Early detection of cardiac involvement in Miyoshi myopathy: 2D strain echocardiography and late gadolinium enhancement cardiovascular magnetic resonance

Journal of Cardiovascular Magnetic Resonance

Research

Seung Woo Park¹  Duk-Kyung Kim¹  Sung-Ji Park¹  Jae K Oh¹  E Ryoung Choi¹  Sung-A Chang¹  Sang-Chol Lee¹  Dong Ryeol Ryu¹  Jin-Oh Choi¹  Yeon Hyeon Choe¹  Byoung Joon Kim² 

[1] Cardiovascular Imaging Center, Cardiac and vascular center, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, 135-710, Seoul, Seoul, Republic of Korea;Department of Neurology, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, 135-710, Seoul, Seoul, Republic of Korea;

关键词: Cardiovascular Magnetic Resonance;    Late Gadolinium Enhancement;    Duchenne Muscular Dystrophy;    Cardiac Involvement;    Global Left Ventricular Longitudinal Strain;   

DOI  :  10.1186/1532-429X-12-31

 received in 2009-12-22, accepted in 2010-05-24,  发布年份 2010

来源: Springer

【 摘 要 】

BackgroundMiyoshi 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).MethodFive 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.ResultsAll 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.ConclusionsPatients 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.

【 授权许可】

Unknown   
© Choi et al; licensee BioMed Central Ltd. 2010. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.