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

Congenit Heart Dis. 2016 Jan-Feb;11(1):7-18. doi: 10.1111/chd.12288. Epub 2015 Jul 29.


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.

Keywords: Arrhythmia; Atrioventricular Block; Heterotaxy; Isomerism; Supraventricular Tachycardia.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Arrhythmias, Cardiac / diagnosis
  • Arrhythmias, Cardiac / etiology*
  • Arrhythmias, Cardiac / therapy
  • Cardiac Pacing, Artificial
  • Child
  • Child, Preschool
  • Chronic Disease
  • Disease-Free Survival
  • Electrocardiography
  • Heterotaxy Syndrome / complications*
  • Heterotaxy Syndrome / diagnosis
  • Humans
  • Infant
  • Kaplan-Meier Estimate
  • Middle Aged
  • Predictive Value of Tests
  • Prognosis
  • Risk Assessment
  • Risk Factors
  • Young Adult