PMID- 30257686
OWN - NLM
STAT- MEDLINE
DCOM- 20190314
LR  - 20240117
IS  - 1532-429X (Electronic)
IS  - 1097-6647 (Print)
IS  - 1097-6647 (Linking)
VI  - 20
IP  - 1
DP  - 2018 Sep 27
TI  - Cardiovascular magnetic resonance evidence of myocardial fibrosis and its 
      clinical significance in adolescent and adult patients with Ebstein's anomaly.
PG  - 69
LID - 10.1186/s12968-018-0488-1 [doi]
LID - 69
AB  - BACKGROUND: Myocardial fibrosis is a common pathophysiological process that is 
      related to ventricular remodeling in congenital heart disease. However, the 
      presence, characteristics, and clinical significance of myocardial fibrosis in 
      Ebstein's anomaly have not been fully investigated. This study aimed to evaluate 
      myocardial fibrosis using cardiovascular magnetic resonance (CMR) late gadolinium 
      enhancement (LGE) and T1 mapping techniques, and to explore the significance of 
      myocardial fibrosis in adolescent and adult patients with Ebstein's anomaly. 
      METHODS: Forty-four consecutive patients with unrepaired Ebstein's anomaly 
      (34.0 +/- 16.2 years; 18 males), and an equal number of age- and gender-matched 
      controls, were included. A comprehensive CMR protocol consisted of cine, LGE, and 
      T1 mapping by modified Look-Locker inversion recovery (MOLLI) sequences were 
      performed. Ventricular functional parameters, native T1, extracellular volume 
      (ECV), and LGE were analyzed. Associations between myocardial fibrosis and 
      disease severity, ventricular function, and NYHA classification were analyzed. 
      RESULTS: LGE was found in 10 (22.7%) patients. Typical LGE in Ebstein's anomaly 
      was located in the endocardium of the septum within the right ventricle (RV). The 
      LV ECV of Ebstein's anomaly were significantly higher than those of the controls 
      (30.0 +/- 3.8% vs. 25.3 +/- 2.3%, P < 0.001). An increased ECV was found to be 
      independent of the existence of LGE. Positive LGE or higher ECV (>/=30%) was 
      associated with larger fRV volume, aRV volume, increased disease severity, and 
      worse NYHA functional class. In addition, ECV was significantly correlated with 
      the LV ejection fraction (P <  0.001). CONCLUSIONS: Both focal and diffuse 
      myocardial fibrosis were observed in adolescent and adult patients with Ebstein's 
      anomaly. Increased diffuse fibrosis is associated with worse LV function, 
      increased Ebstein's severity, and worse clinical status.
FAU - Yang, Dan
AU  - Yang D
AD  - Department of Cardiology, West China Hospital, Sichuan University, Chengdu, 
      610041, Sichuan Province, China.
FAU - Li, Xiao
AU  - Li X
AD  - Department of Cardiovascular Surgery/Pediatric Heart Center, West China Hospital, 
      Sichuan University, Chengdu, 610041, Sichuan Province, China.
FAU - Sun, Jia-Yu
AU  - Sun JY
AD  - Department of Radiology, West China Hospital, Sichuan University, Chengdu, 
      610041, Sichuan Province, China.
FAU - Cheng, Wei
AU  - Cheng W
AD  - Department of Radiology, West China Hospital, Sichuan University, Chengdu, 
      610041, Sichuan Province, China.
FAU - Greiser, Andreas
AU  - Greiser A
AD  - Siemens Healthcare GmbH, Erlangen, Germany.
FAU - Zhang, Tian-Jing
AU  - Zhang TJ
AD  - Northeast Asia MR Collaboration, Siemens Healthcare, Beijing, China.
FAU - Liu, Hong
AU  - Liu H
AD  - Department of Cardiology, West China Hospital, Sichuan University, Chengdu, 
      610041, Sichuan Province, China.
FAU - Wan, Ke
AU  - Wan K
AD  - Department of Cardiology, West China Hospital, Sichuan University, Chengdu, 
      610041, Sichuan Province, China.
FAU - Luo, Yong
AU  - Luo Y
AD  - Department of Cardiology, West China Hospital, Sichuan University, Chengdu, 
      610041, Sichuan Province, China.
