Rhabdomyoma in the Fetus: Illustration of Tumor Growth During the Second Half of Gestation

Rhabdomyomata are the most common cardiac tumors in childhood and are associated with tuberous sclerosis. These tumors tend to regress in the first years of life. Little is known about their intrauterine growth pattern. We describe three fetuses with cardiac rhabdomyomata and illustrate the tumor growth by serial echocardiographic views during the second half of gestation. Tumor growth is proportional to cardiac growth and tends to be somewhat slower toward the end of pregnancy.     

Tuberous sclerosis complex and Wolff-Parkinson-White syndrome

This report highlights the association between tuberous sclerosis and Wolff-Parkinson-White syndrome. Ten patients with concurrent diagnoses of Wolff-Parkinson-White syndrome and tuberous sclerosis were identified. Wolff-Parkinson-White syndrome presented early in life, nine cases being diagnosed in the first year. Eight of the 10 cases were male. In eight cases, the syndrome was associated with supraventricular tachycardias, and in nine with cardiac rhabdomyomata. One child died from cardiac failure secondary to obstruction of the left ventricular outflow tract by a rhabdomyoma. Five of nine survivors showed resolution of Wolff-Parkinson-White syndrome on follow up. The accessory pathway was localised in nine patients from surface electrocardiograms: six children had left sided pathways and three had right sided pathways.     

Cardiac rhabdomyomata in tuberous sclerosis: their course and diagnostic value.

Echocardiography was performed in 60 people with tuberous sclerosis to ascertain the prevalence and course of cardiac rhabdomyomata at different ages. Twenty five (58%) of 43 children had tumours, but only three (18%) of 17 adults. The tumours tended to remain the same size through childhood. Only three infants were included but evidence from this and some published case reports suggest that the tumours tend to regress in early infancy and again in adolescence. The prevalence of tumours in young infants with tuberous sclerosis is likely to be considerably above 50%. As other signs of tuberous sclerosis are usually absent at this age echocardiography may afford the most useful diagnostic test in early infancy.     

监测心排量在重症手足口病患儿中的临床应用

目的：部分重症手足口病患儿有明显心功能改变,心功能衰竭是其致死的主要原因,对重症患儿进行左心功能检测对其救治具有重要意义。本研究探讨监测重症手足口病患儿心排量的临床应用价值。方法：选取2011年4～9月入住儿科重症监护室的重症手足口病患儿107例,临床分期为2、3、4期分别有73、23、11例。采用超声心输出量监测仪（USCOM） 床旁监测患儿的心排量,包括心输出量、每搏输出量。其中95例患儿进行了MRI检查,并按照不同MRI结果分组。MRI异常41例（其中9例延髓受累）,未见异常54例。比较不同临床分期及不同MRI结果组间患儿心排量情况。结果：与临床2、3期患儿比较,临床4期患儿心排量明显降低（P<0.05）;心排量在正常、异常MRI组间比较差异无统计学意义,但延髓受累组患儿心排量明显低于其他部位受累组及MRI正常组（P＜0.05）。结论：心排量明显降低提示病情危重,并提示病变可能已累及脑干的心血管中枢位置,监测心排量对重症患儿的救治具有指导意义。     

What is the True Frequency of Carditis in Acute Rheumatic Fever? A Prospective Clinical and Doppler Blind Study of 56 Children with up to 60 Months of Follow-Up Evaluation

