PMID- 27660108
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20240117
IS  - 1532-429X (Electronic)
IS  - 1097-6647 (Print)
IS  - 1097-6647 (Linking)
VI  - 18
IP  - 1
DP  - 2016 Sep 22
TI  - Myocardial late gadolinium enhancement is associated with clinical presentation 
      in Duchenne muscular dystrophy carriers.
PG  - 61
LID - 61
AB  - BACKGROUND: Duchenne muscular dystrophy (DMD) is an X-linked recessive disease 
      that occurs in males leading to immobility and death in early adulthood. Female 
      carriers of DMD are generally asymptomatic, yet frequently develop dilated 
      cardiomyopathy. This study aims to detect early cardiac manifestation in DMD 
      using cardiovascular magnetic resonance (CMR) and to evaluate its association 
      with clinical symptoms. METHODS: Clinical assessment of DMD carriers included six 
      minutes walk tests (6MWT), blood analysis, electrocardiography, echocardiography, 
      and CMR using FLASH sequences to detect late gadolinium enhancement (LGE). 
      T1-mapping using the Modified Look-Locker Inversion recovery (MOLLI) sequence was 
      performed quantify extracellular volume (ECV). RESULTS: Of 20 carriers (age 
      39.47 +/- 12.96 years) 17 (89.5 %) were clinically asymptomatic. ECV was mildly 
      elevated (29.79 +/- 2.92 %) and LGE was detected in nine cases (45 %). LGE positive 
      carriers had lower left ventricular ejection fraction in CMR (64.36 +/- 5.78 vs. 
      56.67 +/- 6.89 %, p = 0.014), higher bothCK (629.89 +/- 317.48 vs. 256.18 +/- 109.10 
      U/l, p = 0.002) and CK-MB (22.13 +/- 5.25 vs. 12.11 +/- 2.21 U/l, p = 0.001), as well 
      as shorter walking distances during the 6MWT (432.44 +/- 96.72 vs. 
      514.91 +/- 66.80 m, p = 0.037). 90.9 % of subjects without LGE had normal pro-BNP, 
      whereas in 66.7 % of those presenting LGE pro-BNP was elevated (p = 0.027). All 
      individuals without LGE were in the NYHA class I, whereas all those in NYHA 
      classes II and III showed positive for LGE (p = 0.066). CONCLUSIONS: Myocardial 
      involvement shown as LGE in CMR occurs in a substantial number of DMD carriers; 
      it is associated with clinical and morphometric signs of incipient heart failure. 
      LGE is thus a sensitive parameter for the early diagnosis of cardiomyopathy in 
      DMD carriers. TRIAL REGISTRATION: Clinicaltrials.gov, NCT01712152 Trial 
      registration: October 19, 2012. First patient enrolled: September 27, 2012 
      (retrospectively registered).
FAU - Wexberg, Paul
AU  - Wexberg P
AD  - 2nd Medical Department, Krankenanstalt Rudolfstiftung, Vienna, Austria. 
      paul.wexberg@svagw.at.
AD  - SVA-Gesundheitszentrum, Hartmanngasse 2b, Vienna, A-1051, Austria. 
      paul.wexberg@svagw.at.
FAU - Avanzini, Marion
AU  - Avanzini M
AD  - 2nd Medical Department, Krankenanstalt Rudolfstiftung, Vienna, Austria.
FAU - Mascherbauer, Julia
AU  - Mascherbauer J
AD  - Division of Cardiology, Department Of Internal Medicine II, Medical University of 
      Vienna, Vienna, Austria.
FAU - Pfaffenberger, Stefan
AU  - Pfaffenberger S
AD  - Division of Cardiology, Department Of Internal Medicine II, Medical University of 
      Vienna, Vienna, Austria.
FAU - Freudenthaler, Birgit
AU  - Freudenthaler B
AD  - 2nd Medical Department, Krankenanstalt Rudolfstiftung, Vienna, Austria.
FAU - Bittner, Reginald
AU  - Bittner R
AD  - Neuromuscular Research Department, Center of Anatomy & Cell Biology, Medical 
      University of Vienna, Vienna, Austria.
FAU - Bernert, Gunther
AU  - Bernert G
AD  - Gottfried von Preyer Children Hospital, Vienna, Austria.
FAU - Weidinger, Franz
AU  - Weidinger F
AD  - 2nd Medical Department, Krankenanstalt Rudolfstiftung, Vienna, Austria.
LA  - eng
SI  - ClinicalTrials.gov/NCT01712152
PT  - Journal Article
DEP - 20160922
PL  - England
TA  - J Cardiovasc Magn Reson
JT  - Journal of cardiovascular magnetic resonance : official journal of the Society 
      for Cardiovascular Magnetic Resonance
JID - 9815616
PMC - PMC5034448
OTO - NOTNLM
OT  - Cardiomyopathy
OT  - Cardiovascular magnetic resonance
OT  - Duchenne muscular dystrophy
OT  - T1-mapping
EDAT- 2016/09/24 06:00
MHDA- 2016/09/24 06:01
PMCR- 2016/09/22
CRDT- 2016/09/24 06:00
PHST- 2016/04/29 00:00 [received]
PHST- 2016/09/05 00:00 [accepted]
PHST- 2016/09/24 06:00 [entrez]
PHST- 2016/09/24 06:00 [pubmed]
PHST- 2016/09/24 06:01 [medline]
PHST- 2016/09/22 00:00 [pmc-release]
AID - S1097-6647(23)01002-5 [pii]
AID - 281 [pii]
AID - 10.1186/s12968-016-0281-y [doi]
PST - epublish
SO  - J Cardiovasc Magn Reson. 2016 Sep 22;18(1):61. doi: 10.1186/s12968-016-0281-y.