複雜性先天心臟病術前術後心律不整之電生理學機轉：以同步電位及之體結構定位法研究(1/3)
Date Issued
2000
Date
2000
Author(s)
吳美環
DOI
892314B002037
Abstract
Objectives. To determine the prevalence and the electrophysiological mechanisms of rhythm
disturbances in patients with left atrial isomerism (LAI).
Background. Defective sinus node and atrioventricular conduction tissue have been described
in the hearts associated with LAI.
Methods. From 1984 to 1998, a total of 22 patients, and from 1995 to 1998, 3 fetuses, were
identified as LAI and constituted the study population. Pathological confirmation was
obtained in 14 patients and 1 fetus.
Results. The age at the last follow-up ranged from 2 to 276 months (90± 70 months). Three
fetuses (100%) developed sinus bradycardia and intermittent AV block, and were ended with
heart failure and termination. Associated cardiovascular anomalies of the 22 pediatric
patients were interruption of inferior vena cava (18, 82%), common atrium (9, 41%), AV
canal (14, 64%), double-outlet right ventricle (8, 36%) and pulmonary stenosis (15, 68%).
Over half of the patients (14, 64%) developed bradyarrhythmia (onset age, 1 to 264 months,
median 78 months): junctional escape rhythm as dominant rhythm, 10 (45%), sinus
bradycardia or sinoatrial block, 8 (35%, 5 of the 7 also had dominant junctional rhythm) and
AV block, 2 (9%, both had dominant junctional rhythm). By actuarial analysis, the
probability free from bradyarrhythmia decreased with age and was 80% and 46% at the age of
2 and 6 years, respectively. Two patients experienced fainting attacks. However, junctional
ectopic tachycardia after the cardiac operation occurred in 3 out of 10 patients.
Electrophysiological studies (3 cases) revealed sinus node dysfunction in 2/2 and impaired
AV conduction in 1/2. Besides, a Mahaim-like pathway (progressive shortening of HV
interval during decremental atrial pacing) was identified in both of the patients with His
potential recorded during the study.Conclusions. Over half of the LAI patients may develop bradyarrhythmia at the age of 6 years
mainly due to the subnormal sinus node function. Bradyarrhythmia appeared during the fetal
stage may cause preferential loss of the fetuses. Patients with LAI also have a higher chance
to have junctional tachycardia which may be related to the abnormal AV conduction
properties. The high prevalence of Mahaim-like pathway warrants further investigation.
Subjects
left atrial isomerism
heterotaxy syndrome
sinus bradycardia
junctional rhythm
junctional tachycardia
Publisher
臺北市：國立臺灣大學醫學院小兒科
Type
report
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