PMID- 18983659
OWN - NLM
STAT- MEDLINE
DCOM- 20090122
LR  - 20240117
IS  - 1532-429X (Electronic)
IS  - 1097-6647 (Print)
IS  - 1097-6647 (Linking)
VI  - 10
IP  - 1
DP  - 2008 Nov 4
TI  - Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic 
      and pathophysiological insights by a CMR approach.
PG  - 50
LID - 10.1186/1532-429X-10-50 [doi]
AB  - BACKGROUND: Becker-Kiener muscular dystrophy (BMD) represents an X-linked genetic 
      disease associated with myocardial involvement potentially resulting in dilated 
      cardiomyopathy (DCM). Early diagnosis of cardiac involvement may permit earlier 
      institution of heart failure treatment and extend life span in these patients. 
      Both echocardiography and nuclear imaging methods are capable of detecting later 
      stages of cardiac involvement characterised by wall motion abnormalities. 
      Cardiovascular magnetic resonance (CMR) has the potential to detect cardiac 
      involvement by depicting early scar formation that may appear before onset of 
      wall motion abnormalities. METHODS: In a prospective two-center-study, 15 male 
      patients with BMD (median age 37 years; range 11 years to 56 years) underwent 
      comprehensive neurological and cardiac evaluations including physical 
      examination, echocardiography and CMR. A 16-segment model was applied for 
      evaluation of regional wall motion abnormalities (rWMA). The CMR study included 
      late gadolinium enhancement (LGE) imaging with quantification of myocardial 
      damage. RESULTS: Abnormal echocardiographic results were found in eight of 15 
      (53.3%) patients with all of them demonstrating reduced left ventricular ejection 
      fraction (LVEF) and rWMA. CMR revealed abnormal findings in 12 of 15 (80.0%) 
      patients (p = 0.04) with 10 (66.6%) having reduced LVEF (p = 0.16) and 9 (64.3%) 
      demonstrating rWMA (p = 0.38). Myocardial damage as assessed by LGE-imaging was 
      detected in 11 of 15 (73.3%) patients with a median myocardial damage extent of 
      13.0% (range 0 to 38.0%), an age-related increase and a typical subepicardial 
      distribution pattern in the inferolateral wall. Ten patients (66.7%) were in need 
      of medical heart failure therapy based on CMR results. However, only 4 patients 
      (26.7%) were already taking medication based on clinical criteria (p = 0.009). 
      CONCLUSION: Cardiac involvement in patients with BMD is underdiagnosed by 
      echocardiographic methods resulting in undertreatment of heart failure. The 
      degree and severity of cardiac involvement in this population is best 
      characterised when state-of-the-art CMR methods are applied. Further studies need 
      to demonstrate whether earlier diagnosis and institution of heart failure therapy 
      will extend the life span of these patients.
FAU - Yilmaz, Ali
AU  - Yilmaz A
AD  - Division of Cardiology, Robert-Bosch-Krankenhaus, Stuttgart, Germany. 
      ali.yilmaz@rbk.de
FAU - Gdynia, Hans-Jurgen
AU  - Gdynia HJ
FAU - Baccouche, Hannibal
AU  - Baccouche H
FAU - Mahrholdt, Heiko
AU  - Mahrholdt H
FAU - Meinhardt, Gabriel
AU  - Meinhardt G
FAU - Basso, Cristina
AU  - Basso C
FAU - Thiene, Gaetano
AU  - Thiene G
FAU - Sperfeld, Anne-Dorte
AU  - Sperfeld AD
FAU - Ludolph, Albert C
AU  - Ludolph AC
FAU - Sechtem, Udo
AU  - Sechtem U
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PT  - Multicenter Study
PT  - Research Support, Non-U.S. Gov't
DEP - 20081104
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  - Cardiomyopathy, Dilated/etiology/*pathology/physiopathology/therapy
MH  - Child
MH  - Contrast Media
MH  - Echocardiography
MH  - Electrocardiography
MH  - Gadolinium DTPA
MH  - Germany
MH  - Humans
MH  - *Magnetic Resonance Imaging, Cine
MH  - Male
MH  - Middle Aged
MH  - Muscular Dystrophy, Duchenne/*complications/pathology/physiopathology/therapy
MH  - Myocardium/*pathology
MH  - Predictive Value of Tests
MH  - Prospective Studies
MH  - Severity of Illness Index
MH  - Stroke Volume
MH  - *Ventricular Function, Left
MH  - Young Adult
PMC - PMC2585564
EDAT- 2008/11/06 09:00
MHDA- 2009/01/23 09:00
PMCR- 2008/11/04
CRDT- 2008/11/06 09:00
PHST- 2008/08/04 00:00 [received]
PHST- 2008/11/04 00:00 [accepted]
PHST- 2008/11/06 09:00 [pubmed]
PHST- 2009/01/23 09:00 [medline]
PHST- 2008/11/06 09:00 [entrez]
PHST- 2008/11/04 00:00 [pmc-release]
AID - S1097-6647(23)01267-X [pii]
AID - 1532-429X-10-50 [pii]
AID - 10.1186/1532-429X-10-50 [doi]
PST - epublish
SO  - J Cardiovasc Magn Reson. 2008 Nov 4;10(1):50. doi: 10.1186/1532-429X-10-50.