Abstract

Survival of children with single ventricle heart defects after the total cavopulmonary connection (TCPC) has improved, but impaired cardiac function remains a major cause of morbidity and mortality. Cardiac magnetic resonance imaging (cMRI) is the gold standard in assessing single ventricle volume and function, but high costs and limited availability hamper its routine use. A cheaper and more available alternative is echocardiography. Myocardial function can be studied in more detail using speckle tracking echocardiography (STE). The purpose of the study was to describe the association between myocardial deformation assessed by speckle tracking echocardiography (STE) and single ventricle function assessed by cMRI and to evaluate differences in myocardial deformation in children with single left and single right ventricular morphology. Cross-sectional, multicenter study in 77 children after TCPC was conducted. STE segmental and global longitudinal peak strain and systolic strain rate (SR) of the dominant ventricle were measured. Impaired SV function by cMRI was defined as ejection fraction (EF) < 45%. Mean age was 11.8 (range 9.7–14.3) years. Pearson R for cMRI EF versus global longitudinal strain and SR was − 0.25 (p = 0.06) and − 0.03 (p = 0.82), respectively. Global single ventricle longitudinal strain and SR was similar in patients after TCPC with single left and single right ventricular morphology (− 19.0 ± 3.1% vs 19.2 ± 3.2%, p = 0.94). STE myocardial deformation parameters do not correlate with single ventricle ejection fraction assessed by cMRI.

Highlights

  • The Fontan circulation is a palliative surgical strategy for children with congenital heart disease who cannot be offered a biventricular repair [1]

  • We found no correlation between longitudinal strain of the single ventricle assessed by echocardiography and single ventricle ejection fraction, single ventricle volume and cardiac index assessed by MRI

  • We found that speckle tracking echocardiography (STE)-derived global longitudinal myocardial deformation parameters were comparable in children with left ventricle (LV) and right ventricular (RV) morphology, which is in agreement with a relatively large study by Petko et al in children with a mean age of 7.8 years [9]

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Summary

Introduction

The Fontan circulation is a palliative surgical strategy for children with congenital heart disease who cannot be offered a biventricular repair [1]. Various, yet rather small studies in children with a TCPC, have been published, suggesting that measurement of myocardial deformation is feasible and reproducible, and that lower strain and SR values are measured in patients versus controls as well as in TCPC patients with single right ventricular (RV) versus single LV morphology [7,8,9]. It is unknown whether myocardial strain and SR parameters provide additional information on single ventricular function in patients with a TCPC assessed by cMRI

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