BACKGROUND: Cardiac involvement is a recognised complication in female carriers of Duchenne muscular dystrophy (DMD). Since segmental or global left ventricle (LV) wall motion abnormalities in DMD carriers can arise even without apparent muscle weakness, it is difficult to differentiate cardiac involvement of a DMD carrier from other heart diseases in a non-invasive manner. Cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) enables assessment of regional wall motion abnormality and myocardial damage with high spatial resolution. OBJECTIVE: To assess the utility of CMR for detection of myocardial damage in female carriers of DMD. METHODS AND RESULTS: Gadolinium-enhanced CMR was performed in seven female DMD carriers. Physical examination, electrocardiography, chest radiograph, measurements of total creatinine kinase and brain natriuretic peptide levels, and two-dimensional echocardiography were also performed. Four (57%) of the seven carriers had LGE, and LGE was frequently observed at the subepicardial layer in the inferolateral segment. Two carriers had a focal LGE at the LV inferolateral wall without LV dilation or wall motion abnormalities. CONCLUSION: CMR findings of DMD carriers were characterised by subepicardial LGE, which was localised at inferolateral segments. CMR may be a useful modality for detecting cardiac involvement in DMD carriers.
BACKGROUND: Cardiac involvement is a recognised complication in female carriers of Duchenne muscular dystrophy (DMD). Since segmental or global left ventricle (LV) wall motion abnormalities in DMD carriers can arise even without apparent muscle weakness, it is difficult to differentiate cardiac involvement of a DMD carrier from other heart diseases in a non-invasive manner. Cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) enables assessment of regional wall motion abnormality and myocardial damage with high spatial resolution. OBJECTIVE: To assess the utility of CMR for detection of myocardial damage in female carriers of DMD. METHODS AND RESULTS:Gadolinium-enhanced CMR was performed in seven female DMD carriers. Physical examination, electrocardiography, chest radiograph, measurements of total creatinine kinase and brain natriuretic peptide levels, and two-dimensional echocardiography were also performed. Four (57%) of the seven carriers had LGE, and LGE was frequently observed at the subepicardial layer in the inferolateral segment. Two carriers had a focal LGE at the LV inferolateral wall without LV dilation or wall motion abnormalities. CONCLUSION: CMR findings of DMD carriers were characterised by subepicardial LGE, which was localised at inferolateral segments. CMR may be a useful modality for detecting cardiac involvement in DMD carriers.
Authors: Manuel D Cerqueira; Neil J Weissman; Vasken Dilsizian; Alice K Jacobs; Sanjiv Kaul; Warren K Laskey; Dudley J Pennell; John A Rumberger; Thomas Ryan; Mario S Verani Journal: Circulation Date: 2002-01-29 Impact factor: 29.690
Authors: R J Kim; D S Fieno; T B Parrish; K Harris; E L Chen; O Simonetti; J Bundy; J P Finn; F J Klocke; R M Judd Journal: Circulation Date: 1999-11-09 Impact factor: 29.690
Authors: E M Hoogerwaard; P A van der Wouw; A A Wilde; E Bakker; P F Ippel; J C Oosterwijk; D F Majoor-Krakauer; A J van Essen; N J Leschot; M de Visser Journal: Neuromuscul Disord Date: 1999-07 Impact factor: 4.296
Authors: J A McCrohon; J C C Moon; S K Prasad; W J McKenna; C H Lorenz; A J S Coats; D J Pennell Journal: Circulation Date: 2003-06-23 Impact factor: 29.690
Authors: Josef Finsterer; Claudia Stöllberger; Birgit Freudenthaler; Desiree De Simoni; Romana Höftberger; Klaus Wagner Journal: Intractable Rare Dis Res Date: 2018-05