PMID- 31660802
OWN - NLM
STAT- MEDLINE
DCOM- 20200420
LR  - 20200420
IS  - 1527-1315 (Electronic)
IS  - 0033-8419 (Linking)
VI  - 294
IP  - 1
DP  - 2020 Jan
TI  - Left Ventricular Hypertrophy and Late Gadolinium Enhancement at Cardiac MRI Are 
      Associated with Adverse Cardiac Events in Fabry Disease.
PG  - 42-49
LID - 10.1148/radiol.2019191385 [doi]
AB  - Background Cardiac involvement is the leading cause of mortality in patients with 
      Fabry disease. Identification of imaging findings that predict adverse cardiac 
      events is needed to enable identification of high-risk patients. Purpose To 
      establish the prognostic value of cardiac MRI findings in men and women with 
      Fabry disease. Materials and Methods Consecutive women and men with gene-positive 
      Fabry disease who had undergone cardiac MRI at a single large tertiary referral 
      hospital between March 2008 and January 2019 were included in this retrospective 
      cohort study. Evaluators of cardiac MRI studies were blinded to all clinical 
      information. Adverse cardiac events were assessed as a composite end point, 
      defined as ventricular tachycardia, bradycardia requiring device implantation, 
      severe heart failure, and cardiac death. Statistical analysis included Cox 
      proportional hazard models adjusted for age and Mainz Severity Score Index (a 
      measure of the severity of Fabry disease). Results Ninety patients (mean age, 44 
      years +/- 15 [standard deviation]; 59 women) were evaluated. After a median 
      follow-up period of 3.6 years, the composite end point was reached in 21 patients 
      (incidence rate, 7.6% per year). Left ventricular hypertrophy (LVH) and late 
      gadolinium enhancement (LGE) were independent predictors of the composite end 
      point in adjusted analysis (LVH hazard ratio [HR], 3.0; 95% confidence interval 
      [CI]: 1.1, 8.1; P = .03; and LGE HR, 7.2; 95% CI: 1.5, 34; P = .01). Patients 
      with extensive LGE (>/=15% of left ventricular mass) were at highest risk (HR, 12; 
      95% CI: 2.0, 67; P = .006). Sex did not modify the relationship between the 
      composite end point and any of the cardiac MRI parameters, including LVH (P = .15 
      for interaction term) and LGE (P = .38 for interaction term). Conclusion Cardiac 
      MRI findings of left ventricular hypertrophy and late gadolinium enhancement can 
      be used to identify patients with Fabry disease who are at high risk of adverse 
      cardiac events. (c) RSNA, 2019 See also the editorial by Zimmerman in this issue.
FAU - Hanneman, Kate
AU  - Hanneman K
AUID- ORCID: 0000-0002-3077-2218
AD  - From the Toronto Joint Department of Medical Imaging, Toronto General Hospital, 
      Peter Munk Cardiac Centre, University Health Network, University of Toronto, 585 
      University Ave, 1 PMB-298, Toronto, ON, Canada M5G 2N2 (K.H., G.R.K., R.M.W.); 
      Fred A. Litwin Centre in Genetic Medicine, University Health Network & Mount 
      Sinai Hospital, University of Toronto, Toronto, Canada (S.W., C.F.M.); and 
      Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, 
      University of Toronto, Toronto, Canada (R.M.W., R.M.I.).
FAU - Karur, Gauri R
AU  - Karur GR
AD  - From the Toronto Joint Department of Medical Imaging, Toronto General Hospital, 
      Peter Munk Cardiac Centre, University Health Network, University of Toronto, 585 
      University Ave, 1 PMB-298, Toronto, ON, Canada M5G 2N2 (K.H., G.R.K., R.M.W.); 
      Fred A. Litwin Centre in Genetic Medicine, University Health Network & Mount 
      Sinai Hospital, University of Toronto, Toronto, Canada (S.W., C.F.M.); and 
      Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, 
      University of Toronto, Toronto, Canada (R.M.W., R.M.I.).
