Symptomatic myocardial bridging in a child without hypertrophic cardiomyopathy

Myocardial bridging (MB) is a rare coronary anomaly in children that is typically associated with hypertrophic cardiomyopathy or left ventricular hypertrophy. Several reports, mainly in adults, have suggested an association between MB and sudden death or ischemia without other cardiac abnormalities. In this report, we describe an 11-year-old girl with syncope and manifestations of cardiac ischemia associated with MB of the middle segment of the left anterior descending artery. The coronary anomaly was not associated with left ventricular hypertrophy. Surgical unroofing of the affected coronary artery segment resulted in complete recovery. MB should be included in the differential diagnosis of children presenting with syncope and signs of ischemia even in the absence of ventricular hypertrophy.     

Isolierte kongenitale Koronaranomalien

Background

Isolated coronary anomalies are rare congenital malformations. The anomalous origin of the coronary artery from the pulmonary artery (ALCAPA, ARCAPA), the anomalous origination of the coronary ostium from the opposite, facing “coronary” sinus, coronary fistulas, and coronary aneurysms have to be mentioned.

Diagnosis

The symptoms vary from life-threatening events (e.g., myocardial infarction, cardiac syncope and ventricular dysrhythmia) to atrial or ventricular volume overload and asymptomatic courses. Echocardiography is used as a screening method; noninvasive modalities such as magnetic resonance imaging and computed tomography are additionally used for diagnosis, but coronary angiography is the gold standard to depict the morphology discussed in the individual case.

Therapy

The indication for conservative, interventional, or operative treatment must be decided for each patient individually.     

青少年冠状动脉异位起源于对侧冠状动脉窦的经胸超声心动图诊断

背景 冠状动脉异位起源于对侧冠状动脉窦(ACAOS)是非常罕见的冠状动脉畸形,如果在青少年时期发病常会引起非常严重的后果,例如心绞痛、心肌梗死甚至心源性猝死(SCD),诊断ACAOS主要的影像学检查方法包括心脏多层螺旋CT(MDCT)及冠状动脉造影(CAG),而通过经胸超声心动图(TTE)诊断ACAOS的报道较少.目的...     

晕厥儿童病因学及其临床特征的研究

目的分析晕厥儿童的病因分布并对各种病因晕厥儿童的临床特征加以分析,以提高其诊断效率。方法回顾性分析在北京大学第一医院儿科就诊的以“晕厥”为主诉的154例患儿的临床资料。结果自主神经介导的反射性晕厥是晕厥患儿的最常见的病因,占所有患儿的65．6％。其次为心源性晕厥,占所有患儿的6．5％。此外,还包括精神性疾病、神经源性及代谢性疾病导致的晕厥。自主神经介导的反射性晕厥、精神性晕厥患儿多见于青春期女孩,发病年龄大于心源性、神经源性及代谢性疾病导致的晕厥患儿（均P〈0．05）;心源性及精神性晕厥患儿的晕厥发作次数则显著多于自主神经介导的反射性晕厥、神经源性及代谢性疾病导致的晕厥患儿（均P〈0.05）。通过logistic回归分析显示,患儿的晕厥次数及晕厥发生时的体位对判断晕厥病因具有重要的意义（P〈0．05）。心电图的检查对发现心源性晕厥儿童具有重要意义。其他检查包括脑电图、Holter心电图、超声心动图检查及精神学评估在寻找晕厥儿童病因方面的意义有限。直立倾斜试验对诊断及鉴别诊断自主神经介导的反射性晕厥具有重要的意义。结论导致儿童晕厥的病因众多,可选择的辅助检查也有多种,但详细的病史收集、体格检查及心电图检查是诊断晕厥儿童的基础。     

Congenital Coronary Arteriovenous Fistula Presenting with Syncope

Previous reports of syncope in patients with coronary arteriovenous fistula (CAVF) have theorized that it occurs secondary to a coronary steal phenomenon. We present a case of syncope in a young woman with a CAVF and no anatomic substrate for coronary steal.     

Fainting Attacks in Children

Fainting attack or syncope in children is a common occurrence, with vasovagal syncope being the commonest cause for majority of pediatric syncope. The aim of emergency room evaluation is not to miss the rare serious underlying disorder causing syncope. A complete detailed history of the event followed by physical examination helps in categorising syncope into the three major categories—neurally mediated, cardiovascular and non cardiovascular syncope. Investigations will remain normal in majority of the cases. A 12-lead ECG and standing test should be done in all the cases which helps in establishing the cause for syncope. Management varies depending upon the cause and majority of them do not require hospital admission.     

