Toggle Main Menu Toggle Search

Open Access padlockePrints

Chronic Arrhythmias in the Setting of Heterotaxy: Differences between Right and Left Isomerism

Lookup NU author(s): Professor Bob Anderson


Full text for this publication is not currently held within this repository. Alternative links are provided below where available.


So-called heterotaxy affects lateralization of the thoracic and abdominal organs. Congenital malformations may be present in one of several organ systems. Cardiac involvement includes both structural and conduction abnormalities. Data regarding arrhythmias in heterotaxy come from case reports and small case series. We pooled available data to further characterize arrhythmias in heterotaxy. A systematic review of the literature for manuscripts describing arrhythmias in heterotaxy patients was conducted. Databases including PubMed, EMBASE, and Ovid were searched. Studies describing arrhythmias in patients with heterotaxy were included if they were in English and presented characteristics of the arrhythmias. Arrhythmia characteristics were abstracted and are presented as pooled data. Freedom from arrhythmia by age was then analyzed using Kaplan-Meier analysis. A total of 19 studies with 121 patients were included in the pooled analysis. Those with right isomerism were found to be more likely to have atrial flutter, atrial tachycardia, junctional tachycardia, and ventricular tachycardia. Those with left isomerism were more likely to have atrioventricular block, intraventricular conduction delay, sick sinus syndrome, and atrioventricular nodal reentry tachycardia. Median age of onset for all arrhythmias was 4 years with no difference by specific arrhythmia or isomerism. Those with right and left isomerism are at risk for different arrhythmias but are likely to develop arrhythmias at the same age. Those with left isomerism are more likely to require pacemaker placement due to atrioventricular block. Understanding these differences allows for focused surveillance of development of these arrhythmias.

Publication metadata

Author(s): Loomba RS, Willes RJ, Kovach JR, Anderson RH

Publication type: Review

Publication status: Published

Journal: Congenital Heart Disease

Year: 2016

Volume: 11

Issue: 1

Pages: 7-18

Print publication date: 01/01/2016

Online publication date: 29/07/2015

Acceptance date: 21/06/2015

ISSN (print): 1747-079X

ISSN (electronic): 1747-0803



DOI: 10.1111/chd.12288