PMID- 25008475
OWN - NLM
STAT- MEDLINE
DCOM- 20150330
LR  - 20240117
IS  - 1532-429X (Electronic)
IS  - 1097-6647 (Print)
IS  - 1097-6647 (Linking)
VI  - 16
IP  - 1
DP  - 2014 Jul 9
TI  - Patterns of late gadolinium enhancement in Duchenne muscular dystrophy carriers.
PG  - 45
LID - 10.1186/1532-429X-16-45 [doi]
AB  - BACKGROUND: This study was designed to assess whether cardiovascular magnetic 
      resonance imaging (CMR) in Duchenne muscular dystrophy carriers (DMDc) may index 
      any cell milieu elements of LV dysfunction and whether this cardiac phenotype may 
      be related to genotype. The null hypothesis was that myocardial fibrosis, 
      assessed by late gadolinium enhancement (LGE), might be similarly accounted for 
      in DMDc and gender and age-matched controls. METHODS: Thirty DMDc patients had 
      CMR and genotyping with 37 gender and age-matched controls. Systolic and 
      diastolic LV function was assessed by 2D-echocardiography. RESULTS: Absolute and 
      percent LGE were higher in muscular symptomatic (sym) than asymptomatic (asy) 
      DMDc (1.77 +/- 0.27 vs 0.76 +/- 0.17 ml; F = 19.6, p < 0.0001 and 1.86 +/- 0.26% vs 
      0.68 +/- 0.17%, F = 22.1, p < 0.0001, respectively). There was no correlation 
      between LGE and age. LGE was seen most frequently in segments 5 and 6; segment 5 
      was involved in all asy-DMDc. Subepicardial LGE predominated, compared to the 
      mid-myocardial one (11 out of 14 DMDc). LGE was absent in the subendocardium. No 
      correlations were seen between genotyping (type of mutation, gene region and 
      protein domain), confined to the exon's study, and cardiac phenotype. 
      CONCLUSIONS: A typical myocardial LGE-pattern location (LV segments 5 and 6) was 
      a common finding in DMDc. LGE was more frequently subepicardial plus 
      midmyocardial in sym-DMDc, with normal LV systolic and diastolic function. No 
      genotype-phenothype correlation was found.
FAU - Giglio, Vincenzo
AU  - Giglio V
AD  - Center for Neuromuscular Disease, Uildm, Prospero Santacroce St, 5, Rome 00167, 
      Italy. giglio.echo@libero.it.
FAU - Puddu, Paolo Emilio
AU  - Puddu PE
FAU - Camastra, Giovanni
AU  - Camastra G
FAU - Sbarbati, Stefano
AU  - Sbarbati S
FAU - Della Sala, Sabino Walter
AU  - Della Sala SW
FAU - Ferlini, Alessandra
AU  - Ferlini A
FAU - Gualandi, Francesca
AU  - Gualandi F
FAU - Ricci, Enzo
AU  - Ricci E
FAU - Sciarra, Federico
AU  - Sciarra F
FAU - Ansalone, Gerardo
AU  - Ansalone G
FAU - Di Gennaro, Marco
AU  - Di Gennaro M
LA  - eng
PT  - Journal Article
DEP - 20140709
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  - Case-Control Studies
MH  - Child
MH  - *Contrast Media
MH  - Cross-Sectional Studies
MH  - Female
MH  - Fibrosis
MH  - *Gadolinium DTPA
MH  - Genetic Predisposition to Disease
MH  - Heart Ventricles/*pathology/physiopathology
MH  - Humans
MH  - *Magnetic Resonance Imaging, Cine
MH  - Male
MH  - Middle Aged
MH  - Muscular Dystrophy, Duchenne/*complications/genetics
MH  - Myocardium/*pathology
MH  - Phenotype
MH  - Predictive Value of Tests
MH  - Ventricular Dysfunction, Left/*diagnosis/etiology/pathology/physiopathology
MH  - Ventricular Function, Left
MH  - Young Adult
PMC - PMC4096415
EDAT- 2014/07/11 06:00
MHDA- 2015/03/31 06:00
PMCR- 2014/07/09
CRDT- 2014/07/11 06:00
PHST- 2014/01/07 00:00 [received]
PHST- 2014/05/22 00:00 [accepted]
PHST- 2014/07/11 06:00 [entrez]
PHST- 2014/07/11 06:00 [pubmed]
PHST- 2015/03/31 06:00 [medline]
PHST- 2014/07/09 00:00 [pmc-release]
AID - S1097-6647(23)00112-6 [pii]
AID - 1532-429X-16-45 [pii]
AID - 10.1186/1532-429X-16-45 [doi]
PST - epublish
SO  - J Cardiovasc Magn Reson. 2014 Jul 9;16(1):45. doi: 10.1186/1532-429X-16-45.