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摘要


先天性完全房室傳導阻斷大部分是由新生兒狼瘡症候群引起。本研究主要之目的是分析本院先天性完全房室傳導阻斷病人之臨床表征。我們回溯性追蹤從1994到1999的9個病人。出生史,心電圖,24小時心電圖記錄,心律調節器植入,併發症,母親疾病史,以及母親和嬰孩之自體免疫抗體效價增有記錄。所有9個病人均在産前即被診斷出來。5個(55.6%)有胎兒水腫。6個活産,其餘爲人工流産。最初心電圖記錄顯示心房速率每分鐘150到166次,最低心室速率每分鐘46至80次。所有活産嬰孩均經由劍突下途逕植入心律調節器(VVI模式),3個(50%)有併發症。8個母親(100%)有陽性抗核抗體及anti-SSA/Ro抗體,6個母親有陽性anti-SSB/La抗體。5個嬰孩有陽性anti-SSA/Ro抗體,所有嬰孩均沒有anti-SSB/La抗體。我們的結論:所有的先天性完全房室傳導阻斷均與母親自體免疫抗體有關。因爲在新生兒時期,置放永久性心律調節器具有相當高之併發症風險,其應保留在特定的狀況下實施。

並列摘要


Con genital complete heart blocks (CCHB) are mostly related to the neonatal lupus syndrome. The purpose of this paper was to assess the clinical spectrum of CCHB in our hospital. Nine patients were retrospectively enrolled between 1994 and 1999. The birth history, electrocardiography, 24-hour Holter monitoring, pacemaker insertion and its complications, maternal disease, and maternal and infant autoantibody levels were studied. All nine cases were diagnosed prenatally. Hydrops fetalis was noted in five (55.6%). Six cases were live births and the other three were terminated. No anatomical heart defects were noted. Initial electrocardiography revealed the atrial rates ranged from 150 to 166 beats per minute. The minimal ventricular rates ranged from 46 to 80 beats per minute. VVJ mode pacemakers were inserted through xyphoid approach in all live-birth infants. Complications were noted in three of them (50%). Antinuclear antibody and anti-SSA/Ro antibody were positive in all 8 mothers (100%). The anti-SSB/La antibody was positive in 6 of the eight mothers (75%). Five infants tested positive for anti-SSA/Ro antibody. None of the infants tested positive for anti-SSB/La antibody. In conclusion, all CCHBs in our series were associated with maternal autoantibodies. Because of high complication rate of permanent pacemaker insertion during the neonatal period, it should be restricted in certain conditions.

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