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Decreased right ventricular longitudinal strain in children with hypoplastic left heart syndrome during staged repair and follow-up: does it have implications in clinically stable patients?

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Abstract

The principal aim of this study was to evaluate changes in systolic function in the single right ventricle (SRV), during progression of the same patient through the three stages of surgical repair for hypoplastic left heart syndrome and during a 5-year follow-up. We hypothesize that, SRV global longitudinal strain (GLS) will be low during 3 stages of repair even in stable patients. We retrospectively evaluated 140 echocardiograms in 20 patients with HLHS (ages 0–11.3 years), before and after 3 stages of surgical palliation. Five-year follow-up data were available in all 20 patients. Controls with structurally normal hearts and in the same age group were used for comparison. We utilized speckle-tracking imaging for assessment of SRV segmental and global longitudinal and circumferential strains, from previously acquired 4-chamber and mid-cavity short-axis views prior to and within 1–3 months of each surgical stage. Longitudinal strain (LS) remained low through all 3 stages of repair and during follow-up. The pre-Fontan stage demonstrated significant interstage improvement compared to the post-Glenn stage despite similar volume status. Global LS was (− 15.6 ± 4.5% after Fontan surgery and remained similar (− 15.32 ± 3.2%) 5 years later. The SRV also showed increased dominance of circumferential strain compared to the normal RV, where the longitudinal deformation was dominant. In SRV, longitudinal strain may be a useful clinical index for evaluating both segmental and global function in an objective manner. Due to lack of significant clinical deterioration over a 10-year period, we speculate that a “lower-than-normal” longitudinal strain may be used as an objective measure of SRV function in clinically stable patients, particularly after the Fontan operation. Compensatory mechanisms where the longitudinal pattern of contraction switches to a more circumferential pattern, may play a role in asymptomatic patients with HLHS.

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Abbreviations

RV:

Right ventricle

LV:

Left ventricle

HLHS:

Hypoplastic left ventricle

DICOM:

Digital imaging and communications in medicine

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Funding

This study has not received any funding. However, Dr. Mercer-Rosa was supported by a K01HL125521 Grant.

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Authors and Affiliations

  1. Division of Cardiology, The Children’s Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, 34th and Civic Center Boulevard, Philadelphia, PA, 19104, USA

    Roshan D’Souza, Yan Wang, Renzo J. C. Calderon-Anyosa, Andrea E. Montero, Maalika M. Banerjee, Omoni Ekhomu, Daisuke Matsubara, Laura Mercer-Rosa, Tomoyuki Sato & Anirban Banerjee

  2. Tufts University School of Medicine, Boston, MA, 02116, USA

    Maalika M. Banerjee

  3. Dept. of Pediatrics and Adolescent Medicine and Dept. of Clinical Medicine, Aarhus University Hospital, Aarhus, Denmark

    Peter Agger

Authors
  1. Roshan D’Souza
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  2. Yan Wang
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  3. Renzo J. C. Calderon-Anyosa
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  4. Andrea E. Montero
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  5. Maalika M. Banerjee
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  7. Daisuke Matsubara
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  8. Laura Mercer-Rosa
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  9. Peter Agger
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  10. Tomoyuki Sato
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  11. Anirban Banerjee
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Corresponding author

Correspondence to Anirban Banerjee.

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All authors declare that he/she has no conflict of interest.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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This study was approved by the IRB of The Children’s Hospital of Philadelphia. Informed consent was waived as this was a retrospective study.

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D’Souza, R., Wang, Y., Calderon-Anyosa, R.J.C. et al. Decreased right ventricular longitudinal strain in children with hypoplastic left heart syndrome during staged repair and follow-up: does it have implications in clinically stable patients?. Int J Cardiovasc Imaging 36, 1667–1677 (2020). https://doi.org/10.1007/s10554-020-01870-0

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  • DOI: https://doi.org/10.1007/s10554-020-01870-0

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