Fatal Epstein-barr Virus Myocarditis in a Child with Repetitive Myocarditis

Fatal Epstein-Barr virus (EBV) myocarditis occurred in a 9-year-old female with a history of two prior discrete episodes of myocarditis, the first associated with chicken pox and the second of undetermined origin. Serologic studies during the fatal episode were characteristic of acute EBV infection, and EBV genome was detected by polymerase chain reaction (PCR) amplification of DNA extracted from cutopsy heart and liver. PCRs for enteroviruses and cardiac viral culture were negative. An intense mononuclear cell infiltrate in the myocardium consisted entirely of T cells, without identifiable B cells. Human leukocyte antigen HLA-DR analysis using frozen tissue obtained postmortem revealed antigens DR4 and DR13. DR4 is associated with some autoimmune disorders, as well as idiopathic dilated cardiomyopathy. We postulate that an aberrant immune response, possibly associated with the DR4 locus, was responsible for the repetitive episodes of myocarditis in this patient.     

Cardiac Troponin T: A Marker in the Diagnosis of Acute Myocarditis in Children

This study was conducted to assess the use of serum cardiac troponin T (cTnT) level as a noninvasive indicator to diagnose acute myocarditis in children. Noninvasive conventional methods often fail to diagnose myocarditis, A median cTnT level of 0.088 ng/ml (0.04–3.11) was reported in pediatric patients with acute myocarditis in our previous study. Hence, we attempted to determine the cutfoff level of cTnT to diagnose acute myocarditis in children. Pediatric patients with clinically suspected myocarditis or dilated cardiomyopathy (DCM) and a control group were recruited. History, physical examination, elctrocardiogram, chest roentgenogram, echocardiogram, cTnT level, and/or endomyocardial biopsy and clinical course were studied. The gold standard to diagnose acute myocarditis was endomyocardial biopsy proved according to the Dallas criteria and/or recovery from cardiovascular problems within 6 months of follow-up. Forty-three patients were admitted due to cardiovascular problems from primary myocardial dysfunction. Twenty-four patients were diagnosed as acute myocarditis (group 1), 19 were idiopathic chronic DCM (group 2), and 21 patients had moderate to large ventricular septal defect and congestive heart failure (group 3). Median cTnT level was statistically higher in (group 1) compared to groups 2 and 3. Ejection fraction (EF) and left ventricular end diastolic dimension (LVEDd) z score of acute myocarditis were 38.5% (range, 21–67) and 1.3 (range, –0.8–3.0), respectively, which were significantly better than DCM [28.0% (range, 17–45) and 6.0 (range, 2.0–10.0)]. The cutoff point of cTnT level to diagnose acute myocarditis was 0.052 ng/ml (sensitivity, 71%; specificity, 86%). cTnT level, EF, and LVEDd z score did not predict short-term outcomes of patients. In acute myocarditis, cTnT level and EF were significantly higher and LVEDd z score was significantly lower than in DCM. However, the three parameters had no significant effect on outcomes of the patients. Our data show that cardiac a cTnT level of 0.052 ng/ml is an appropriate cutoff point for the diagnosis of acute myocarditis.     

The Clinical Course of Acquired Complete Heart Block in Children with Acute Myocarditis

A case of biopsy-proven myocarditis in a 7-year-old complicated by advanced atrioventricular (AV) block prompted a review of the medical literature to determine the prognosis for recovery of AV conduction or need for pacemaker implantation. A total of 40 patients younger than 20 years of age were identified, with return of AV conduction within 7 days in 27 patients (67%), permanent pacing in 11 patients (28%) with persistent AV block, and death in 2 patients (5%).     

