PMID- 21744250
OWN - NLM
STAT- MEDLINE
DCOM- 20120713
LR  - 20220331
IS  - 1826-6983 (Electronic)
IS  - 0033-8362 (Linking)
VI  - 117
IP  - 1
DP  - 2012 Feb
TI  - Role of cardiac MRI in evaluating patients with Anderson-Fabry disease: assessing 
      cardiac effects of long-term enzyme replacement therapy.
PG  - 19-28
LID - 10.1007/s11547-011-0710-9 [doi]
AB  - PURPOSE: Anderson-Fabry disease is a multisystemic disorder of lipid metabolism 
      secondary to X-chromosome alterations and is frequently associated with cardiac 
      manifestations such as left ventricular (LV) hypertrophy, gradually leading to an 
      alteration in cardiac performance. The purpose of this study was to monitor, 
      using magnetic resonance imaging (MRI), any changes produced by enzyme 
      replacement therapy with agalsidase beta at the cardiac level in patients with 
      Anderson-Fabry disease. MATERIALS AND METHODS: Sixteen (ten men, six women) 
      patients with genetically confirmed Anderson-Fabry disease underwent cardiac MRI 
      before starting enzyme replacement therapy (baseline study) and after 48 months 
      of treatment with agalsidase beta at the dose of 1 mg/kg (follow-up study). 
      RESULTS: After 48 months of treatment, a significant reduction in LV mass and 
      wall thickness was observed: 187+/-59 g vs. 149+/-44 g, and 16+/-3 mm vs. 13+/-3 mm, 
      respectively. A significant reduction in T2 relaxation time was noted at the 
      level of the interventricular septum (81+/-3 ms vs. 67+/-7 ms), at the apical level 
      (80+/-8 ms vs. 63+/-6 ms) and at the level of the lateral wall (82+/-8 ms vs. 63+/-10 ms) 
      (p<0.05). No significant variation was observed in ejection fraction between the 
      two studies (65+/-3% vs. 64+/-2%; p>0.05) (mean bias 1.0); however, an improvement 
      was noted in the New York Heart Association (NYHA) class of the majority of 
      patients (12/16) (p<0.05). CONCLUSIONS: In patients with Anderson-Fabry disease 
      undergoing enzyme replacement therapy with agalsidase beta, MRI documented a 
      significant reduction in myocardial T2 relaxation time, a significant decrease in 
      maximal myocardial thickness and in total LV mass. MRI did not reveal significant 
      improvements in LV global systolic function; however, improvement in NYHA 
      functional class was noted, consistent with improved diastolic function.
FAU - Messalli, G
AU  - Messalli G
AD  - Dipartimento di Scienze Biomorfologiche e Funzionali, Universita degli Studi 
      Federico II, Napoli, Italy.
FAU - Imbriaco, M
AU  - Imbriaco M
FAU - Avitabile, G
AU  - Avitabile G
FAU - Russo, R
AU  - Russo R
FAU - Iodice, D
AU  - Iodice D
FAU - Spinelli, L
AU  - Spinelli L
FAU - Dellegrottaglie, S
AU  - Dellegrottaglie S
FAU - Cademartiri, F
AU  - Cademartiri F
FAU - Salvatore, M
AU  - Salvatore M
FAU - Pisani, A
AU  - Pisani A
LA  - eng
LA  - ita
PT  - Journal Article
DEP - 20110709
PL  - Italy
TA  - Radiol Med
JT  - La Radiologia medica
JID - 0177625
RN  - 0 (Isoenzymes)
RN  - EC 3.2.1.22 (alpha-Galactosidase)
RN  - RZD65TSM9U (agalsidase beta)
SB  - IM
MH  - Adult
MH  - Enzyme Replacement Therapy/*methods
MH  - Fabry Disease/*drug therapy/*physiopathology
MH  - Female
MH  - Heart Diseases/*diagnosis/*physiopathology
MH  - Humans
MH  - Isoenzymes/*therapeutic use
MH  - Magnetic Resonance Imaging, Cine/*methods
MH  - Male
MH  - Risk Factors
MH  - Vectorcardiography
MH  - alpha-Galactosidase/*therapeutic use
EDAT- 2011/07/12 06:00
MHDA- 2012/07/14 06:00
CRDT- 2011/07/12 06:00
PHST- 2010/12/10 00:00 [received]
PHST- 2011/01/12 00:00 [accepted]
PHST- 2011/07/12 06:00 [entrez]
PHST- 2011/07/12 06:00 [pubmed]
PHST- 2012/07/14 06:00 [medline]
AID - 10.1007/s11547-011-0710-9 [doi]
PST - ppublish
SO  - Radiol Med. 2012 Feb;117(1):19-28. doi: 10.1007/s11547-011-0710-9. Epub 2011 Jul 
      9.