Key result
In male patients, papillary muscles and trabeculations contributed to a significantly greater percentage of left ventricular mass in those with Fabry disease compared to healthy controls (20% vs 13%, p=0.01).
Why the study?
Does including papillary muscles and trabeculations in CMR analysis improve the accuracy of left ventricular mass and volume assessment in patients with Fabry disease?
Cross-Sectional (n=40)
Yes
Does including papillary muscles and trabeculations in CMR analysis improve the accuracy of left ventricular mass and volume assessment in patients with Fabry disease?
Absolute Event Rate: 20% vs 13%
p-value: p=0.01
In Fabry disease, papillary muscles and trabeculations contribute disproportionately to left ventricular mass, making their inclusion in CMR analysis critical to avoid underestimation and misclassification.
May warrant including papillary muscles/trabeculations in Fabry CMR to reduce underestimation; leaves open effects on classification and outcomes.
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 (M inc P&T) or excluding (M ex P&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 (M inc P&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 (M inc P&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.
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Kozor et al. (2015) conducted a cross-sectional in Fabry disease (n=40). Fabry disease vs. Healthy controls was evaluated on Percentage contribution of papillary muscles and trabeculations to total left ventricular mass (p=0.01). In male patients, papillary muscles and trabeculations contributed to a significantly greater percentage of left ventricular mass in those with Fabry disease compared to healthy controls (20% vs 13%, p=0.01).
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