Sección Reumatología, Servicio de Clínica Médica, Hospital Italiano de Buenos Aires, Peron 4190 (C1199ABB), Buenos Aires, Argentina.
Instituto Universitario del Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.
Clin Rheumatol. 2019 Aug;38(8):2211-2217. doi: 10.1007/s10067-019-04556-8. Epub 2019 Apr 17.
INTRODUCTION/OBJECTIVES: Complete congenital atrioventricular block (AVB) may be due to cardiac malformations or the presence of maternal antibodies (autoimmune AVB). Our objective was to estimate the prevalence of autoimmune AVB among all AVB in newborns treated at our hospital. Secondly, we estimated the prevalence of AVB among mothers with anti-Ro/La antibodies and examined the relationship of those fetal AVB with mother's use of hydroxychloroquine during pregnancy.
Retrospective cohort in which we reviewed electronic medical records from years 2000 to 2014 of (a) all mothers with children born with third degree AVB and (b) all pregnant women with anti-Ro/La-positive antibodies.
Twenty-three AVBs were diagnosed. Ten (43.5%, 95% CI 23.2-65.5) were associated with maternal rheumatologic disease. The remaining 13 were associated with cardiac malformations. Sixty-two pregnancies in 47 mothers with Ro/La antibodies were identified; eight (12.9%, 95% CI 5.7-23.8) suffered AVB. Fourteen mothers consumed hydroxychloroquine during full pregnancy (one newborn (7.1%) suffered AVB) and 48 did not (7 newborns with AVB (14.6%); p = 0.5).
All congenital AVB diagnosed at our hospital without cardiac malformations were associated with a maternal rheumatologic disease/antibodies. Therefore, if a AVB is diagnosed in a newborn without structural heart disease, the mother should be studied for an autoimmune disease. We found a high prevalence of AVB among mothers with anti-Ro/La antibodies. Although not statistically significant, AVBs in mothers with Ro/La antibodies were numerically more frequent in those not using hydroxychloroquine.Key Points• Although structural heart malformations were the predominant cause of third-degree AVB, autoimmune AVB was still a significant cause.• The distinction between structural or non-structural cause of AVB constitutes an essential issue since it determines the prognostic of these fetuses in terms of complications.• Although not statistically significant, AVBs in mothers with Ro/La antibodies were more frequent in those not using hydroxychloroquine.• If an AVB is diagnosed in a newborn without structural heart disease, the mother should be studied for an autoimmune disease.
介绍/目的：完全性先天性房室传导阻滞（AVB）可能由心脏畸形或母体抗体引起（自身免疫性 AVB）。我们的目的是估计我院治疗的所有新生儿 AVB 中自身免疫性 AVB 的患病率。其次，我们估计了抗 Ro/La 抗体阳性孕妇中 AVB 的患病率，并检查了这些胎儿 AVB 与母亲在怀孕期间使用羟氯喹之间的关系。
回顾性队列研究，我们回顾了 2000 年至 2014 年期间（a）所有患有三度 AVB 的儿童的母亲和（b）所有抗 Ro/La 阳性抗体的孕妇的电子病历。
诊断出 23 例 AVB。10 例（43.5%，95%CI 23.2-65.5）与母体风湿性疾病相关。其余 13 例与心脏畸形有关。在 47 名抗 Ro/La 抗体的孕妇中发现了 62 例妊娠，其中 8 例（12.9%，95%CI 5.7-23.8）患有 AVB。14 名母亲在整个孕期服用羟氯喹（1 名新生儿（7.1%）患有 AVB），48 名未服用（7 名新生儿患有 AVB（14.6%）；p=0.5）。
我院诊断的所有无心脏畸形的先天性 AVB 均与母体风湿性疾病/抗体有关。因此，如果在无结构性心脏病的新生儿中诊断出 AVB，应研究母亲是否患有自身免疫性疾病。我们发现抗 Ro/La 抗体阳性母亲的 AVB 患病率较高。尽管没有统计学意义，但在未使用羟氯喹的 Ro/La 抗体阳性母亲中，AVB 的发生率略高。
尽管结构性心脏畸形是三度 AVB 的主要原因，但自身免疫性 AVB 仍然是一个重要原因。
区分 AVB 的结构性或非结构性原因是一个至关重要的问题，因为它决定了这些胎儿在并发症方面的预后。
尽管没有统计学意义，但在未使用羟氯喹的 Ro/La 抗体阳性母亲中，AVB 的发生率略高。
如果在无结构性心脏病的新生儿中诊断出 AVB，应研究母亲是否患有自身免疫性疾病。
