PMID- 25976093 OWN - NLM STAT- MEDLINE DCOM- 20160411 LR - 20240117 IS - 1532-429X (Electronic) IS - 1097-6647 (Print) IS - 1097-6647 (Linking) VI - 17 IP - 1 DP - 2015 May 15 TI - Focal myocardial fibrosis assessed by late gadolinium enhancement cardiovascular magnetic resonance in children and adolescents with dilated cardiomyopathy. PG - 34 LID - 10.1186/s12968-015-0142-0 [doi] LID - 34 AB - BACKGROUND: 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 <18 years 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 +/- 4.2 years) with severe LV dilatation (mean indexed LVEDV 136 +/- 48 ml/m(2)) 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. FAU - Latus, Heiner AU - Latus H AD - Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. heiner.latus@googlemail.com. FAU - Gummel, Kerstin AU - Gummel K AD - Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. kerstin.gummel@web.de. FAU - Klingel, Karin AU - Klingel K AD - Department of Molecular Pathology, University Hospital Tubingen, Tubingen, Germany. karin.klingel@med.uni-tuebingen.de. FAU - Moysich, Axel AU - Moysich A AD - Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. axel.moysich@paediat.med.uni-giessen.de. FAU - Khalil, Markus AU - Khalil M AD - Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. markus.khalil@paediat.med.uni-giessen.de. FAU - Mazhari, Nona AU - Mazhari N AD - Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. nona.mazhari@paediat.med.uni-giessen.de. FAU - Bauer, Juergen AU - Bauer J AD - Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. juergen.bauer@paediat.med.uni-giessen.de. FAU - Kandolf, Reinhard AU - Kandolf R AD - Department of Molecular Pathology, University Hospital Tubingen, Tubingen, Germany. reinhard.kandolf@med.uni-tuebingen.de. FAU - Schranz, Dietmar AU - Schranz D AD - Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. dietmar.schranz@paediat.med.uni-giessen.de. FAU - Apitz, Christian AU - Apitz C AD - Pediatric Heart Centre, University Children's Hospital Giessen, Feulgenstr. 12, D-35392, Giessen, Germany. capitz@aol.com. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150515 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 - Age Factors MH - Biopsy MH - Cardiac Catheterization MH - Cardiomyopathy, Dilated/complications/*diagnosis/pathology/physiopathology/surgery MH - Child MH - Child, Preschool MH - Contrast Media/*administration & dosage MH - Female MH - Fibrosis MH - Gadolinium DTPA/*administration & dosage MH - Heart Failure/diagnosis/etiology/surgery MH - Heart Transplantation MH - Humans MH - Hypertrophy, Left Ventricular/diagnosis/etiology MH - Infant MH - Infant, Newborn MH - *Magnetic Resonance Imaging, Cine MH - Male MH - Myocardium/*pathology MH - Predictive Value of Tests MH - Retrospective Studies MH - Severity of Illness Index MH - Ventricular Dysfunction, Left/diagnosis/etiology MH - Ventricular Function, Left MH - Ventricular Remodeling PMC - PMC4432888 EDAT- 2015/05/16 06:00 MHDA- 2016/04/12 06:00 PMCR- 2015/05/15 CRDT- 2015/05/16 06:00 PHST- 2015/01/30 00:00 [received] PHST- 2015/05/01 00:00 [accepted] PHST- 2015/05/16 06:00 [entrez] PHST- 2015/05/16 06:00 [pubmed] PHST- 2016/04/12 06:00 [medline] PHST- 2015/05/15 00:00 [pmc-release] AID - S1097-6647(23)00859-1 [pii] AID - 142 [pii] AID - 10.1186/s12968-015-0142-0 [doi] PST - epublish SO - J Cardiovasc Magn Reson. 2015 May 15;17(1):34. doi: 10.1186/s12968-015-0142-0.