BACKGROUND: This study aimed to evaluate prospectively clinical and echocardiographic findings of patients who had rheumatic fever with and without clinical features of cardiac involvement. METHODS: For this study, 56 consecutive patients (mean age, 11.4 years) with acute rheumatic fever diagnosed according to the 1992 modified Jones criteria were evaluated at diagnosis, after 3 and 6 months, then at 2 and 5 years. All assessments were performed blindly and included physical and cardiac examination, electrocardiogram, chest X-ray, and two-dimensional color-flow Doppler echocardiography. RESULTS: Initial clinical carditis was observed for 27 patients (48.2%), all of whom had positive echocardiographic abnormalities. Echocardiographic abnormalities were observed in 11 patients who had arthritis or chorea presentation without initial clinical carditis. Persistence of the abnormalities was observed at a late follow-up evaluation in 72.7% of the cases. Sydenham's chorea was present in 8 patients with clinical carditis and in 10 without this disorder, 8 of whom had echocardiographic abnormalities. CONCLUSION: Patients who had acute rheumatic fever without clinical signs of carditis showed acute and late follow-up echocardiographic abnormalities suggestive of cardiac involvement. Clinicians should be attentive for the presence of cardiac involvement among patients with chorea.     

小儿心源性晕厥的临床特征分析

目的 探讨小儿心源性晕厥(cardiac syncope,CS)的临床特点,提高小儿 CS的诊断水平.方法 应用结构性晕厥病史问卷,详细记录并分析23例CS儿童的病史特征及标准体表心电图表现,以找出CS儿童的临床特征.结果 7例为病态窦房结综合征、4例为先天性长QT综合征、2例为Ⅲ度房室传导阻滞、2例为阵发性室上性心动过速、1例为阵发性室性心动过速、1例为心房颤动、1例为Ⅲ度房室传导阻滞安装起搏器后起搏器工作不良、3例为特发性肺动脉高压、1例为肥厚型心肌病、1例为扩张型心肌病.CS儿童发病年龄较小,平均为9.0岁;23例患儿中劳累诱发晕厥的为14/23(60.9%);各种体位均有晕厥发作者为7/23(30.4%).CS儿童晕厥发作前存在先兆症状者较少,仅为12/23(52.2%);晕厥发生时存在伴随症状的患儿较多,尤其发生大小便失禁的几率较高,共4/23(17.4%).在Cs患儿中,具有心脏病史者为4/23(17.4%);仪1例有猝死家族史(占4.3%);23例中有21例的标准体表心电图存在异常,占91.7%.结论 CS患儿具有明显的临床特征,尤其是心电图异常和劳累诱发晕厥这2项特征发生率最高.临床上识别这些临床特征,对于提高儿童晕厥的诊断效率有重要意义.     

超声心输出量监护仪在重症肺炎患儿心功能评价中的应用

目的 研究便携式超声心输出量监护仪 （ultrasonic cardiac output monitor,USCOM）在重症肺炎患儿的临床应用。方法 应用USCOM分别测量29例重症肺炎患儿 （观察组）及43例轻症肺炎患儿 （对照组）心功能相关指标,将两组结果进行比较分析差异,并比较观察组治疗前后心功能指标变化。结果 观察组较对照组相比心率、心输出量、每搏输出量、速度峰值差异有统计学意义 （P < 0.05）,心脏指数、外周血管阻力差异无统计学意义 （P > 0.05）。观察组治疗后心率、心输出量、每搏输出量、速度峰值、心脏指数、外周血管阻力较治疗前改善,差异有统计学意义 （P < 0.05）。结论 应用USCOM可快速、简便、准确、动态了解重症肺炎患儿的心功能状态及患儿整体循环状态,为诊断、治疗及评估病情提供依据,具有很强的实用性。     

以心脏杂音为首发症状的WHIM综合征1例

对2017年12月在首都医科大学附属北京儿童医院就诊的1例以心脏杂音为首发症状的WHIM综合征患儿临床资料进行回顾性分析。患儿,女,5岁,临床表现为心脏杂音,粒细胞减少及反复感染。基因检测结果提示 CXCR4基因杂合突变(c.1000C>T)。心脏超声及心脏CT提示心脏畸形。WHIM综合征非常罕见,以心脏杂音...     

