of these patients, 79 (57%) had severe systolic dysfunction (left ventricular ejection fraction of < 30%), 79 (57.2%) developed DCM before 12 months of age, 62 (45%) were male, 121 (87.7%) presented with advanced heart failure (cardiac functional class III/IV), and 54 (39.1%) presented with arrhythmia. At a median follow-up of 12 months, the overall cardiac mortality rate was 33%, and 40 of 46 deaths occurred within 6 months following DCM diagnosis. A multivariate Cox regression analysis identified several independent cardiac death predictors, including an age of 12 months to 5 years [hazard ratio (HR) 2.799; 95% confidence interval (CI) 1.160-6.758; P = 0.022] or 10-15 years (HR 3.617; 95% CI 1.336-9.788; P = 0.011) at diagnosis, an elevated serum alanine aminotransferase (ALT) concentration (≥ 51.5 U/L) (HR 2.219; 95% CI 1.06-4.574; P = 0.031), and use of mechanical ventilation (HR 4.223; 95% CI 1.763-10.114; P = 0.001).

Conclusion: The mortality rate of primary DCM without transplantation is high. Age, an elevated serum ALT concentration, and the need for mechanical ventilation predict mortality in patients with primary DCM, providing new insights into DCM risk stratification.

Keywords: dilated cardiomyopathy; heart failure; mortality; pediatric patient; risk factor.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

FIGURE 1
FIGURE 1
Prognostic value of alanine aminotransferase (ALT) in pediatric patients with DCM. ROC curves showing ALT levels in patients with DCM predicting the cardiac death. AUC, area under the curve; DCM, dilated cardiomyopathy; ROC, receiver operating characteristic.
FIGURE 2
FIGURE 2
The comparison of mortality according the follow-up time after diagnosis of DCM.
FIGURE 3
FIGURE 3
Survival rates from Kaplan–Meier estimates among the study population by sex (A) and age at diagnosis (B).
See this image and copyright information in PMC

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