FAU - An, Qi
AU  - An Q
AD  - Department of Cardiovascular Surgery/Pediatric Heart Center, West China Hospital, 
      Sichuan University, Chengdu, 610041, Sichuan Province, China.
FAU - Chung, Yiu-Cho
AU  - Chung YC
AD  - Paul C. Lauterbur Research Centre for Biomedical Imaging, Shenzhen Key Laboratory 
      for MRI, Shenzhen Institutes of Advanced Technology, Chinese Academy of Sciences, 
      Shenzhen, Guangdong, China.
FAU - Han, Yuchi
AU  - Han Y
AD  - Department of Medicine (Cardiovascular Division), University of Pennsylvania, 
      Philadelphia, PA, USA.
FAU - Chen, Yu-Cheng
AU  - Chen YC
AUID- ORCID: 0000-0002-1771-4769
AD  - Department of Cardiology, West China Hospital, Sichuan University, Chengdu, 
      610041, Sichuan Province, China. chenyucheng2003@126.com.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20180927
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  - K2I13DR72L (Gadolinium DTPA)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Age Factors
MH  - Case-Control Studies
MH  - Child
MH  - China/epidemiology
MH  - Contrast Media/administration & dosage
MH  - Cross-Sectional Studies
MH  - Disease Progression
MH  - Ebstein Anomaly/*diagnostic imaging/epidemiology/pathology/physiopathology
MH  - Female
MH  - Fibrosis
MH  - Gadolinium DTPA/administration & dosage
MH  - Humans
MH  - *Magnetic Resonance Imaging, Cine
MH  - Male
MH  - Myocardium/*pathology
MH  - Predictive Value of Tests
MH  - Prevalence
MH  - Prognosis
MH  - Registries
MH  - Risk Factors
MH  - Severity of Illness Index
MH  - Stroke Volume
MH  - Ventricular Dysfunction, Left/*diagnostic 
      imaging/epidemiology/pathology/physiopathology
MH  - *Ventricular Function, Left
MH  - *Ventricular Remodeling
MH  - Young Adult
PMC - PMC6158838
OTO - NOTNLM
OT  - Cardiovascular magnetic resonance imaging
OT  - Ebstein's anomaly
OT  - Extracellular volume
OT  - Late gadolinium enhancement
OT  - Left ventricular function
OT  - Myocardial fibrosis
COIS- ETHICS APPROVAL AND CONSENT TO PARTICIPATE: The study was approved by the 
      Clinical Trial and Biological Ethics Committee of Sichuan University, and all 
      patients provided written informed consent. The study of the healthy subjects was 
      approved by the Clinical Trial and Biological Ethics Committee of Sichuan 
      University, and all healthy subjects provided written informed consent. CONSENT 
      FOR PUBLICATION: Written informed consent was obtained from the patient (or 
      guardian) for publication of their individual details and accompanying images in 
      this manuscript. The consent form is held by the authors/the authors' institution 
      and is available for review by the Editor-in-Chief. COMPETING INTERESTS: Dr. 
      Chung is the investigator of Shenzhen Institutes of Advanced Technology. Andreas 
      Greiser and Tian-Jing Zhang are the investigators on a cooperative Research and 
      Development Agreement with Siemens. The remaining authors declare that they have 
      no competing interests. PUBLISHER'S NOTE: Springer Nature remains neutral with 
      regard to jurisdictional claims in published maps and institutional affiliations
EDAT- 2018/09/28 06:00
MHDA- 2019/03/15 06:00
PMCR- 2018/09/27
CRDT- 2018/09/28 06:00
PHST- 2017/06/13 00:00 [received]
PHST- 2018/09/05 00:00 [accepted]
PHST- 2018/09/28 06:00 [entrez]
PHST- 2018/09/28 06:00 [pubmed]
PHST- 2019/03/15 06:00 [medline]
PHST- 2018/09/27 00:00 [pmc-release]
AID - S1097-6647(23)00606-3 [pii]
AID - 488 [pii]
AID - 10.1186/s12968-018-0488-1 [doi]
PST - epublish
SO  - J Cardiovasc Magn Reson. 2018 Sep 27;20(1):69. doi: 10.1186/s12968-018-0488-1.