Autoimmune-associated congenital heart block: Demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry
Autoimmune-associated congenital heart block: Demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry
复制标题
DOI：
10.1016/s0735-1097(98)00161-2
复制
发表时间：
1998-06-01
影响因子：
24
通讯作者：
Skovron, ML
中科院分区：
文献类型：
--
作者：
Buyon, JP;Hiebert, R;Skovron, ML
目标.本研究使用新生儿狼疮研究登记处的信息描述了自身免疫相关先天性心脏传导阻滞（CHB）的人口统计学、死亡率、发病率和复发率。背景。出生时或出生前检测到的孤立性CHB与48-kD SSB/La，52-kD SSA/Ro和60-kD SSA/Ro核糖核蛋白的母体自身抗体密切相关，并且是新生儿狼疮综合征（NLS）的永久表现。可用的数据是有限的罕见的疾病。该队列包括105名母亲，其血清含有抗SSA/Ro或抗SSB/La抗体，或两者，以及1970年至1997年期间诊断为CHB的113名婴儿（56名男孩，57名女孩）。在有足够医疗记录的57例妊娠中，71例（82%）（中位时间23周）在妊娠30周前首次检测到被证实为CHB的缓慢性心律失常。没有病例认为主要先天性心脏解剖缺陷是CHB发展的原因; 14例有轻微异常。113例患儿中22例（19%）死亡，16例（73%）在出生后3个月内死亡，3年累积生存率为79%。107例活产婴儿中有67例（63%）需要植入起搏器：35例在出生后9天内植入，15例在1年内植入，17例在1年后植入。49名母亲随后怀孕：8名（16%）有另一名患有CHB的婴儿，3名（6%）有一名患有与NLS一致的孤立皮疹的孩子。来自这个大系列的数据证实，自身抗体相关性CHB与主要结构异常不一致，最常在妊娠中期晚期发现，新生儿期死亡率很高，经常需要起搏。CHB的复发率至少是抗SSA/Ro-SSB/La抗体的母亲从未有过受影响的孩子的复发率的2 - 3倍，支持在所有随后的妊娠中进行密切的超声心动图监测，并在妊娠18周兰德24周之间加强监测。(C)1998年，美国心脏病学会。
Objectives. The present study describes the demographics, mortality, morbidity and recurrence rates of autoimmune-associated congenital heart block (CHB) using information from the Research Registry for Neonatal Lupus.Background. Isolated CHB detected at or before birth is strongly associated with maternal autoantibodies to 48-kD SSB/La, 52-kD SSA/Ro and 60-kD SSA/Ro ribonucleoproteins and is a permanent manifestation of the neonatal lupus syndromes (NLS). Available data are limited by the rarity of the disease.Results. The cohort includes 105 mothers whose sera contain anti-SSA/Ro or anti-SSB/La antibodies, or both, and their 113 infants diagnosed with CHB between 1970 and 1997 (56 boys, 57 girls). Of 57 pregnancies in which sufficient medical records were available, bradyarrhythmia confirmed to be CHB was initially detected before 30 weeks of gestation in 71 (82%) (median time 23 weeks). There were no cases in which major congenital cardiac anatomic defects were considered causal for the development of CHB; in 14 there were minor abnormalities. Twenty-two (19%) of the 113 children died, 16 (73%) within 3 months after birth.Cumulative probability of 3-year survival was 79%. Sixty-seven (63%) of 107 live-born children required pacemakers: 35 within 9 days of life, 15 within 1 year, and 17 after 1 year. Forty-nine of the mothers had subsequent pregnancies: 8 (16%) had another infant with CHB and 3 (6%) had a child with an isolated rash consistent with NLS.Conclusions. Data from this large series substantiate that autoantibody-associated CHB is not coincident with major structural abnormalities, is most often identified in the late second trimester, carries a substantial mortality in the neonatal period and frequently requires pacing. The recurrence rate of CHB is at least two- to three-fold higher than the rate for a mother with anti-SSA/Ro-SSB/La antibodies who never had an affected child, supporting close echocardiographic monitoring in all subsequent pregnancies, with heightened surveillance between 18 rand 24 weeks of gestation. (C) 1998 by the American College of Cardiology.