Ventricular Tachycardia in Acute Fulminant Myocarditis: Medical Management and Follow-up

The combination of ventricular tachycardia (VT) and severe left ventricular dysfunction presents a serious challenge in management of acute fulminant myocarditis (AFM). We report a case of a 17-month-old girl with AFM, presented with hypotension and VT, successfully treated with respiratory and inotropic support, high-dose intravenous immunoglobulin, and amiodarone. The myocardial function improved significantly within 2 weeks of treatment. The clinical course was complicated by significant amiodarone-induced hepatotoxicity, disseminated intravascular coagulation, and deep-vein thrombosis. She was later diagnosed with congenital dysfibrinogenemia and treated with chronic Lovenox therapy.     

Predictors of Outcome of Myocarditis

Heart failure from myocarditis may be transient or may progress to unremitting severe cardiac failure. This study was performed to determine the outcomes and prognostic features of pediatric patients with myocarditis. Patients with the diagnosis of myocarditis between 1990 and 2001 were identified through the coding system of Yale–New Haven Hospital. A total of 28 patients were included, with ages ranging from 1 day to 20 years. Before discharge, 11 patients developed unremitting severe cardiac failure. Of the remaining 17 patients, at the time of discharge 10 had normal systolic function and 7 had decreased systolic function. Unremitting cardiac failure developed in 9 of 14 patients (64%) with an ejection fraction <30% and in only 2 of 14 (14%) of those with an ejection fraction 30% on admission (p < 0.01). Furthermore, shortening fraction <15%, left ventricular dilatation, and moderate to severe mitral regurgitation on admission as well as arrhythmia were significantly associated with development of unremitting severe cardiac failure. In this series of patients with myocarditis, by the time of discharge 39% of the patients had developed unremitting severe cardiac failure, 25% had depressed systolic function, and 36% had normal systolic function. Predictive factors at admission for poor outcome were ejection fraction <30%, shortening fraction <15%, left ventricular dilatation, and moderate to severe mitral regurgitation.Data from this study was presented as a poster at the 2002 annual meeting of the Society for Pediatric Research and Pediatric Academic Societies.     

Myocarditis and pericarditis in children

Myocarditis and pericarditis are rare but important causes of pediatric chest pain. The diagnostic criteria, clinical course, causes, and treatment of myocarditis is reviewed. There is particular attention to the relationship of myocarditis with dilated cardiomyopathy. Supportive therapy remains the standard of care for pump dysfunction. The identification and treatment of pericarditis with associated large pericardial effusion can be lifesaving. This article reviews the important clinical features that might lead the clinician to diagnose either myocarditis or pericarditis and thus separate the few patients with either of these conditions from the legions of children with noncardiac chest pain.     

Severe Transient Left Ventricular Hypertrophy in an Infant with Acute Myocarditis and Heart Failure

A case of rare acute myocarditis with heart failure and transient left ventricular hypertrophy in a 4.5-month-old male infant is described. Initial echocardiogram demonstrated severe concentric left ventricular hypertrophy with depressed left ventricular systolic function and reduced left ventricular cavity. Left ventricular hypertrophy was transient; it decreased significantly within a couple of weeks concomitantly with the improvement of ejection fraction. The myocarditis resolved without sequelae. The natural course of this rare mode of presentation of myocarditis and its relationship to immunosuppressive and immunomodulatory treatment are discussed.     

非病毒感染性心肌炎

心肌炎在儿童中并不常见,是导致儿童扩张型心肌病及猝死的一个重要原因。其中以病毒性心肌炎最常见,主要以柯萨奇病毒感染后导致的心肌炎为主。近年来,非病毒感染性心肌炎受到重视,其病因包括自身免疫疾病、细菌及寄生虫感染、中毒、药物反应等。现就非病毒性心肌炎的病因及目前诊疗方法综述如下。     

Successful Heart Transplantation Following Neonatal Necrotic Enterovirus Myocarditis

Enterovirus myocarditis is a potentially devastating diagnosis in the neonatal setting, with an associated high mortality and risk for chronically impaired cardiac function. Transplantation may be a possible therapeutic option, but, due to the comparative scarcity of the presentation, the limited donor pool, and fears of viral persistence and recurrence of myocarditis (especially in the immunocompromised, post-transplant setting), there are no reported cases in the literature. This case report illustrates an encouraging one-year outcome following heart transplantation for necrotic enterovirus myocarditis in a neonate.     

