小儿暴发型心肌炎引起的致命性及很危险心律失常的诊断与处理

国内多数学者认为所谓“暴发型心肌炎”是指那些起病急骤、病情发展迅猛、预后凶险的急性重型心肌炎〔1 - 3〕。临床常表现为急性充血性心力衰竭、心源性休克和 /或致命性及很危险的心律失常 ,此型患儿如不被及时有效救治 ,病死率很高 ,有的病例甚至发生猝死。近年来对暴发型心肌炎的治疗有不少进展 ,下面仅就小儿暴发型心肌炎引起的致命性及很危险的心律失常的诊断和处理进行讨论。1　致命性心律失常和很危险心律失常的概念对小儿心律失常的分类方法较多 ,目前常按病理生理 (激动的起源和传导 )分类 ,也可以按心律是否规则、心率快慢进行分…     

小儿暴发型心肌炎合并阿-斯综合征的急救治疗

近年来小儿病毒性心肌炎发病率有上升趋势 ,由于急性炎症累及心肌组织的广泛性及严重度不同 ,其临床表现及结局差异甚大。多数普通型病例预后良好 ,但少数危重病例 ,起病急骤呈暴发型 ,可导致心源性休克、急性心功能不全及严重心律失常 ,后者导致阿 斯综合征 ,预后严重 ,本文重点讨论暴发型心肌炎所致阿 斯综合征的临床特点及急救治疗。1　暴发型心肌炎合并心脑综合征 (阿 斯综合征 ,Adams Stokessyndrome,ASS)的临床表现暴发型心肌炎患者 ,主要由于严重广泛的心肌炎症 ,不仅影响心肌收缩功能使心脏排血量骤降 ,同时累及心脏传导组织 ,导…     

儿童暴发性心肌炎的临床特点及预后的影响因素

目的 调查儿童暴发性心肌炎的临床特点及预后的影响因素,为临床诊治及预后评估提供参考。方法 回顾性分析24例暴发性心肌炎患儿的临床资料。根据患儿预后分为存活组(n=12)和死亡组(n=12)。应用logistic回归分析筛选出影响暴发性心肌炎患儿预后的危险因素。结果 24例暴发性心肌炎患儿中,入院首发症状为消化系统症状者14例,神经系统症状12例,呼吸系统症状1例,循环系统症状2例。入院时血清肌酸激酶MB同工酶、肌钙蛋白I、脑钠肽水平均升高;左室射血分数减低22例(92%);心胸比值增大10例;Ⅲ度房室传导阻滞8例,ST段改变11例,室性心律2例。死亡组患儿左室射血分数低于存活组(PPOR=7.418,P结论 儿童暴发性心肌炎临床特点缺乏特异性。左室射血分数减低是暴发性心肌炎患儿预后不良的危险因素。     

儿童暴发性心肌炎诊断与救治的几个问题

儿童暴发性心肌炎起病急、病情严重、进展迅速,常有严重心律失常、心源性休克或心力衰竭等发生,容易导致急性期死亡.部分患儿以呼吸急促、发绀等呼吸道症状或呕吐、腹痛等消化道症状起病,容易延误诊断.发病早期及时识别并给予恰当治疗可降低病死率.该文同时介绍暴发性心肌炎血流动力学监测、体外膜肺、临时心脏起搏器等在救治中应用近况.     

