TY - JOUR
T1 - Hybrid positron emission tomography-magnetic resonance imaging for assessing different stages of cardiac impairment in patients with Anderson-Fabry disease
T2 - AFFINITY study group
AU - Imbriaco, Massimo
AU - Nappi, Carmela
AU - Ponsiglione, Andrea
AU - Pisani, Antonio
AU - Dell'Aversana, Serena
AU - Nicolai, Emanuele
AU - Spinelli, Letizia
AU - Aiello, Marco
AU - Diomiaiuti, Claudio Tommaso
AU - Riccio, Eleonora
AU - Esposito, Roberta
AU - Galderisi, Maurizio
AU - Losi, Mariangela
AU - Greiser, Andreas
AU - Chow, Kelvin
AU - Cuocolo, Alberto
PY - 2019/9/1
Y1 - 2019/9/1
N2 - Aims: Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder associated with multi-organ dysfunction. While native myocardial T1 mapping by magnetic resonance (MR) allow non-invasive measurement of myocyte sphingolipid accumulation, 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) and MR are able to identify different pathological patterns of disease progression. We investigated the relationship between T1 mapping and 18F-FDG uptake by hybrid PET-MR cardiac imaging in AFD female patients. Methods and results: Twenty AFD females without cardiac symptoms underwent cardiac PET-MR using 18F-FDG for glucose uptake. In all patients and in seven age- and sex-matched control subjects, T1 mapping was performed using native T1 Modified Look-Locker Inversion-recovery prototype sequences. 18F-FDG myocardial uptake was quantified by measuring the coefficient of variation (COV) of the standardized uptake value using a 17-segment model. T1 values of AFD patients were lower compared with control subjects (1236 ± 49 ms vs. 1334 ± 27 ms, P < 0.0001). Focal 18F-FDG uptake with COV >0.17 was detected in seven patients. COV was 0.32 ± 0.1 in patients with focal 18F-FDG uptake and 0.12 ± 0.04 in those without (P < 0.001). Patients with COV >0.17 had higher T1 values of lateral segments of the mid ventricular wall, compared with those with COV ≤0.17 (1216 ± 22 ms vs. 1160 ± 59 ms, P < 0.05). Conclusion: In females with AFD, focal 18F-FDG uptake with a trend towards a pseudo-normalization of abnormal T1 mapping values, may represent an intermediate stage before the development of myocardial fibrosis. These findings suggest a potential relationship between progressive myocyte sphingolipid accumulation and inflammation.
AB - Aims: Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder associated with multi-organ dysfunction. While native myocardial T1 mapping by magnetic resonance (MR) allow non-invasive measurement of myocyte sphingolipid accumulation, 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) and MR are able to identify different pathological patterns of disease progression. We investigated the relationship between T1 mapping and 18F-FDG uptake by hybrid PET-MR cardiac imaging in AFD female patients. Methods and results: Twenty AFD females without cardiac symptoms underwent cardiac PET-MR using 18F-FDG for glucose uptake. In all patients and in seven age- and sex-matched control subjects, T1 mapping was performed using native T1 Modified Look-Locker Inversion-recovery prototype sequences. 18F-FDG myocardial uptake was quantified by measuring the coefficient of variation (COV) of the standardized uptake value using a 17-segment model. T1 values of AFD patients were lower compared with control subjects (1236 ± 49 ms vs. 1334 ± 27 ms, P < 0.0001). Focal 18F-FDG uptake with COV >0.17 was detected in seven patients. COV was 0.32 ± 0.1 in patients with focal 18F-FDG uptake and 0.12 ± 0.04 in those without (P < 0.001). Patients with COV >0.17 had higher T1 values of lateral segments of the mid ventricular wall, compared with those with COV ≤0.17 (1216 ± 22 ms vs. 1160 ± 59 ms, P < 0.05). Conclusion: In females with AFD, focal 18F-FDG uptake with a trend towards a pseudo-normalization of abnormal T1 mapping values, may represent an intermediate stage before the development of myocardial fibrosis. These findings suggest a potential relationship between progressive myocyte sphingolipid accumulation and inflammation.
KW - Anderson-Fabry disease
KW - Cardiac
KW - hybrid PET-MR
KW - T1 mapping
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U2 - 10.1093/ehjci/jez039
DO - 10.1093/ehjci/jez039
M3 - Article
C2 - 30879055
AN - SCOPUS:85068889740
VL - 20
SP - 1004
EP - 1011
JO - European Heart Journal Cardiovascular Imaging
JF - European Heart Journal Cardiovascular Imaging
SN - 2047-2404
IS - 9
ER -