Maternal anti-Ro and anti-La antibody-associated endocardial fibroelastosis
Maternal anti-Ro and anti-La antibody-associated endocardial fibroelastosis
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DOI：
10.1161/hc0702.104182
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发表时间：
2002-02-19
期刊：
影响因子：
37.8
通讯作者：
Hornberger, LK
中科院分区：
文献类型：
--
作者：
Nield, LE;Silverman, ED;Hornberger, LK
母体抗Ro和抗La抗体与先天性心脏传导阻滞（CHB）有关。虽然内皮弹力纤维增生症（EFE）已被描述在孤立的情况下，自身抗体介导的慢性乙型肝炎，这种疾病的自然史和发病机制知之甚少。方法和结果，我们回顾性地审查了临床病史，超声心动图，和病理的胎儿和儿童EFE与慢性乙型肝炎出生的母亲阳性抗Ro或抗La抗体在5个中心。确定了13例患者，6例产前诊断，7例产后诊断。6名母亲抗Ro和抗La抗体阳性，7名母亲仅抗Ro抗体阳性。只有1名母亲患有自身免疫性疾病。所有胎儿和出生后病例均出现严重的心室功能障碍。4例胎儿和3例出生后病例在最初出现时有EFE。然而，2例胎儿和4例出生后病例分别在CHB诊断后6至12周和7个月至5年发生EFE，即使在6例出生后病例中进行了心室起搏。11例（85%）死亡（n=9）或接受心脏移植（n=2）继发于EFE：病理评估的心脏，在10例，显示中度至重度EFE 7例和轻度EFE 3例，主要涉及左心室。免疫组化在4例（包括3个胎儿）表现出沉积的IgG在4和IgM在3例和T细胞浸润，这表明免疫反应的受影响的胎儿或child.Conclusions EFE发生在存在自身抗体介导的慢性乙型肝炎，尽管有足够的心室起搏。自身抗体相关性EFE具有非常高的死亡率，无论是在胎儿还是出生后发展。
Background-Maternal anti-Ro and anti-La antibodies are associated with congenital heart block (CHB). Although endocardial fibroelastosis (EFE) has been described in isolated cases of autoantibody-mediated CHB, the natural history and pathogenesis of this disease are poorly understood.Methods and Results-We retrospectively reviewed the clinical history, echocardiography, and pathology of fetuses and children with EFE associated with CHB born to mothers positive for anti-Ro or anti-La antibodies at 5 centers. Thirteen patients were identified, 6 with a prenatal and 7 with a postnatal diagnosis. Six mothers were positive for anti-Ro and anti-La antibodies, and 7 were positive for anti-Ro antibodies only. Only 1 mother had autoimmune disease. Severe ventricular dysfunction was seen in all fetal and postnatal cases. Four fetal and 3 postnatal cases had EFE at initial presentation. However, 2 fetal and 4 postnatal cases developed EFE 6 to 12 weeks and 7 months to 5 years from CHB diagnosis, respectively, even despite ventricular pacing in 6 postnatal cases. Eleven (85%) either died (n=9) or underwent cardiac transplantation (n=2) secondary to the EFE: Pathologic assessment of the explanted heart, available in 10 cases, revealed moderate to severe EFE in 7 and mild EFE in 3 cases, predominantly involving the left ventricle. Immunohistochemistry in 4 cases (including 3 fetuses) demonstrated deposition of IgG in 4 and IgM in 3 and T-cell infiltrates in 3 cases, suggesting an immune response by the affected fetus or child.Conclusions-EFE occurs in the presence of autoantibody-mediated CHB despite adequate ventricular pacing. Autoantibody-associated EFE has a very high mortality rate, whether developing in fetal or postnatal life.