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Reduced native right ventricular T 1 in Anderson-Fabry disease as compared to patients with pulmonary hypertension

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Recently, native (non-contrast) T1 mapping has demonstrated low left ventricular myocardial values in patients with Anderson-Fabry disease (FD), potentially due to accumulation of glycosphingolipids... 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... Figure 2 shows T1 values for all subjects, demonstrating lower myocardial T1 in both the LV and RV for all FD subjects (p = 0.0143 for either ventricles)... Interobserver agreement (coefficient of variation) was 4.0% and 2.41% for the RV and septum, respectively... No FD subjects were positive for LGE in the inferior RV or septum; however one was positive at the RV insertion point... No PH subjects were positive for LGE in the inferior wall; however 2 showed mid-wall septal enhancement at locations within ROIs and all were positive at the RV insertion site... However, as indicated in Figure 2, septal T1 values are similar in those positive (1340.7 & 1249.7 ms) and or negative (1459.4 & 1215.0 ms) for LGE... 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.

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Myocardial T1 values by ventricle and condition. Circles and line segments denote values for a given individual, and * marks cases of septal late gadolinium enhancement.
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Figure 2: Myocardial T1 values by ventricle and condition. Circles and line segments denote values for a given individual, and * marks cases of septal late gadolinium enhancement.

Mentions: Analysis was performed in 5 patients with FD and 4 patients with PH (WT(LV-FD) = 11.7 mm, WT(RV-FD) = 5.7 mm, WT(LV-PH) = 10.8 mm, WT(RV-PH) = 7.5 mm), as the remaining cases had inadequate hypertrophy and spatial resolution to proceed with analysis. Sample SASHA images are shown in Figure 1. Figure 2 shows T1 values for all subjects, demonstrating lower myocardial T1 in both the LV and RV for all FD subjects (p = 0.0143 for either ventricles). Interobserver agreement (coefficient of variation) was 4.0% and 2.41% for the RV and septum, respectively. No FD subjects were positive for LGE in the inferior RV or septum; however one was positive at the RV insertion point. No PH subjects were positive for LGE in the inferior wall; however 2 showed mid-wall septal enhancement at locations within ROIs and all were positive at the RV insertion site. However, as indicated in Figure 2, septal T1 values are similar in those positive (1340.7 & 1249.7 ms) and or negative (1459.4 & 1215.0 ms) for LGE.


Reduced native right ventricular T 1 in Anderson-Fabry disease as compared to patients with pulmonary hypertension
Myocardial T1 values by ventricle and condition. Circles and line segments denote values for a given individual, and * marks cases of septal late gadolinium enhancement.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4042516&req=5

Figure 2: Myocardial T1 values by ventricle and condition. Circles and line segments denote values for a given individual, and * marks cases of septal late gadolinium enhancement.
Mentions: Analysis was performed in 5 patients with FD and 4 patients with PH (WT(LV-FD) = 11.7 mm, WT(RV-FD) = 5.7 mm, WT(LV-PH) = 10.8 mm, WT(RV-PH) = 7.5 mm), as the remaining cases had inadequate hypertrophy and spatial resolution to proceed with analysis. Sample SASHA images are shown in Figure 1. Figure 2 shows T1 values for all subjects, demonstrating lower myocardial T1 in both the LV and RV for all FD subjects (p = 0.0143 for either ventricles). Interobserver agreement (coefficient of variation) was 4.0% and 2.41% for the RV and septum, respectively. No FD subjects were positive for LGE in the inferior RV or septum; however one was positive at the RV insertion point. No PH subjects were positive for LGE in the inferior wall; however 2 showed mid-wall septal enhancement at locations within ROIs and all were positive at the RV insertion site. However, as indicated in Figure 2, septal T1 values are similar in those positive (1340.7 & 1249.7 ms) and or negative (1459.4 & 1215.0 ms) for LGE.

View Article: PubMed Central - HTML

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Recently, native (non-contrast) T1 mapping has demonstrated low left ventricular myocardial values in patients with Anderson-Fabry disease (FD), potentially due to accumulation of glycosphingolipids... 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... Figure 2 shows T1 values for all subjects, demonstrating lower myocardial T1 in both the LV and RV for all FD subjects (p = 0.0143 for either ventricles)... Interobserver agreement (coefficient of variation) was 4.0% and 2.41% for the RV and septum, respectively... No FD subjects were positive for LGE in the inferior RV or septum; however one was positive at the RV insertion point... No PH subjects were positive for LGE in the inferior wall; however 2 showed mid-wall septal enhancement at locations within ROIs and all were positive at the RV insertion site... However, as indicated in Figure 2, septal T1 values are similar in those positive (1340.7 & 1249.7 ms) and or negative (1459.4 & 1215.0 ms) for LGE... 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.

No MeSH data available.


Related in: MedlinePlus