PMID- 25808629
OWN - NLM
STAT- MEDLINE
DCOM- 20160209
LR  - 20181113
IS  - 1619-7089 (Electronic)
IS  - 1619-7070 (Linking)
VI  - 42
IP  - 7
DP  - 2015 Jun
TI  - First experience of simultaneous PET/MRI for the early detection of cardiac 
      involvement in patients with Anderson-Fabry disease.
PG  - 1025-31
LID - 10.1007/s00259-015-3036-3 [doi]
AB  - PURPOSE: Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder 
      associated with severe multiorgan dysfunction and premature death. Early 
      diagnosis and treatment strategies play a key role in patient outcome. We 
      investigated the potential role of hybrid PET/MR imaging in the assessment of 
      early cardiac involvement in AFD patients. METHODS: Thirteen AFD patients without 
      cardiac symptoms and with normal left ventricular function underwent simultaneous 
      cardiac PET/MR imaging after administration of (18)F-FDG. Cardiac FDG uptake was 
      quantified by measuring the standardized uptake value in 17 myocardial segments 
      in each subject. The coefficient of variation (COV, i.e. the standard deviation 
      divided by the average) of the uptake of the 17 segments was calculated as an 
      index of heterogeneity in the heart. RESULTS: Six patients exhibited focal late 
      gadolinium enhancement (LGE) indicating intramyocardial fibrosis, and four of 
      these also had positive short inversion time inversion recovery (STIR) sequences. 
      All patients with LGE and positive STIR MR images showed focal FDG uptake in the 
      corresponding myocardial segments indicating inflammation. Of the seven patients 
      with negative LGE and STIR images, five showed homogeneous FDG cardiac uptake and 
      two showed heterogeneous FDG uptake. The COV was significantly greater in 
      patients with focal FDG uptake (0.25 +/- 0.02) than in those without (0.14 +/- 0.07, 
      p < 0.01). CONCLUSION: PET/MR imaging is clinically feasible for the early 
      detection of cardiac involvement in patients with AFD. Further studies evaluating 
      the role of hybrid PET/MR imaging in management of the disease in larger patient 
      populations are warranted.
FAU - Nappi, Carmela
AU  - Nappi C
AD  - Department of Advanced Biomedical Sciences, University Federico II, Naples, 
      Italy.
FAU - Altiero, Michele
AU  - Altiero M
FAU - Imbriaco, Massimo
AU  - Imbriaco M
FAU - Nicolai, Emanuele
AU  - Nicolai E
FAU - Giudice, Caterina Anna
AU  - Giudice CA
FAU - Aiello, Marco
AU  - Aiello M
FAU - Diomiaiuti, Claudio Tommaso
AU  - Diomiaiuti CT
FAU - Pisani, Antonio
AU  - Pisani A
FAU - Spinelli, Letizia
AU  - Spinelli L
FAU - Cuocolo, Alberto
AU  - Cuocolo A
LA  - eng
PT  - Journal Article
DEP - 20150326
PL  - Germany
TA  - Eur J Nucl Med Mol Imaging
JT  - European journal of nuclear medicine and molecular imaging
JID - 101140988
RN  - 0 (Radiopharmaceuticals)
RN  - 0Z5B2CJX4D (Fluorodeoxyglucose F18)
SB  - IM
MH  - Adult
MH  - Early Diagnosis
MH  - Fabry Disease/*diagnostic imaging
MH  - Female
MH  - Fibrosis/diagnostic imaging
MH  - Fluorodeoxyglucose F18
MH  - Heart Ventricles/*diagnostic imaging/pathology
MH  - Humans
MH  - *Magnetic Resonance Imaging
MH  - Male
MH  - Middle Aged
MH  - *Multimodal Imaging
MH  - *Positron-Emission Tomography
MH  - Radiopharmaceuticals
EDAT- 2015/03/27 06:00
MHDA- 2016/02/10 06:00
CRDT- 2015/03/27 06:00
PHST- 2014/12/10 00:00 [received]
PHST- 2015/03/05 00:00 [accepted]
PHST- 2015/03/27 06:00 [entrez]
PHST- 2015/03/27 06:00 [pubmed]
PHST- 2016/02/10 06:00 [medline]
AID - 10.1007/s00259-015-3036-3 [doi]
PST - ppublish
SO  - Eur J Nucl Med Mol Imaging. 2015 Jun;42(7):1025-31. doi: 
      10.1007/s00259-015-3036-3. Epub 2015 Mar 26.