Literature DB >> 28618061

Electrical storm in a patient with Duchenne muscular dystrophy cardiomyopathy triggered by abrupt β-blocker interruption.

Nikolaos Fragakis1, Melani Sotiriadou1, Lydia Krexi1, Vassilios Vassilikos1.   

Abstract

We present a case of a young man with Duchenne muscular dystrophy cardiomyopathy (DMDC) having an implantable cardioverter defibrillator for secondary prevention, who presented with electrical storm shortly after β-blocker interruption. The patient was stabilized and remained free of ventricular arrhythmias soon after reinitiating b-adrenoreceptor antagonists. The present case highlights the importance of sympathetic blockage in patients with DMDC due to existing pathophysiology of excess diastolic Ca2+ leak from sarcoplasmic reticulum as a result of ryanodine receptor dysfunction.
© 2017 Wiley Periodicals, Inc.

Entities:  

Keywords:  cardiomyopathy; duchenne muscular dystrophy; electrical storm; implantable cardioverter defibrillator; β-blocker

Mesh：

Substances：

Year:  2017        PMID： 28618061      PMCID： PMC6931609          DOI： 10.1111/anec.12477

Source DB:  PubMed          Journal:  Ann Noninvasive Electrocardiol        ISSN： 1082-720X            Impact factor:   1.468


  12 in total

1.  Carvedilol can prevent cardiac events in Duchenne muscular dystrophy.

Authors:  Tsuyoshi Matsumura; Takuhisa Tamura; Satoshi Kuru; Yasuki Kikuchi; Mitsuru Kawai
Journal:  Intern Med       Date:  2010-07-15       Impact factor: 1.271

Review 2.  Inherited dysfunction of sarcoplasmic reticulum Ca2+ handling and arrhythmogenesis.

Authors:  Silvia G Priori; S R Wayne Chen
Journal:  Circ Res       Date:  2011-04-01       Impact factor: 17.367

3.  Why are some patients with Duchenne muscular dystrophy dying young: An analysis of causes of death in North East England.

Authors:  H J A Van Ruiten; C Marini Bettolo; T Cheetham; M Eagle; H Lochmuller; V Straub; K Bushby; M Guglieri
Journal:  Eur J Paediatr Neurol       Date:  2016-07-30       Impact factor: 3.140

4.  Leaky RyR2 trigger ventricular arrhythmias in Duchenne muscular dystrophy.

Authors:  Jérémy Fauconnier; Jérôme Thireau; Steven Reiken; Cécile Cassan; Sylvain Richard; Stefan Matecki; Andrew R Marks; Alain Lacampagne
Journal:  Proc Natl Acad Sci U S A       Date:  2010-01-04       Impact factor: 11.205

5.  Complete cloning of the Duchenne muscular dystrophy (DMD) cDNA and preliminary genomic organization of the DMD gene in normal and affected individuals.

Authors:  M Koenig; E P Hoffman; C J Bertelson; A P Monaco; C Feener; L M Kunkel
Journal:  Cell       Date:  1987-07-31       Impact factor: 41.582

6.  Circumferential strain analysis identifies strata of cardiomyopathy in Duchenne muscular dystrophy: a cardiac magnetic resonance tagging study.

Authors:  Kan N Hor; Janaka Wansapura; Larry W Markham; Wojciech Mazur; Linda H Cripe; Robert Fleck; D Woodrow Benson; William M Gottliebson
Journal:  J Am Coll Cardiol       Date:  2009-04-07       Impact factor: 24.094

Review 7.  Calcium misregulation and the pathogenesis of muscular dystrophy.

Authors:  F W Hopf; P R Turner; R A Steinhardt
Journal:  Subcell Biochem       Date:  2007

8.  Dystrophic cardiomyopathy: amplification of cellular damage by Ca2+ signalling and reactive oxygen species-generating pathways.

Authors:  Carole Jung; Adriano S Martins; Ernst Niggli; Natalia Shirokova
Journal:  Cardiovasc Res       Date:  2007-12-04       Impact factor: 10.787

9.  Enhanced currents through L-type calcium channels in cardiomyocytes disturb the electrophysiology of the dystrophic heart.

Authors:  Xaver Koenig; Lena Rubi; Gerald J Obermair; Rene Cervenka; Xuan B Dang; Peter Lukacs; Stefan Kummer; Reginald E Bittner; Helmut Kubista; Hannes Todt; Karlheinz Hilber
Journal:  Am J Physiol Heart Circ Physiol       Date:  2013-12-13       Impact factor: 5.125

10.  Sarcospan Regulates Cardiac Isoproterenol Response and Prevents Duchenne Muscular Dystrophy-Associated Cardiomyopathy.

Authors:  Michelle S Parvatiyar; Jamie L Marshall; Reginald T Nguyen; Maria C Jordan; Vanitra A Richardson; Kenneth P Roos; Rachelle H Crosbie-Watson
Journal:  J Am Heart Assoc       Date:  2015-12-23       Impact factor: 5.501
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  1 in total

1.  Exploring the acceptability of implantable defibrillators in patients with cardiac dystrophinopathy and carers.

Authors:  Ursula M Hiermeier; Christine Baker; John P Bourke
Journal:  Open Heart       Date:  2020-04-21
  1 in total

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