Buyon J P, Hiebert R, Copel J, Craft J, Friedman D, Katholi M, Lee L A, Provost T T, Reichlin M, Rider L, Rupel A, Saleeb S, Weston W L, Skovron M L
Department of Rheumatology and Medicine, Hospital for Joint Diseases, New York University School of Medicine, New York 10003, USA.
J Am Coll Cardiol. 1998 Jun;31(7):1658-66. doi: 10.1016/s0735-1097(98)00161-2.
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.
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.
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 87 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.
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 and 24 weeks of gestation.
本研究利用新生儿狼疮研究登记处的信息，描述自身免疫相关先天性心脏传导阻滞（CHB）的人口统计学特征、死亡率、发病率和复发率。
出生时或出生前检测到的孤立性CHB与母体针对48-kD SSB/La、52-kD SSA/Ro和60-kD SSA/Ro核糖核蛋白的自身抗体密切相关，是新生儿狼疮综合征（NLS）的一种永久性表现。现有数据因该疾病的罕见性而受到限制。
该队列包括105名血清中含有抗SSA/Ro或抗SSB/La抗体，或两者皆有的母亲，以及她们在1970年至1997年间被诊断为CHB的113名婴儿（56名男孩，57名女孩）。在87例有足够医疗记录的妊娠中，最初在妊娠30周前检测到确诊为CHB的心律失常的有71例（82%）（中位时间为23周）。没有病例认为主要先天性心脏解剖缺陷是CHB发生的原因；14例有轻微异常。113名儿童中有22例（19%）死亡，16例（73%）在出生后3个月内死亡。3年生存率的累积概率为79%。107名活产儿童中有67例（63%）需要起搏器：35例在出生后9天内，15例在1年内，17例在1年后。49名母亲随后再次怀孕：8例（16%）又有一名婴儿患CHB，3例（6%）有一名患有与NLS一致的孤立性皮疹的儿童。
来自这个大型系列的数据证实，自身抗体相关的CHB与主要结构异常无关，最常在孕中期晚期被发现，在新生儿期有相当高的死亡率，并且经常需要起搏治疗。CHB的复发率比从未有过患病孩子的抗SSA/Ro-SSB/La抗体母亲的复发率至少高出两到三倍，这支持在所有后续妊娠中进行密切的超声心动图监测，在妊娠18至24周期间加强监测。
