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The planimetric Grothoff's criteria by cardiac magnetic resonance can improve the specificity of left ventricular non-compaction diagnosis in thalassemia intermedia

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Abstract

We differentiated the left ventricle non-compaction (LVNC) from hypertrabeculated myocardium due to a negative remodeling in thalassemia intermedia (TI) patients applying linear and planimetric criteria and comparing the cardiovascular magnetic resonance (CMR) findings. CMR images were analyzed in 181 TI patients enrolled in the Myocardial Iron Overload in Thalassemia Network and 27 patients with proved LVNC diagnosis. The CMR diagnostic criteria applied in TI patients were: a modified linear CMR Petersen’s criterion based on a more restrictive ratio of diastolic NC/C > 2.5 at segmental level and the combination of planimetric Grothoff's criteria (percentage of trabeculated LV myocardial mass LV–MM ≥ 25% of global LV mass and total LV–MMI NC ≥ 15 g/m2). Seventeen TI patients showed at least one positive NC/C segment. Compared to LVNC patients, these patients showed a lower frequency of segments with non-compaction areas (2.41 ± 1.33 vs 5.48 ± 2.26; P < 0.0001), significantly lower LV–MM NC percentage (10.99 ± 4.09 vs 28.20 ± 4.27%; P < 0.0001), LV–MMI (7.58 ± 4.86 vs 19.88 ± 5.02 g/m2; P < 0.0001) and extension of macroscopic fibrosis (0.44 ± 0.18 vs 4.65 ± 2.89; P = 0.004), and significantly higher LV ejection fraction (61.29 ± 5.17 vs 48.50 ± 17.55%; P = 0.016) and cardiac index (4.80 ± 1.49 vs 3.46 ± 1.11 l/min/m2; P = 0.002). No TI patient fulfilled the Grothoff's criteria. All TI patients with an NC/C ratio > 2.5 showed morphological and functional CMR parameters significantly different from the patients with a proved diagnosis of LVNC. Differentiation of LVNC from hypertrabeculated LV in β-TI patients due to a negative heart remodeling depends on the selected CMR criterion. We suggest using planimetric Grothoff's criteria to improve the specificity of LVNC diagnosis.

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Acknowledgements

We would like to thank all the colleagues involved in the MIOT project (https://miot.ftgm.it/). We thank Claudia Santarlasci for her skillful secretarial work. We finally thank all patients for their cooperation.

Funding

The MIOT project receives “no-profit support” from industrial sponsorships (Chiesi Farmaceutici S.p.A. and ApoPharma Inc.).

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Authors and Affiliations

  1. U.O.C. Risonanza Magnetica, Fondazione G. Monasterio CNR-Regione Toscana, Via Moruzzi 1, 56124, Pisa, Italy

    Francesca Macaione, Antonella Meloni, Vincenzo Positano, Laura Pistoia, Andrea Barison & Alessia Pepe

  2. Department of Cardiology, Policlinico “P. Giaccone”, Palermo, Italy

    Francesca Macaione, Giuseppina Novo & Salvatore Novo

  3. Department of Cardiology, Ospedale Civico, Palermo, Italy

    Daniele Di Lisi

  4. UOSD Centro per le Microcitemie, AORN Cardarelli, Napoli, Italy

    Anna Spasiano

  5. Unità Operativa Semplice Talassemia, Presidio Ospedaliero “Umberto I”, Siracusa, Italy

    Saveria Campisi

  6. U.O.C. Clinica Pediatrica Trasfusionale e Malattie Rare, P.O. Pediatrico Microcitemico “A. CAO”, Cagliari, Italy

    Alessandra Spiga

  7. U.O. di Diagnostica per immagini e Radiologia Interventistica, Ospedale del Delta, Lagosanto, Ferrara, Italy

    Riccardo Righi

Authors
  1. Francesca Macaione
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  2. Antonella Meloni
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  3. Vincenzo Positano
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  4. Laura Pistoia
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  5. Andrea Barison
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  6. Daniele Di Lisi
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  7. Anna Spasiano
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  8. Saveria Campisi
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  9. Alessandra Spiga
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  10. Riccardo Righi
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  11. Giuseppina Novo
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  12. Salvatore Novo
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  13. Alessia Pepe
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Corresponding author

Correspondence to Alessia Pepe.

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The authors do not have any conflict of interest to declare.

Ethical standards

This human study has been approved by the institutional ethics committee and has therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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All persons gave their informed consent prior to their inclusion in the study.

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Macaione, F., Meloni, A., Positano, V. et al. The planimetric Grothoff's criteria by cardiac magnetic resonance can improve the specificity of left ventricular non-compaction diagnosis in thalassemia intermedia. Int J Cardiovasc Imaging 36, 1105–1112 (2020). https://doi.org/10.1007/s10554-020-01797-6

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  • DOI: https://doi.org/10.1007/s10554-020-01797-6

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