Electrocardiographic Abnormalities and Sudden Death in Myotonic Dystrophy Type 1

Groh, William J.; Groh, Miriam R.; Saha, Chandan; Kincaid, John C.; Simmons, Zachary; Ciafaloni, Emma; Pourmand, Rahman; Otten, Richard F.; Bhakta, Deepak; Nair, Girish V.; Marashdeh, Mohammad; Zipes, Douglas P.; Pascuzzi, Robert M.

doi:10.1056/nejmoa062800

Cited by 449 publications

(447 citation statements)

References 30 publications

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“…109 The presence of conduction defects and atrial arrhythmias are independent risk factors for sudden death. 103,110 In Emery-Dreifuss and Limb-Girdle type IB disease, widespread atrial fibrosis can lead to atrial standstill. 101 In Emery-Dreifuss, AF and atrial flutter with slow ventricular responses and asystolic pauses can be observed, coupled with the occurrence of thromboembolism and stroke.…”

Section: Hereditary Muscular Dystrophiesmentioning

confidence: 99%

EHRA/HRS/APHRS/SOLAECE expert consensus on atrial cardiomyopathies: definition, characterization, and clinical implication

et al. 2016

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Section: Hereditary Muscular Dystrophiesmentioning

confidence: 99%

EHRA/HRS/APHRS/SOLAECE expert consensus on atrial cardiomyopathies: definition, characterization, and clinical implication

et al. 2016

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“…It is well established that sudden death can occur as a consequence of cardiac-conduction abnormalities in the DM1 patients. 18 However, it is not known whether the same risk applies to DM2 patients. In one series of 297 DM2 patients, sudden death occurred in four patients.…”

Section: Discussionmentioning

confidence: 99%

Anesthésie et dystrophie myotonique de type 2: une série de cas

Weingarten

Höfer

Milone

et al. 2010

Can J Anesth/J Can Anesth

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Background Myotonic dystrophy type 2 (DM2) is a genetically distinct disorder that shares some phenotypical features of myotonic dystrophy type 1 (DM1). However, anesthetic management of patients with DM2 has not been described. The purpose of this study is to report the anesthetic management of a series of patients with DM2 and to describe their response to anesthesia. Methods We performed a computerized search of the Mayo Clinic medical records database looking for patients with DM2 who underwent general anesthesia. The medical records were reviewed for anesthetic technique, medications used, and postoperative complications. Results We identified 19 patients with DM2 who underwent 39 general anesthetics, 17 monitored anesthetic care cases, and two regional anesthetics. The patients exhibited normal responses to succinylcholine, nondepolarizing neuromuscular blockers, neostigmine, induction agents, and volatile anesthetics. Serious postoperative complications related to DM2 did not occur. Conclusion In our series, patients with DM2 tolerated commonly used anesthetics without obvious complications, and they exhibited normal responses to muscle relaxants. These observations suggest that these medications may be used safely in patients with DM2.

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“…13 These ECG findings have been associated with worse patient outcome. [14][15][16][17] Thus, an abnormal ECG may be a prognostic indicator for development of future ventricular dysfunction and identify a high risk group of patients within the larger population of patients with cardiomyopathy. The mechanisms underlying conduction system abnormalities in patients with Alström syndrome are not known and potentially multi-factorial.…”

Section: Discussionmentioning

confidence: 99%

Cardiac Electrical System Involvement in Alström Syndrome: Uncommon Causes of Dilated Cardiomyopathies

et al. 2012

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Alström syndrome is a rare autosomal recessive disorder with dilated cardiomyopathy in 60% of patients. Despite the frequency of cardiac involvement in Alström syndrome, conduction system abnormalities or arrhythmias have not been characterized previously. We report two siblings with Alström syndrome with conduction system involvement with left bundle branch block on electrocardiogram (ECG). One patient had first degree atrioventricular block in addition to bundle branch block and underwent pacemaker implantation. This same patient developed intra-atrial reentry tachycardia requiring anti-arrhythmic medication and eventual trans-catheter ablation. The second patient developed atrial and ventricular arrhythmias and underwent placement of a bi-ventricular defibrillator. These findings suggest that cardiac conduction system involvement and clinical arrhythmia may be significant yet under-recognized complications in patients with Alström syndrome. Patients should be routinely screened with ECG and Holter monitoring in addition to echocardiographic assessment and a cardiologist experienced with cardiomyopathy should be an integral part of the care team.

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Electrocardiographic Abnormalities and Sudden Death in Myotonic Dystrophy Type 1

Abstract: Patients with adult myotonic dystrophy type 1 are at high risk for arrhythmias and sudden death. A severe abnormality on the ECG and a diagnosis of an atrial tachyarrhythmia predict sudden death. (ClinicalTrials.gov number, NCT00622453.)

Cited by 449 publications

References 30 publications

EHRA/HRS/APHRS/SOLAECE expert consensus on atrial cardiomyopathies: definition, characterization, and clinical implication

EHRA/HRS/APHRS/SOLAECE expert consensus on atrial cardiomyopathies: definition, characterization, and clinical implication

Anesthésie et dystrophie myotonique de type 2: une série de cas

Cardiac Electrical System Involvement in Alström Syndrome: Uncommon Causes of Dilated Cardiomyopathies

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