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Cardiogenetics
Volume 2
Issue 1
10.4081/cardiogenetics.2012.e2
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Cardiogenetics is published by MDPI from Volume 10 Issue 2 (2020). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
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Brief Report

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

by
Richard J. Czosek
1,*,
Paula Goldenberg
1,
Erin M. Miller
1,
Robert Spicer
1,
Jeffrey A. Towbin
1 and
Stephanie M. Ware
1
1
Division of Pediatric Cardiology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH
2
Division of Molecular Cardiovascular Biology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH
*
Author to whom correspondence should be addressed.
Cardiogenetics 2012, 2(1), e2; https://doi.org/10.4081/cardiogenetics.2012.e2
Submission received: 7 November 2011 / Revised: 16 January 2012 / Accepted: 17 January 2012 / Published: 20 January 2012

Abstract

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.
Keywords: Alström syndrome; arrhythmia; cardiomyopathy Alström syndrome; arrhythmia; cardiomyopathy

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MDPI and ACS Style

Czosek, R.J.; Goldenberg, P.; Miller, E.M.; Spicer, R.; Towbin, J.A.; Ware, S.M. Cardiac Electrical System Involvement in Alström Syndrome: Uncommon Causes of Dilated Cardiomyopathies. Cardiogenetics 2012, 2, e2. https://doi.org/10.4081/cardiogenetics.2012.e2

AMA Style

Czosek RJ, Goldenberg P, Miller EM, Spicer R, Towbin JA, Ware SM. Cardiac Electrical System Involvement in Alström Syndrome: Uncommon Causes of Dilated Cardiomyopathies. Cardiogenetics. 2012; 2(1):e2. https://doi.org/10.4081/cardiogenetics.2012.e2

Chicago/Turabian Style

Czosek, Richard J., Paula Goldenberg, Erin M. Miller, Robert Spicer, Jeffrey A. Towbin, and Stephanie M. Ware. 2012. "Cardiac Electrical System Involvement in Alström Syndrome: Uncommon Causes of Dilated Cardiomyopathies" Cardiogenetics 2, no. 1: e2. https://doi.org/10.4081/cardiogenetics.2012.e2

APA Style

Czosek, R. J., Goldenberg, P., Miller, E. M., Spicer, R., Towbin, J. A., & Ware, S. M. (2012). Cardiac Electrical System Involvement in Alström Syndrome: Uncommon Causes of Dilated Cardiomyopathies. Cardiogenetics, 2(1), e2. https://doi.org/10.4081/cardiogenetics.2012.e2

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