Abstract
Aim: To explore the correlation between different phenotypes of arrhythmia and the prognosis in children with EFE/LVNC/DCM.Methods: A total of 167 children with cardiomyopathy diagnosed and treated in Shengjing Hospital between January 2010 and May 2019 were evaluated. After patient screening, 31 patients with endomyocardial fibroelastosis (EFE), left ventricular non-compaction, or dilated cardiomyopathy with significant arrhythmias were selected. In addition, 42 children with primary EFE were selected to evaluate the prognosis with or without arrhythmia. Follow-up was undertaken 0, 1, 3, 6, 9, and 12 months after treatment.Results: We revealed the outcomes for five types of cardiomyopathy: EFE patients with Wolff–Parkinson–White syndrome B and supraventricular tachycardia, intraventricular block and complete left bundle branch block recovered slower than EFE patients with atrial flutter and atrial fibrillation, even slower than EFE with ventricular tachycardia. The average recovering time for LVEF and LVED in EFE patients without arrythmia was 10 months after diagnosis, while 76.9% (3/13 cases) of those with significant arrythmia hadn't recovered until 24 months after diagnosis. Three of patients died at 6, 7, and 6 and half years after diagnosis.Conclusion: The long-term prognosis in children with cardiomyopathy is associated with the type of arrhythmia and time of intervention. The prognosis of EFE patients with arrhythmia is worse than EFE patients without arrhythmia. Patients with Wolff–Parkinson–White syndrome B, especially a significantly widen QRS complex, carry a poor prognosis if radiofrequency ablation is not undertaken. CLBBB patients have similar poor prognosis if proper pacemaker is not implanted timely.
Highlights
Endomyocardial fibroelastosis (EFE), left ventricular non-compaction (LVNC), and dilated cardiomyopathy (DCM) are common phenotypes of cardiomyopathy in children
The cardiac troponin I (cTnI) level in each group was higher before treatment than after one, while the cTnI level in Group 2 was higher than Group 1 and 3, the differences were not significant (Table 1)
The high sensitivity cardiac troponin T (hs-cTnT) level in each group was higher before treatment than after one, but the difference was not significant
Summary
Endomyocardial fibroelastosis (EFE), left ventricular non-compaction (LVNC), and dilated cardiomyopathy (DCM) are common phenotypes of cardiomyopathy in children. EFE, LVNC, and DCM in children, are usually accompanied with supraventricular tachycardia (SVT), atrial fibrillation (Af), atrial flutter (AF), VT, frequent premature ventricular contraction (PVC) and even involvement of the conduction system [e.g., inter-ventricular conduction block (IVB) and complete left bundle branch block (CLBBB)]. Most of these patients are diagnosed at
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