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Longitudinal echocardiographic follow-up in children with congenital complete atrioventricular block
Gertie C M Beaufort-Krol1, Miek J M Schasfoort-van Leeuwen, Ymkje Stienstra
1Beatrix Children's Hospital, Division of Pediatric Cardiology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Groningen, The Netherlands.
Insights
Children with congenital complete atrioventricular block (CCAVB) experience left ventricular (LV) dilatation, which improves with physiologic pacing. This LV dilatation is linked to a lower heart rate (HR) but does not affect shortening fraction (SF).
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Echocardiography
Background:
- Congenital complete atrioventricular block (CCAVB) can lead to a low heart rate (HR), potentially causing increased left ventricular (LV) stroke volume.
- This may result in enlarged LV dimensions and, in some cases, dilated cardiomyopathy (DCM).
Purpose of the Study:
- To determine if children with CCAVB develop LV dilatation.
- To investigate the effect of pacing on LV dilatation in these children.
Main Methods:
- Longitudinal evaluation of echocardiographic data, including LVEDD and LVESD, in 36 children with CCAVB.
- Follow-up duration averaged 10.6 years, with initial assessments around 2.5 years of age.
Main Results:
- Children with CCAVB showed larger LVEDD and LVESD Z scores compared to controls, with scores inversely related to HR.
- LV dilatation progressed in children eligible for pacing but remained stable in non-paced individuals.
- Physiologic pacing led to a decrease in LVEDD and LVESD Z scores, while shortening fraction (SF) remained normal throughout.
Conclusions:
- Children with CCAVB exhibit LV dilatation, which is progressive if pacing is not initiated.
- Physiologic pacing effectively regresses LV dilatation.
- LV dilatation is more pronounced with lower HR, but SF is not compromised.
- DCM is an early finding and does not develop during childhood in this cohort.
Background:
Due to a low heart rate (HR) in children with congenital complete atrioventricular block (CCAVB), a larger stroke volume of the left ventricle (LV) may be expected. If so, end-diastolic (LVEDD) and end-systolic (LVESD) diameters may be enlarged and even dilated cardiomyopathy (DCM) may occur. The aim of this study was to answer the question if children with CCAVB develop LV dilatation. Furthermore, we investigated whether LV dilatation would decrease after pacing.
Methods:
We longitudinally evaluated echocardiographic data (LVEDD, LVESD, shortening fraction [SF]) in 36 children with CCAVB. Age at the first visit was 2.5 +/- 3.3 years (mean +/- SD); follow-up 10.6 +/- 7.3 years.
Results:
Three children had DCM, already at 1st visit. LVEDD and LVESD Z scores in all children with CCAVB were larger than in normal controls (LVEDD Z score 1.38 +/- 1.80; LVESD Z score 0.64 +/- 1.35). Both Z scores were larger when HR was lower. Both Z scores increased over time in children who met criteria for pacing, but did not change in non-paced children. Physiologic pacing decreased both Z scores. SF of all children was normal and remained normal during follow-up (0.39 +/- 0.05 1st visit vs 0.39 +/- 0.06 last visit).
Conclusions:
We conclude that children with CCAVB have LV dilatation, which is progressive in children who met criteria for pacing. LV dilatation regressed by physiologic pacing. LV dilatation was larger when HR was lower. SF does not deteriorate over time. DCM occurs early in the disease and does not develop during childhood, not even in children with LV dilatation.
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