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- Open Access
Reduced native right ventricular T1 in Anderson-Fabry disease as compared to patients with pulmonary hypertension
© Pagano et al.; licensee BioMed Central Ltd. 2014
- Published: 16 January 2014
- Pulmonary Hypertension
- Right Ventricle
- Late Gadolinium Enhancement
- Right Ventricle Wall
- Right Ventricle Hypertrophy
Recently, native (non-contrast) T1 mapping has demonstrated low left ventricular myocardial values in patients with Anderson-Fabry disease (FD)[1, 2], potentially due to accumulation of glycosphingolipids. Autopsy studies have shown biventricular involvement, however quantitative T1 imaging of the right ventricle (RV) has not been performed.
In images from 20 subjects with FD and 7 subjects with pulmonary hypertension (PH), the inferior RV wall was assessed for the appearance of hypertrophy, to identify subjects with sufficient wall thickness for T1 analysis. Images were acquired on 1.5T Siemens systems (Sonata and Avanto) using the SASHA T1 mapping method for all studies: 70° flip angle, 1.31 ms echo time, 2.62 ms repetition time, 9 images with 85-995 ms saturation recovery times plus a non-saturated image, 8 mm slice thickness, 360 × 270 FOV, 192 × 108 acquisition matrix before interpolation, and 75% phase resolution. Either rate 2 parallel imaging (GRAPPA) or 6/8 partial Fourier was used for image acceleration. Regions of interest were drawn on the septum and inferior RV by two observers, avoiding the septal insertion point, fat and blood pool. Analysis was repeated 10 times per subject to evaluate T1 variability with ROI placement. Late gadolinium enhancement (LGE) and diastolic regional wall thickness (WT), measured on bSSFP cine images, were assessed at a comparable slice location.
In FD patients with RV hypertrophy, both RV and LV native T1 values are reduced as compared to patients with PH and RV hypertrophy. Significant improvement in spatial resolution is required for T1 mapping of the normal right ventricle to establish healthy native RV T1 values.
The authors acknowledge financial support from CIHR, AIHS, WCHRI.
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