ArticlePediatric cardiology2019
Cardiac Rhythm Disturbances in Heterotaxy Syndrome.
Article in Pediatric cardiology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 29 citations in OpenAlex.
- Outcomes of Cardiac Repair in Heterotaxy Syndrome Patients-Tertiary Center Experience.CJC pediatric and congenital heart disease · 2026Article
- Early Currents: Developmental Electrophysiology and Arrhythmia in Pediatric Congenital Heart Disease.Journal of cardiovascular development and disease · 2025Review
- Sudden cardiac death in congenital heart disease-a narrative review and update.Frontiers in cardiovascular medicine · 2025Review
- Cardiac Laterality: Surgical Results of Right Atrial Isomerism.Diseases (Basel, Switzerland) · 2023Article
- Advances in the Prenatal Management of Fetal Cardiac Disease.Children (Basel, Switzerland) · 2022Article
- Article
- Successful atrioventricular nodal reentrant tachycardia ablation in a female patient with left isomerism.Anatolian journal of cardiology · 2021Article
- Junctional ectopic tachycardia in infants and children.Journal of arrhythmia · 2020Review
- Prenatal diagnosis of fetal bradyarrhythmia and postnatal outcome.Indian pacing and electrophysiology journalArticle
- Left atrial isomerism associated with aneurysmal enlargement of right atrial appendage: A case report with literature review.The Indian journal of radiology & imagingArticle
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveHeterotaxy syndrome is associated with complex cardiac malformations and cardiac conduction system abnormalities. Those with right atrial isomerism (RAI) have dual sinus nodes and dual atrioventricular nodes predisposing them to supraventricular tachycardia (SVT). Those with left atrial isomerism (LAI) lack a normal sinus node and are at risk of sinus node dysfunction (SND) and atrioventricular block (AV block). We report the occurrence and risk factors associated with arrhythmias in heterotaxy syndrome.
methodsA retrospective review of all heterotaxy syndrome patients born and treated at our institution between 2000 and 2014 was performed.
resultsA total of 40 patients were identified; 16/40 (40%) with LAI and 24/40 (60%) with RAI. There were 12 deaths during follow-up [LAI 3/16 (19%), RAI 9/24 (38%); p = 0.30]. Twenty-one patients had arrhythmias during a mean follow-up period of 5.4 years; 14/16 (87%) in LAI and 7/24 (29%) in RAI (p < 0.001). Freedom from arrhythmia at 1,3,5 years of age was 75.0%, 37.9%, 22.7% in LAI, and 83.3%, 77.5%, 69.6% in RAI, respectively(p = 0.00261). LAI had a three-fold increase in developing arrhythmias. Left atrial isomerism was the only factor identified to be associated with arrhythmia occurrence.
conclusionsArrhythmias were commonly seen in heterotaxy syndrome particularly in left isomerism with more than half of the patients having arrhythmias by 3 years of age. Atrial situs was the only risk factor identified to be associated with arrhythmias, and close follow-up is warranted in these patients.
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