Indiana University School of Medicine, Indianapolis, IN, 46202, USA.
Department of Dermatology, Indiana University School of Medicine, Indianapolis, IN, 46202, USA.
BMC Cardiovasc Disord. 2020 Jul 29;20(1):352. doi: 10.1186/s12872-020-01637-4.
Cardiac manifestations of neonatal lupus include an array of structural and conduction abnormalities due to placental transference of maternal anti-SSA/Ro and anti-SSB/La autoantibodies. Late-onset neonatal lupus cardiomyopathies, occurring outside the neonatal period, is an infrequently reported manifestation with unknown pathophysiology and poorly defined treatment regimens. Due to the rarity of this condition, additional studies and case reports are required to better understand and manage late-onset neonatal lupus cardiomyopathies.
A 4-week-old female, born to a mother with known anti-SSA/Ro and anti-SSB/La autoantibodies, presents with classic cutaneous manifestations for neonatal lupus and is found to have left bundle branch block, severely dilated cardiomyopathy with an ejection fraction of 25%, and a thin echogenic dyskinetic ventricular septum. Weekly second trimester and 30-week fetal echocardiograms showed no signs of structural or conduction abnormalities. There were no histologic signs of inflammation on cardiac tissue biopsy. After a complicated hospital course, she was successfully treated with biventricular pacemaker, intravenous immunoglobulin, and plasmapheresis.
We present a case of late-onset neonatal lupus with severe dilated cardiomyopathy, a dyskinetic ventricular septum, and left bundle branch block. To our knowledge, the dyskinetic ventricular septum has never been reported and left bundle branch block is rarely reported in NL. This case further validates the need for long term cardiac follow up for patients born with NL, even if lacking cardiac manifestations in the peripartum period. We characterize a unique presentation of a rare clinical entity, highlighting the diagnostic challenges, and describe a successful treatment course.
新生儿狼疮的心脏表现包括由于母体抗 SSA/Ro 和抗 SSA/La 自身抗体的胎盘转移而导致的一系列结构和传导异常。晚发型新生儿狼疮性心肌病发生在新生儿期之外，是一种罕见的表现，其发病机制尚不清楚，治疗方案也未明确定义。由于这种情况很少见，需要更多的研究和病例报告来更好地理解和管理晚发型新生儿狼疮性心肌病。
一名 4 周大的女性，出生于已知携带抗 SSA/Ro 和抗 SSA/La 自身抗体的母亲，患有新生儿狼疮的典型皮肤表现，并发现存在左束支传导阻滞、严重扩张型心肌病，射血分数为 25%，以及薄的回声运动失调室间隔。每周的中期和 30 周胎儿超声心动图均未显示结构或传导异常的迹象。心脏组织活检无组织学炎症迹象。经过复杂的住院治疗，她成功地接受了双心室起搏器、静脉注射免疫球蛋白和血浆置换治疗。
我们报告了一例晚发型新生儿狼疮伴严重扩张型心肌病、运动失调室间隔和左束支传导阻滞的病例。据我们所知，运动失调室间隔从未被报道过，而左束支传导阻滞在 NL 中也很少被报道。该病例进一步证实了即使在围产期缺乏心脏表现，出生时患有 NL 的患者也需要长期的心脏随访。我们描述了一种罕见临床实体的独特表现，强调了诊断挑战，并描述了成功的治疗过程。
