PMID- 31366357
OWN - NLM
STAT- MEDLINE
DCOM- 20200504
LR  - 20240117
IS  - 1532-429X (Electronic)
IS  - 1097-6647 (Print)
IS  - 1097-6647 (Linking)
VI  - 21
IP  - 1
DP  - 2019 Aug 1
TI  - Loss of base-to-apex circumferential strain gradient assessed by cardiovascular 
      magnetic resonance in Fabry disease: relationship to T1 mapping, late gadolinium 
      enhancement and hypertrophy.
PG  - 45
LID - 10.1186/s12968-019-0557-0 [doi]
LID - 45
AB  - BACKGROUND: Cardiac involvement is common and is the leading cause of mortality 
      in Fabry disease (FD). We explored the association between cardiovascular 
      magnetic resonance (CMR) myocardial strain, T1 mapping, late gadolinium 
      enhancement (LGE) and left ventricular hypertrophy (LVH) in patients with FD. 
      METHODS: In this prospective study, 38 FD patients (45.0 +/- 14.5 years, 37% male) 
      and 8 healthy controls (40.1 +/- 13.7 years, 63% male) underwent 3 T CMR including 
      cine balanced steady-state free precession (bSSFP), LGE and modified Look-Locker 
      Inversion recovery (MOLLI) T1 mapping. Global longitudinal (GLS) and 
      circumferential (GCS) strain and base-to-apex longitudinal strain (LS) and 
      circumferential strain (CS) gradients were derived from cine bSSFP images using 
      feature tracking analysis. RESULTS: Among FD patients, 8 had LVH (FD LVH+, 21%) 
      and 17 had LGE (FD LGE+, 45%). Nineteen FD patients (50%) had neither LVH nor LGE 
      (FD LVH- LGE-). None of the healthy controls had LVH or LGE. FD patients and 
      healthy controls did not differ significantly with respect to GLS (- 15.3 +/- 3.5% 
      vs. - 16.3 +/- 1.5%, p = 0.45), GCS (- 19.4 +/- 3.0% vs. -19.5 +/- 2.9%, p = 0.84) or 
      base-to-apex LS gradient (7.5 +/- 3.8% vs. 9.3 +/- 3.5%, p = 0.24). FD patients had 
      significantly lower base-to-apex CS gradient (2.1 +/- 3.7% vs. 6.5 +/- 2.2%, 
      p = 0.002) and native T1 (1170.2 +/- 37.5 ms vs. 1239.0 +/- 18.0 ms, p < 0.001). 
      Base-to-apex CS gradient differentiated FD LVH- LGE- patients from healthy 
      controls (OR 0.42, 95% CI: 0.20 to 0.86, p = 0.019), even after controlling for 
      native T1 (OR 0.24, 95% CI: 0.06 to 0.99, p = 0.049). In a nested logistic 
      regression model with native T1, model fit was significantly improved by the 
      addition of base-to-apex CS gradient (chi(2)(df = 1) = 11.04, p < 0.001). Intra- 
      and inter-observer agreement were moderate to good for myocardial strain 
      parameters: GLS (ICC 0.849 and 0.774, respectively), GCS (ICC 0.831 and 0.833, 
      respectively), and base-to-apex CS gradient (ICC 0.737 and 0.613, respectively). 
      CONCLUSIONS: CMR reproducibly identifies myocardial strain abnormalities in FD. 
      Loss of base-to-apex CS gradient may be an early marker of cardiac involvement in 
      FD, with independent and incremental value beyond native T1.
FAU - Mathur, Shobhit
AU  - Mathur S
AD  - Toronto Joint Department of Medical Imaging, Toronto General Hospital, University 
      of Toronto, 585 University Avenue, 1PMB-298, Toronto, ON, M5G 2N2, Canada.
FAU - Dreisbach, John G
AU  - Dreisbach JG
AD  - Toronto Joint Department of Medical Imaging, Toronto General Hospital, University 
      of Toronto, 585 University Avenue, 1PMB-298, Toronto, ON, M5G 2N2, Canada.
FAU - Karur, Gauri R
AU  - Karur GR
AD  - Toronto Joint Department of Medical Imaging, Toronto General Hospital, University 
      of Toronto, 585 University Avenue, 1PMB-298, Toronto, ON, M5G 2N2, Canada.