Eosinophilic Myocarditis Presenting with Pediatric Myocardial Infarction

Determination of the etiology of myocardial infarction (MI) in children can present a challenge to the practitioner since cases of pediatric MI are rare and the causes can be diverse. We report an unusual case of pediatric eosinophilic myocarditis that presented with MI.     

病毒性心肌炎诊断的研究进展

病毒性心肌炎是一种自然病程变化很大的疾病,其临床表现与多数辅助检查均缺乏特异性,致使确诊非常困难,特别是亚临床型及暴发型的病例.因此,应根据患者的症状、体征结合辅助检查的特点,对不同时期的患者予以针对性检查,以进一步确诊病毒性心肌炎.现将有助于病毒性心肌炎诊断的各种检查的特点综述如下.     

Myocarditis in infants and children: A review for the paediatrician

Clinical myocarditis is uncommon in infants and children. The most common pathogen is Coxsackievirus B. The offending agent triggers an immune response, which results in myocardial edema with subsequent impairment of systolic and diastolic function. Newborns and infants are more severely affected because the immature myocardium has limited ways of adapting to an acute insult. Children typically present with sinus tachycardia and gallop on auscultation, cardiomegaly on chest x-ray and small voltages on electrocardiogram. The echocardiogram shows reduced ventricular function. Viral studies can isolate the pathogen. Myocardial biopsy is useful diagnostically, but carries a significant risk for the sick infant. The first line of treatment includes measures such as rest, oxygen and diuretics. Inotropic agents are useful in moderate to severe heart failure. The role of immunosuppressive therapy is not yet clearly established in the paediatric age group. Prognosis is guarded in newborns but more favourable in older children.     

小鼠免疫性心肌炎与细胞凋亡的关系

目的 探讨猪心肌球蛋白致小鼠免疫性心肌炎（AM）心肌细胞损伤的变化，观察细胞凋亡在AM心肌损伤中的作用。方法 采用猪心肌球蛋白制备小鼠AM动物模型，取血清检测其抗肌球蛋白抗体和肌钙蛋白水平，取心肌组织切片HE染色了解心肌损伤情况；电镜和原位缺口末端标记技术（TUNEL）法观察心肌中细胞凋亡。结果 心肌炎组血清抗肌球蛋白抗体、肌钙蛋白水平及细胞凋亡指数（AI）均明显高于对照组；心肌炎组可见心肌细胞坏死和单核、淋巴细胞浸润，后期可见钙化灶和部分心肌纤维化。结论 小鼠AM中心肌损伤坏死与凋亡共存。其中异常的心肌细胞凋亡参与AM的发病过程。     

Pediatric Giant Cell Myocarditis and Orbital Myositis

Giant cell myocarditis is a rare but often fatal form of myocarditis that often requires cardiac transplantation and has been associated with autoimmune diseases. We describe a 14-year-old female who developed painful proptosis and was diagnosed clinically and histologically with orbital myositis that improved with corticosteroid therapy. Approximately 2 months later, she developed abdominal pain, vomiting, weight gain, and fatigue. She was diagnosed with congestive heart failure and cardiomyopathy, and endomyocardial biopsy revealed giant cell myocarditis. She was treated with immunosuppressive agents and has responded well, without the need for cardiac transplantation. Three previous case reports have described an association between giant cell myocarditis and orbital myositis, but we present the first pediatric case report. We conclude that if orbital myositis is diagnosed in a patient, regardless of age, cardiac function should be closely monitored to detect myocarditis, which may affect the overall outcome.     

