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Native T1 mapping in ATTR cardiac amyloidosis - comparison with AL cardiac amyloidosis - a 200 patient study

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Since new therapies are imminent which aim to treat ATTR amyloidosis the lack of a quantitative test represents a critical step for drug development... In cardiac AL amyloidosis, T1 has high diagnostic accuracy and tracks disease... We hypothesised similar results would occur for ATTR... We hypothesized that the native myocardial T1 would be elevated in ATTR amyloid; that T1 elevation would track the cardiac amyloid burden as measured by DPD grading; and that T1 elevation would be an early disease marker. 3 groups were studied: ATTR amyloid patients (n = 85; 70 male; age 73 ± 10); healthy mutations carriers (n = 8; 3 male; age 47 ± 6); and AL amyloid patients (n = 79; 55 male; age 62 ± 10)... These were compared with 52 healthy volunteers and 46 patients with hypertrophic cardiomyopathy (HCM)... ATTR patients and mutation carriers also underwent cardiac DPD scintigraphy... T1 was elevated in ATTR patients compared to HCM and normal subjects (1097 ± 43 ms vs 1026 ± 64 ms vs 967 ± 34 ms, both p < 0.0001)... In established cardiac ATTR amyloidosis, T1 elevation was not as high as in AL amyloidosis (AL 1130 ± 68 ms, p = 0.01) (Figure 1 and 2)... T1 correlated with cardiac amyloid burden as determined semi-quantitatively by DPD scintigraphy (p < 0.0001)... T1 was not elevated in mutation carriers (952 ± 35 ms) but was in isolated DPD grade 1 (n = 9, 1037 ± 60 ms, p = 0.001)... Native myocardial T1 mapping represents the first test able in ATTR amyloidosis to quantify the cardiac amyloid burden.

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Characteristic examples from CMR scans - CMR end-diastolic cine still (upper panel); ShMOLLI native T1 map (middle) and late gadolinium enhancement (LGE) images (lower) in (left to right) healthy volunteer, HCM, definite AL and definite ATTR patients. Note the markedly elevated myocardial T1 time in the AL cardiac amyloid patient and ATTR patient into the red range of the colour scale (the elevation is higher in AL, i.e. more red) compared to the normal control (green) and the patient with hypertrophic cardiomyopathy.
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Figure 1: Characteristic examples from CMR scans - CMR end-diastolic cine still (upper panel); ShMOLLI native T1 map (middle) and late gadolinium enhancement (LGE) images (lower) in (left to right) healthy volunteer, HCM, definite AL and definite ATTR patients. Note the markedly elevated myocardial T1 time in the AL cardiac amyloid patient and ATTR patient into the red range of the colour scale (the elevation is higher in AL, i.e. more red) compared to the normal control (green) and the patient with hypertrophic cardiomyopathy.

Mentions: T1 was elevated in ATTR patients compared to HCM and normal subjects (1097 ± 43 ms vs 1026 ± 64 ms vs 967 ± 34 ms, both p < 0.0001). In established cardiac ATTR amyloidosis, T1 elevation was not as high as in AL amyloidosis (AL 1130 ± 68 ms, p = 0.01) (Figure 1 and 2). Diagnostic performance was similar for AL and ATTR amyloid (vs HCM: AL AUC 0.84 (95%CI 0.76-0.92); ATTR 0.85 (0.77-0.92) P < 0.0001) (Figure 2). T1 correlated with cardiac amyloid burden as determined semi-quantitatively by DPD scintigraphy (p < 0.0001). T1 was not elevated in mutation carriers (952 ± 35 ms) but was in isolated DPD grade 1 (n = 9, 1037 ± 60 ms, p = 0.001).


Native T1 mapping in ATTR cardiac amyloidosis - comparison with AL cardiac amyloidosis - a 200 patient study
Characteristic examples from CMR scans - CMR end-diastolic cine still (upper panel); ShMOLLI native T1 map (middle) and late gadolinium enhancement (LGE) images (lower) in (left to right) healthy volunteer, HCM, definite AL and definite ATTR patients. Note the markedly elevated myocardial T1 time in the AL cardiac amyloid patient and ATTR patient into the red range of the colour scale (the elevation is higher in AL, i.e. more red) compared to the normal control (green) and the patient with hypertrophic cardiomyopathy.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4043793&req=5

Figure 1: Characteristic examples from CMR scans - CMR end-diastolic cine still (upper panel); ShMOLLI native T1 map (middle) and late gadolinium enhancement (LGE) images (lower) in (left to right) healthy volunteer, HCM, definite AL and definite ATTR patients. Note the markedly elevated myocardial T1 time in the AL cardiac amyloid patient and ATTR patient into the red range of the colour scale (the elevation is higher in AL, i.e. more red) compared to the normal control (green) and the patient with hypertrophic cardiomyopathy.
Mentions: T1 was elevated in ATTR patients compared to HCM and normal subjects (1097 ± 43 ms vs 1026 ± 64 ms vs 967 ± 34 ms, both p < 0.0001). In established cardiac ATTR amyloidosis, T1 elevation was not as high as in AL amyloidosis (AL 1130 ± 68 ms, p = 0.01) (Figure 1 and 2). Diagnostic performance was similar for AL and ATTR amyloid (vs HCM: AL AUC 0.84 (95%CI 0.76-0.92); ATTR 0.85 (0.77-0.92) P < 0.0001) (Figure 2). T1 correlated with cardiac amyloid burden as determined semi-quantitatively by DPD scintigraphy (p < 0.0001). T1 was not elevated in mutation carriers (952 ± 35 ms) but was in isolated DPD grade 1 (n = 9, 1037 ± 60 ms, p = 0.001).

View Article: PubMed Central - HTML

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Since new therapies are imminent which aim to treat ATTR amyloidosis the lack of a quantitative test represents a critical step for drug development... In cardiac AL amyloidosis, T1 has high diagnostic accuracy and tracks disease... We hypothesised similar results would occur for ATTR... We hypothesized that the native myocardial T1 would be elevated in ATTR amyloid; that T1 elevation would track the cardiac amyloid burden as measured by DPD grading; and that T1 elevation would be an early disease marker. 3 groups were studied: ATTR amyloid patients (n = 85; 70 male; age 73 ± 10); healthy mutations carriers (n = 8; 3 male; age 47 ± 6); and AL amyloid patients (n = 79; 55 male; age 62 ± 10)... These were compared with 52 healthy volunteers and 46 patients with hypertrophic cardiomyopathy (HCM)... ATTR patients and mutation carriers also underwent cardiac DPD scintigraphy... T1 was elevated in ATTR patients compared to HCM and normal subjects (1097 ± 43 ms vs 1026 ± 64 ms vs 967 ± 34 ms, both p < 0.0001)... In established cardiac ATTR amyloidosis, T1 elevation was not as high as in AL amyloidosis (AL 1130 ± 68 ms, p = 0.01) (Figure 1 and 2)... T1 correlated with cardiac amyloid burden as determined semi-quantitatively by DPD scintigraphy (p < 0.0001)... T1 was not elevated in mutation carriers (952 ± 35 ms) but was in isolated DPD grade 1 (n = 9, 1037 ± 60 ms, p = 0.001)... Native myocardial T1 mapping represents the first test able in ATTR amyloidosis to quantify the cardiac amyloid burden.

No MeSH data available.


Related in: MedlinePlus