Management of Pediatric Chest Pain,Palpitations, Syncope,and Murmur Presenting to the Emergency Department

Chest pain, palpitations and syncope are amongst the most common symptoms presenting to the emergency department (ED). A finding of a murmur, although rare to present as primary cause for ED visit, can often be an associated finding noted during the evaluation. Although these symptoms can be very alarming to patients and parents, they are infrequently associated with significant cardiac disease. Although the cardiac etiologies for these common symptoms are rare, these cardiac conditions can sometimes be life threatening. This review examines these presenting symptoms of chest pain, syncope and palpitations or the finding of murmur for possible differential diagnoses.     

Cardiac syncope in children]

Syncope is a frequent problem in childhood; generally, it is an isolated event and the common causes are benign. However, in some circumstances, syncope can herald a potentially lethal problem, especially when occurring during exercise. Routine evaluation includes history, physical examination and a 12-lead standard ECG should be performed in all cases. Worrying features which should be an indication for further investigation include syncope during exercise, collapse in a swimming pool, history of familial sudden death, and abnormalities on clinical exam or ECG. Structural cardiac abnormalities that may cause syncope and sudden death include aortic stenosis, hypertrophic cardiomyopathy and coronary malformations. All children with unrepaired or repaired congenital heart disease who experienced a syncope should be referred to a specialist. Primary arrhythmias that are easily diagnosed on ECG are the long QT syndrome, complete atrio-ventricular block and Wolff-Parkinson-White syndrome; ST elevation in V1-V3 may reveal a Brugada syndrome. Another arrhythmia which is known to be potentially fatal if undiagnosed is the catecholaminergic ventricular tachycardia; the baseline ECG is normal but the arrhythmia is easily reproduced during exercise testing. Finally, vasovagal syncope is the most likely cause of syncope in the young and it usually easily recognized.     

儿童左冠状动脉异常起源于右冠状窦伴动脉间行程四例分析

目的分析儿童左冠状动脉异常起源于右冠状窦伴动脉间行程(ALCA-R-IAC)的临床特征和诊治要点.方法对2015年11月至2018年6月在首都医科大学附属北京儿童医院住院确诊的4例ALCA-R-IAC患儿的临床表现、实验室检查、影像学特点、治疗及预后进行回顾性分析.结果4例患儿均为女童,发病年龄7.5~14.7岁,全部经CT冠状动脉成像(CTCA)检查确诊为ALCA-R-IAC.4例患儿均有运动性晕厥、急性心肌梗死的临床表现,伴急性左心衰竭3例、心源性休克1例、心跳骤停1例;1例患儿出现神经系统受累.所有患儿肌钙蛋白Ⅰ均明显增高(20.65~50.00μg/L);心电图显示左主干闭塞的图形特征;超声心动图发现3例射血分数降低(25%~45%),仅1例提示左冠状动脉异常起源可能.CTCA均表现为左冠状动脉异常起源于右冠状窦,并走行于主动脉和肺动脉之间,左主干受压纤细等.2例行冠状动脉去顶术治疗,2例保守对症予严格限制活动.门诊规律随访12~43个月,4例患儿均存活,无症状反复发作,预后良好.结论ALCA-R-IAC可导致青少年运动性晕厥、急性心肌梗死甚至猝死的临床表现.CTCA有助于及早明确诊断,一旦确诊应避免剧烈活动,外科手术是主要治疗手段.     

Sudden Cardiac Death in Young Athletes

Sudden cardiovascular death in young athletes is a rare event. However, when it does occur, it is devastating. An understanding of potentially worrisome history and physical examination elements, including symptoms of syncope, near syncope, dizziness, or shortness of breath with exercise, is important in the determination of risk for potentially lethal anatomical and functional cardiac defects. In active children and adolescents, HCM is the most common cardiac condition causing sudden death. Most often, HCM is clinically silent. The best tool for diagnosing HCM is 2-dimensional echocardiography. However, the use of this as a screening tool is prohibitively expensive. Any child presenting to the ED with exertional chest pain, syncope, near syncope, and/or shortness of breath should be evaluated further to rule out cardiac conditions as the cause.     

