A disproportionate contribution of papillary muscles and trabeculations to total left ventricular mass makes choice of cardiovascular magnetic resonance analysis technique critical in Fabry disease

Kozor, Rebecca, Callaghan, Fraser, Tchan, Michel, Hamilton-Craig, Christian, Figtree, Gemma A and Grieve, Stuart M (2015) A disproportionate contribution of papillary muscles and trabeculations to total left ventricular mass makes choice of cardiovascular magnetic resonance analysis technique critical in Fabry disease. Journal of Cardiovascular Magnetic Resonance, 17 22: . doi:10.1186/s12968-015-0114-4


Author Kozor, Rebecca
Callaghan, Fraser
Tchan, Michel
Hamilton-Craig, Christian
Figtree, Gemma A
Grieve, Stuart M
Title A disproportionate contribution of papillary muscles and trabeculations to total left ventricular mass makes choice of cardiovascular magnetic resonance analysis technique critical in Fabry disease
Journal name Journal of Cardiovascular Magnetic Resonance   Check publisher's open access policy
ISSN 1532-429X
1097-6647
Publication date 2015-02-21
Year available 2015
Sub-type Article (original research)
DOI 10.1186/s12968-015-0114-4
Open Access Status DOI
Volume 17
Issue 22
Total pages 7
Place of publication London, United Kingdom
Publisher BioMed Central Ltd
Collection year 2016
Language eng
Formatted abstract
Background

Sphingolipid deposition in Fabry disease causes left ventricular (LV) hypertrophy, of which the accurate assessment is essential. Cardiovascular magnetic resonance (CMR) has been proposed as the gold standard. However, there is debate in the literature as to whether papillary muscles and trabeculations (P&T) should be included in LV mass (LVM).

Methods/results

We examined the accuracy of 2 CMR methods of assessing LVM and LV volumes, including (MincP&T) or excluding (MexP&T) P&T, in a cohort of Fabry disease subjects (n = 20) compared to a matched control group (n = 20). Significant differences between the two measurement methods were observed for LV end-diastolic volume, LV end-systolic volume, LVM, and LV ejection fraction (LVEF) in both groups. These differences were significantly greater in the Fabry group compared to controls, except for LVEF. P&T contributed to a greater percentage of LVM in Fabry subjects than controls (20 ± 1% vs 13 ± 2%, p = 0.01). In the control group, both volume-derived methods (MincP&T or MexP&T) provided accurate SV measurements compared with the internal reference of velocity-encoded aortic flow. In the Fabry group, inclusion of P&T (MincP&T) resulted in good concordance with phase contrast flow imaging (difference between flow and volume techniques: 1 ± 3 ml, p = 0.7).

Conclusion

The volumetric contribution of P&T in Fabry disease is markedly increased relative to healthy controls. Failure to account for this results in significant underestimation of LVM and results in misclassification of a proportion of subjects.

Keyword Fabry disease
Papillary muscles
Left ventricular hypertrophy
Cardiovascular magnetic resonance imaging
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Official 2016 Collection
School of Medicine Publications
 
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