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
Zeppenfeld, K.; Tfelt-Hansen, J.; Riva, M. de; Winkel, B.G.; Behr, E.R.; Blom, N.A.; ... ; ESC Scientific Document Group 2022
Aims The 12-lead electrocardiogram (ECG) is routinely performed in children with hypertrophic cardiomyopathy (HCM). An ECG risk score has been suggested as a useful tool for risk stratification,... Show moreAims The 12-lead electrocardiogram (ECG) is routinely performed in children with hypertrophic cardiomyopathy (HCM). An ECG risk score has been suggested as a useful tool for risk stratification, but this has not been independently validated. This aim of this study was to describe the ECG phenotype of childhood HCM in a large, international, multi-centre cohort and investigate its role in risk prediction for arrhythmic events. Methods and results Data from 356 childhood HCM patients with a mean age of 10.1 years (+/- 4.5) were collected from a retrospective, multi-centre international cohort. Three hundred and forty-seven (97.5%) patients had ECG abnormalities at baseline, most commonly repolarization abnormalities (n = 277, 77.8%); left ventricular hypertrophy (n = 240, 67.7%); abnormal QRS axis (n = 126, 35.4%); or QT prolongation (n = 131, 36.8%). Over a median follow-up of 3.9 years (interquartile range 2.0-7.7), 25 (7%) had an arrhythmic event, with an overall annual event rate of 1.38 (95% CI 0.93-2.04). No ECG variables were associated with 5-year arrhythmic event on univariable or multivariable analysis. The ECG risk score threshold of >5 had modest discriminatory ability [C-index 0.60 (95% CI 0.484-0.715)], with corresponding negative and positive predictive values of 96.7% and 6.7% Conclusion In a large, international, multi-centre cohort of childhood HCM, ECG abnormalities were common and varied. No ECG characteristic, either in isolation or combined in the previously described ECG risk score, was associated with 5-year sudden cardiac death risk. This suggests that the role of baseline ECG phenotype in improving risk stratification in childhood HCM is limited. Show less
Although both ICD and CRT have proven to be an effective treatment strategy for selected patients in large clinical trials, many issues of the effects of defibrillator treatment in routine clinical... Show moreAlthough both ICD and CRT have proven to be an effective treatment strategy for selected patients in large clinical trials, many issues of the effects of defibrillator treatment in routine clinical practice remain unclear. In the current thesis, some of these unresolved questions are clarified based on data from a large cohort of ICD and CRT-D patients with long-term follow-up in routine clinical practice. In the first part the thesis was specifically focused on clinical aspects such as pocket related complications, the mode of death, ventricular arrhythmias and the suitability for subcutaneous device implantation in ICD recipients. In the second part the thesis was focused on socio-economic implications of ICD therapy such as the cost-effectiveness of ICD therapy, device longevity and an evidence-based approach regarding driving restrictions in ICD patients. In addition, the last chapter focused on the development and implementation of criteria that allow better identification of high risk patients and to limit the number of defibrillator implants in patients who will not benefit Show less
Maury, P.; Baratto, F.; Zeppenfeld, K.; Klein, G.; Delacretaz, E.; Sacher, F.; ... ; Chillou, C. de 2014
On one hand, clinicians have expressed concern that the number-needed-to-treat for primary prevention implantable cardioverter defibrillator (ICD) might be too high and that the population eligible... Show moreOn one hand, clinicians have expressed concern that the number-needed-to-treat for primary prevention implantable cardioverter defibrillator (ICD) might be too high and that the population eligible for primary prevention ICD treatment is of such magnitude that ICD therapy will strain financial resources and the pool of trained personnel. On the other hand, the currently implanted population has a relatively low incidence of ventricular arrhythmia. Therefore, additional clinically applicable tools are necessary to aid in the optimal allocation of ICD treatment. The current thesis explores possibilities for risk stratification for mortality and ventricular arrhythmia using easily available clinical parameters and evaluates the usefulness of novel, not routinely acquired parameters. The results of these studies should assist clinicians in the identification of patients who, although currently indicated, should not receive ICD treatments because of ery low risk of ventricular arrhythmia or very high risk of non arrhythmic death. Finally, this thesis clearly maps the drawbacks, accompanying ICD treatment. Show less
