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Atrial arrhythmias (including AV block) in congenital heart disease

Samuel Asirvatham, MD
Heidi M Connolly, MD, FASE
Christopher J McLeod, MB, ChB, PhD
Section Editors
Hugh Calkins, MD
Charles I Berul, MD
Deputy Editor
Gordon M Saperia, MD, FACC


One of the striking successes in caring for patients with congenital heart disease (CHD) over the last few decades is the improved longevity. Over one million adults with CHD are now living in the United States [1-3]; up to half having undergone at least one open heart surgical procedure resulting in one or more residual atrial scars [4]. For the purposes of this topic, CHD does not include bicuspid valves.

As a consequence of both the added longevity and the atrial scarring from surgical procedures, atrial arrhythmias are increasingly recognized in this group. They are a major cause of hospital admission and morbidity in patients with CHD [5-7]. These rhythm abnormalities may be poorly tolerated and are associated with an almost 50 percent increase in mortality compared with those patients without atrial arrhythmias [7].

Although all forms of atrial bradycardia and tachycardia can adversely affect patients with CHD, there are particular considerations in this group because of the anatomy and prior surgical repairs. Although some arrhythmias are intrinsic to the cardiac maldevelopment itself, most are secondary to surgical scars and chronic hemodynamic burden.

This review will focus on the management of these arrhythmias (table 1), which should involve a comprehensive multidisciplinary approach.


Excluding bicuspid aortic valve, around 1 percent of infants are born with congenital heart disease (CHD) (table 2), with minor reported racial differences in the United States [8]. Many of the congenital cardiac lesions (45 percent) are classified as simple forms, such as atrial septal defects (ASD) and ventricular septal defects (VSD); moderate forms of CHD such as tetralogy of Fallot occur less commonly, and complex CHD occurs infrequently [9]. The epidemiology of CHD is discussed in detail elsewhere. (See "Identifying newborns with critical congenital heart disease", section on 'Epidemiology'.)

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Literature review current through: Nov 2017. | This topic last updated: Sep 05, 2017.
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