PMID- 31046780 OWN - NLM STAT- MEDLINE DCOM- 20200128 LR - 20240117 IS - 1532-429X (Electronic) IS - 1097-6647 (Print) IS - 1097-6647 (Linking) VI - 21 IP - 1 DP - 2019 May 2 TI - Myocardial fibrosis by late gadolinium enhancement cardiovascular magnetic resonance in myotonic muscular dystrophy type 1: highly prevalent but not associated with surface conduction abnormality. PG - 26 LID - 10.1186/s12968-019-0535-6 [doi] LID - 26 AB - BACKGROUND: Conduction disease and arrhythmias represent a major cause of mortality in myotonic muscular dystrophy type 1 (MMD1). Permanent pacemaker (PPM) implantation is the cornerstone of therapy to reduce cardiovascular mortality in MMD1. Cardiovascular magnetic resonance (CMR) studies demonstrate a high prevalence of myocardial fibrosis in MMD1, however the association between CMR myocardial fibrosis with late gadolinium enhancement (CMR-LGE) and surface conduction abnormality is not well established in MMD1. We investigated whether myocardial fibrosis by CMR-LGE is associated with surface conduction abnormalities meeting criteria for PPM implantation according to current guidelines in a cohort of patients with genetically confirmed MMD1. METHODS: Patients with genetically confirmed MMD1 were retrospectively evaluated. 12-lead electrocardiography (ECG) performed within 6 months of CMR was necessary for inclusion. The severity and extent of MMD1 was quantified using a validated Muscular Impairment Rating Scale (MIRS). Based on current guidelines for device-based therapy of cardiac rhythm abnormalities, we defined surface conduction abnormality as the presence of ECG alterations meeting criteria for PPM implant (class I or II indications): PR interval > 200 ms (type I atrioventricular (AV) block) and/or mono or bifascicular block (QRS > 120 ms), or evidence of advanced AV block. Balanced steady-state free precession sequences (bSSFP) were used for assessment of left ventricular (LV) volumes and ejection fraction. MOdified Look-Locker Inversion Recovery (MOLLI) acquisition schemes were used to acquire T1 maps. Patients' charts were reviewed up to 12 months post-CMR for occurrence of PPM implantation. RESULTS: Fifty-two patients (38% male, 41 +/- 14 years) were included. Overall, 31 (60%) patients had a surface conduction abnormality and 22 (42%) demonstrated midwall myocardial fibrosis by CMR-LGE. After a median of 57 days from CMR exam, 15 patients (29%) underwent PPM implantation. Subjects with vs. without surface conduction abnormality had significantly longer disease length (15.5 vs. 7.8 years, p = 0.015) and higher disease severity on the MIRS scale (p = 0.041). High prevalence of myocardial fibrosis by CMR-LGE was detected in subjects with and without surface conduction abnormality with no significant difference between the two cohorts (42% vs. 43%, p = 0.999). By multivariate logistic regression analysis, disease length was the only independent variable associated with surface conduction abnormality (OR 1.071, 95%CI 1.003-1.144, p = 0.040); while CMR-LGE was not associated with conduction abnormality (rho = - 0.009, p = 0.949). CONCLUSIONS: Myocardial fibrosis by CMR-LGE is highly prevalent in MMD1 but not related to surface conduction abnormality meeting current guideline criteria for PPM implantation . FAU - Cardona, Andrea AU - Cardona A AD - Division of Cardiovascular Medicine, The Ohio State University Wexner Medical Center, 473 W 12th Ave, Suite 200, Columbus, OH 43210, USA. AD - Division of Cardiology, University of Perugia, Rome, Italy. FAU - Arnold, William D AU - Arnold WD AD - Division of Neuromuscular Disorders, The Ohio State University Wexner Medical Center, Columbus, OH, USA. FAU - Kissel, John T AU - Kissel JT AD - Division of Neuromuscular Disorders, The Ohio State University Wexner Medical Center, Columbus, OH, USA. FAU - Raman, Subha V AU - Raman SV AD - Division of Cardiovascular Medicine, The Ohio State University Wexner Medical Center, 473 W 12th Ave, Suite 200, Columbus, OH 43210, USA. FAU - Zareba, Karolina M AU - Zareba KM AUID- ORCID: 0000-0002-4891-4106 AD - Division of Cardiovascular Medicine, The Ohio State University Wexner Medical Center, 473 W 12th Ave, Suite 200, Columbus, OH 43210, USA. kzarebamd@gmail.com. LA - eng GR - L30 HL134084/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20190502 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) SB - IM MH - Adult MH - Arrhythmias, Cardiac/diagnosis/*epidemiology/therapy MH - Cardiac Pacing, Artificial MH - Cardiomyopathies/*diagnostic imaging/epidemiology/pathology MH - Contrast Media/*administration & dosage MH - Female MH - Fibrosis MH - Humans MH - *Magnetic Resonance Imaging, Cine MH - Male MH - Middle Aged MH - Myocardium/*pathology MH - Myotonic Dystrophy/diagnosis/*epidemiology/genetics MH - Ohio/epidemiology MH - Predictive Value of Tests MH - Prevalence MH - Retrospective Studies MH - Risk Factors PMC - PMC6498496 OTO - NOTNLM OT - Cardiovascular magnetic resonance OT - Electrocardiogram OT - Late gadolinium enhancement OT - Myocardial fibrosis OT - Myotonic muscular dystrophy OT - Pacemaker COIS- AUTHORS' INFORMATION: No third parties were involved in the authorship of review of the manuscript. CONSENT FOR PUBLICATION: Not applicable. All data presented in this article is non-identifiable. COMPETING INTERESTS: The authors have declared 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- 2019/05/03 06:00 MHDA- 2020/01/29 06:00 PMCR- 2019/05/02 CRDT- 2019/05/04 06:00 PHST- 2018/06/15 00:00 [received] PHST- 2019/03/26 00:00 [accepted] PHST- 2019/05/04 06:00 [entrez] PHST- 2019/05/03 06:00 [pubmed] PHST- 2020/01/29 06:00 [medline] PHST- 2019/05/02 00:00 [pmc-release] AID - S1097-6647(23)00199-0 [pii] AID - 535 [pii] AID - 10.1186/s12968-019-0535-6 [doi] PST - epublish SO - J Cardiovasc Magn Reson. 2019 May 2;21(1):26. doi: 10.1186/s12968-019-0535-6.