Complete atrioventricular block in Duchenne muscular dystrophy

Kuru, Satoshi; Tanahashi, Tamotsu; Matsumoto, Shinjirou; Kitamura, Tatsuya; Konagaya, Masaaki

doi:10.5692/clinicalneurol.52.685

Cited by 8 publications

(3 citation statements)

References 5 publications

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“…One of the 47 patients developed complete AV block. Three cases of complete AV block have previously been reported in patients with DMD diagnosed by gene analysis, [ 11 – 13 ] with the ages at onset being 30, 33, and 40 years. Two reports described that the ECGs before the onset of the disorder showed complete right branch block.…”

Section: Discussionmentioning

confidence: 99%

“…Two reports described that the ECGs before the onset of the disorder showed complete right branch block. [ 11 , 13 ] Complete AV blocks are rarely associated with DMD, but given that the age of onset is relatively high, improved survival among patients with DMD might be attributable to the development of complete AV block in older patients. Further studies are necessary with regard to the indication for prophylactic pacemaker implantation.…”

Section: Discussionmentioning

confidence: 99%

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Cardiac conduction disturbances and aging in patients with Duchenne muscular dystrophy

et al. 2017

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The majority of patients with Duchenne muscular dystrophy (DMD) have electrocardiographic abnormalities, but the clinical significance of conduction disturbances remains unclear. This study aimed to evaluate age-dependent cardiac conduction disturbances by electrocardiogram (ECG) to identify risks of complete atrioventricular (AV) block in this patient population.In total, 47 patients with DMD (age, ≥20 ys) who recorded ECGs at our hospital from July 2015 to June 2016 were included in this study. The PR interval and QRS duration in their previous ECGs were analyzed retrospectively. Associations between ECG findings and left ventricular (LV) systolic function obtained from the latest echocardiography were examined.The mean age of patients was 27.6 ± 6.0 years, and the mean observation period was 9.8 ± 3.7 years. The PR interval gradually increased with age, but no ECGs showed an abnormally prolonged PR interval. On the other hand, the QRS duration tended to increase progressively with age, and some patients had an abnormally prolonged QRS duration. The QRS duration was not correlated with LV systolic function (P = 0.867). One patient who developed a complete AV block had a drastically prolonged QRS duration before the onset of the disorder.The QRS duration tended to increase progressively with age, irrespective of LV systolic function in patients with DMD. Attention should be paid to the QRS duration as an indicator of risk for complete AV block in older patients.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Cardiac conduction disturbances and aging in patients with Duchenne muscular dystrophy

et al. 2017

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“…Figure 1 summarises current best practice recommendations in the cardiac care of paediatric patients with cardiac dystrophinopathy, including the need for observations and checks of renal function and biochemistry 9–68. The three-drug combination outlined may have to be reduced or suspended temporarily during serious illness such as sepsis, but it should be restarted during convalescence.…”

Section: Resultsmentioning

confidence: 99%

Cardiac care of children with dystrophinopathy and females carrying DMD-gene variations

et al. 2022

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ObjectiveWe provide succinct, evidence-based and/or consensus-based best practice guidance for the cardiac care of children living with Duchenne muscular dystrophy (DMD) as well as recommendations for screening and management of female carriers of mutations in theDMD-gene.MethodsInitiated by an expert working group of UK-based cardiologists, neuromuscular clinicians and DMD-patient representatives, draft guidelines were created based on published evidence, current practice and expert opinion. After wider consultation with UK-cardiologists, consensus was reached on these best-practice recommendations for cardiac care in DMD.ResultsThe resulting recommendations are presented in the form of a succinct care pathway flow chart with brief justification. The guidance signposts evidence on which they are based and acknowledges where there have been differences in opinion. Guidelines for cardiac care of patients with more advanced cardiac dystrophinopathy at any age have also been considered, based on the previous published work of Quinlivanet aland are presented here in a similar format. The recommendations have been endorsed by the British Cardiovascular Society.ConclusionThese guidelines provide succinct, reasoned recommendations for all those managing paediatric patients with early or advanced stages of cardiomyopathy as well as females with cardiac dystrophinopathy. The hope is that this will result in more uniform delivery of high standards of care for children with cardiac dystrophinopathy, so improving heart health into adulthood through timely earlier interventions across the UK.

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Interventions for preventing and treating cardiac complications in Duchenne and Becker muscular dystrophy and X-linked dilated cardiomyopathy

Bourke

Bueser

Quinlivan

2018

Cochrane Database of Systematic Reviews

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Complete atrioventricular block in Duchenne muscular dystrophy

Cited by 8 publications

References 5 publications

Cardiac conduction disturbances and aging in patients with Duchenne muscular dystrophy

Cardiac conduction disturbances and aging in patients with Duchenne muscular dystrophy

Cardiac care of children with dystrophinopathy and females carrying DMD-gene variations

Interventions for preventing and treating cardiac complications in Duchenne and Becker muscular dystrophy and X-linked dilated cardiomyopathy

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