Gordon P A
Department of Rheumatology, King's College Hospital, London, UK.
Lupus. 2007;16(8):642-6. doi: 10.1177/0961203307079041.
Isolated congenital heart block is strongly associated with anti-Ro antibodies. It occurs in 2% of anti-Ro antibody positive pregnancies with a recurrence rate of 17-19%. Mortality is high in the first year of life (12-41%) and is predominantly due to dilated cardiomyopathy. A prolonged QTc occurs in 15-22% of cases and minor structural defects such as atrial septal defects and patent arterial ducts are well recognized. The 'mechanical' PR interval can now be measured in utero allowing for the detection of first-degree heart block. Both first and second-degree heart block detected in utero respond to therapy with fluorinated steroids. Complete congenital heart block is not reversible. Progression from a normal PR interval to complete heart block can occur within a week. IVIG is under investigation for the prevention of recurrence of congenital heart block, while dexamethasone should not be used for this purpose due to unacceptable toxicity. Data on the use of fluorinated steroids for established complete heart block is conflicting, although their use in cases where there is evidence of hydrops, poor ventricular function or both is not controversial.
孤立性先天性心脏传导阻滞与抗Ro抗体密切相关。在抗Ro抗体阳性的妊娠中，其发生率为2%，复发率为17% - 19%。出生后第一年死亡率很高（12% - 41%），主要原因是扩张型心肌病。15% - 22%的病例出现QTc延长，并且诸如房间隔缺损和动脉导管未闭等轻微结构缺陷也很常见。现在可以在子宫内测量“机械性”PR间期，从而能够检测一度心脏传导阻滞。在子宫内检测到的一度和二度心脏传导阻滞对氟化类固醇治疗有反应。完全性先天性心脏传导阻滞不可逆转。从正常PR间期进展到完全性心脏传导阻滞可在一周内发生。静脉注射免疫球蛋白正在研究用于预防先天性心脏传导阻滞的复发，而地塞米松因毒性不可接受不应为此目的使用。关于使用氟化类固醇治疗已确诊的完全性心脏传导阻滞的数据存在矛盾，尽管在有水肿、心室功能不良或两者皆有的情况下使用氟化类固醇并无争议。
