Cardiac electrical system involvement in Alström syndrome: uncommon causes of dilated cardiomyopathies

  • Richard J. Czosek | richard.czosek@cchmc.org Division of Pediatric Cardiology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH, United States.
  • Paula Goldenberg Division of Pediatric Cardiology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH, United States.
  • Erin M. Miller Division of Pediatric Cardiology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH, United States.
  • Robert Spicer Division of Pediatric Cardiology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH, United States.
  • Jeffrey A. Towbin Division of Pediatric Cardiology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH, United States.
  • Stephanie M. Ware Division of Pediatric Cardiology; Division of Molecular Cardiovascular Biology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH, United States.

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.

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Author Biographies

Richard J. Czosek, Division of Pediatric Cardiology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH

Department of Pediatrics

Division of Cardiology

Assistant Professor

Cardiologist/Electrophysiologist

Paula Goldenberg, Division of Pediatric Cardiology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH
Assistant Professor

Geneticist

Erin M. Miller, Division of Pediatric Cardiology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH

Genetic Counselor

Robert Spicer, Division of Pediatric Cardiology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH
Professor
Jeffrey A. Towbin, Division of Pediatric Cardiology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH

Director of Cardiology

Stephanie M. Ware, Division of Pediatric Cardiology; Division of Molecular Cardiovascular Biology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, OH
Associate Professor

References

Published
2012-01-20
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Section
Brief Reports
Supporting Agencies
No direct funding or support for the work in this submission
Keywords:
Alström syndrome, arrhythmia, cardiomyopathy.
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How to Cite
Czosek, R., Goldenberg, P., Miller, E., Spicer, R., Towbin, J., & Ware, S. (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