PMID- 24886403
OWN - NLM
STAT- MEDLINE
DCOM- 20141021
LR  - 20240117
IS  - 1532-429X (Electronic)
IS  - 1097-6647 (Print)
IS  - 1097-6647 (Linking)
VI  - 16
IP  - 1
DP  - 2014 May 14
TI  - Myocardial fibrosis in Eisenmenger syndrome: a descriptive cohort study exploring 
      associations of late gadolinium enhancement with clinical status and survival.
PG  - 32
LID - 10.1186/1532-429X-16-32 [doi]
AB  - BACKGROUND: A relationship between myocardial fibrosis and ventricular 
      dysfunction has been demonstrated using late gadolinium enhancement (LGE) in the 
      pressure-loaded right ventricle from congenital heart defects. In patients with 
      Eisenmenger syndrome (ES), the presence of LGE has not been investigated. The 
      aims of this study were to detect any myocardial fibrosis in ES and describe 
      major clinical variables associated with the finding. METHODS: From 45 subjects 
      screened, 30 subjects (age 43 +/- 13 years, 20 female) underwent prospective 
      cardiovascular magnetic resonance with LGE to quantify biventricular volume and 
      function as well as maximal and submaximal exercise during a single visit. 
      Standard cine acquisitions were obtained for ventricular volume and function. 
      Further imaging was performed after administration of 0.1 mmol/kg gadolinium 
      contrast. Regions of LGE were evaluated qualitatively and quantitatively by 
      manual contouring of identified areas, with total area expressed as a percentage 
      of mass. Patients were followed prospectively (mean follow up 7.4 +/- 0.4 years) 
      and any deaths recorded. Patients with LGE findings were compared to those 
      without. RESULTS: LGE was present in 22/30 (73%) patients, specifically in RV 
      myocardium (70%), RV trabeculae (60%), LV myocardium (33%) or LV papillary 
      muscles (30%), though in small amounts (mean 1.4% of total ventricular mass, 
      range 0.16 - 6.0%). Those with any LGE were not different in age, history of 
      arrhythmia, desaturation, nor hemoglobin, nor ventricular size, mass, or 
      function. Exercise capacity was low, but also not different between those with 
      and without LGE. Similarly no significant associations were found with amount of 
      fibrosis. There were five deaths among patients with LGE, versus two in patients 
      without, but no difference in survival (log rank =0.03, P = 0.85). CONCLUSIONS: 
      Myocardial fibrosis by LGE is common in ES, though not extensive. The presence 
      and quantity of LGE did not correlate with ventricular size, function, degree of 
      cyanosis, exercise capacity, or survival in this pilot study. More data are 
      clearly required before recommendations for routine use of LGE in these patients 
      can be made.
FAU - Broberg, Craig S
AU  - Broberg CS
AD  - Adult Congenital Heart Program, Knight Cardiovascular Institute, Oregon Health & 
      Science University, Portland, Oregon, USA. brobergc@ohsu.edu.
FAU - Prasad, Sanjay K
AU  - Prasad SK
FAU - Carr, Chad
AU  - Carr C
FAU - Babu-Narayan, Sonya V
AU  - Babu-Narayan SV
FAU - Dimopoulos, Konstantinos
AU  - Dimopoulos K
FAU - Gatzoulis, Michael A
AU  - Gatzoulis MA
LA  - eng
GR  - FS/11/38/28864/British Heart Foundation/United Kingdom
GR  - K23HL093024/HL/NHLBI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
DEP - 20140514
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)
SB  - IM
MH  - Adult
MH  - *Contrast Media
MH  - Eisenmenger Complex/complications/*diagnosis/mortality/pathology/physiopathology
MH  - Exercise Test
MH  - Exercise Tolerance
MH  - Female
MH  - Fibrosis
MH  - Humans
MH  - Kaplan-Meier Estimate
MH  - *Magnetic Resonance Imaging, Cine
MH  - Male
MH  - Middle Aged
MH  - Myocardium/*pathology
MH  - Pilot Projects
MH  - Predictive Value of Tests
MH  - Prognosis
MH  - Prospective Studies
MH  - Risk Factors
MH  - Time Factors
MH  - Ventricular Function, Left
MH  - Ventricular Function, Right
PMC - PMC4051886
EDAT- 2014/06/03 06:00
MHDA- 2014/10/22 06:00
PMCR- 2014/05/14
CRDT- 2014/06/03 06:00
PHST- 2013/12/16 00:00 [received]
PHST- 2014/05/02 00:00 [accepted]
PHST- 2014/06/03 06:00 [entrez]
PHST- 2014/06/03 06:00 [pubmed]
PHST- 2014/10/22 06:00 [medline]
PHST- 2014/05/14 00:00 [pmc-release]
AID - S1097-6647(23)00091-1 [pii]
AID - 1532-429X-16-32 [pii]
AID - 10.1186/1532-429X-16-32 [doi]
PST - epublish
SO  - J Cardiovasc Magn Reson. 2014 May 14;16(1):32. doi: 10.1186/1532-429X-16-32.