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Peri-operative myocardial performance in infants with Down syndrome undergoing CHD repair
Lyudmyla Zakharchenko1,2, Afif El-Khuffash3,4, Eleanor J Molloy5,6,7
1Department of Paediatric Cardiology, Children's Health Ireland at Crumlin, Dublin, Ireland.
Insights
Infants with Down syndrome and congenital heart disease (CHD) show altered myocardial performance and higher right ventricular pressure peri-operatively. Down syndrome independently predicts longer ventilation, inotrope use, and intensive care stays.
Area of Science:
- Cardiology
- Pediatrics
- Genetics
Background:
- Infants with Down syndrome often have congenital heart disease (CHD).
- Understanding the peri-operative cardiac impact in these infants is crucial.
Purpose of the Study:
- To characterize the impact of Down syndrome on myocardial performance and loading conditions in infants with CHD.
- To compare these infants with controls matched for cardiac lesion and normal microarray.
Main Methods:
- Measured left ventricular global longitudinal strain, right ventricular free wall longitudinal strain, left ventricular end-systolic wall stress, and right ventricular systolic pressure.
- Compared infants with Down syndrome (n=55) to control infants (n=29) over the peri-operative period.
Main Results:
- Left ventricular global longitudinal strain showed some recovery in the Down syndrome group pre-discharge.
- Right ventricular longitudinal strain recovery was better in controls.
- The Down syndrome group had lower end-systolic wall stress and higher right ventricular systolic pressure.
- Down syndrome independently predicted longer ventilation, inotrope use, and intensive care stay.
Conclusions:
- Down syndrome significantly impacts left and right ventricular function peri-operatively.
- The findings highlight the additional myocardial performance burden associated with Down syndrome in infants with CHD.
Background:
We aimed to characterise the impact of Down syndrome on myocardial performance and loading conditions in infants with Down syndrome and CHD over the peri-operative period by comparing them with infants matched for cardiac lesion with a normal microarray.
Methods:
Left ventricular global longitudinal strain, right ventricular free wall longitudinal strain, left ventricular end-systolic wall stress, and right ventricular systolic pressure were measured in the two groups over the peri-operative period.
Results:
Fifty-five infants had a diagnosis of Down syndrome and these were compared with 29 control infants. Left ventricular global longitudinal strain decreased in both groups post-operatively with the Down syndrome group demonstrating some recovery pre-discharge (18 ± 3 versus 16 ± 3 %, p = 0.01). Right ventricular longitudinal strain significantly decreased in both groups post-operatively with the control group demonstrating better recovery by hospital discharge (14 ± 4 versus 18 ± 6 %, p < 0.01). End-systolic wall stress was lower and right ventricular systolic pressure was higher in the Down syndrome group throughout the study period (all p < 0.05). Down syndrome was an independent predictor of the duration of ventilation, post-operative use of inotropes, and intensive care stay. Right ventricular longitudinal strain was an independent predictor of duration of intensive care stay.
Conclusion:
This study demonstrates the difference between the two groups in relation to left and right ventricular function, particularly prior to discharge, and outlines the additional impact a diagnosis of Down syndrome has on myocardial performance during the peri-operative period.
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