This international multidisciplinary document is intended to guide electrophysiologists, cardiologists, other clinicians, and health care professionals in caring for patients with arrhythmic... Show moreThis international multidisciplinary document is intended to guide electrophysiologists, cardiologists, other clinicians, and health care professionals in caring for patients with arrhythmic complications of neuromuscular disorders (NMDs). The document presents an overview of arrhythmias in NMDs followed by detailed sections on specific disorders: Duchenne muscular dystrophy, Becker muscular dystrophy, and limb-girdle muscular dystrophy type 2; myotonic dystrophy type 1 and type 2; Emery-Dreifuss muscular dystrophy and limb-girdle muscular dystrophy type 1B; facioscapulohumeral muscular dystrophy; and mitochondrial myopathies, including Friedreich ataxia and Kearns-Sayre syndrome, with an emphasis on managing arrhythmic cardiac manifestations. Endof-life management of arrhythmias in patients with NMDs is also covered. The document sections were drafted by the writing committee members according to their area of expertise. The recommendations represent the consensus opinion of the expert writing group, graded by class of recommendation and level of evidence utilizing defined criteria. The recommendations were made available for public comment; the document underwent review by the Heart Rhythm Society Scientific and Clinical Documents Committee and external review and endorsement by the partner and collaborating societies. Changes were incorporated based on these reviews. By using a breadth of accumulated available evidence, the document is designed to provide practical and actionable clinical information and recommendations for the diagnosis and management of arrhythmias and thus improve the care of patients with NMDs. Show less
Turkbey, E.B.; Backlund, J.Y.C.; Gai, N.; Nacif, M.; Geest, R.J. van der; Lachin, J.M.; ... ; DCCT/EDIC Reseach Group 2022
Alterations in myocardial structure, function, tissue composition (e.g., fibrosis) may be associated with metabolic syndrome (MetS). This study aimed to determine the relation of MetS and its... Show moreAlterations in myocardial structure, function, tissue composition (e.g., fibrosis) may be associated with metabolic syndrome (MetS). This study aimed to determine the relation of MetS and its individual components to markers of cardiovascular disease in patients with type 1 Diabetes Mellitus (T1DM). A total of 978 subjects of the Diabetes Control and Complications Trial/Epidemiology of Diabetes Interventions and Complications T1DM cohort (age: 49 +/- 7 years, 47% female, DM duration 28 +/- 5 years) underwent cardiovascular magnetic resonance. In a subset of 200 patients, myocardial tissue composition was measured with cardiovascular magnetic resonance T1 mapping after contrast administration. MetS was defined as T1DM plus 2 other abnormalities based on the American Heart Association/National Cholesterol Education Program criteria. MetS was present in 34.1% of subjects. After adjustment for age, height, scanner, study cohort, gender, smoking, mean glycated hemoglobin levels, history of macroalbuminuria and end-stage renal disease, left ventricle mass was greater by 12.3 g, end-diastolic volume was higher by 5.4 ml, and mass to end-diastolic volume ratio was higher by 5% in patients with MetS versus those without MetS (p<0.001 for all). Myocardial T1 times were lower by 29ms in patients with MetS than those without (p<0.001). Elevated waist circumference showed the strongest associations with left ventricle mass (+10.1 g), end-diastolic volume (+6.7 ml), and lower myocardial T1 times (+31 ms) in patients with MetS compared with those without (p<0.01). In conclusion, in a large cohort of patients with T1DM, 34.1% of subjects met MetS criteria. MetS was associated with adverse myocardial structural remodeling and change in myocardial tissue composition. (C) 2022 Elsevier Inc. All rights reserved. Show less
Park, J.; Desjardins, B.; Liang, J.J.; Zghaib, T.; Xie, S.L.; Lucena-Padros, I.; ... ; Nazarian, S. 2020