Perioperative Care of the Adult with Congenital Heart Disease in a Free-Standing Tertiary Pediatric Facility

Adult patients with congenital heart disease are presenting more frequently for cardiac surgery. Frequently, pediatric congenital heart surgeons perform these procedures at pediatric hospitals. Between July 1995 and June 2000, a retrospective review of adult patients (> or = 18 years old) who had undergone cardiothoracic operations was performed. A total of 112 operations were performed and divided into two groups--81 cardiac operations in 79 patients and 31 noncardiac operations in 23 patients. One patient had a cardiac and noncardiac operation performed. The overall early operative mortality was 6% (6/101). There were 3 late deaths. New-onset cardiac arrhythmias requiring treatment were diagnosed after 5/81 (6%) cardiac operations. Six of 79 (7%) patients were diagnosed with postoperative clinical depression. An acceptable mortality can be achieved when adult patients undergo cardiothoracic operations at a pediatric facility. New-onset arrhythmias necessitating treatment are relatively common, and postoperative clinical depression should be anticipated.     

Progressive cardiac failure following orthotopic liver transplantation for type IV glycogenosis

Orthotopic liver transplantation (OLT) has been proposed to treat patients with type IV glycogenosis because of early progressive cirrhosis. Reports have shown absence of disease progression in other organs after OLT and even regression of cardiac amylopectin infiltration in one case. We describe a 15-month-old child in whom a liver transplant was performed for type IV glycogenosis. There were no clinical signs of extrahepatic disease before OLT. Nine months later, the patient developed progressive cardiac insufficiency and died from cardiac failure. Because of massive amylopectin deposits, decreased myofibrils in cardiac cells, and exclusion of other causes of cardiac failure, death was attributed to amylopectinosis. Our observation contrasts with the Pittsburgh experience and suggests that cardiac amylopectionosis may progress after OLT.     

Cardiac tamponade after varicella infection

Infection with varicella zoster virus is common in childhood and generally associated with few complications. Myopericarditis following varicella infection is rare but may result in severe rhythm disturbances and congestive cardiac failure. The case is presented of a 4-month-old infant presenting with a large pericardial effusion and cardiac tamponade 2 weeks after the onset of a varicella exanthem. Although Streptococcus was noted in the pericardial fluid, it could not be grown on bacterial culture and the subsequent clinical course was in keeping with a viral myopericarditis. Conclusion Varicella infection in children may be complicated by myopericardial disease ranging from subclinical ECG changes to fulminant cardiac failure and/or cardiac tamponade. The clinical spectrum of this unusual complication is reviewed and the importance of early recognition emphasised. Received: 1 September 1997 / Accepted in revised form: 17 November 1997     

Using multidetector-row CT in neonates with complex congenital heart disease to replace diagnostic cardiac catheterization for anatomical investigation: initial experiences in technical and clinical feasibility

Background Echocardiography is the first-line modality for the investigation of neonatal congenital heart disease. Diagnostic cardiac catheterization, which has a small but recognized risk, is usually performed if echocardiography fails to provide a confident evaluation of the lesions. Objective To verify the technical and clinical feasibilities of replacing diagnostic cardiac catheterization with multidetector-row CT (MDCT) in neonatal complex congenital heart disease. Materials and methods Over a 1-year period we prospectively enrolled all neonates with complex congenital heart disease referred for diagnostic cardiac catheterization after initial assessment by echocardiography. MDCT was performed using a 40-detector-row CT scanner with dual syringe injection. A multidisciplinary congenital heart disease team evaluated the MDCT images and decided if further diagnostic cardiac catheterization was necessary. The accuracy of MDCT in detecting separate cardiovascular anomalies and bolus geometry of contrast enhancement were calculated. Results A total of 14 neonates were included in the study. No further diagnostic cardiac catheterization was needed in any neonate. The accuracy of MDCT in diagnosing separate cardiovascular anomalies was 98% (53/54) with only one atrial septal defect missed in a patient with coarctation syndrome. The average cardiovascular enhancement in evaluated chambers was 471 HU. No obvious beam-hardening artefact was observed. Conclusion The technical and clinical feasibility of MDCT in complex congenital heart disease in neonates is confirmed. After initial assessment with echocardiography, MDCT could probably replace diagnostic cardiac catheterization for further anatomical clarification in neonates.     

