Correlation Between Arrhythmia and the Prognosis in Children With EFE/LVNC/DCM

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.

Keywords: LVEF; Wolff–Parkinson–White syndrome; arrhythmia; cardiomyopathy; children; prognosis.

Figure 1

The trends of cTnI. In each group, cTnI was higher before treatment than after. It was recovered in all groups at 12 months of treatment point (Group 1, WPW-B+SVT/IVB/CLBBB; Group 2, AF/Af/AT; Group 3, PVC/VT; Group A, EFE with arrythmia; Group B, EFE without arrythmia).

Figure 2

The trends of hs-cTnT. In each group, the hs-cTnT level was higher before treatment, and was almost recovered after 12 months treatment.

Figure 3

The trends of NT pro-BNP. Before treatment, NT pro-BNP were significantly elevated in every group. After treatment, cardiac functions were improved, and NT pro-BNP was quickly decreased and remained at a low level except in Group 1.

Figure 4

The trends of LVEF. Before treatment, all patients had low LVEF. After treatment, LVEF in patients with ventricular unsynchronization in Group 1 and A remained at a low level.

Figure 5

The trends of ΔLVED. Before treatment, all patients with increased LVED (ΔLVED). After treatment, only patients with ventricular unsynchronization in Group 1 and A had higher ΔLVED.

Figure 6

By Kaplan–Meier analysis, EFE/LVNC/DCM patients with arrythmia of WPW-B/IVB/CLBBB showed a high risk of poor outcome. The hazard ratio for persistent LVEF of ≤35% or for event was 7.0 (95% CI, 5.6–28.9; p = 0.052).

Figure 7

By Kaplan–Meier analysis, EFE patients with arrythmia showed a high risk of poor outcome. The hazard ratio for persistent LVEF of ≤35% or for event was 20.6 (95% CI, 11.6–29.7; p = 0.002).

Figure 8

ECG showed WPW-B, left axis deviation in case one at her 6.5 years old.

Figure 9

Echo showed LVED 46.5 mm, LVEF 0.22 (A); endocardial thickness 3.5 mm (B) in case one at her 2 years old.

Figure 10

Echo showed LVED 64 mm, LVEF 0.24, endocardial thickness 3–5 mm in a 10 years old patient (about 6 years after pacemaker installed).