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(Circulation. 2007;115:534-545.)
© 2007 American Heart Association, Inc.

Congenital Heart Disease for the Adult Cardiologist

Arrhythmias in Adult Patients With Congenital Heart Disease

Edward P. Walsh, MD; Frank Cecchin, MD

From the Electrophysiology Division, Department of Cardiology, Children’s Hospital Boston, and Department of Pediatrics, Harvard Medical School, Boston, Mass.

Correspondence to Edward P. Walsh, MD, Cardiac Electrophysiology, Children’s Hospital Boston, 300 Longwood Ave, Boston MA 02115. E-mail epwalsh@cardio.tch.harvard.edu

Key Words: ablation • atrial flutter • heart defects, congenital • arrhythmia • pacemakers • electrophysiology • pediatrics

An extract of the first 250 words of the full text is provided, because this article has no abstract.

	Introduction

 
By the time patients with congenital heart disease (CHD) reach adolescence and early adulthood, rhythm status has often become an active issue, if not the central issue, in their cardiac management. For some, arrhythmias are intrinsic to the structural malformation itself, as is the case with Wolff-Parkinson-White syndrome in the setting of Ebstein’s anomaly, or atrioventricular (AV) block in the setting of "congenitally corrected" transposition of the great arteries (L-TGA). For most other CHD patients, arrhythmias represent an acquired condition related to the unique myocardial substrate created by surgical scars in conjunction with cyanosis and abnormal pressure/volume loads of long duration. This review will attempt to address the major rhythm difficulties faced by adults with CHD, concentrating on electrophysiological features that distinguish CHD from more conventional forms of adult heart disease. A listing of specific arrhythmias and commonly associated congenital defects is provided in Table 1 to serve as an outline for the discussion.

View this table:   TABLE 1. Specific Arrhythmias and Associated Defects in Adults With Congenital Heart Disease

	Prevalence

 
As of 2001, there were estimated to be 800 000 adults with CHD living in the United States,¹ a number that has grown steadily as more survivors of childhood surgery reach maturity. Of these, roughly 45% are considered to have mild forms of CHD (eg, atrial septal defect, valvar pulmonary stenosis), 40% are classified as having moderate disease (eg, tetralogy of Fallot, Ebstein’s anomaly), and 15% are considered to have complex disease (eg, single ventricle, transposition of the great arteries). Although arrhythmias can . . . [Full Text of this Article]

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