晕厥的病因分析及鉴别诊断

目的了解儿童晕厥病因组成特点,探讨以发病诱因及症状区分心源性与神经介导性晕厥的可行性。方法对111例住院患儿病例进行综合评估,以规范的诊断步骤对确诊晕厥的患儿进行病因分类。总结导致晕厥的诱因、先兆症状及发作症状,对心源性及神经介导性晕厥患儿资料中行统计学处理,试图发现两种晕厥的特征性表现。结果非心源性晕厥者占60.4%,直立性低血压占18.9%,血管迷走性晕厥及心源性晕厥分别占18.0%和9.0%。非心源性晕厥中以神经介导性晕厥为主。心源性仅占全部晕厥患者的极小部分。晕厥发作前持久站立及恶心是神经介导性晕厥的特征性表现。心源性晕厥往往与运动相关。结论非心源性晕厥者占晕厥患儿的大多数,其中以神经介导性晕厥为主。运动相关的晕厥为心源性晕厥的特征。发作前直立体位、伴恶心症状的多为神经介导性晕厥。     

Autonomic syncope in pediatrics: a practice-oriented approach to classification, pathophysiology, diagnosis, and management

This paper presents a practice-oriented approach to the problem of syncope in pediatrics. Autonomic syncope is the major etiologic category in pediatrics and consists of 2 types: reflex and dysautonomic. The latter type is rare in pediatrics. Reflex syncope has 4 subtypes: neurocardiogenic, central, situational, and cerebral. Neurocardiogenic syncope, the most common subtype, is easily diagnosed by taking a careful, detailed history; identifying diagnostic red flags; performing a complete physical examination; and ordering a minimum of laboratory tests. Patient and parent education is essential, and usually, without medication, outcomes are good.     

儿童体位性心动过速综合征的临床特征及随访研究

目的　探讨儿童体位性心动过速综合征 (POTS)的临床特征、诊断标准及治疗方案。方法　以符合诊断标准的 28例POTS患儿为研究对象,分析年龄分布、病程及基础血流动力学指标,同时观察其各种临床表现的发生频率及诊治效果。结果　诊断为POTS的患儿 28例,占 88例不明原因晕厥或起立后头晕就诊患儿的 32%,其中男 11例,女 17例。男∶女约为 1∶1.5。年龄 6~16岁,平均为(11.6±2.2)岁,其中 6~10岁 5例,占 18%, 10~16岁(包括 10岁)23例,占 82%。病程为 1个月~6年,平均(13.3±19.6)个月,半数以上在 6个月以内;最常见的临床表现为起立后出现头晕或眩晕、晕厥、胸闷、头痛、心悸、面色改变、视物模糊、倦怠、晨起不适等直立不耐受或直立调节障碍症状, 14例伴有恶心或呕吐等消化道症状;在直立试验(先安静平卧 10min,然后直立 10min)或直立倾斜试验(HUT)过程中,POTS患儿最常见的异常表现为在直立或倾斜后 10min内,心率增加≥35次 /min,部分患儿心率最大值≥120次 /min,出现异常表现的时间平均为 5min左右;但仅有 10例的患儿在直立后即出现异常表现, 18例患儿需要HUT确定诊断。12例POTS患儿曾被误诊为癫痫或心肌炎,误诊率达 43%。经过生活指导治疗及药物治疗的综合治疗后,大多数患儿症状可缓解或消失。结论　POTS常见     

Abrupt aggravation of atrioventricular block and syncope in hypertrophic cardiomyopathy.

A 10 year old girl with hypertrophic cardiomyopathy (HCM) developed high grade atrioventricular (A-V) block unexpectedly, which instantly led to syncope; she required a permanent pacemaker. High grade A-V block, a rare complication of HCM, relates closely to syncope or sudden death in this disease and if progressive the use of cardiac pacing should be considered without delay.     

Abrupt aggravation of atrioventricular block and syncope in hypertrophic cardiomyopathy

A 10 year old girl with hypertrophic cardiomyopathy (HCM) developed high grade atrioventricular (A-V) block unexpectedly, which instantly led to syncope; she required a permanent pacemaker. High grade A-V block, a rare complication of HCM, relates closely to syncope or sudden death in this disease and if progressive the use of cardiac pacing should be considered without delay.     