暴发性心肌炎14例

目的 探讨小儿暴发性心肌炎的临床特点及诊治策略.方法　对2008年1月-2010年5月在本院住院治疗的14例暴发性心肌炎病例进行回顾性分析.其中男8例,女6例;年龄4个月8 d～11岁,平均4.9岁.患儿均符合暴发性心肌炎的诊断标准,伴不同程度心功能不全、心源性休克、心律失常等,并除外先天性心脏病、风湿性心脏病等其他心脏疾病.确诊后给予卧床休息、吸氧、镇静,应用大剂量丙种球蛋白、肾上腺皮质激素、磷酸肌酸,并予抗病毒、纠正心律失常等治疗.结果　14例暴发性心肌炎患儿中,9例初诊时误诊,误诊率为64.29%(9/14例).经治疗11例治愈或好转,1例于发病4个月转为扩张型心肌病,1例放弃治疗,1例死亡(发病2周转为扩张型心肌病).患儿均存在心电图改变,包括Ⅲ度房室传导阻滞3例,Ⅰ度房室传导阻滞及不完全性右束支传导阻滞各2例,频发室性期前收缩及窦性心动过速各2例,左束支传导阻滞1例,广泛ST-T改变及低电压各1例.7例患儿CK-MB和肌钙蛋白I均升高,1例仅有肌钙蛋白I升高.结论　暴发性心肌炎临床表现缺乏特异性,易误诊,但若能及时诊断、及时抢救,预后相对较好.其心电图的改变要早于心肌酶谱的升高.     

病毒性心肌炎患儿免疫状况的研究

目的：探讨病毒性心肌炎 (VMC)患儿在细胞免疫、体液免疫和自身免疫方面的改变,从而指导免疫治疗。方法：用流式细胞技术分别测定 30例急性期、2 2例迁延期VMC患儿及30例对照的血清CD2 3,CD3/HLA DR,用比浊法测IgG,IgA和IgM。 结果：VMC患儿急性期、迁延期的CD2 3,IgG ,IgA ,IgM及CD3+ /HLA -DR+,CD3-/HLA -DR+ 细胞比例均高于正常对照组 (P均 <0 .0 1)。VMC患儿CD2 3和IgG ,IgA ,IgM呈正相关 (r分别=0.30,0.17,0.40,P<0.0 5),而急性期、迁延期VMC患儿的CD3+ /HLA DR-细胞表达率与正常对照组相比均无明显差异 (P均 >0 .0 5 )。迁延期患儿的各项指标和急性期相比无明显差异(P均>0.05)。结论：病毒性心肌炎患儿急性期、迁延期的细胞免疫、体液免疫均处于增强状态,提示细胞免疫、体液免疫可能参与了VMC患儿的心肌损伤。     

儿童过敏性肺炎1例并文献复习

目的 总结儿童过敏性肺炎的临床、影像学和病理学特点。方法 对病理确诊的1例9岁6个月的过敏性肺炎患儿的临床、影像学和病理学资料进行总结,并复习文献进行讨论。结果 以咳嗽4个月、气促为主要表现,无咳痰、无三凹征,肺内未闻及啰音,无杵状指趾。肺功能示混合性通气功能障碍。肺部高分辨CT表现为弥漫的细小结节影,以上肺为重,肺组织病理为细支气管炎、细支气管周围的间质淋巴细胞炎症和间质的多核巨细胞,无纤维化和结核结节,可见大量的泡沫细胞。脱离环境并予甲泼尼龙2 mg·kg-1·d-1治疗2周后,患儿咳嗽、气促症状有所减轻,肺功能有所改善,肺部CT的小结节影明显改善。糖皮质激素治疗2个月后呼吸道症状消失,肺部影像学和肺功能恢复正常。结论 过敏性肺炎为无特异的沉淀抗体的病例,主要依靠肺活检病理细支气管炎、细支气管周围间质炎症和找到非干酪的不典型肉芽肿或多核巨细胞确定诊断。亚急性过敏性肺炎用糖皮质激素治疗有效。