Pediatric Cardiology

, Volume 33, Issue 3, pp 484–490 | Cite as

Sudden Cardiac Death and Malignant Arrhythmias: The Scope of the Problem in Adult Congenital Heart Patients

  • James C. PerryEmail author
Review Article


A key component of recognizing sudden cardiac death (SCD) risk in the adult congenital heart disease (ACHD) patient is the recognition of heart failure risk for each physiology. The risk of SCD is an accrued phenomenon, representing the influences of anatomy, genetics, surgical and catheter interventions, and long-term sequelae of residual hemodynamic issues. These all lead to a substrate for tachyarrhythmia. It is beneficial in thinking about all of the potential combinations of CHD anatomy and physiologies to categorize SCD risk for the ACHD patient in terms of systemic left-ventricular failure, systemic right-ventricular failure, subpulmonary ventricular failure, the dyssynchronous contractility states due to bundle branch block, and single-site ventricular pacing. This article reviews important issues in arrhythmogenesis for ACHD patients with all of these physiologies and discusses potential cardiac rhythm device-management needs.


Adult congenital heart Sudden death Electrophysiology 


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Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Electrophysiology and Adult Congenital Heart ProgramsUCSD/Rady Children’s Hospital San DiegoSan DiegoUSA

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