PMID- 18066751
OWN - NLM
STAT- MEDLINE
DCOM- 20080225
LR  - 20071210
IS  - 1097-6647 (Print)
IS  - 1097-6647 (Linking)
VI  - 9
IP  - 6
DP  - 2007
TI  - Mid-myocardial fibrosis by cardiac magnetic resonance in patients with lamin A/C 
      cardiomyopathy: possible substrate for diastolic dysfunction.
PG  - 907-13
AB  - AIMS: We sought to identify patterns of myocardial fibrosis in vivo in patients 
      with lamin cardiomyopathy, and to determine its functional significance. METHODS 
      AND RESULTS: Eleven patients sharing the identical mutation in LMNA without 
      contraindication to magnetic resonance were identified from a 1016-member 
      pedigree. Eight autopsy hearts from deceased relatives were reviewed. Patients 
      and age-matched controls underwent cardiac magnetic resonance that included 
      measures of cardiac function and late gadolinium enhancement (LGE). LGE-CMR 
      identified midmyocardial fibrosis of the basal interventricular septum in 5 of 11 
      LMNA patients that was identical to that seen in 6 autopsy specimens of related 
      genotype-positive family members; this was not present in any of 11 controls. 
      LGE-CMR was positive in the 5 oldest patients in the cohort, age 46 +/- 6 years 
      compared to 24 +/- 10 years for LGE-negative subjects (p = 0.003). Systolic 
      function was abnormal in 2 subjects, both with myocardial fibrosis. 
      LGE-positivity distinguished patients with diastolic dysfunction by mitral inflow 
      velocities from those with normal diastolic function; these patients also had 
      significant left atrial enlargement compared to controls (p < 0.05). CONCLUSIONS: 
      LGE-CMR can identify myocardial fibrosis under genetic control in vivo in 
      patients with heritable cardiomyopathy similar in distribution to that observed 
      at autopsy. Mid-myocardial fibrosis may form the substrate for diastolic 
      dysfunction in these patients.
FAU - Raman, Subha V
AU  - Raman SV
AD  - Department of Internal Medicine, The Ohio State University Medical Center, 
      Columbus, OH 43210-1252, USA. Raman.1@osu.edu
FAU - Sparks, Elizabeth A
AU  - Sparks EA
FAU - Baker, Peter M
AU  - Baker PM
FAU - McCarthy, Beth
AU  - McCarthy B
FAU - Wooley, Charles F
AU  - Wooley CF
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
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 (Lamin Type A)
SB  - IM
MH  - Adult
MH  - Autopsy
MH  - Cardiomyopathy, Dilated/*genetics/*physiopathology
MH  - Case-Control Studies
MH  - Diastole/physiology
MH  - Female
MH  - Fibrosis
MH  - Genotype
MH  - Humans
MH  - Lamin Type A/*genetics
MH  - Longitudinal Studies
MH  - *Magnetic Resonance Imaging, Cine
MH  - Male
MH  - Mutation
MH  - Prospective Studies
MH  - Statistics, Nonparametric
EDAT- 2007/12/11 09:00
MHDA- 2008/02/26 09:00
CRDT- 2007/12/11 09:00
PHST- 2007/12/11 09:00 [pubmed]
PHST- 2008/02/26 09:00 [medline]
PHST- 2007/12/11 09:00 [entrez]
AID - 786988307 [pii]
AID - 10.1080/10976640701693733 [doi]
PST - ppublish
SO  - J Cardiovasc Magn Reson. 2007;9(6):907-13. doi: 10.1080/10976640701693733.