PMID- 29284499 OWN - NLM STAT- MEDLINE DCOM- 20190314 LR - 20240117 IS - 1532-429X (Electronic) IS - 1097-6647 (Print) IS - 1097-6647 (Linking) VI - 19 IP - 1 DP - 2017 Dec 28 TI - Left Atrial structure and function in hypertrophic cardiomyopathy sarcomere mutation carriers with and without left ventricular hypertrophy. PG - 107 LID - 10.1186/s12968-017-0420-0 [doi] LID - 107 AB - BACKGROUND: Impaired left atrial (LA) function is an early marker of cardiac dysfunction and predictor of adverse cardiac events. Herein, we assess LA structure and function in hypertrophy in hypertrophic cardiomyopathy (HCM) sarcomere mutation carriers with and without left ventricular hypertrophy (LVH). METHOD: Seventy-three participants of the HCMNet study who underwent cardiovascular magnetic resonance (CMR) imaging were studied, including mutation carriers with overt HCM (n = 34), preclinical mutation carriers without HCM (n = 24) and healthy, familial controls (n = 15). RESULTS: LA volumes were similar between preclinical, control and overt HCM cohorts after covariate adjustment. However, there was evidence of impaired LA function with decreased LA total emptying function in both preclinical (64 +/- 8%) and overt HCM (59 +/- 10%), compared with controls (70 +/- 7%; p = 0.002 and p = 0.005, respectively). LA passive emptying function was also decreased in overt HCM (35 +/- 11%) compared with controls (47 +/- 10%; p = 0.006). Both LAtotal emptying function and LA passive emptying function were inversely correlated with the extent of late gadolinium enhancement (LGE; p = 0.005 and p < 0.05, respectively), LV mass (p = 0.02 and p < 0.001) and interventricular septal thickness (p < 0.001 for both) and serum NT-proBNP levels (p < 0.001 for both). CONCLUSION: LA dysfunction is detectable by CMR in preclinical HCM mutation carriers despite non-distinguishable LV wall thickness and LA volume. LA function appears most impaired in subjects with overt HCM and a greater extent of LV fibrosis. FAU - Farhad, Hoshang AU - Farhad H AD - Non-Invasive Cardiovascular Imaging Program and the Cardiovascular Division, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, 75 Francis St, Boston, MA, 02115, USA. FAU - Seidelmann, Sara B AU - Seidelmann SB AD - Non-Invasive Cardiovascular Imaging Program and the Cardiovascular Division, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, 75 Francis St, Boston, MA, 02115, USA. FAU - Vigneault, Davis AU - Vigneault D AD - Radiology and Imaging Sciences, National Institutes of Health, Bethesda, MD, USA. FAU - Abbasi, Siddique A AU - Abbasi SA AD - Non-Invasive Cardiovascular Imaging Program and the Cardiovascular Division, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, 75 Francis St, Boston, MA, 02115, USA. FAU - Yang, Eunice AU - Yang E AD - Department of Cardiology, Johns Hopkins School of Medicine, Baltimore, MD, USA. FAU - Day, Sharlene M AU - Day SM AD - Departments of Internal Medicine and Pediatrics, University of Michigan, Ann Arbor, MI, USA. FAU - Colan, Steven D AU - Colan SD AD - Department of Cardiology, Boston Children's Hospital, Boston, MA, USA. FAU - Russell, Mark W AU - Russell MW AD - Departments of Internal Medicine and Pediatrics, University of Michigan, Ann Arbor, MI, USA. FAU - Towbin, Jeffrey AU - Towbin J AD - The Heart Institute and Pediatric Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. FAU - Sherrid, Mark V AU - Sherrid MV AD - New York University Langone Medical Center, New