TY - JOUR
T1 - Cardiac rhythm disturbances in patients with left atrial isomerism
AU - Wu, Mei Hwan
AU - Wang, Jou Kou
AU - Lin, Jiunn Lee
AU - Lai, Ling Ping
AU - Lue, Hung Chi
AU - Hsieh, Fon Jou
PY - 2001/1/1
Y1 - 2001/1/1
N2 - 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.
AB - 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.
KW - Heterotaxy syndrome
KW - Junctional rhythm
KW - Junctional tachycardia
KW - Left atrial isomerism
KW - Sinus bradycardia
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U2 - 10.1046/j.1460-9592.2001.01631.x
DO - 10.1046/j.1460-9592.2001.01631.x
M3 - Article
C2 - 11816632
AN - SCOPUS:0035165432
VL - 24
SP - 1631
EP - 1638
JO - PACE - Pacing and Clinical Electrophysiology
JF - PACE - Pacing and Clinical Electrophysiology
SN - 0147-8389
IS - 11
ER -