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Negative Impact of the Left Ventricular Remnant Morphology on Systemic Right Ventricular Myocardial Deformation in Hypoplastic Left Heart Syndrome

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Abstract

Left ventricular (LV) morphology may affect right ventricular (RV) function before and after Fontan palliation in patients with hypoplastic left heart syndrome (HLHS). We sought to assess the potential impact of LV morphology on RV function in patients with HLHS using cardiac magnetic resonance (CMR) imaging. A retrospective analysis of available CMR scans from all patients with HLHS was performed. LV morphology was categorized as absent/slit-like or globular/miniaturized. Volumetric analysis was performed using manual disc-summation method on steady-state free precession (SSFP) stack obtained in short-axis orientation of the ventricles. 4-chamber and short-axis SSFP images were used to measure strain on a semi-automated feature-tracking (FT) module. Two sample t-test was used to compare the groups. A total of 48 CMR scans were analyzed. Of those, 12 patients had absent/slit-like and 36 had globular/miniaturized LV morphology. Averaged 4-chamber longitudinal RV strain was significantly higher for absent/slit-like (− 17.6 ± 4.7%) than globular/miniaturized (− 13.4 ± 3.5; P = 0.002). Averaged 4-chamber radial RV strain was also significantly higher for absent/slit-like (33.1 ± 14.9%) than globular/miniaturized (21.6 ± 7.1; P = 0.001). For globular/miniaturized LV morphology, the decreases of 4-chamber longitudinal and radial strains were mainly attributable to the septal basilar and septal mid-ventricular segments. No differences were found in short-axis RV global circumferential strain between the morphologic subtypes (absent/slit-like − 15.0 ± 6.5, globular/miniaturized − 15.7 ± 4.7; P = 0.68). Larger LV remnants, with globular/miniaturized LV morphology, demonstrated diminished strain in the septal base and mid-ventricle segments. Patients with globular/miniaturized LV morphology may benefit with closer monitoring and lower threshold to start heart failure medications. These results exemplify the utility of including both septal and regional deformation in systemic RV strain analysis.

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Acknowledgments

This study was supported and funded by the Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome. The members of Wanek Family Program for Hypoplastic Left Heart Syndrome Imaging Pipeline are Timothy J. Nelson, MD, PhD, Dawit T. Haile, MD, Angela R. Miller, RDCS, Sara E. Martineau, RDCS, Amanda L. Breuer, RDCS, Karen S. Miller, CCRP, Lori A. Riess, CCRP, Karen M. Cavanaugh, CCRP, Lucas Timmons, CCRP.

Funding

This study was funded by Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome.

Author information

Authors and Affiliations

  1. Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN, USA

    Alan P. Wang, Patrick W. O’Leary & M. Yasir Qureshi

  2. Division of Pediatric Cardiology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Angela M. Kelle, Chelsea L. Reece, Patrick W. O’Leary & M. Yasir Qureshi

  3. Division of Biostatistics, Mayo Clinic, Rochester, MN, USA

    Meredith Hyun

  4. Department of Radiology, Mayo Clinic, Rochester, MN, USA

    Phillip M. Young

  5. Department of Pediatrics, Division of Pediatric Cardiology, Penn State Hershey Medical Center, Hershey, PA, USA

    Angela M. Kelle

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  1. Alan P. Wang
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Wanek Family Program for Hypoplastic Left Heart Syndrome Imaging Pipeline

  • Timothy J. Nelson
  • , Dawit T. Haile
  • , Angela R. Miller
  • , Sara E. Martineau
  • , Amanda L. Breuer
  • , Karen S. Miller
  • , Lori A. Riess
  • , Karen M. Cavanaugh
  •  & Lucas Timmons

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Correspondence to M. Yasir Qureshi.

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Written informed consent was obtained from parents of patients less than 18 years of age or from the study participants who were 18 or greater of age.

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The members of Wanek Family Program for Hypoplastic Left Heart Syndrome Imaging Pipeline are listed in Acknowledgements section.

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Wang, A.P., Kelle, A.M., Hyun, M. et al. Negative Impact of the Left Ventricular Remnant Morphology on Systemic Right Ventricular Myocardial Deformation in Hypoplastic Left Heart Syndrome. Pediatr Cardiol 42, 278–288 (2021). https://doi.org/10.1007/s00246-020-02480-2

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