体外膜肺氧合在儿童暴发型心肌炎应用的多中心调查

目的 探讨中国大陆PICU应用体外膜肺氧合(extracorporeal membrane oxygenation,ECMO)支持儿童暴发型心肌炎治疗的效果.方法 采用问卷调查方法回顾自2006年4月中国大陆PICU首次开展ECMO 治疗以来,国内三级儿童专科医院或综合医院的PICU应用ECMO支持暴发型心肌炎的治疗情况;总结分析临床特点及预后.结果 共有3家医院的23例儿童急性暴发型心肌炎患者接受ECMO治疗,男18例,女5例;平均年龄(86.3±48.8)个月,平均体重(25.8±12.1)kg,ECMO治疗前左室射血分数(39.5±15.6)%,ECMO平均治疗时间(119.1±57.3)h.18例患儿存活出院,5例死亡.所有患儿经ECMO治疗24h后平均动脉压均有上升,从ECMO前的(60.7±23.7)mmHg(1mmHg=0.133kPa)升至(72.1±9.8)mmHg,并且存活者上升水平显著高于死亡者(P=0.04);血清乳酸水平从ECMO前的(6.8±5.1) mmol/L降至(2.9±2.6)mmol/L,存活者血清乳酸水平降低幅度显著大于死亡者(P<0.001).23例患儿中,成功撤离ECMO 21例,成功撤离率为91.3%;3例患儿撤离ECMO后30d内死亡;18例好转出院,整体存活率为78.3%.死亡患儿ECMO支持时间长于存活患者,但两者比较差异无统计学意义(P=0.41).所有患儿平均医疗花费(16.4±4.9)万元,存活者与死亡者比较差异无统计学意义(P=0.24).18例存活患儿中,共有15例随访,发生神经系统后遗症2例,1例颈总动脉血栓形成,1例心功能不全.结论 ECMO可为儿童急性暴发型心肌炎患者提供有效的循环支持,促进血流动力学稳定,提高存活率.     

新生儿心肌炎的临床特征

目的：了解新生儿心肌炎的临床特征。方法：将该科1997年12月至2000年5月收治的可疑心肌炎患儿,依据诊断性检查,结合起病情况进行分析。结果：新生儿心肌炎以生后24h内发病最多,为71.1%,首发症状及体征以呼吸急促、面色青紫、心律失常等表现为主。诊断性检查,心电图表现绝大多数为异位心律失常,占82.4%,彩色多普勒超声心动图以三尖瓣返流占第一位,其次是先天性心脏病和心包积液。心肌酶谱生后1周内普遍明显增高而且持续时间长。结论：新生儿心肌炎临床表现不典型,呈非特异性,大多数为呼吸系统症状,其次是循环系统体征,部分新生儿心肌炎与先天性心脏病并存。     

儿童暴发性心肌炎12例临床分析

目的 探讨儿童暴发性心肌炎的临床特点及诊治策略.方法 对2007年1月至2011年12月在本院住院治疗的12例暴发性心肌炎病例进行回顾性分析.结果 男3例,女9例.年龄1个月～14岁,其中1个月～1岁5例,6岁以上5例.发病季节5月～9月8例.首发症状以精神反应差、呕吐、气促、面色苍白或发绀、发热多见.8例患儿初诊时被误诊.所有患儿CK-MB、肌钙蛋白升高,伴有心电图异常,表现为ST-T改变及低电压(9例)、传导阻滞(7例)、心律失常(8例).12例心脏彩超见左室收缩和舒张功能减低.7例X线胸片见心影增大.确诊后给予抗病毒、营养心肌、抗心力衰竭、抗休克、纠正心律失常、早期静脉滴注甲泼尼龙和大剂量丙种球蛋白,并给予镇静、吸氧等对症支持治疗.10例呼吸机辅助通气.7例安装心脏临时起搏器.经治疗,7例治愈或好转出院,目前随访一般情况良好,无活动受限的表现.病死率41.7％.结论 暴发性心肌炎起病急,进展迅速,病死率高,表现缺乏特异性,部分患儿以心外表现起病,易误诊,若能尽早诊断和及时抢救,预后相对较好.     