FAU - Iwanochko, Robert M
AU  - Iwanochko RM
AD  - Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, 
      University of Toronto, 585 University Ave, Toronto, ON, M5G 2N2, Canada.
FAU - Morel, Chantal F
AU  - Morel CF
AD  - Fred A. Litwin Centre in Genetic Medicine, University Health Network & Mount 
      Sinai Hospital, University of Toronto, 60 Murray St, Toronto, ON, M5T 3L9, 
      Canada.
FAU - Wasim, Syed
AU  - Wasim S
AD  - Fred A. Litwin Centre in Genetic Medicine, University Health Network & Mount 
      Sinai Hospital, University of Toronto, 60 Murray St, Toronto, ON, M5T 3L9, 
      Canada.
FAU - Nguyen, Elsie T
AU  - Nguyen ET
AD  - Toronto Joint Department of Medical Imaging, Toronto General Hospital, University 
      of Toronto, 585 University Avenue, 1PMB-298, Toronto, ON, M5G 2N2, Canada.
FAU - Wintersperger, Bernd J
AU  - Wintersperger BJ
AD  - Toronto Joint Department of Medical Imaging, Toronto General Hospital, University 
      of Toronto, 585 University Avenue, 1PMB-298, Toronto, ON, M5G 2N2, Canada.
FAU - Hanneman, Kate
AU  - Hanneman K
AUID- ORCID: 0000-0002-3077-2218
AD  - Toronto Joint Department of Medical Imaging, Toronto General Hospital, University 
      of Toronto, 585 University Avenue, 1PMB-298, Toronto, ON, M5G 2N2, Canada. 
      kate.hanneman@uhn.ca.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20190801
PL  - England
TA  - J Cardiovasc Magn Reson
JT  - Journal of cardiovascular magnetic resonance : official journal of the Society 
      for Cardiovascular Magnetic Resonance
JID - 9815616
RN  - 0 (Contrast Media)
RN  - 0 (Organometallic Compounds)
RN  - 1BJ477IO2L (gadobutrol)
RN  - Fabry Disease, Cardiac Variant
SB  - IM
MH  - Adult
MH  - Cardiomyopathies/*diagnosis/physiopathology
MH  - Case-Control Studies
MH  - Contrast Media/*administration & dosage
MH  - Fabry Disease/*diagnostic imaging/physiopathology
MH  - Female
MH  - Humans
MH  - Hypertrophy, Left Ventricular/*diagnosis/physiopathology
MH  - *Magnetic Resonance Imaging, Cine
MH  - Male
MH  - Middle Aged
MH  - *Myocardial Contraction
MH  - Observer Variation
MH  - Organometallic Compounds/*administration & dosage
MH  - Predictive Value of Tests
MH  - Prognosis
MH  - Prospective Studies
MH  - Reproducibility of Results
MH  - *Ventricular Function, Left
MH  - *Ventricular Remodeling
PMC - PMC6670217
OTO - NOTNLM
OT  - Cardiomyopathy
OT  - Cardiovascular magnetic resonance
OT  - Fabry disease
OT  - Magnetic resonance imaging
OT  - Myocardial strain
OT  - T1 mapping
COIS- Syed Wasim has received honoraria and other research support from Shire Pharma 
      Canada ULC and Sanofi Genzyme. Dr. Morel has received honoraria and research 
      grant from Shire Pharma Canada ULC and Sanofi Genzyme. Dr. Iwanochko has received 
      honoraria from Shire Pharma Canada ULC and Sanofi Genzyme. The other authors 
      report no conflicts of interest. The other authors declare that they have no 
      competing interests.
EDAT- 2019/08/02 06:00
MHDA- 2020/05/06 06:00
PMCR- 2019/08/01
CRDT- 2019/08/02 06:00
PHST- 2018/10/29 00:00 [received]
PHST- 2019/06/17 00:00 [accepted]
PHST- 2019/08/02 06:00 [entrez]
PHST- 2019/08/02 06:00 [pubmed]
PHST- 2020/05/06 06:00 [medline]
PHST- 2019/08/01 00:00 [pmc-release]
AID - S1097-6647(23)00218-1 [pii]
AID - 557 [pii]
AID - 10.1186/s12968-019-0557-0 [doi]
PST - epublish
SO  - J Cardiovasc Magn Reson. 2019 Aug 1;21(1):45. doi: 10.1186/s12968-019-0557-0.