Editor:
Dr O’Brien and colleagues should be commended for their important study published in the June 2020 issue of Radiology: Cardiothoracic Imaging which provides novel data highlighting the potential differences in measurements of left ventricular mass (LVM) between transthoracic echocardiography (TTE) and cardiac MRI among patients with Fabry disease (1). The authors conclude that TTE overestimates LVM compared with cardiac MRI. We suggest, however, that the decision not to include papillary muscles in cardiac MRI measurement of LVM will have underestimated the true LVM and significantly contributed to the discrepancy between imaging modalities.
The authors of the current study appear to have used the same methodology as the UK Biobank project, on which their recent publication of normal reference ranges was based. This used ellipsoid “smoothed” contouring of the compacted endocardial border and excluded both papillary muscles and trabeculae from LVM (2). This technique may offer improved reproducibility but sacrifices accuracy (closeness of the measured LVM to the true value). Our opinion is that papillary muscles are myocardial tissue and should routinely be excluded from blood volumes and included in LVM using “detailed” contouring. Use of smoothed contouring can lead to errors in the measurement of derived left ventricular parameters—our data in patients with hypertrophic cardiomyopathy suggest that using smoothed contours misses hyperdynamic function in the presence of large papillary muscles and extensive trabeculations (3). The choice of myocardial contouring technique has potentially even greater importance in phenocopies such as Fabry disease; papillary muscle hypertrophy is an early diagnostic sign and contributes disproportionately to the overall LVM (4).
This issue needs national and international guidance. It is notable that the authors’ analysis conforms with updated 2019 Society for Cardiovascular Magnetic Resonance guidelines (5). These still do not specify whether papillary muscles should be routinely included in measurement of LVM (and excluded from volumes), nor stipulate that any one normal reference range should be adopted, suggesting instead that the reference range is aligned with the reporting technique.
We would be interested to learn from the current authors if intermodality differences in LVM measurements persisted if detailed cardiac MRI contouring was used in this cohort of patients with Fabry disease.
Footnotes
Disclosures of Conflicts of Interest: W.E.M. Activities related to the present article: disclosed no relevant relationships. Activities not related to the present article: author received consultancy fees from Pfizer, Alnylam Pharmaceuticals, and Akcea Therapeutics. Other relationships: disclosed no relevant relationships R.V. Activities related to the present article: disclosed no relevant relationships. Activities not related to the present article: author is consultant for Amicus Therapeutics; author received travel accommodations to WORLD Congress 2018 from Amicus Therapeutics; author receives consultancy fees from Takeda. Other relationships: disclosed no relevant relationships. R.P.S. Activities related to the present article: disclosed no relevant relationships. Activities not related to the present article: author is consultant for Freeline Therapeutics; institution has grant from Takeda Shire for separate project in Fabry disease; author receives payment for educational lecture from Amicus; author receives payment for development of educational presentations from Amicus. Other relationships: disclosed no relevant relationships.
References
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