PMID- 27363857 OWN - NLM STAT- MEDLINE DCOM- 20170509 LR - 20180912 IS - 1942-0080 (Electronic) IS - 1941-9651 (Linking) VI - 9 IP - 7 DP - 2016 Jul TI - Cardiac Involvement in Myotonic Dystrophy Type 2 Patients With Preserved Ejection Fraction: Detection by Cardiovascular Magnetic Resonance. LID - e004615 [pii] LID - 10.1161/CIRCIMAGING.115.004615 [doi] AB - BACKGROUND: Myotonic dystrophy type 2 (DM2) is a genetic disorder characterized by skeletal muscle symptoms, metabolic changes, and cardiac involvement. Histopathologic alterations of the skeletal muscle include fibrosis and fatty infiltration. The aim of this study was to investigate whether subclinical cardiac involvement in DM2 is already detectable in preserved left ventricular function by cardiovascular magnetic resonance. METHODS AND RESULTS: Twenty-seven patients (mean age, 54+/-10 years; 20 females) with a genetically confirmed diagnosis of DM2 were compared with 17 healthy age- and sex-matched controls using a 1.5 T magnetic resonance imaging. For myocardial tissue differentiation, T1 and T2 mapping, fat/water-separated imaging, focal fibrosis imaging (late gadolinium enhancement [LGE]), and (1)H magnetic resonance spectroscopy were performed. Extracellular volume fraction was calculated. Conduction abnormalities were diagnosed based on Groh criteria. LGE located subepicardial basal inferolateral was detectable in 22% of the patients. Extracellular volume was increased in this region and in the adjacent medial inferolateral segment (P=0.03 compared with healthy controls). In 21% of patients with DM2, fat deposits were detectable (all women). The control group showed no abnormalities. Myocardial triglycerides were not different in LGE-positive and LGE-negative subjects (P=0.47). Six patients had indicators for conduction disease (60% of LGE-positive patients and 12.5% of LGE-negative patients). CONCLUSIONS: In DM2, subclinical myocardial injury was already detectable in preserved left ventricular ejection fraction. Extracellular volume was also increased in regions with no focal fibrosis. Myocardial fibrosis was related to conduction abnormalities. CI - (c) 2016 American Heart Association, Inc. FAU - Schmacht, Luisa AU - Schmacht L AD - From the Working Group on Cardiovascular Magnetic Resonance, Experimental, and Clinical Research Center, a joint cooperation between the Charite University Medicine Berlin and the Max-Delbrueck Center for Molecular Medicine, and HELIOS Klinikum Berlin Buch, Department of Cardiology and Nephrology, Germany (L.S., J.T., W.U., M.A.D., E.B., F.v.K.-B., J.S.-M.); DZHK (German Center for Cardiovascular Research), partner site Berlin, Germany (L.S., F.v.K.-B, J.S.-M.); Muscle Research Unit, Experimental and Clinical Research Center, a joint cooperation between the Charite Medical Faculty and the Max-Delbrueck Center for Molecular Medicine, Berlin, Germany (U.G., S.S.); and Laboratory of Cardiac Energetics, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.). FAU - Traber, Julius AU - Traber J AD - From the Working Group on Cardiovascular Magnetic Resonance, Experimental, and Clinical Research Center, a joint cooperation between the Charite University Medicine Berlin and the Max-Delbrueck Center for Molecular Medicine, and HELIOS Klinikum Berlin Buch, Department of Cardiology and Nephrology, Germany (L.S., J.T., W.U., M.A.D., E.B., F.v.K.-B., J.S.-M.); DZHK (German Center for Cardiovascular Research), partner site Berlin, Germany (L.S., F.v.K.-B, J.S.-M.); Muscle Research Unit, Experimental and Clinical Research Center, a joint cooperation between the Charite Medical Faculty and the Max-Delbrueck Center for Molecular Medicine, Berlin, Germany (U.G., S.S.); and Laboratory of Cardiac Energetics, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.). FAU - Grieben, Ulrike AU - Grieben U AD - From the Working Group on Cardiovascular Magnetic Resonance, Experimental, and Clinical Research Center, a joint cooperation between the Charite University Medicine Berlin and the Max-Delbrueck Center for Molecular Medicine, and HELIOS Klinikum Berlin Buch, Department of Cardiology and Nephrology, Germany (L.S., J.T., W.U., M.A.D., E.B., F.v.K.