Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 Summer;26(1):101038.
doi: 10.1016/j.jocmr.2024.101038. Epub 2024 Mar 16.

Cardiovascular magnetic resonance reference values of right ventricular volumetric variables in patients with hypoplastic left heart syndrome

Andrik Ballenberger  1 , Amke Caliebe  2 , Sylvia Krupickova  3 , Anselm Uebing  4 , Dominik Daniel Gabbert  4 , Inga Voges  5
Affiliations

Cardiovascular magnetic resonance reference values of right ventricular volumetric variables in patients with hypoplastic left heart syndrome

Andrik Ballenberger et al. J Cardiovasc Magn Reson. 2024 Summer.

Abstract

Background: Cardiovascular magnetic resonance (CMR) has established itself as the gold standard for serial assessment of systemic right ventricular (RV) performance but due to the lack of standardized RV reference values for hypoplastic left heart syndrome (HLHS) patients, the interpretation of RV volumetric data in HLHS remains difficult. Therefore, this study aimed to close this gap by providing CMR reference values for the systemic RV in HLHS patients.

Methods: CMR scans of 160 children, adolescents, and young adults (age range 2.2-25.2 years, 106 males) with HLHS were retrospectively evaluated. All patients were studied following total cavopulmonary connection. Short-axis stacks were used to measure RV end-diastolic and end-systolic volumes (RVEDV, RVESV), RV stroke volume (RVSV), RV ejection fraction (RVEF), and RV end-diastolic myocardial mass (RVEDMM). Univariable and multiple linear regression analyses were performed to assess associations between RV parameters and demographic and anthropometric characteristics. Following the results of the regression analysis, reference graphs and tables were created with the Lambda-Mu-Sigma method.

Results: Multiple linear regression analysis showed strong associations between body height and RVEDV, RVESV as well as RVSV. Age was highly associated with RVEDMM. Therefore, percentile curves and tables were created with respect to body height (RVEDV, RVESV, RVSV) and age (RVEDMM). The influence of demographic and anthropometric parameters on RVEF was mild, thus no percentile curves and tables for RVEF are provided.

Conclusion: We were able to define CMR reference values for RV volumetric variables for HLHS patients. These data might be useful for the assessment and interpretation of CMR scans in these patients and for research in this field.

Keywords: Cardiovascular magnetic resonance; Children; Hypoplastic left heart syndrome; Reference values.

PubMed Disclaimer

Conflict of interest statement

Declaration of competing interests The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Figures

ga1
Graphical abstract
Fig. 1
Fig. 1
Assessment of ventricular volumes in HLHS patients without (A-C) and with (D-F) a visible left ventricle. HLHS, hypoplastic left heart syndrome
Fig. 2
Fig. 2
Barplot of the study population according to age and sex. y: years.
Fig. 3
Fig. 3
Percentile curves for RVEDV, RVESV, RVSV and RVEDMM with respect to body height or age. RVEDMM: right ventricular end-diastolic mass, RVEDV: right ventricular end-diastolic volume, RVESV: right ventricular end-systolic volume, RVSV: right ventricular stroke volume.
See this image and copyright information in PMC

References

    1. Feinstein J.A., Benson D.W., Dubin A.M., Cohen M.S., Maxey D.M., Mahle W.T., et al. Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol. 2012;59:S1–S42. doi: 10.1016/j.jacc.2011.09.022. - DOI - PMC - PubMed
    1. Best K.E., Miller N., Draper E., Tucker D., Luyt K., Rankin J. The improved prognosis of hypoplastic left heart: a population-based register study of 343 cases in England and Wales. Front Pedia. 2021;9 doi: 10.3389/fped.2021.635776. - DOI - PMC - PubMed
    1. Wald R.M., Mertens L.L. Hypoplastic left heart syndrome across the lifespan: clinical considerations for care of the fetus, child, and adult. Can J Cardiol. 2022;38:930–945. doi: 10.1016/j.cjca.2022.04.028. - DOI - PubMed
    1. Rychik J., Atz A.M., Celermajer D.S., Deal B.J., Gatzoulis M.A., Gewillig M.H., et al. Evaluation and management of the child and adult with fontan circulation: a scientific statement from the American Heart Association. Circulation. 2019;140:e234–e284. doi: 10.1161/cir.0000000000000696. Cir0000000000000696. - DOI - PubMed
    1. Kanngiesser L.M., Freitag-Wolf S., Boroni Grazioli S., Gabbert D.D., Hansen J.H., Uebing A.S., et al. Serial assessment of right ventricular deformation in patients with hypoplastic left heart syndrome: a cardiovascular magnetic resonance feature tracking study. J Am Heart Assoc. 2022;11 doi: 10.1161/jaha.122.025332. - DOI - PMC - PubMed

MeSH terms