Neurally Mediated Syncope in Children: Results of Tilt Testing, Treatment, and Long-Term Follow-Up

Despite a great deal of literature concerning children with neurally mediated syncope (NMS) there is no clear standard of diagnosis or consistent approach to therapy. This report reviews our experience with tilt testing all patients who presented with syncope during a 3-year period. All patients referred for evaluation of syncope who underwent tilt testing were retrospectively reviewed. Follow-up was obtained at return visits or by telephone interview. Sixty patients were identified. The average age was 13.5 ± 3.0 years. Twenty-six patients (43%) had positive tilt tests. Follow-up was available for 56 patients. Twelve of these patients had causes other than NMS found for their symptoms. The remaining 44 patients all had histories consistent with NMS. Nineteen of these patients had positive tilt tests. All 44 patients reported either a decrease or no recurrence of syncope, and 41 patients reported a decrease or no recurrence of presyncopal symptoms. Recurrence of syncope or symptoms was not related to the results of the tilt test. Twenty-five of the 44 patients used conservative measures (extra fluids or supplemental salt) and only 3 patients were taking medications. The use of conservative measures or medications was not related to the tilt test results. Tilt testing has a high false-negative and false-positive rate and should not be used as the standard for identifying patients with NMS. The long-term follow-up of patients with NMS demonstrates that regardless of the results of the tilt test, almost all have improved or resolved symptoms with simple interventions.     

Coronary Artery Emergencies in Children

Coronary artery issues are rare in children, leading to challenges for the pediatric emergency medicine practitioner. The differential diagnosis can be grouped into congenital or acquired coronary abnormalities. Congenital coronary anomalies leading to myocardial ischemia or aborted sudden cardiac death are most often due to an anomalous origin of a coronary artery. Acquired coronary artery disease is most commonly related to the sequelae of Kawasaki disease. Lastly, rare, specific patient populations are at higher risk for early coronary disease. This review discusses the most common pediatric causes of coronary artery events, providing an overview of the evaluation and management in an emergency department setting.     

Catecholaminergic polymorphic ventricular tachycardia in a child: a case report

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare arrythmogenic disease characterized by exercise--or stress--induced ventricular tachyarrythmias, syncope, or sudden death, usually in the pediatric age group. Familial occurrence has been noted in about 30% of cases. Inheritance may be autosomal dominant or recessive, usually with high penetrance. The causative genes have been mapped to chromosome 1. Mutations of the cardiac ryanodine receptor gene (RyR2) have been identified in autosomal dominant pedigrees, while calsequestrin gene (CASQ2) mutations are seen in recessive cases. CONCLUSION: Due to its potential lethal outcome, exclusion or confirmation of catecholaminergic polymorphic ventricular tachycardia in children with physical and emotional syncope is mandatory. We report a case of catecholaminergic polymorphic ventricular tachycardia in a three-year-old child only diagnosed by genetic mapping.     

Head-up tilt for the evaluation of syncope of unknown origin in children

Fifteen patients aged 10 to 18 years with syncope of unknown origin, and 10 healthy control children aged 11 to 18 years, were evaluated by head-up tilt to 60 degrees for 60 minutes. Six patients (43%) reproduced symptoms of syncope during the examination. Four had a typical vasovagal reaction; two had marked hyperventilation. None of the children in the control group had syncope. The head-up tilt test offers a simple, noninvasive, high-yielding diagnostic tool for evaluation of syncope in children.     

小儿心源性晕厥57例治疗及随诊分析

目的总结小儿心源性晕厥病例的治疗经验。方法研究对象为1997年1月至2008年3月广东省人民医院收治的心源性晕厥患儿57例,其中男25例,女32例;年龄平均(3.0±3.9)岁(0.6~14岁)。35例法洛四联症缺氧发作晕厥的病例予β受体阻滞剂及心脏手术治疗。9例Ⅲ度房室阻滞晕厥病例予心室起搏及针对基础心脏疾病治疗。5例长QT综合征(LQTS)晕厥发作的病例分别予美西律、β受体阻滞剂及联合起搏治疗。致心律失常性右室心肌病及扩张型心肌病室性心动过速晕厥的病例予电击复律、索他洛尔、胺碘酮等治疗。室颤及电机械分离病例予除颤等复苏急救及治疗原发心脏病。患儿出院后均予随诊。结果50例住院期间晕厥得到控制,住院期间死亡3例。本组随访时间1个月至10年,5例出院后失访。随访中45例无晕厥发作,1例晕厥发作减少,2例仍反复发作,1例死亡。结论心源性晕厥除治疗引发晕厥的直接因素外,还应积极治疗原发心脏疾病。单纯药物治疗只对部分病例有效,药物联合起搏或联合植入型心脏复律除颤器有望在恶性心律失常晕厥病例治疗中发挥重要作用。