-B., J.S.-M.); DZHK (German Center for Cardiovascular Research), partner site Berlin, Germany (L.S., F.v.K.-B, J.S.-M.); Muscle Research Unit, Experimental and Clinical Research Center, a joint cooperation between the Charite Medical Faculty and the Max-Delbrueck Center for Molecular Medicine, Berlin, Germany (U.G., S.S.); and Laboratory of Cardiac Energetics, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.). FAU - Utz, Wolfgang AU - Utz W AD - From the Working Group on Cardiovascular Magnetic Resonance, Experimental, and Clinical Research Center, a joint cooperation between the Charite University Medicine Berlin and the Max-Delbrueck Center for Molecular Medicine, and HELIOS Klinikum Berlin Buch, Department of Cardiology and Nephrology, Germany (L.S., J.T., W.U., M.A.D., E.B., F.v.K.-B., J.S.-M.); DZHK (German Center for Cardiovascular Research), partner site Berlin, Germany (L.S., F.v.K.-B, J.S.-M.); Muscle Research Unit, Experimental and Clinical Research Center, a joint cooperation between the Charite Medical Faculty and the Max-Delbrueck Center for Molecular Medicine, Berlin, Germany (U.G., S.S.); and Laboratory of Cardiac Energetics, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.). FAU - Dieringer, Matthias A AU - Dieringer MA AD - From the Working Group on Cardiovascular Magnetic Resonance, Experimental, and Clinical Research Center, a joint cooperation between the Charite University Medicine Berlin and the Max-Delbrueck Center for Molecular Medicine, and HELIOS Klinikum Berlin Buch, Department of Cardiology and Nephrology, Germany (L.S., J.T., W.U., M.A.D., E.B., F.v.K.-B., J.S.-M.); DZHK (German Center for Cardiovascular Research), partner site Berlin, Germany (L.S., F.v.K.-B, J.S.-M.); Muscle Research Unit, Experimental and Clinical Research Center, a joint cooperation between the Charite Medical Faculty and the Max-Delbrueck Center for Molecular Medicine, Berlin, Germany (U.G., S.S.); and Laboratory of Cardiac Energetics, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.). FAU - Kellman, Peter AU - Kellman P AD - From the Working Group on Cardiovascular Magnetic Resonance, Experimental, and Clinical Research Center, a joint cooperation between the Charite University Medicine Berlin and the Max-Delbrueck Center for Molecular Medicine, and HELIOS Klinikum Berlin Buch, Department of Cardiology and Nephrology, Germany (L.S., J.T., W.U., M.A.D., E.B., F.v.K.-B., J.S.-M.); DZHK (German Center for Cardiovascular Research), partner site Berlin, Germany (L.S., F.v.K.-B, J.S.-M.); Muscle Research Unit, Experimental and Clinical Research Center, a joint cooperation between the Charite Medical Faculty and the Max-Delbrueck Center for Molecular Medicine, Berlin, Germany (U.G., S.S.); and Laboratory of Cardiac Energetics, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.). FAU - Blaszczyk, Edyta AU - Blaszczyk E AD - From the Working Group on Cardiovascular Magnetic Resonance, Experimental, and Clinical Research Center, a joint cooperation between the Charite University Medicine Berlin and the Max-Delbrueck Center for Molecular Medicine, and HELIOS Klinikum Berlin Buch, Department of Cardiology and Nephrology, Germany (L.S., J.T., W.U., M.A.D., E.B., F.v.K.-B., J.S.-M.); DZHK (German Center for Cardiovascular Research), partner site Berlin, Germany (L.S., F.v.K.-B, J.S.