小儿脱髓鞘疾病糖皮质激素治疗效果初步探讨

目的探讨小儿脱髓鞘疾病的临床特征和糖皮质激素的治疗效果.方法总结11例脱髓鞘患儿的临床资料和磁共振(MRI)特点,观察其临床转归和糖皮质激素的治疗效应.结果11例患儿,以急性或亚急性起病,7例起病前1～4周有发热、呼吸道或消化道感染症状,出现神经系统异常时感染征象已消失.主要表现有乏力、嗜睡、头痛、颅神经异常和肢体瘫痪.头部MRI检查全部异常,表现为白质、基底节、脑干和小脑T1WI等信号、低信号病灶和T2WI高信号病灶.甲基泼尼松龙或泼尼松治疗4～7天症状好转,4～12周后治愈6例,好转5例.其中3例有复发.结论儿童急性脱髓鞘疾病①多数与感染有关;②临床多样化,颅神经受累和肢体瘫痪是主要神经系统表现;③MRI为诊断本病的主要辅助手段之一;④糖皮质激素治疗有效,但部分患儿有复发     

超声引导下经皮穿刺无水酒精注射治疗儿童单纯肾囊肿

目的 探讨超声引导下经皮穿刺尤水酒精注射治疗儿童单纯性肾囊肿的疗效.方法 我院采用超声引导下经皮穿刺尤水酒精注射治疗14例单纯性肾囊肿患儿(男6例.女8例).术后随访1～24个月,观察患儿症状改善情况,并复查B超.结果 14例患儿治疗1个月后囊肿体积均较治疗前减少;治疗6个月后,7例患儿8个囊肿完全消失.3例体积缩小50%以上;治疗12个月后,10例患儿11个囊肿完全消失.2例体积缩小50%以卜.随访期问.患儿的症状均得到不同程度的缓解.结论 对于有症状的儿童单纯性肾囊肿,超声引导下经皮穿刺无水洒精注射治疗具有安全、有效、经济和微创的特点.     

甲亢危象并发多器官衰竭1 例报告

目的探讨儿童甲亢危象的临床特征。方法回顾分析1例甲亢危象并发多器官衰竭患儿的临床资料。结果女性患儿,13岁,既往无甲状腺疾病史。患儿以恶心、呕吐、神萎起病,入院时以心律失常、心源性休克、心力衰竭为主要表现,考虑为暴发性心肌炎。有轻度突眼。游离T3、游离T4明显增高,促甲状腺激素明显降低。入院后相继出现呼吸衰竭、肾衰竭及肝衰竭,甲亢危象评分120分,确诊为甲亢危象。经积极抗心律失常、应用血管活性药物、呼吸支持、丙硫氧嘧啶、碘剂、糖皮质激素、血液净化等综合治疗,患儿好转出院。结论甲亢危象在儿童较为罕见,但可以作为儿童甲状腺疾病的首发症状出现。甲亢危象表现隐匿,早期识别、及早综合治疗是治疗成功的关键。     

儿童暴发性心肌炎的早期诊断及治疗进展

暴发性心肌炎是一种以急性血流动力学障碍为主要表现的心肌炎,临床表现多样且病死率极高。暴发性心肌炎早期诊断需依靠血清学指标、心电图、超声心动图等进行综合分析,心脏磁共振成像检查在心肌炎诊断中也具有很好的应用价值。传统的暴发性心肌炎治疗,包括对症支持、抗病毒及免疫疗法等,但对危重患儿往往难以奏效。体外膜肺氧合技术(ECMO)的开展,大大提高了儿童暴发性心肌炎的抢救成功率,成为救治儿童暴发性心肌炎的可靠手段。文章综述目前儿童暴发性心肌炎的诊断及最新治疗进展。     

A Cluster of Fulminant Myocarditis Cases in Children,Baltimore, Maryland, 1997

The true incidence of myocarditis in children is difficult to estimate because many mild cases go undetected. This study describes an unusual cluster of myocarditis cases that occurred in young children living in the greater Baltimore area between May and October 1997. A search of multiple comprehensive databases and interviews with area pediatric cardiologists were conducted to identify unreported cases and determine the background rate of myocarditis in the area. Seven cases of myocarditis were found as well as two with a similar clinical picture and myocardial fibrosis on tissue examination. Six case patients with active myocarditis and one child with fibrosis died. The case children were predominantly black (eight of nine) and male (seven of nine), with no identifiable risk factors. The disease was characterized by a fulminant course with malignant arrhythmias. The greatest number of pediatric myocarditis deaths reported in 1 year prior to 1997 was three. Myocardial tissues were examined using immunohistochemistry, in situ hybridization, and polymerase chain reaction but no etiologic agent was identified. This outbreak is unusual because of both the number of cases and the fulminant course of the disease in this group of children.     

