PMID- 20497525
OWN - NLM
STAT- MEDLINE
DCOM- 20101026
LR  - 20240117
IS  - 1532-429X (Electronic)
IS  - 1097-6647 (Print)
IS  - 1097-6647 (Linking)
VI  - 12
IP  - 1
DP  - 2010 May 24
TI  - Early detection of cardiac involvement in Miyoshi myopathy: 2D strain 
      echocardiography and late gadolinium enhancement cardiovascular magnetic 
      resonance.
PG  - 31
LID - 10.1186/1532-429X-12-31 [doi]
AB  - BACKGROUND: Miyoshi myopathy (MM) is an autosomal recessive distal myopathy 
      characterized by early adult onset. Cardiomyopathy is a major clinical 
      manifestation in other muscular dystrophies and an important prognostic factor. 
      Although dysferlin is highly expressed in cardiac muscle, the effect of dysferlin 
      deficiency in cardiac muscle has not been studied. We hypothesized that early 
      myocardial dysfunction could be detected by 2D strain echocardiography and late 
      gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR). METHOD: 
      Five consecutive MM patients (3 male) in whom we detected the DYSF gene mutation 
      and age-matched healthy control subjects were included. None of the patients had 
      history of cardiac disease or signs and symptoms of overt heart failure. Patients 
      were studied using 2D strain echocardiography and CMR, with 2D strain being 
      obtained using the Automated Function Imaging technique. RESULTS: All patients 
      had preserved left ventricular systolic function. However, segmental Peak 
      Systolic Longitudinal Strain (PSLS) was decreased in 3 patients. Global PSLS was 
      significantly lower in patients with MM than in control subjects (p = 0.005). 
      Basal anterior septum, basal inferior septum, mid anterior, and mid inferior 
      septum PSLS were significantly lower in patients with MM than in control subjects 
      (P < 0.0001, < 0.0001, 0.038 and 0.003, respectively). Four patients showed 
      fibrosis by LGE. The reduced PSLS lesion detected by 2D strain tended to be in 
      the same area as that which showed fibrosis by LGE. CONCLUSIONS: Patients with MM 
      showed subclinical involvement of the heart. 2D strain and LGE are sensitive 
      methods for detecting myocardial dysfunction prior to the development of 
      cardiovascular symptoms. The prognostic significance of these findings warrants 
      further longitudinal follow-up.
FAU - Choi, E Ryoung
AU  - Choi ER
AD  - Cardiovascular Imaging Center, Samsung Medical Center, Sungkyunkwan University 
      School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul, 135-710, Seoul, Republic of 
      Korea.
FAU - Park, Sung-Ji
AU  - Park SJ
FAU - Choe, Yeon Hyeon
AU  - Choe YH
FAU - Ryu, Dong Ryeol
AU  - Ryu DR
FAU - Chang, Sung-A
AU  - Chang SA
FAU - Choi, Jin-Oh
AU  - Choi JO
FAU - Lee, Sang-Chol
AU  - Lee SC
FAU - Park, Seung Woo
AU  - Park SW
FAU - Kim, Byoung Joon
AU  - Kim BJ
FAU - Kim, Duk-Kyung
AU  - Kim DK
FAU - Oh, Jae K
AU  - Oh JK
LA  - eng
PT  - Journal Article
DEP - 20100524
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 (DYSF protein, human)
RN  - 0 (Dysferlin)
RN  - 0 (Membrane Proteins)
RN  - 0 (Muscle Proteins)
RN  - 0 (Radiopharmaceuticals)
RN  - AU0V1LM3JT (Gadolinium)
SB  - IM
MH  - Adult
MH  - Cardiomyopathies/*diagnosis/etiology
MH  - Case-Control Studies
MH  - Dysferlin
MH  - Echocardiography
MH  - Female
MH  - Gadolinium
MH  - Humans
MH  - Magnetic Resonance Imaging
MH  - Male
MH  - Membrane Proteins
MH  - Middle Aged
MH  - Muscle Proteins
MH  - Muscular Diseases/complications/*diagnosis
MH  - Radiopharmaceuticals
PMC - PMC2887439
EDAT- 2010/05/26 06:00
MHDA- 2010/10/27 06:00
PMCR- 2010/05/24
CRDT- 2010/05/26 06:00
PHST- 2009/12/22 00:00 [received]
PHST- 2010/05/24 00:00 [accepted]
PHST- 2010/05/26 06:00 [entrez]
PHST- 2010/05/26 06:00 [pubmed]
PHST- 2010/10/27 06:00 [medline]
PHST- 2010/05/24 00:00 [pmc-release]
AID - S1097-6647(23)01311-X [pii]
AID - 1532-429X-12-31 [pii]
AID - 10.1186/1532-429X-12-31 [doi]
PST - epublish
SO  - J Cardiovasc Magn Reson. 2010 May 24;12(1):31. doi: 10.1186/1532-429X-12-31.