PMID- 27919765
OWN - NLM
STAT- MEDLINE
DCOM- 20180124
LR  - 20220129
IS  - 1556-3871 (Electronic)
IS  - 1547-5271 (Linking)
VI  - 14
IP  - 4
DP  - 2017 Apr
TI  - Late gadolinium enhancement in Brugada syndrome: A marker for subtle underlying 
      cardiomyopathy?
PG  - 583-589
LID - S1547-5271(16)31159-6 [pii]
LID - 10.1016/j.hrthm.2016.12.004 [doi]
AB  - BACKGROUND: There is increasing evidence that the Brugada ECG pattern is a marker 
      of subtle structural heart disease. OBJECTIVE: The purpose of this study was to 
      characterize patients with Brugada syndrome (BrS) using cardiovascular magnetic 
      resonance (CMR) with late gadolinium enhancement (LGE). METHODS: BrS was 
      diagnosed according to international guidelines. Twenty-six percent of patients 
      with BrS carried SCN5A mutations. CMR data from 78 patients with BrS were 
      compared with 78 healthy controls (44 +/- 15 vs 42 +/- 14 years; P = .434; and 64% vs 
      64% male; P = 1). RESULTS: Right ventricular (RV) ejection fraction was slightly 
      lower (61 +/- 8% vs 64 +/- 5%; P = .004) and RV end-systolic volume slightly greater 
      (31 +/- 10 mL/m(2) vs 28 +/- 6 mL/m(2); P = .038) in BrS compared with controls. 
      These values remained within the normal range. LGE was demonstrated in 8% of 
      patients with BrS (left ventricular midwall LGE in 5%) but not in controls (P = 
      .028). In patients with BrS with midwall LGE there were no other features of 
      cardiomyopathy at the time of CMR, but genetic testing and follow-up revealed a 
      desmoplakin mutation in 1 patient and evolution of T-wave inversion throughout 
      all precordial ECG leads in another. Neither patient fulfils diagnostic criteria 
      for arrhythmogenic right ventricular cardiomyopathy. CONCLUSION: Some patients 
      with BrS have left ventricular midwall LGE consistent with an underlying 
      cardiomyopathic process. Even cases without LGE show greater RV volumes and 
      reduced RV function. These findings lend further support to the presence of 
      subtle structural abnormalities in BrS. The BrS pattern with LGE may serve as 
      early markers for evolution of a cardiomyopathic phenotype over time. CMR is a 
      potentially useful adjunct investigation in the clinical evaluation of BrS.
CI  - Copyright (c) 2016 Heart Rhythm Society. Published by Elsevier Inc. All rights 
      reserved.
FAU - Bastiaenen, Rachel
AU  - Bastiaenen R
AD  - Molecular and Clinical Sciences Research Institute, St. George's University of 
      London, United Kingdom; Cardiology Clinical Academic Group, St. George's 
      University Hospitals NHS Foundation Trust, London, United Kingdom. Electronic 
      address: rbastiae@sgul.ac.uk.
FAU - Cox, Andrew T
AU  - Cox AT
AD  - Molecular and Clinical Sciences Research Institute, St. George's University of 
      London, United Kingdom; Cardiology Clinical Academic Group, St. George's 
      University Hospitals NHS Foundation Trust, London, United Kingdom.
FAU - Castelletti, Silvia
AU  - Castelletti S
AD  - Center for Cardiac Arrhythmias of Genetic Origin, IRCCS Istituto Auxologico 
      Italiano "San Carlo." Milan, Italy.
FAU - Wijeyeratne, Yanushi D
AU  - Wijeyeratne YD
AD  - Molecular and Clinical Sciences Research Institute, St. George's University of 
      London, United Kingdom; Cardiology Clinical Academic Group, St. George's 
      University Hospitals NHS Foundation Trust, London, United Kingdom.
FAU - Colbeck, Nicholas
AU  - Colbeck N
AD  - Molecular and Clinical Sciences Research Institute, St. George's University of 
      London, United Kingdom.
