Arrhythmia-related workup in hereditary myopathies

Finsterer, Josef; Stöllberger, Claudia; Keller, Hans

doi:10.1016/j.jelectrocard.2012.02.003

Cited by 24 publications

(21 citation statements)

References 46 publications

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“…Phenotypic variability results in a wide spectrum of clinical manifestations even amongst members of the same family [24].…”

Section: Clinical Cardiac Manifestationsmentioning

confidence: 99%

“…Cardiac fibrosis and fatty infiltration most commonly affect the His-Purkinje system but may also involve the sino-atrial and atrioventricular (AV) nodes. These provide a substrate for conduction block, ectopic activity and re-entrant arrhythmias and can present with palpitations, syncope and sudden cardiac death (0.56% per year of follow-up) [24,30]. A meta-analysis of 1828 patients with MD revealed 1st degree AV block in 28.2%, QTc N440 ms in 22.0%, QRS N120 ms in 19.9%, frequent premature ventricular contractions in 14.6%, atrial fibrillation/flutter in 5.0%, right bundle branch block in 4.4%, left bundle branch block in 5.7% and non-sustained ventricular tachycardia in 4.1% [30].…”

Section: Arrhythmiasmentioning

confidence: 99%

“…Approximately 65% of patients with MD have ECG abnormalities, the most common being prolongation of the PR interval and the QRS duration [15,24,41]. QRS prolongation appears to progress with age, approximately at the rate of 0.54 ms/year, and prolongation of the PR interval and QRS duration are correlated [10].…”

Section: Electrocardiogrammentioning

confidence: 99%

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Myotonic dystrophy and the heart: A systematic review of evaluation and management

Lau

Corbett

et al. 2015

International Journal of Cardiology

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“…Phenotypic variability results in a wide spectrum of clinical manifestations even amongst members of the same family [24].…”

Section: Clinical Cardiac Manifestationsmentioning

confidence: 99%

Section: Arrhythmiasmentioning

confidence: 99%

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Myotonic dystrophy and the heart: A systematic review of evaluation and management

Lau

Corbett

et al. 2015

International Journal of Cardiology

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“…52,53 In this case the pattern of inheritance could be helpful. 54 Many genetic causes have also manifestations in other organ systems: for example, a familial history of neuromuscular disease and a personal history of diabetes and sensorineural deafness could suggest mitochondriopathies. 17 If genetic causes are unlikely or have been without success by performing genetic tests, other non-genetic disorders should be considered.…”

Section: Dilated Phenotypementioning

confidence: 99%

Diagnosis of Cardiomyopathies: Tips and Tricks for Internists and General Practitioners

2017

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Cardiomyopathies are little known to internists and general practitioners (GPs), and not always able to arouse the interest of cardiologists. Probably, this happens because cardiomyopathies are perceived as rare and complex disorders, a prerogative of a few dedicated centers. This may partly explain why the diagnosis of cardiomyopathy is often missed and, consequently, why cardiomyopathies are largely underdiagnosed. Internists and general practitioners should have an interest in these conditions, because cardiomyopathies are not as rare as generally perceived, and because their complexity can be unravelled with knowledge and methodology. Cardiomyopathies are defined as myocardial disorders in which the heart is structurally and functionally abnormal in the absence of coronary artery disease or abnormal loading conditions. Irrespective of the cardiac imaging technique used, a limited number of phenotypes are defined based on ventricular morphology and function. These basic phenotypes include hypertrophic, dilated, restrictive and right ventricular arrhythmogenic cardiomyopathies. Aim of this review is to describe a simplified approach to the detection of the underlying causes of specific phenotypes. We will focus our attention on the basic phenotypes, presenting a diagnostic work-up and a suggestive clinical case for each phenotype.

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“…Thanks to their ability to register the patients' electrocardiogram during spontaneous symptoms, ILRs have gained popularity and are now recommended as an early diagnostic tool in the evaluation of unexplained syncope [3]. They are also recommended in patients with other neuromuscular disorders [4].…”

mentioning

confidence: 99%

Expanded Diagnostic and Therapeutic Options for Cardiac Disease in Duchenne Muscular Dystrophy

Stöllberger

Finsterer

2018

JND

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With interest we read the article by Fayssoil et al. about cardiac involvement classification and therapy in patients with Duchenne muscular dystrophy (DMD) [1]. We agree with the authors that search for cardiac involvement and cardiac therapy are important in the care of patients with DMD, however we want to add some comments and suggestions.Regarding symptoms, it is mentioned that "classical" symptoms of heart failure like exertional dyspnea or symptoms of arrhythmias like palpitations may be absent in DMD because of the limited mobility. Thus, instrumental investigations to assess the cardiac function are important. The authors recommend electrocardiogram, echocardiography and cardiac magnetic resonance imaging. However, the armamentarium of the cardiologists to detect cardiac dysfunction is larger. First, we recommend measurement of the serum levels of brain natriuretic peptide (BNP) which is a robust indicator for cardiac dysfunction, useful for diagnostic purposes and for control of therapeutic effects, as recommended by current heart failure guidelines [2]. Secondly, we suggest that in patients with suspected arrhythmia 24-Holter monitoring or, if the arrhythmias occur only rarely, implantable loop recorders (ILRs) should be considered. ILRs are small devices, implanted or injected subcutaneously in the left side of the chest under local anaesthesia. They continuously record and delete a

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Arrhythmia-related workup in hereditary myopathies

Cited by 24 publications

References 46 publications

Myotonic dystrophy and the heart: A systematic review of evaluation and management

Myotonic dystrophy and the heart: A systematic review of evaluation and management

Diagnosis of Cardiomyopathies: Tips and Tricks for Internists and General Practitioners

Expanded Diagnostic and Therapeutic Options for Cardiac Disease in Duchenne Muscular Dystrophy

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