Duchenne Muscular Dystrophy: Prevalence and patterns of cardiac involvement

In about 10% cases of Duchenne muscular dystrophy (DMD), death is due to cardiac dysfunction. The recognition of cardiomyopathy in DMD is thus important.Objective: To assess cardiac involvement in DMD patients by clinical, radiographic, electrocardiographic (ECG) and echocardiographic monitoring and correlate clinical parameters, CPK levels, presence of gene deletion and steroid therapy with cardiac involvement.Methods: Thirty patients beyond 6 years age, with DMD in advanced stage disease/non-ambulatory were recalled. A detailed clinical evaluation, CPK levels, gene deletion studies were carried out. Cardiac investigations included Chest X-ray, 12 lead ECG and echocardiography.Results: Nineteen patients were non-ambulatory at the time of enrollment. Symptoms or signs suggestive of cardiac dysfunction were seen in only 10%. Gene deletion was identified in 70.3%. Around one-third patients had cardiomegaly. ECG abnormalities were present in 93.3% patients and commonest abnormality was R> 4 mm in V1. Ejection fraction (EF) < 55% was observed in 64.2% and EF < 50% in 17.8%.Conclusion: Cardiomyopathy of DMD is characterized by lack of symptoms and few physical signs. Presence of subtle changes like sinus tachycardia may suggest early cardiac involvement. Thus echocardiography is required for evaluation of cardiac dysfunction. Presence of gene deletion was associated with higher CT ratio. Older children have been found to have higher heart rates. No other significant correlation with clinical parameters, CPK levels, genotype and steroid therapy was observed. Early detection possibly leads to appropriate treatment thus reducing the morbidity.     

Glucagon: Use in cardiac resuscitation of a propranolol-treated infant

Summary An 8-month-old child had a cardiorespiratory arrest one hour after receiving her usual maintenance dose of propranolol hydrochloride. Intracardiac administration of epinephrine, isoproterenol, and sodium bicarbonate was not followed by resumption of cardiac activity, but administration of glucagon was followed by immediate and sustained cardiac activity. Both experimental and clinical observations suggest that glucagon may be particularly useful for treating acute cardiac depression in the propranolol-treated patient.Supported in part by the Dwan Family Fund     

动态监测新生儿早期休克患儿左心排量变化及临床意义

目的探讨动态监测新生儿休克患儿的左心排量变化及临床意义。方法采用USCOM技术无创监测30例休克新生儿和30例正常新生儿的左心输出量及每搏输出量,并监测休克患儿扩容及多巴胺[2～4μg/(kg·min)]治疗后的心排量变化。结果休克患儿左心输出量及每搏输出量[分别为(0.36±0.24)L/min,(3.5±1.5)cm3]与正常对照组[(0.49±0.15)L/min,(2.7±1.6)cm3]比较显著降低(P均<0.05);休克患儿经扩容及多巴胺治疗2～6h后,左心输出量及每搏输出量[(0.52±0.28)L/min,(3.8±1.8)cm3]较扩容前明显改善(P均<0.05),且治疗后尿量明显增加(P<0.05)。结论动态监测新生儿休克患儿心排量,可指导临床液体复苏。     

Correlation of 8-isoprostane, interleukin-6 and cardiac functions with clinical score in childhood obstructive sleep apnoea