FAU - Pakroo, Nadia
AU  - Pakroo N
AD  - Molecular and Clinical Sciences Research Institute, St. George's University of 
      London, United Kingdom.
FAU - Ahmed, Hammad
AU  - Ahmed H
AD  - Molecular and Clinical Sciences Research Institute, St. George's University of 
      London, United Kingdom.
FAU - Bunce, Nick
AU  - Bunce N
AD  - Molecular and Clinical Sciences Research Institute, St. George's University of 
      London, United Kingdom.
FAU - Anderson, Lisa
AU  - Anderson L
AD  - Molecular and Clinical Sciences Research Institute, St. George's University of 
      London, United Kingdom; Cardiology Clinical Academic Group, St. George's 
      University Hospitals NHS Foundation Trust, London, United Kingdom.
FAU - Moon, James C
AU  - Moon JC
AD  - Bart's Heart Centre, St. Bartholomew's Hospital, London, United Kingdom.
FAU - Prasad, Sanjay
AU  - Prasad S
AD  - Royal Brompton Hospital, Royal Brompton and Harefield NHS Foundation Trust, 
      London, United Kingdom.
FAU - Sharma, Sanjay
AU  - Sharma S
AD  - Molecular and Clinical Sciences Research Institute, St. George's University of 
      London, United Kingdom; Cardiology Clinical Academic Group, St. George's 
      University Hospitals NHS Foundation Trust, London, United Kingdom.
FAU - Behr, Elijah R
AU  - Behr ER
AD  - Molecular and Clinical Sciences Research Institute, St. George's University of 
      London, United Kingdom; Cardiology Clinical Academic Group, St. George's 
      University Hospitals NHS Foundation Trust, London, United Kingdom.
LA  - eng
GR  - FS/10/40/28260/BHF_/British Heart Foundation/United Kingdom
GR  - FS/12/56/29723/BHF_/British Heart Foundation/United Kingdom
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20161203
PL  - United States
TA  - Heart Rhythm
JT  - Heart rhythm
JID - 101200317
RN  - 0 (Contrast Media)
RN  - 0 (NAV1.5 Voltage-Gated Sodium Channel)
RN  - 0 (SCN5A protein, human)
RN  - AU0V1LM3JT (Gadolinium)
SB  - IM
CIN - Heart Rhythm. 2017 Apr;14 (4):590-591. PMID: 27919766
MH  - Adult
MH  - *Brugada Syndrome/diagnosis/genetics/physiopathology
MH  - Cardiomyopathies/diagnosis/physiopathology
MH  - Contrast Media/pharmacology
MH  - Female
MH  - Gadolinium/pharmacology
MH  - *Heart Ventricles/diagnostic imaging/pathology/physiopathology
MH  - Humans
MH  - Image Enhancement/methods
MH  - Magnetic Resonance Imaging, Cine/*methods
MH  - Male
MH  - Middle Aged
MH  - Mutation
MH  - NAV1.5 Voltage-Gated Sodium Channel/genetics
MH  - Organ Size
MH  - Reproducibility of Results
MH  - Stroke Volume
OTO - NOTNLM
OT  - Arrhythmogenic right ventricular cardiomyopathy
OT  - Brugada syndrome
OT  - Cardiac magnetic resonance
OT  - Cardiomyopathy
OT  - Late gadolinium enhancement
EDAT- 2016/12/07 06:00
MHDA- 2018/01/25 06:00
CRDT- 2016/12/07 06:00
PHST- 2016/07/01 00:00 [received]
PHST- 2016/12/07 06:00 [pubmed]
PHST- 2018/01/25 06:00 [medline]
PHST- 2016/12/07 06:00 [entrez]
AID - S1547-5271(16)31159-6 [pii]
AID - 10.1016/j.hrthm.2016.12.004 [doi]
PST - ppublish
SO  - Heart Rhythm. 2017 Apr;14(4):583-589. doi: 10.1016/j.hrthm.2016.12.004. Epub 2016 
      Dec 3.