York, NY, USA. FAU - Canter, Charles E AU - Canter CE AD - Department of Pediatrics, Washington University School of Medicine, St. Louis, MO, USA. FAU - Shi, Ling AU - Shi L AD - New England Research Institutes, Watertown, MA, USA. FAU - Jerosch-Herold, Michael AU - Jerosch-Herold M AD - Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA. FAU - Bluemke, David A AU - Bluemke DA AD - Radiology and Imaging Sciences, National Institutes of Health, Bethesda, MD, USA. FAU - Ho, Carolyn AU - Ho C AD - Non-Invasive Cardiovascular Imaging Program and the Cardiovascular Division, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, 75 Francis St, Boston, MA, 02115, USA. cho@partners.org. FAU - Neilan, Tomas G AU - Neilan TG AD - Cardiac MR PET CT Program, Division of Cardiology and Department of Radiology, Massachusetts General Hospital, Boston, USA. tneilan@mgh.harvard.edu. AD - Division of Cardiology, Department of Medicine, Cardiac MR PET CT Program, Department of Radiology, Massachusetts General Hospital, 165 Cambridge St, Boston, MA, 02114, USA. tneilan@mgh.harvard.edu. LA - eng GR - T32 HL094301/HL/NHLBI NIH HHS/United States GR - 2T32HL094301-06/NH/NIH HHS/United States GR - R01 HL137562/HL/NHLBI NIH HHS/United States GR - 1P20HL101408/HL/NHLBI NIH HHS/United States GR - R01 HL130539/HL/NHLBI NIH HHS/United States GR - P30 AI060354/AI/NIAID NIH HHS/United States GR - P20 HL101408/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Observational Study PT - Research Support, N.I.H., Extramural PT - Research Support, N.I.H., Intramural PT - Research Support, Non-U.S. Gov't DEP - 20171228 PL - England TA - J Cardiovasc Magn Reson JT - Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance JID - 9815616 SB - IM MH - Adolescent MH - Adult MH - *Atrial Function, Left MH - Cardiomyopathy, Hypertrophic/complications/diagnostic imaging/*genetics/physiopathology MH - Case-Control Studies MH - Cross-Sectional Studies MH - DNA Mutational Analysis MH - Female MH - Fibrosis MH - Genetic Predisposition to Disease MH - Heart Atria/*diagnostic imaging/physiopathology MH - Humans MH - Hypertrophy, Left Ventricular/*diagnostic imaging/etiology/physiopathology MH - *Magnetic Resonance Imaging, Cine MH - Male MH - Middle Aged MH - *Mutation MH - Phenotype MH - Predictive Value of Tests MH - Sarcomeres/*genetics MH - Ventricular Function, Left MH - Ventricular Remodeling MH - Young Adult PMC - PMC5745877 OTO - NOTNLM OT - Cardiovascular magnetic resonance (CMR) OT - Hypertrophic cardiomyopathy OT - Left Atrial function COIS- ETHICS APPROVAL AND CONSENT TO PARTICIPATE: Institutional review boards (IRB) at each participating site (all sites's IRB/ethics boards listed in supplemental 1 approved the study protocol and all participants provided written informed consent or parental consent/ youth assent. CONSENT FOR PUBLICATION: Not applicable COMPETING INTERESTS: The authors declare that they have no competing interests. PUBLISHER'S NOTE: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. EDAT- 2017/12/30 06:00 MHDA- 2019/03/15 06:00 PMCR- 2017/12/28 CRDT- 2017/12/30 06:00 PHST- 2017/01/06 00:00 [received] PHST- 2017/11/27 00:00 [accepted] PHST- 2017/12/30 06:00 [entrez] PHST- 2017/12/30 06:00 [pubmed] PHST- 2019/03/15 06:00 [medline] PHST- 2017/12/28 00:00 [pmc-release] AID - S1097-6647(23)01141-9 [pii] AID - 420 [pii] AID - 10.1186/s12968-017-0420-0 [doi] PST - epublish SO - J Cardiovasc Magn Reson. 2017 Dec 28;19(1):107. doi: 10.1186/s12968-017-0420-0.