Abstract Objective: Adeno-tonsillar hypertrophy is the commonest cause of childhood obstructive sleep apnoea (OSA). Our aim of the study is to correlate the severity of OSA with levels of 8-isoprostane and interleukin-6 (IL-6) and with cardiac diastolic dysfunctions. Methods: Forty children with adenoidal hypertrophy and 20 control children were recruited. The OSA clinical score was evaluated and IL-6 and 8-isoprostane were measured in exhaled breath condensate. The cardiac functions were evaluated by conventional and tissue Doppler echocardiography (TDE). Results: Higher concentrations of isoprostane-8 and IL-6 were found in group with clinical score >40 (58.595 +/- 2.86 pg/mL and 38 +/- 1.77 pg/mL, respectively) than in control group (34.9 +/- 1.5 pg/mL and 7.02 +/- 0.3 pg/mL, respectively) {p < 0.0001*}. There was positive correlation between level of isoprostane-8 and IL-6 and value of clinical score {p < 0.0001*} and also with the degree of the cardiac dysfunction in those children. Conclusion: The severity of OSA as indicated by clinical score was positively correlated with degree of elevation of 8-Isoprostane and IL-6 in breath condensate of children with OSA and also with degree of cardiac dysfunction. Echocardiography and tissue Doppler modality are advised to examine these children.     

DIAGNOSIS OF SEVERE CONGENITAL HEART DISEASE IN INFANTS

In a series of 103 consecutive cases of severe cardiac disease in infants the diagnostic value of clinical examination, electrocardiogram and conventional roentgen examination was found to be very limited. Emergency use of cardiac catheterization and angiocardiography is necessary to achieve a correct diagnosis in these patients.     

双胎输血综合征早产儿合并心脏发育异常的临床分析

目的 探讨双胎输血综合征（TTTS）早产儿合并症发生情况,分析TTTS受血者合并心脏发育异常的临床情况及预后。方法 对2009年6月至2012年12月收治的17对确诊TTTS的早产儿进行回顾性分析。结果 TTTS早产儿供血者体重（1.4±0.6 kg）较受血者体重（1.9±0.6 kg）明显减轻（P结论 TTTS受血者心脏瓣膜病变和心肌病变合并症发生率高,受血者应该尽早完善胎儿超声心动图,评估心功能,早期治疗以改善预后。     

婴儿先天性心脏病体外循环术后的治疗

目的 探讨婴儿先天性心脏病体外循环术后的治疗及转归.方法 对我院2005年1月至2010年11月经体外循环治疗的217例婴儿的临床资料进行回顾性分析.结果 全组术后死亡11例,住院病死率为5.1%(11/217).其中因重症低心排出量综合征死亡4例,肺动脉高压危象死亡2例,低心排出量综合征合并急性肾功能不全死亡2例,重症感染死亡1例,严重低氧血症死亡1例,慢性心包填塞导致心跳骤停死亡1例.术后住院时间7～52 d,平均14 d.结论 随着外科治疗技术的提高,婴儿体外循环下心脏手术治疗可以取得良好的治疗效果.体外循环术后采取积极有效的治疗是降低围手术期病死率的关键.

Abstract：

Objective To study the treatment and outcome in infants with congenital heart disease after cardiopulmonary bypass. Methods The clinical data of 217 infants with congenital heart disease after cardiopulmonary bypass in Shengjing Hospital from Jan 2005 to Nov 2010 were retrospectively reviewed. Results Eleven infants died and the mortality was 5. 1% ( 11/217 ). Four infants died of severe low cardiac output syndrome,2 died of pulmonary hypertension crisis,2 died of low cardiac output syndrome complicated with acute renal insufficiency, 1 died of severe infection, 1 died of severe hypoxemia,and 1 died of chronic pericardial tamponade. The postoperation length of hospital stay was 7 ～ 52 d, 14 d in average. Conclusion With the improvement of surgical techniques,cardiac surgery with cardiopulmonary bypass on infants can result in good clinical effect. Active and effective treatment can reduce the perioperative mortality after cardiopulmonary bypass.     

Neonates with congenital heart disease: an overview

The chest x-ray can be a valuable tool as part of the initial evaluation of a neonate with clinical signs of congenital heart disease. The radiographic appearance of pulmonary vascularity, as well as cardiac size and configuration, can provide information about the pathophysiologic effect of a cardiac lesion. Along with clinical evaluation of the infant, information gathered from the x-ray may be helpful in indicating which cardiac defect is involved. However, final identification of a defect would require further evaluation by echocardiogram or cardiac catheterization.