Focal myocardial fibrosis assessed by late gadolinium enhancement cardiovascular magnetic resonance in children and adolescents with dilated cardiomyopathy

dc.contributor.authorLatus, Heiner
dc.contributor.authorGummel, Kerstin
dc.contributor.authorKlingel, Karin
dc.contributor.authorMoysich, Axel
dc.contributor.authorKhalil, Markus
dc.contributor.authorMazhari, Nona
dc.contributor.authorBauer, Juergen
dc.contributor.authorKandolf, Reinhard
dc.contributor.authorSchranz, Dietmar
dc.contributor.authorApitz, Christian
dc.description.abstractBACKGROUND: Different patterns of late gadolinium enhancement (LGE) including mid-wall fibrosis using cardiovascular magnetic resonance (CMR) have been reported in adult patients presenting with non-ischemic dilated cardiomyopathy (DCM). In these studies, LGE was associated with pronounced LV remodelling and predicted adverse cardiac outcomes. Accordingly, the purpose of our study was to determine the presence and patterns of LGE in children and adolescents with DCM. METHODS: Patients <18years of age presenting with severe congestive heart failure who were admitted for evaluation of heart transplantation at our centre underwent CMR examination which consisted of ventricular functional analysis and assessment of LGE for detection of myocardial fibrosis. Ischemic DCM was excluded by coronary angiography, and right ventricular endomyocardial biopsies ruled out acute myocarditis.RESULTS:Thirty-one patients (mean age 2.1+/-4.2years) with severe LV dilatation (mean indexed LVEDV 136+/-48ml/m2) and LV dysfunction (mean LV-EF 23+/-8%) were examined. LGE was detected in 5 of the 31 patients (16%) appearing in various patterns characterized as mid-wall (n=1), focal patchy (n=1), RV insertion site (n=1) and transmural (n=2). Based on histopathological analysis, 4 of the 5 LGE positive patients had lymphocytic myocarditis, whereas one patient was diagnosed with idiopathic DCM. CONCLUSIONS: In children and adolescents with DCM, focal histologically proven myocardial fibrosis is rarely detected by LGE CMR despite marked LV dilatation and severely depressed LV function. LGE occurred in various patterns and mostly in patients with inflammatory cardiomyopathy. It remains unclear whether myocardial fibrosis in childhood DCM reflects different endogenous repair mechanisms that enable favourable reverse remodelling. Larger trials are needed to assess the prognostic implications of LGE in childhood DCM.en
dc.rightsNamensnennung 3.0 International*
dc.subjectchildhood dilated cardiomyopathyen
dc.subjectmyocardial fibrosisen
dc.subjectlate gadolinium enhancementen
dc.subjectreverse ventricular remodellingen
dc.titleFocal myocardial fibrosis assessed by late gadolinium enhancement cardiovascular magnetic resonance in children and adolescents with dilated cardiomyopathyen
local.affiliationFB 11 - Medizinde_DE
local.opus.institutePediatric Heart Centrede_DE
local.source.freetextJournal of Cardiovascular Magnetic Resonance 17:34de_DE


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