Introduction The association of late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) with epicardial and surface ventricular tachycardia (VT) electrogram features, in nonischemic... Show moreIntroduction The association of late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) with epicardial and surface ventricular tachycardia (VT) electrogram features, in nonischemic cardiomyopathy (NICM), is unknown. We sought to define the association of LGE and viable wall thickness with epicardial electrogram features and exit site paced QRS duration in patients with NICM. Methods A total of 19 patients (age 53.5 +/- 11.5 years) with NICM (ejection fraction 40.2 +/- 13.2%) underwent CMR before VT ablation. LGE transmurality was quantified on CMR and coregistered with 2294 endocardial and 2724 epicardial map points. Results Both bipolar and unipolar voltage were associated with transmural signal intensity on CMR. Longer electrogram duration and fractionated potentials were associated with increased LGE transmurality, but late potentials or local abnormal ventricular activity were more prevalent in nontransmural versus transmural LGE regions (p < .05). Of all critical VT sites, 19% were located adjacent to regions with LGE but normal bipolar and unipolar voltage. Exit site QRS duration was affected by LGE transmurality and intramural scar location, but not by wall thickness, at the impulse origin. Conclusions In patients with NICM and VT, LGE is associated with epicardial electrogram features and may predict critical VT sites. Additionally, exit site QRS duration is affected by LGE transmurality and intramural location at the impulse origin or exit. Show less
Xie, S.L.; Kubala, M.; Liang, J.J.; Yang, J.D.; Desjardins, B.; Santangeli, P.; ... ; Nazarian, S. 2019
OBJECTIVES This study hypothesized that paroxysmal atrial fibrillation (PAF) reflects the presence of a more severe cardiac hypertrophic cardiomyopathy (HCM) phenotype.BACKGROUND HCM is... Show moreOBJECTIVES This study hypothesized that paroxysmal atrial fibrillation (PAF) reflects the presence of a more severe cardiac hypertrophic cardiomyopathy (HCM) phenotype.BACKGROUND HCM is characterized by myocyte hypertrophy, fibrosis, and a high prevalence of PAF. It is currently unresolved whether atrial fibrillation (AF) is a marker or a mediator of adverse outcomes in HCM.METHODS This study retrospectively examined 45 HCM patients who underwent cardiovascular magnetic resonance (CMR) imaging in sinus rhythm. The function of all 4 cardiac chambers was assessed, as well as late gadolinium enhancement (LGE) in the left atrium (LA) and left ventricle (LV), as indicators of fibrosis. A fat-saturated, 3-dimensional inversion recovery-prepared, fast-spoiled, gradient-recalled echo sequence, and the image intensity ratio method were used to measure LA-LGE; LGE in the LV was quantified using a semi-automated threshold technique.RESULTS HCM patients (n = 45) were divided into 2 groups (PAF, no AF) based on history of PAF. All HCM patients had LGE in the LA posterior wall. The PAF group (n = 18) had higher LA volume, a lower LA ejection fraction, a lower global peak longitudinal LA strain (PLAS), and a higher amount of LA-LGE compared with the no AF group (n = 27). A modest inverse association was noted between the LA ejection fraction, PLAS, and LA-LGE; a positive association was present between LV-LGE and LA-LGE. The PAF group had lower ejection fractions in the LV, right atrium, and right ventricle compared with those in the no AF group.CONCLUSIONS PAF is associated with a greater degree of structural LA remodeling and global myopathy, which suggests a more severe cardiac HCM phenotype. (c) 2019 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). Show less
Quantitative T-1 mapping of delayed gadolinium-enhanced cardiac magnetic resonance imaging has shown promise in identifying diffuse myocardial fibrosis. Despite careful control of magnetic... Show moreQuantitative T-1 mapping of delayed gadolinium-enhanced cardiac magnetic resonance imaging has shown promise in identifying diffuse myocardial fibrosis. Despite careful control of magnetic resonance imaging parameters, comparison of T-1 times between different patients may be problematic because of patient specific factors such as gadolinium dose, differing glomerular filtration rates, and patient specific delay times. In this work, a model driven approach to account for variations between patients to allow for comparison of T-1 data is provided. Kinetic model parameter values were derived from healthy volunteer time-contrast curves. Correction values for the factors described above were used to normalize T-1 values to a matched state. Examples of pre- and postcorrected values for a pool of normal subjects and in a patient cohort of type 1 diabetic patients shows tighter clustering and improved discrimination of disease state. Magn Reson Med 65:1407-1415, 2011. (C) 2010 Wiley-Liss, Inc. Show less
