PMID- 30848402 OWN - NLM STAT- MEDLINE DCOM- 20190422 LR - 20200225 IS - 1875-8312 (Electronic) IS - 1569-5794 (Print) IS - 1569-5794 (Linking) VI - 35 IP - 2 DP - 2019 Feb TI - Myocardial tissue characterization in patients with hereditary gelsolin (AGel) amyloidosis using novel cardiovascular magnetic resonance techniques. PG - 351-358 LID - 10.1007/s10554-019-01570-4 [doi] AB - Gelsolin (AGel) amyloidosis is a hereditary condition with common neurological effects. Myocardial involvement, especially strain, T1, or extracellular volume (ECV), in this disease has not been investigated before. Local myocardial effects and possible amyloid accumulation were the targets of interest in this study. Fifty patients with AGel amyloidosis were enrolled in the study. All patients underwent cardiovascular magnetic resonance imaging, including cine imaging, T1 mapping, tagging, and late gadolinium enhancement (LGE) imaging at 1.5 T. Results for volumetry, myocardial feature-tracking strain, rotation, torsion, native T1, ECV, and LGE were investigated. The population mean native T1 values in different segments of the left ventricle (LV) varied between 1003 and 1080 ms. Myocardial mean T1 was 1031 +/- 37 ms. T1 was highest in the basal plane of the LV (1055 +/- 40 ms), similarly to ECV (30.0% +/- 4.4%). ECV correlated with native T1 in all LV segments (p < 0.005). Basal LGE was detected in 76% of patients, and mid-ventricular LGE in 32%. LV longitudinal strain was impaired (- 17.4% +/- 2.6%), significantly decreasing apical rotation (p = 0.018) and concurrently myocardial torsion (p = 0.005). LV longitudinal strain correlated with mean T1 and ECV of different LV planes (p < 0.04; basal p < 0.01). Myocardial involvement in AGel amyloidosis is significant, but the effects are local, focusing on the basal plane of the LV. FAU - Lehmonen, Lauri AU - Lehmonen L AUID- ORCID: 0000-0002-7523-3867 AD - HUS Medical Imaging Center, Radiology, University of Helsinki and Helsinki University Hospital, Haartmaninkatu 4, PO Box 340, 00029 HUS, Helsinki, Finland. lauri.lehmonen@helsinki.fi. AD - Department of Physics, University of Helsinki, Helsinki, Finland. lauri.lehmonen@helsinki.fi. FAU - Kaasalainen, Touko AU - Kaasalainen T AD - HUS Medical Imaging Center, Radiology, University of Helsinki and Helsinki University Hospital, Haartmaninkatu 4, PO Box 340, 00029 HUS, Helsinki, Finland. FAU - Atula, Sari AU - Atula S AD - Clinical Neurosciences, Neurology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland. FAU - Mustonen, Tuuli AU - Mustonen T AD - Faculty of Medicine, University of Helsinki, Helsinki, Finland. FAU - Holmstrom, Miia AU - Holmstrom M AD - HUS Medical Imaging Center, Radiology, University of Helsinki and Helsinki University Hospital, Haartmaninkatu 4, PO Box 340, 00029 HUS, Helsinki, Finland. LA - eng PT - Journal Article DEP - 20190308 PL - United States TA - Int J Cardiovasc Imaging JT - The international journal of cardiovascular imaging JID - 100969716 RN - 0 (Gelsolin) RN - Meretoja syndrome SB - IM MH - Amyloidosis/*diagnostic imaging/genetics/pathology/physiopathology MH - Biomechanical Phenomena MH - Cardiomyopathies/*diagnostic imaging/genetics/pathology/physiopathology MH - Corneal Dystrophies, Hereditary/*diagnostic imaging/genetics/pathology/physiopathology MH - Gelsolin/genetics MH - Genetic Predisposition to Disease MH - Humans MH - *Magnetic Resonance Imaging, Cine MH - Mutation MH - *Myocardial Contraction MH - Myocardium/pathology MH - Phenotype MH - Predictive Value of Tests MH - Prospective Studies MH - Registries MH - Stroke Volume MH - *Ventricular Function, Left MH - Ventricular Function, Right PMC - PMC6428907 OTO - NOTNLM OT - Amyloidosis OT - Cardiovascular magnetic resonance OT - Extracellular volume OT - Feature tracking OT - T1 mapping OT - Tagging COIS- CONFLICT OF INTEREST: The authors declare that they have no conflict of interest. ETHICAL APPROVAL: The study protocol was approved by the local ethics committee. All participants gave their written consent prior to their inclusion in the study. EDAT- 2019/03/09 06:00 MHDA- 2019/04/23 06:00 PMCR- 2019/03/08 CRDT- 2019/03/09 06:00 PHST- 2018/11/30 00:00 [received] PHST- 2019/02/21 00:00 [accepted] PHST- 2019/03/09 06:00 [pubmed] PHST- 2019/04/23 06:00 [medline] PHST- 2019/03/09 06:00 [entrez] PHST- 2019/03/08 00:00 [pmc-release] AID - 10.1007/s10554-019-01570-4 [pii] AID - 1570 [pii] AID - 10.1007/s10554-019-01570-4 [doi] PST - ppublish SO - Int J Cardiovasc Imaging. 2019 Feb;35(2):351-358. doi: 10.1007/s10554-019-01570-4. Epub 2019 Mar 8.