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首页> 外文期刊>PACE: Pacing and clinical electrophysiology >Is isolated congenital heart block associated to neonatal lupus requiring pacemaker a distinct cardiac syndrome?
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Is isolated congenital heart block associated to neonatal lupus requiring pacemaker a distinct cardiac syndrome?

机译:与需要起搏器的新生儿狼疮相关的孤立性先天性心脏阻滞是否是一种独特的心脏综合征?

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Isolated congenital heart block (ICHB) is frequently associated with neonatal lupus syndrome (NLS). Therefore few data are available regarding the long-term cardiac outcome of newborns with ICHB and the pathogenic mechanisms are not yet defined. In order to compare demographic features and cardiological outcome of patients with ICHB submitted to pacemaker (PM) implantation with and without NLS, forty ICHB patients were evaluated pre- and post-PM implantation, by clinical, electrocardiogram, Holter Monitoring, treadmill test, and electrophysiological study. According to the presence of antibodies to 52 and 60 kDa Ro/SSA and La/SSB proteins in mother's sera, it was found that 60% (24/40) of patients had ICHB associated to NLS (ICHB/NL+). Twenty-three of 24 ICHB/NL+ patients were asymptomatic, and 16 (67%) were female (P = 0.013). The frequency of syncope, mitral insufficiency (MI), and congestive heart failure (CHF) was similar pre-PM implantation in both ICHB/NL+ and ICHB/NL- groups (P > 0.05). After PMimplantation, MI and CHF were only observed in ICHB/NL+ patients, although not statistically significant. Interestingly, 67% of ICHB/NL+ were noticed before one year of age while only one fourth of ICHB/NL- was diagnosed in this period (P = 0.024). Almost half (46%) of ICHB/NL+ patients required PMs in the first 24 months of life, whereas only one in the ICHB/NL- received a PM at the same age (P = 0.02). In ICHB patients requiring PM implantation, the antibody-mediated lesion seems to be associated with an earlier onset and a more severe heart disease, in spite of the uniform criteria for PM indication.
机译:孤立性先天性心脏传导阻滞(ICHB)通常与新生儿狼疮综合征(NLS)相关。因此,关于ICHB新生儿的长期心脏预后的资料很少,其致病机制尚未确定。为了比较接受和不接受NLS的起搏器(PM)植入的ICHB患者的人口统计学特征和心脏病结局,通过临床,心电图,动态心电图监测,跑步机测试和电生理研究。根据母亲血清中存在针对52和60 kDa Ro / SSA和La / SSB蛋白的抗体,发现60%(24/40)的患者患有与NLS相关的ICHB(ICHB / NL +)。 24例ICHB / NL +患者中有23例无症状,女性为16例(67%)(P = 0.013)。在ICHB / NL +和ICHB / NL-组中,晕厥,二尖瓣关闭不全(MI)和充血性心力衰竭(CHF)的发生频率与PM植入前相似(P> 0.05)。 PM植入后,仅在ICHB / NL +患者中观察到MI和CHF,尽管无统计学意义。有趣的是,在这一年龄段之前发现了67%的ICHB / NL +,而在此期间仅诊断出四分之一的ICHB / NL-(P = 0.024)。 ICHB / NL +患者中近一半(46%)在出生后的前24个月内需要PM,而ICHB / NL-中只有一个在同一年龄接受过PM(P = 0.02)。在需要PM植入的ICHB患者中,尽管有统一的PM指征标准,但抗体介导的病变似乎与更早发作和更严重的心脏病有关。

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