2021 年第 7 期 第 16 卷

心脏磁共振对新生儿狼疮心脏损害早期评估的临床价值

The clinical value of cardiac magnetic resonance in early evaluation of cardiac damage in neonatal lupus

作者：李佳周茜杨曦苏瑛刘桂英

英文作者：Li Jia Zhou Qian Yang Xi Su Ying Liu Guiying

单位：首都医科大学附属北京安贞医院儿科100029

英文单位：Department of Pediatrics Beijing Anzhen Hospital Capital Medical University Beijing 100029 China

关键词：新生儿狼疮；心脏磁共振；心脏损害；激素；预后 

英文关键词：Neonatallupus;Cardiacmagneticresonance;Cardiacdamage;Glucocorticoid;Prognosis

• 摘要：

• 目的 探讨心脏磁共振（CMR）对新生儿狼疮（NL）心脏损害早期评估的临床价值。方法 收集2017年6月至2019年12月首都医科大学附属北京安贞医院儿科收治的5例进行了CMR检查的NL患儿的临床资料，对其临床表现、实验室检查资料及相关治疗和随访情况进行回顾性分析。结果 5例NL患儿中男2例、女3例。宫内发现心律失常2例；出生心电图提示Ⅱ度窦房传导阻滞1例，Ⅰ度房室传导阻滞1例，Ⅱ度房室传导阻滞4例，窦性心动过缓3例，室性期前收缩2例，房性扑动2例，阵发性室上性心动过速1例。3例贫血，2例肝功能异常。5例患儿的抗核抗体均为阳性，抗SSA抗体阳性4例，抗SSB抗体阳性1例，抗核糖核蛋白抗体阳性4例，抗Ro-52抗体阳性3例。心肌标志物检查发现心肌肌钙蛋白I升高4例，肌酸激酶升高4例，肌酸激酶同工酶升高4例。心脏超声检查均未见异常。CMR提示4例患儿显示有延迟强化［晚期钆增强（LGE）］，1例心包积液，其中延迟强化伴有心肌水肿1例。5例患儿母亲孕前疾病：3例系统性红斑狼疮，2例干燥综合征。结合CMR检查5例患儿在营养心肌治疗基础上加用糖皮质激素治疗，其中1例左心室壁可见散在斑片状LGE伴心肌水肿联合丙种球蛋白治疗，2例肝功能损害给予保肝治疗，5例患儿心律失常在出生后2周均消失，出院后3、6个月复查抗核抗体滴度均较前有所下降，有3例新生儿在出院后6个月复查CMR，心包积液及心肌水肿均消失，LGE范围较治疗前缩小。结论 NL患儿最常见的心脏损害是心动过缓、传导阻滞；早期NL合并心脏损害患儿CMR检查可见LGE；出现LGE的NL患儿早期加用糖皮质激素针对性治疗，可改善预后。

• Objective To investigate the clinical value of cardiac magnetic resonance(CMR) in early evaluation of cardiac damage in neonatal lupus(NL). Methods From June 2017 to December 2019, clinical materials of 5 children with NL undergoing CMR detection admitted to Beijing Anzhen Hospital, Capital Medical University were collected, and the clinical manifestations, laboratory data, treatment and follow-up of them were retrospectively analyzed. Results There were 2 males and 3 females among 5 children with NL. Arrhythmia in utero was found in 2 cases. Electrocardiogram at birth indicated 1 case of Ⅱ degree sinoatrial block, 1 case of Ⅰ degree atrioventricular block, 4 cases of Ⅱ degree atrioventricular block, 3 cases of sinus bradycardia, 2 cases of ventricular premature contraction, 2 cases of atrial flutter, and 1 case of paroxysmal supraventricular tachycardia. There were 3 cases of anemia and 2 cases of abnormal liver function. Antinuclear antibody was positive in all 5 children, anti-Sjogren′s syndrome antigen A antibody was positive in 4 cases, anti-Sjogren′s syndrome antigen B antibody was positive in 1 case, anti-ribonucleoprotein antibody was positive in 4 cases and anti-Ro-52 antibody was positive in 3 cases. The examination of myocardial markers showed that 4 cases of cardiac troponin I increased, 4 cases of creatine kinase increased, and 4 cases of creatine kinase isoenzyme increased. Echocardiography showed no abnormality. CMR indicated that 4 cases delayed enhancement［late gadolinium-enhanced(LGE)］, 1 case of pericardial effusion, one of LGE case with myocardial edema. Among the pre-pregnancy diseases of 5 mothers, 3 cases were diagnosed with systemic lupus erythematosus and 2 cases had Sjogren′s syndrome. Combined with CMR examination, 5 chlidren were treated with glucocorticoid on the basis of nutritional myocardial therapy. Among them, 1 case of left ventricular wall showed scattered patchy LGE with myocardial edema who was treated by glucocorticoid and gamma globulin, and 2 cases of liver damage were given liver protection treatment. All the chlidren with arrhythmia disappeared in 2 weeks after birth. The antinuclear antibody titers in 3 and 6 months after discharge were all lower than before. Three cases of neonates reviewed CMR 6 months after discharge, and showed that pericardial effusion and myocardial edema disappeared, and LGE range was narrower than before. Conclusions Bradycardia and conduction block are the most common myocardial damage in children with NL. LGE is observed on CMR in early NL complicated with myocardial damage. Early treatment with glucocorticoid can improve the prognosis of NL children with LGE.