-M.); Muscle Research Unit, Experimental and Clinical Research Center, a joint cooperation between the Charite Medical Faculty and the Max-Delbrueck Center for Molecular Medicine, Berlin, Germany (U.G., S.S.); and Laboratory of Cardiac Energetics, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.). FAU - von Knobelsdorff-Brenkenhoff, Florian AU - von Knobelsdorff-Brenkenhoff F AD - From the Working Group on Cardiovascular Magnetic Resonance, Experimental, and Clinical Research Center, a joint cooperation between the Charite University Medicine Berlin and the Max-Delbrueck Center for Molecular Medicine, and HELIOS Klinikum Berlin Buch, Department of Cardiology and Nephrology, Germany (L.S., J.T., W.U., M.A.D., E.B., F.v.K.-B., J.S.-M.); DZHK (German Center for Cardiovascular Research), partner site Berlin, Germany (L.S., F.v.K.-B, J.S.-M.); Muscle Research Unit, Experimental and Clinical Research Center, a joint cooperation between the Charite Medical Faculty and the Max-Delbrueck Center for Molecular Medicine, Berlin, Germany (U.G., S.S.); and Laboratory of Cardiac Energetics, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.). FAU - Spuler, Simone AU - Spuler S AD - From the Working Group on Cardiovascular Magnetic Resonance, Experimental, and Clinical Research Center, a joint cooperation between the Charite University Medicine Berlin and the Max-Delbrueck Center for Molecular Medicine, and HELIOS Klinikum Berlin Buch, Department of Cardiology and Nephrology, Germany (L.S., J.T., W.U., M.A.D., E.B., F.v.K.-B., J.S.-M.); DZHK (German Center for Cardiovascular Research), partner site Berlin, Germany (L.S., F.v.K.-B, J.S.-M.); Muscle Research Unit, Experimental and Clinical Research Center, a joint cooperation between the Charite Medical Faculty and the Max-Delbrueck Center for Molecular Medicine, Berlin, Germany (U.G., S.S.); and Laboratory of Cardiac Energetics, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.). FAU - Schulz-Menger, Jeanette AU - Schulz-Menger J AD - From the Working Group on Cardiovascular Magnetic Resonance, Experimental, and Clinical Research Center, a joint cooperation between the Charite University Medicine Berlin and the Max-Delbrueck Center for Molecular Medicine, and HELIOS Klinikum Berlin Buch, Department of Cardiology and Nephrology, Germany (L.S., J.T., W.U., M.A.D., E.B., F.v.K.-B., J.S.-M.); DZHK (German Center for Cardiovascular Research), partner site Berlin, Germany (L.S., F.v.K.-B, J.S.-M.); Muscle Research Unit, Experimental and Clinical Research Center, a joint cooperation between the Charite Medical Faculty and the Max-Delbrueck Center for Molecular Medicine, Berlin, Germany (U.G., S.S.); and Laboratory of Cardiac Energetics, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (P.K.). Jeanette.schulz-menger@charite.de. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Circ Cardiovasc Imaging JT - Circulation. Cardiovascular imaging JID - 101479935 RN - 0 (Triglycerides) SB - IM CIN - Circ Cardiovasc Imaging. 2016 Jul;9(7):. PMID: 27363858 MH - Adult MH - Arrhythmias, Cardiac/etiology/physiopathology MH - Asymptomatic Diseases MH - Cardiomyopathies/*diagnostic imaging/etiology/metabolism/physiopathology MH - Case-Control Studies MH - Electrocardiography MH - Female MH - Fibrosis MH - Heart Rate MH - Humans MH - *Magnetic Resonance Imaging, Cine MH - Male MH - Middle Aged MH - Myocardium/chemistry/pathology MH - Myotonic Dystrophy/*complications/diagnostic imaging MH - Predictive Value of Tests MH - Prospective Studies MH - Proton Magnetic Resonance Spectroscopy MH - *Stroke Volume MH - Triglycerides/analysis MH - *Ventricular Function, Left OTO - NOTNLM OT - arrythmias, cardiac OT - cardiomyopathy OT - fibrosis OT - magnetic resonance imaging OT - myotonic dystrophies OT - triglycerides EDAT- 2016/07/02 06:00 MHDA- 2017/05/10 06:00 CRDT- 2016/07/02 06:00 PHST- 2015/11/30 00:00 [received] PHST- 2016/05/24 00:00 [accepted] PHST- 2016/07/02 06:00 [entrez] PHST- 2016/07/02 06:00 [pubmed] PHST- 2017/05/10 06:00 [medline] AID - CIRCIMAGING.115.004615 [pii] AID - 10.1161/CIRCIMAGING.115.004615 [doi] PST - ppublish SO - Circ Cardiovasc Imaging. 2016 Jul;9(7):e004615. doi: 10.1161/CIRCIMAGING.115.004615.