Cardiac rhythm disturbances in patients with left atrial isomerism

Mei Hwan Wu, Jou Kou Wang, Jiunn Lee Lin, Ling Ping Lai, Hung Chi Lue, Fon Jou Hsieh

Research output: Contribution to journalArticle

47 Citations (Scopus)

Abstract

This long-term study sought to determine the clinical implication of defective sinus node and A V conduction tissue in patients with left atrial isomerism (LAI). From 1984 to 1998, a total of 22 patients were identified as LAI. Patient age at the last follow-up ranged from 2 to 276 months (90 ± 70 months). Associated cardiac anomalies were interruption of the inferior vend cava (n = 18, 82%), common atrium (n = 9, 41%), AV canal (n = 14, 64%), double-outlet right ventricle (n = 8, 36%), and pulmonary stenosis (n = 15, 68%). Palliative interventions were performed in 16 patients (Fontan-type operation in 4 patients, shunt followed by Fontan-type operation in 2, repair of septal defect in 4, and extracardiac intervention in 6). During the follow-up, over half of the patients (n = 14, 64%) developed bradyarrhythmia (onset age: from i to 264 months; median 78 months): junctional rhythm (n = 11), sinus bradycardia (n = 8) (5 patients also had junctional rhythm), and A V block (n = 2, both also had junctional rhythm). The probability free from bradyarrhythmia was 80% and 46% at the age of 2 and 6 years, respectively. None of the bradyarrhythmias were directly related to open-heart surgery. Besides, junctional ectopic tachycardia occurred after Fontan-type operation in three of six patients. In two patients, a Mahaim-like pathway was identified during the electrophysiological study. The patients with LAI had a high probability of developing bradyarrhythmias clue to abnormal sinus node function. Varied A V conduction abnormalities may include compromised A V conduction, junctional ectopic tachycardia after Fontan-type operation, and an association of Mahaim-like pathway.

Original languageEnglish
Pages (from-to)1631-1638
Number of pages8
JournalPACE - Pacing and Clinical Electrophysiology
Volume24
Issue number11
DOIs
Publication statusPublished - Jan 1 2001
Externally publishedYes

Fingerprint

Heterotaxy Syndrome
Fontan Procedure
Bradycardia
Ectopic Junctional Tachycardia
Sinoatrial Node
Double Outlet Right Ventricle
Pulmonary Valve Stenosis
Age of Onset
Thoracic Surgery

Keywords

  • Heterotaxy syndrome
  • Junctional rhythm
  • Junctional tachycardia
  • Left atrial isomerism
  • Sinus bradycardia

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Cardiac rhythm disturbances in patients with left atrial isomerism. / Wu, Mei Hwan; Wang, Jou Kou; Lin, Jiunn Lee; Lai, Ling Ping; Lue, Hung Chi; Hsieh, Fon Jou.

In: PACE - Pacing and Clinical Electrophysiology, Vol. 24, No. 11, 01.01.2001, p. 1631-1638.

Research output: Contribution to journalArticle

Wu, Mei Hwan ; Wang, Jou Kou ; Lin, Jiunn Lee ; Lai, Ling Ping ; Lue, Hung Chi ; Hsieh, Fon Jou. / Cardiac rhythm disturbances in patients with left atrial isomerism. In: PACE - Pacing and Clinical Electrophysiology. 2001 ; Vol. 24, No. 11. pp. 1631-1638.
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abstract = "This long-term study sought to determine the clinical implication of defective sinus node and A V conduction tissue in patients with left atrial isomerism (LAI). From 1984 to 1998, a total of 22 patients were identified as LAI. Patient age at the last follow-up ranged from 2 to 276 months (90 ± 70 months). Associated cardiac anomalies were interruption of the inferior vend cava (n = 18, 82{\%}), common atrium (n = 9, 41{\%}), AV canal (n = 14, 64{\%}), double-outlet right ventricle (n = 8, 36{\%}), and pulmonary stenosis (n = 15, 68{\%}). Palliative interventions were performed in 16 patients (Fontan-type operation in 4 patients, shunt followed by Fontan-type operation in 2, repair of septal defect in 4, and extracardiac intervention in 6). During the follow-up, over half of the patients (n = 14, 64{\%}) developed bradyarrhythmia (onset age: from i to 264 months; median 78 months): junctional rhythm (n = 11), sinus bradycardia (n = 8) (5 patients also had junctional rhythm), and A V block (n = 2, both also had junctional rhythm). The probability free from bradyarrhythmia was 80{\%} and 46{\%} at the age of 2 and 6 years, respectively. None of the bradyarrhythmias were directly related to open-heart surgery. Besides, junctional ectopic tachycardia occurred after Fontan-type operation in three of six patients. In two patients, a Mahaim-like pathway was identified during the electrophysiological study. The patients with LAI had a high probability of developing bradyarrhythmias clue to abnormal sinus node function. Varied A V conduction abnormalities may include compromised A V conduction, junctional ectopic tachycardia after Fontan-type operation, and an association of Mahaim-like pathway.",
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AU - Hsieh, Fon Jou

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KW - Sinus bradycardia

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