小儿暴发性心肌炎危险因素分析

目的：通过对小儿病毒性心肌炎临床表现、体征以及实验室检查指标等方面进行回顾性分析研究,以探讨小儿暴发性心肌炎的危险因素。方法：收集临床诊断为病毒性心肌炎患儿资料71例,分为暴发性心肌炎组(n=16)和非暴发性心肌炎组(n=55),采用χ2检验或t检验对两组患儿入院时的临床表现、心电图、心脏B超与血清生化检查等各项指标进行回顾性分析,对有统计学意义的相关因素,进行logistic多元回归分析,研究暴发性心肌炎的独立高危因素。结果：暴发性心肌炎组死亡率远高于非暴发性心肌炎组（50% vs 0%）。急性病毒性心肌炎患儿入院时血压降低、血清CK-MB水平升高、cTnI阳性、心电图QRS波时限延长、ST段改变、完全性房室传导阻滞、完全性左束支传导阻滞,以及左室射血分数和左室短轴缩短率降低与心肌炎暴发阶段密切相关。QRS波群时限延长（OR＝1.139;CI＝1.014~1.279; P<0.05）和左心室射血分数降低（OR＝0.711;CI＝0.533~0.949;P<0.05）是小儿暴发性心肌炎的独立阳性预测因素。结论：暴发性心肌炎死亡率高。入院时QRS波群时限延长和左心室射血分数降低是小儿暴发性心肌炎的独立危险因素。［中国当代儿科杂志,2009,11（8）：627-630］     

Circulating cardiac troponins levels and cardiac dysfunction in children with acute and fulminant viral myocarditis

Objectives: To study the correlation between cardiac Troponins blood levels and degrees of cardiac dysfunction in children with acute and fulminant viral myocarditis and to study their prognostic role in predicting the outcomes and risk of having dilated cardiomyopathy. Methodology: Troponin I & T blood levels were measured in 65 children with acute or fulminant viral myocarditis. The cardiac functions of RV & LV were assessed by Doppler echocardiography. Results: The levels of cTnI & CTnT were significantly higher in patients with fulminant myocarditis than in controls and children with acute myocarditis (p < 0.05 & <0.001* respectively). The cardiac functions were significantly impaired in fulminant myocarditis than in acute myocarditis (p < 0.001*). There were negative correlations between the cardiac troponins levels and the cardiac functions measured by echocardiography in children with acute and fulminant myocarditis. There were 3 deaths (7.5%), and 10 (25%) children developed dilated cardiomyopathy in acute myocarditis while there were eight deaths (32%) and one patient (4%) who developed dilated cardiomyopathy in fulminant myocarditis group. Conclusion: Cardiac troponins levels can predict the severity of myocarditis and the prognosis on the short‐term level. Fulminant myocarditis was associated with higher levels of both cTn I & cTn T than acute myocarditis. Despite that fulminant myocarditis has a more aggressive course, the risk of developing cardiomyopathy was less than in acute myocarditis.     

The challenges of prompt identification and resuscitation in children with acute fulminant myocarditis: case series and review of the literature

