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Echocardiographic scores for biventricular repair risk prediction of congenital heart disease with borderline left ventricle: a review

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Abstract

The aim of this review is to highlight the strengths and limitations of major echocardiographic biventricular repair (BVR) prediction models for borderline left ventricle (LV) in complex congenital heart disease (CHD). A systematic search in the National Library of Medicine for Medical Subject Headings and free text terms including echocardiography, CHD, and scores, was performed. The search was refined by adding keywords for critical aortic stenosis (AS), borderline LV, complex left ventricular outflow tract (LVOT) obstruction, hypoplastic left heart syndrome/complex (HLHS/HLHC), and unbalanced atrio-ventricular septal defects (uAVSD). Fifteen studies were selected for the final analysis. We outlined what echocardiographic scores for different types of complex CHD with diminutive LV are available. Scores for CHD with LVOT obstruction including critical AS, HLHS/HLHC, and aortic arch hypoplasia have been validated and implemented by several studies. Scores for uAVSD with right ventricle (RV) dominance have also been established and implemented, the first being the atrioventricular valve index (AVVI). In addition to AVII, both LV/RV inflow angle and LV inflow index have all been validated for the prediction of BVR. We conclude with a discussion of limitations in the development and validation of each of these scores, including retrospective design during score development, heterogeneity in echocardiographic parameters evaluated, variability in the definition of outcomes, differences in adopted surgical and Interventional strategies, and institutional differences. Furthermore, scores developed in the past two decades may have little clinical relevance now. In summary, we provide a review of echocardiographic scores for BVR in complex CHD with a diminutive LV that may serve as a guide for use in modern clinical practice.

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Authors and Affiliations

  1. Fondazione G. Monasterio CNR-Regione Toscana, Massa, Pisa, Italy

    Massimiliano Cantinotti, Pietro Marchese, Eliana Franchi & Nadia Assanta

  2. Institute of Clinical Physiology, Pisa, Italy

    Massimiliano Cantinotti

  3. Institute of Life Sciences, Scuola Superiore Sant’Anna, Pisa, Italy

    Pietro Marchese

  4. Adult and Pediatric Cardiac Surgery, Dept. Advanced Biomedical Sciences, University of Naples “Federico II”, 80131, Naples, Italy

    Raffaele Giordano

  5. Division of Pediatric Cardiology, Department of Pediatrics, Medical University of Graz, Graz, Austria

    Martin Koestenberger

  6. Blalock Taussig, Thomas Heart Center, Johns Hopkins Hospital, Baltimore, USA

    Vivek Jani & Shelby Kutty

  7. Children’s Heart Centre, Children’s Health Ireland at Crumlin, Dublin, Ireland

    Sophie Duignan & Colin Joseph McMahon

Authors
  1. Massimiliano Cantinotti
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  2. Pietro Marchese
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  4. Eliana Franchi
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  5. Nadia Assanta
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  6. Martin Koestenberger
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  7. Vivek Jani
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  8. Sophie Duignan
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  9. Shelby Kutty
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  10. Colin Joseph McMahon
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Contributions

Massimiliano Cantinotti, Pietro Marchese, and Raffaele Giordano conceived the study and wrote the draft. Eliana Franchi, Nadia Assanta, Martin Koestenberg, and Sophie Duignan performed the search, and Vivek Jani, Shelby Kutty, and Colin Joseph McMahoN validation and supervision.

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Correspondence to Raffaele Giordano.

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Cantinotti, M., Marchese, P., Giordano, R. et al. Echocardiographic scores for biventricular repair risk prediction of congenital heart disease with borderline left ventricle: a review. Heart Fail Rev 28, 63–76 (2023). https://doi.org/10.1007/s10741-022-10230-0

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