Impact of Neonatal Intervention on Left Ventricular Performance in Ebstein's Anomaly and Tricuspid Valve Dysplasia

Yozo Teramachi; Lisa K Hornberger; Lisa Howley; Mary E van der Velde; Mohammed Al-Aklabi; Luke G Eckersley

doi:10.1016/j.echo.2023.11.019

Impact of Neonatal Intervention on Left Ventricular Performance in Ebstein's Anomaly and Tricuspid Valve Dysplasia

J Am Soc Echocardiogr. 2024 Feb;37(2):248-254. doi: 10.1016/j.echo.2023.11.019. Epub 2023 Nov 25.

Authors

Yozo Teramachi¹, Lisa K Hornberger¹, Lisa Howley², Mary E van der Velde³, Mohammed Al-Aklabi¹, Luke G Eckersley⁴

Affiliations

¹ Fetal and Neonatal Cardiology Program, Division of Cardiology, Department of Pediatrics, Women's and Children Health Research Institute, Stollery Children's Hospital, University of Alberta, Edmonton, Alberta, Canada.
² Division of Cardiology, Department of Pediatrics, University of Colorado, Denver, Colorado.
³ Division of Pediatric Cardiology, Department of Pediatrics, University of Michigan, Ann Arbor, Michigan.
⁴ Fetal and Neonatal Cardiology Program, Division of Cardiology, Department of Pediatrics, Women's and Children Health Research Institute, Stollery Children's Hospital, University of Alberta, Edmonton, Alberta, Canada. Electronic address: leckersl@ualberta.ca.

PMID: 38013061
DOI: 10.1016/j.echo.2023.11.019

Abstract

Background: Severe neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are associated with high perinatal morbidity and mortality. The authors recently demonstrated left ventricular (LV) dysfunction and dyssynchrony to be prevalent in affected newborns and to contribute to poor outcomes. The aim of this study was to investigate the impact of patent ductus arteriosus (PDA) closure, spontaneous or surgical ligation, or right ventricular exclusion (Starnes procedure) on LV performance in neonatal EA and TVD.

Methods: Neonates with EA or TVD encountered from 2004 to 2018 at three institutions were identified. Pre- and postoperative LV function was assessed using two-dimensional, Doppler-derived deformation (six-segment vector velocity imaging) and two measures of mechanical dyssynchrony (the SD of time to peak and global dyssynchrony index), and values were compared using paired t test analysis or the Wilcoxon rank sum test.

Results: Before the intervention, LV function was impaired in the PDA (n = 18) and Starnes (n = 6) groups and was similar between groups. After PDA closure, LV performance did not change. After the Starnes procedure, however, LV function, including synchrony, improved significantly: fractional area change from 45 ± 5% to 58 ± 8% (P = .003), global circumferential strain from -18.2 ± 5.0% to -32.5 ± 5.5% (P = .01), cardiac index from 1.9 ± 0.3 to 3.9 ± 1.5 L/min/m² (P = .05), and circumferential strain dyssynchrony (dyssynchrony index from 0.19 ± 0.09 to 0.04 ± 0.02 [P = .009] and SD of time to peak from 59.8 ± 18.5 to 29.9 ± 8.2 [P = .02]).

Conclusion: The Starnes procedure results in early improvements in LV dysfunction and dyssynchrony, not observed after PDA closure in neonatal severe EA and TVD, which may benefit critically unwell neonates.

Keywords: Ebstein’s anomaly; Echocardiography; Left ventricular dyssynchrony; Left ventricular function; Starnes operation; Vector velocity imaging.

MeSH terms

Ebstein Anomaly* / complications
Ebstein Anomaly* / diagnosis
Ebstein Anomaly* / surgery
Female
Heart Defects, Congenital*
Heart Valve Diseases*
Humans
Infant, Newborn
Pregnancy
Tricuspid Valve / diagnostic imaging
Tricuspid Valve / surgery
Ventricular Dysfunction, Left* / diagnostic imaging
Ventricular Dysfunction, Left* / etiology