Aim: To describe the clinical presentation, triage, resuscitation and outcome of acute fulminant myocarditis in children presenting to district hospitals and referred for cardiac intensive care. Methods: Case series describing five patients (from 2 weeks to 12 years old) with a diagnosis of acute fulminant myocarditis, presented to outlying hospitals between December 2006 and December 2007 and retrieved to a cardiac intensive care unit. Results: All children were admitted with non‐specific symptoms such as vomiting, cough and poor feeding to their local hospital, where various provisional diagnoses such as viral gastroenteritis, bronchitis or renal failure were considered. Acute physiological deterioration usually prompted the referral for intensive care. Two children died at the referring hospital during stabilisation by the retrieval team. Three children survived transport to intensive care and to hospital discharge; two received mechanical support and one underwent urgent orthotopic heart transplantation. Enterovirus and parvovirus were identified as causative agents in two patients. In one case, macrophage activation syndrome was diagnosed although no clear viral trigger was identified. Median length of hospitalisation among survivors was 33 days, and mechanical cardiac support was required for a median of 12 days. Conclusions: The diagnosis and initial management of acute fulminant myocarditis is extremely challenging. Prognosis for patients admitted to a cardiac centre for early mechanical support can be very favourable, while a delay in considering the diagnosis may result in poor outcome. The diagnosis of myocarditis should be considered in any previously well child presenting with a viral prodrome and non‐specific organ dysfunction associated with dysrhythmias, shock or acute heart failure, even in the absence of cardiomegaly.     

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.     

CD8 + T-LYMPHOCYTES INFILTRATE THE MYOCARDIUM IN FULMINANT HERPES VIRUS MYOCARDITIS

We report two cases of fulminant viral myocarditis in previously healthy children. They were caused by herpes simplex virus (HSV)-1 (in a boy aged 3 years) and Epstein-Barr virus (EBV) (in a boy aged 12 months). We obtained the diagnosis of HSV-1 myocarditis by immunohistochemistry and the diagnosis of EBV myocarditis by in situ hybridization. Histologic examination of heart tissue from the two boys revealed mononuclear cell infiltration of the myocardium. Immunohistochemical staining identified these cells as CD8 + T-lymphocytes. CD8 + T-lymphocytes induced by herpes virus infections may play an important role in the damage to heart muscle fibers seen in fulminant myocarditis in previously healthy children. To our knowledge, this is the first report of HSV-1 or EBV myocarditis (at least in children) in which viral infection has been demonstrated in the myocardium.     

CD8+ T-lymphocytes infiltrate the myocardium in fulminant herpes virus myocarditis

We report two cases of fulminant viral myocarditis in previously healthy children. They were caused by herpes simplex virus (HSV)-1 (in a boy aged 3 years) and Epstein-Barr virus (EBV) (in a boy aged 12 months). We obtained the diagnosis of HSV-1 myocarditis by immunohistochemistry and the diagnosis of EBV myocarditis by in situ hybridization. Histologic examination of heart tissue from the two boys revealed mononuclear cell infiltration of the myocardium. Immunohistochemical staining identified these cells as CD8+ T-lymphocytes. CD8+ T-lymphocytes induced by herpes virus infections may play an important role in the damage to heart muscle fibers seen in fulminant myocarditis in previously healthy children. To our knowledge, this is the first report of HSV-1 or EBV myocarditis (at least in children) in which viral infection has been demonstrated in the myocardium.     

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目的分析感染性心肌炎所致小儿完全性房室传导阻滞(CAVB)的临床特点及治疗,以提高对该病的认识。方法对1984-08—2005-03上海市交通大学附属儿童医院收治的感染性心肌炎所致CAVB17例进行回顾性分析。结果17例CAVB患儿出现阿斯综合征者8例,心功能不全者5例。17例胸片检查心影增大13例。13例心脏超声检查示均有左心室增大。心电图示平均心室率为(52·8±10·4)/min。急重症心肌炎6例,均予甲基泼尼松龙静脉冲击和营养心肌治疗,3例安置临时起搏器进行治疗;其余患儿都以改善传导、增快心室率药物治疗。治愈6例,好转5例,仍为CAVB5例,死亡1例。结论急重症心肌炎发生CAVB可以是致命的,如果能早期诊断、及时应用大剂量肾上腺皮质激素及给予急诊临时起搏器处理,将会取得较好的预后。在随访心肌炎所致房室传导阻滞患儿时,如并有束支传导阻滞者、QRS波增宽、结性逸搏时要高度警惕其可能进展为CAVB。