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Right Ventricular Global Longitudinal Strain in Fetuses with Hypoplastic Left Heart Syndrome Does Not Differ Between Those With and Without Genetic Conditions

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Abstract

The presence of a genetic condition is a risk factor for increased mortality in hypoplastic left heart syndrome (HLHS). Speckle tracking strain analysis in interstage echocardiograms have shown promise in identifying patients with HLHS at increased risk of mortality. We hypothesized that fetuses with a genetic condition and HLHS have impaired right ventricular global longitudinal strain compared with fetuses with HLHS and no evident genetic condition. We performed a retrospective analysis of 60 patients diagnosed in fetal life with HLHS from 11/2015 to 11/2019. We evaluated presenting echocardiograms and calculated right ventricular global longitudinal strain (RV GLS) and fractional area of change (FAC) using post-processing software. We first compared RV GLS and FAC between those with genetic conditions to those without. We examined the secondary outcome of mortality among those with and without genetic conditions and among HLHS subgroups. Of the 60 patients with available genetic testing, 11 (18%) had an identified genetic condition. Neither RV GLS nor FAC was significantly different between patients with and without genetic conditions. There was no difference in RV GLS or FAC among HLHS phenotype or those who died or survived as infants. However, patients with a genetic syndrome had increased neonatal and overall mortality. In this cohort, RV GLS did not differ between those with and without a genetic diagnosis, among HLHS phenotypes, or between those surviving and dying as infants. Further analysis of strain throughout gestation and after birth could provide insight into the developing heart in fetuses with HLHS.

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Abbreviations

AA:

Aortic atresia

AS:

Aortic stenosis

CHD:

Congenital heart disease

CMA:

Chromosomal microarray

DICOM:

Digital Imaging and Communications in Medicine

FAC:

Fractional area of change

FISH:

Fluorescence in situ hybridization

FPS:

Frames per second

GA:

Gestational age

GLS:

Right ventricle global longitudinal strain

HLHS:

Hypoplastic left heart syndrome

LV:

Left ventricle

MA:

Mitral atresia

Mb:

Megabase

MS:

Mitral stenosis

PI:

Pulsatility index

Q1:

Quartile 1

Q3:

Quartile 3

RV:

Right ventricle

S/D:

Systolic/diastolic

SPSS:

Statistical Package for the Social Sciences

TR:

Tricuspid regurgitation

VUS:

Variance of unknown significance

WES:

Whole exome sequence

WRS:

Wilcoxon rank sum

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Authors and Affiliations

  1. Department of Pediatrics, Section of Pediatric Cardiology, Baylor College of Medicine/Texas Children’s Hospital, 6651 Main St, FE1920, Houston, TX, 77030, USA

    J. Kevin Wilkes, Tam T. Doan, Shaine A. Morris, Carolyn A. Altman, Nancy A. Ayres, Ricardo Pignatelli & Betul Yilmaz Furtun

  2. Texas Children’s Hospital Fetal Center, 6651 Main St, Suite 420, Houston, TX, 77030, USA

    Lacey Schoppe & Magnolia Nguyen

  3. Department of Pediatrics, Section of Pediatric Cardiology, Baylor College of Medicine/Texas Children’s Hospital, 6651 Main St, E1920, Houston, TX, 77030, USA

    J. Kevin Wilkes

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  1. J. Kevin Wilkes
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  2. Tam T. Doan
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Contributions

JKW, TTD, SAM, RP, and BYF participated in study design, data acquisition, data analysis and interpretation, and manuscript drafting and revision. LS, MN, CAA, and NAA participated in data acquisition and revision of the manuscript. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

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Correspondence to J. Kevin Wilkes.

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Wilkes, J.K., Doan, T.T., Morris, S.A. et al. Right Ventricular Global Longitudinal Strain in Fetuses with Hypoplastic Left Heart Syndrome Does Not Differ Between Those With and Without Genetic Conditions. Pediatr Cardiol 43, 655–664 (2022). https://doi.org/10.1007/s00246-021-02770-3

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  • DOI: https://doi.org/10.1007/s00246-021-02770-3

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