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Atrial arrhythmias (including AV block) in congenital heart disease

Authors
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

INTRODUCTION

One of the striking successes in caring for patients with congenital heart disease (CHD) over the last few decades is the improved longevity. Almost 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 many of the 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, which are often benign in the general population, 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 both children and adults 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.

PREVALENCE AND INCIDENCE

Excluding bicuspid aortic valvulopathy, around 1 percent of live births have some element of congenital heart disease (CHD) (table 2). Minor racial differences in the incidence appear to be the case in the United States, and international variation is not known [8]. Of these, about 45 percent present with simpler forms of disease, such as atrial septal defects (ASD) and ventricular septal defects (VSD), somewhat less frequency of moderate forms of CHD such as tetralogy of Fallot, and the remainder have more complex CHD [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 2016. | This topic last updated: Tue Sep 29 00:00:00 GMT+00:00 2015.
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