FAU - Wasim, Syed
AU  - Wasim S
AD  - From the Toronto Joint Department of Medical Imaging, Toronto General Hospital, 
      Peter Munk Cardiac Centre, University Health Network, University of Toronto, 585 
      University Ave, 1 PMB-298, Toronto, ON, Canada M5G 2N2 (K.H., G.R.K., R.M.W.); 
      Fred A. Litwin Centre in Genetic Medicine, University Health Network & Mount 
      Sinai Hospital, University of Toronto, Toronto, Canada (S.W., C.F.M.); and 
      Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, 
      University of Toronto, Toronto, Canada (R.M.W., R.M.I.).
FAU - Wald, Rachel M
AU  - Wald RM
AD  - From the Toronto Joint Department of Medical Imaging, Toronto General Hospital, 
      Peter Munk Cardiac Centre, University Health Network, University of Toronto, 585 
      University Ave, 1 PMB-298, Toronto, ON, Canada M5G 2N2 (K.H., G.R.K., R.M.W.); 
      Fred A. Litwin Centre in Genetic Medicine, University Health Network & Mount 
      Sinai Hospital, University of Toronto, Toronto, Canada (S.W., C.F.M.); and 
      Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, 
      University of Toronto, Toronto, Canada (R.M.W., R.M.I.).
FAU - Iwanochko, Robert M
AU  - Iwanochko RM
AUID- ORCID: 0000-0001-7455-4469
AD  - From the Toronto Joint Department of Medical Imaging, Toronto General Hospital, 
      Peter Munk Cardiac Centre, University Health Network, University of Toronto, 585 
      University Ave, 1 PMB-298, Toronto, ON, Canada M5G 2N2 (K.H., G.R.K., R.M.W.); 
      Fred A. Litwin Centre in Genetic Medicine, University Health Network & Mount 
      Sinai Hospital, University of Toronto, Toronto, Canada (S.W., C.F.M.); and 
      Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, 
      University of Toronto, Toronto, Canada (R.M.W., R.M.I.).
FAU - Morel, Chantal F
AU  - Morel CF
AUID- ORCID: 0000-0001-9159-2053
AD  - From the Toronto Joint Department of Medical Imaging, Toronto General Hospital, 
      Peter Munk Cardiac Centre, University Health Network, University of Toronto, 585 
      University Ave, 1 PMB-298, Toronto, ON, Canada M5G 2N2 (K.H., G.R.K., R.M.W.); 
      Fred A. Litwin Centre in Genetic Medicine, University Health Network & Mount 
      Sinai Hospital, University of Toronto, Toronto, Canada (S.W., C.F.M.); and 
      Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, 
      University of Toronto, Toronto, Canada (R.M.W., R.M.I.).
LA  - eng
PT  - Journal Article
DEP - 20191029
PL  - United States
TA  - Radiology
JT  - Radiology
JID - 0401260
RN  - 0 (Contrast Media)
RN  - AU0V1LM3JT (Gadolinium)
SB  - IM
CIN - Radiology. 2020 Jan;294(1):50-51. PMID: 31661361
CIN - Radiology. 2020 Aug;296(2):E123. PMID: 32396044
MH  - Adult
MH  - Cohort Studies
MH  - Contrast Media/pharmacokinetics
MH  - Fabry Disease/*complications
MH  - Female
MH  - Follow-Up Studies
MH  - Gadolinium/*pharmacokinetics
MH  - Heart/diagnostic imaging
MH  - Heart Failure/*complications
MH  - Humans
MH  - Hypertrophy, Left Ventricular/*complications/*diagnostic imaging
MH  - Image Enhancement/*methods
MH  - Magnetic Resonance Imaging/*methods
MH  - Male
MH  - Middle Aged
MH  - Retrospective Studies
MH  - Risk Factors
EDAT- 2019/10/30 06:00
MHDA- 2020/04/21 06:00
CRDT- 2019/10/30 06:00
PHST- 2019/10/30 06:00 [pubmed]
PHST- 2020/04/21 06:00 [medline]
PHST- 2019/10/30 06:00 [entrez]
AID - 10.1148/radiol.2019191385 [doi]
PST - ppublish
SO  - Radiology. 2020 Jan;294(1):42-49. doi: 10.1148/radiol.2019191385. Epub 2019 Oct 
      29.