Friedman Dm, Duncanson Lj, Glickstein J, Buyon Jp
Dept. of Pediatrics, St Luke's-Roosevelt Hospital Center, New York, NY.
Images Paediatr Cardiol. 2003 Jul;5(3):36-48.
Congenital heart block is a rare disorder. It has an incidence of about 1 in 22,000 live births. It may be associated with high mortality and morbidity. This should generate a high index of suspicion for early diagnosis and aggressive therapy when appropriate. The congenital heart block associated with neonatal lupus is considered a form of passively acquired autoimmune disease in which maternal autoantibodies to the intracellular ribonucleoproteins Ro (SS-A) and La (SS-B), cross the placenta and injure the previously normal fetal heart. Women with serum titers of anti-Ro antibody carry a 3% risk of having a child with neonatal lupus syndrome. Recurrence rates are about 18%. We believe that serial echocardiograms should be acquired so that early diagnosis is made and aggressive therapy administered, if signs of conduction system disease such as PR interval prolongation by Doppler are found, so as to optimize the outcome. Establishment of guidelines for therapy have been set empirically, should signs of congenital heart block develop. Those patients whose congenital heart block is associated with structural heart disease have a higher morbidity and mortality, which is determined more by the underlying structural congenital heart disease than it is by the need for a pacemaker per se.
先天性心脏传导阻滞是一种罕见的疾病。其发病率约为每22000例活产中有1例。它可能与高死亡率和高发病率相关。这就需要在早期诊断时保持高度怀疑，并在适当的时候积极治疗。与新生儿狼疮相关的先天性心脏传导阻滞被认为是一种被动获得性自身免疫性疾病，其中母亲针对细胞内核糖核蛋白Ro（SS - A）和La（SS - B）的自身抗体穿过胎盘并损害先前正常的胎儿心脏。抗Ro抗体血清滴度高的女性生育患有新生儿狼疮综合征孩子的风险为3%。复发率约为18%。我们认为应进行系列超声心动图检查，以便早期诊断，并在发现诸如多普勒显示PR间期延长等传导系统疾病迹象时进行积极治疗，从而优化治疗结果。如果出现先天性心脏传导阻滞的迹象，已根据经验制定了治疗指南。那些先天性心脏传导阻滞与结构性心脏病相关的患者发病率和死亡率更高，这更多地取决于潜在的结构性先天性心脏病，而非起搏器本身的需求。
