Pediatric Cardiology

Attention-deficit/hyperactivity disorder (ADHD) is the most common neurobehavioral disorder of childhood, and congenital heart disease (CHD) is the most common form of birth defect. Children with CHD are at increased risk for neurodevelopmental disorders such as ADHD. Stimulant medications, specifically methylphenidates and amphetamines, are frequently prescribed and effective in reducing the symptoms of ADHD. Despite their efficacy and long history of use, the safety of these medications has recently come into question due to isolated reports describing sudden unexplained death of children undergoing treatment. This review summarizes the current literature on the cardiovascular risks associated with the use of pharmacologic therapy for ADHD, with an emphasis on patients who had CHD.

The expectations of pediatric cardiac surgeons grow as the specialty evolves and yesterday’s challenges become tomorrow’s routine. The pioneering era of fast-paced major technical advances is behind us. Integration of surgery,
cardiology, anesthesia, intensive care, and nursing is now the basis of incremental improvements in perioperative performance and long-term outcomes.

Surgeons can be natural leaders of this process because their skills, roles, and experience are crucial in the preoperative, intra-operative, and postoperative care of the patient and their family. However, the personality traits that draw physicians to the specialty of surgery and contribute to the drive to become a successful technical surgeon may be at odds with the
collaborative aspects of this microsystem, both inside and outside the operating room. The potential for disruptive behavior on the part of the surgeon to impede the functioning of a large multidisciplinary team providing care of the upmost complexity raises fundamental questions about how to design tools and checks to create reliable pediatric cardiac surgical teams.

In, P.R. Barach, J.P. Jacobs, S.E. Lipshultz, P.C. Laussen (eds.), 293
Pediatric and Congenital Cardiac Care: Volume 2: Quality Improvement and Patient Safety,
DOI 10.1007/978-1-4471-6566-8_23, © Springer-Verlag London 2015

Minimally invasive strategies can be expanded by combining standard surgical and interventional techniques. We performed a longitudinal prospective study of all pediatric patients who have undergone hybrid cardiac surgery at the University of Chicago Children's Hospital. Hybrid cardiac surgery was defined as combined catheter-based and surgical interventions in either one setting or in a planned sequential fashion within 24 hours. Between June 2000 and June 2003, 25 patients were treated with hybrid approaches. Seventeen patients with muscular ventricular septal defects (mVSDs) (mean age, 4 months; range, 2 weeks-4 years) underwent either sequential Amplatzer device closure in the catheterization laboratory followed by surgical completion (group 1A, n = 9) or one-stage intraoperative offpump device closure (group IB, n = 8) with subsequent repair of any concomitant heart lesions. Eight patients with branch pulmonary artery (PA) stenoses (group 2) underwent intraoperative PA stenting or stent balloon dilatation along with concomitant surgical procedures. All patients survived hospitalization. Complications from the hybrid approach were mostly confined to groups 1A and 2. At a mean follow-up of 18 months, 2 group 1A patients died suddenly several months after discharge. All other patients are doing well. Hybrid pediatric cardiac surgery performed in tandem by surgeons and cardiologists is safe and effective in reducing or eliminating cardiopulmonary bypass. Patients with mVSDs who are small, have poor vascular access, or have concomitant cardiac lesions are currently treated in one setting with the perventricular approach.

This article examines the delivery of a theatre initiative with child audiences in a hospital context. It reports on a mixed method evaluation of a bedside site-specific performance at high-risk wards based in a Children’s Hospital, NHS Trust in England. It acknowledges the circumstances of the children and the conditions of the location, and examines the potential of supporting children during their stay in hospital through entertainment and relaxation. The article discusses the experiences of those who participated in the performance including children, parents and guardians. It discusses the experience of taking theatre in healthcare and the strengths and challenges of using hospital space as a ‘stage’. It concludes that a synergy between the artist and the child is important to this type of intervention, seeking to provide children both with entertainment and relaxation as an important strategy for their wellbeing in hospital.

Key words: theatre, children, wellbeing, relaxation, hospital
http://www.intellectbooks.co.uk/journals/view-Article,id=19252/

The healthcare delivery system is overly complex, impersonal and inefficient. Stakeholders are searching for effective remedies to ensure and enable that high quality care is readily available to all no matter their socioeconomic standing and their location. High-performing healthcare organizations differentiate themselves by focusing relentlessly and continuously on process- improvement initiatives to advance patient care. Continuous quality improvement offers a powerful way of thinking about how to transform clinical operations and healthcare teams to this end. Quality improvement methods are ideally suited for applications in complex cardiac care.

In particular, we describe five quality improvement tools—checklists, process maps, Ishikawa diagrams, run charts, and control charts—most relevant to improving the process and outcomes of pediatric cardiac care.

The tools help visualize, analyze, and track process and outcome data for both individual and groups of patients. These tools should be taught to healthcare providers and managers and should routinely be deployed by clinicians and healthcare systems to evaluate and improve care.

The objective of this study was to evaluate combined pulse oximetry and clinical examination as a screening method for congenital heart disease (CHD) in asymptomatic newborns. Asymptomatic newborns were screened for CHD using pulse oximetry and clinical examination before their discharge from the nursery. Oxygen saturation ‡94% was considered normal. Echocardiography was done for newborns with abnormal readings and for those with significant murmurs. Data concerning undetected cases were collected from the pediatric referral hospital. A total of 5211 cases were screened. Echocardiographic evaluations were done based on low pulse oximetry in five cases and on murmur detection in ten others. The sensitivity of the combined method of screening was 77%, whereas it was 31% for oximetry alone and 46% for clinical examination alone. Specificity was 100% for all methods. The positive predictive value of the combined tool was 66.7%. We conclude that combining pulse oximetry and clinical examination can enhance the clinicianÕs ability to detect life-threatening CHD in a timely manner. This screening method should become a part of the discharge plan for every newborn.

As the prevalence of childhood obesity increases, exercise testing of obese children is likely to increase as well. This article discusses the implications of pediatric obesity for exercise testing and provides some recommendations for conducting tests and evaluating results. Studies comparing obese and nonobese children during exercise testing indicate that obese children are capable of meeting the challenges of exercise testing to nearly the same extent as their nonobese peers. Their physiologic responses, at least for the levels of obesity reported in the literature, are not sufficiently different from their nonobese counterparts to necessitate major changes in test protocols. Laboratory staff should pay special attention to fostering confidence in the obese child during the pretest routine.

This study aimed to provide a general description of the cardiovascular and hemodynamic effects of dexmedetomidine and an evidence-based review of the literature regarding its use in infants and children with congenital heart disease (CHD). A computerized bibliographic search of the literature on dexmedetomidine use in infants and children with CHD was performed. The cardiovascular effects of dexmedetomidine have been well studied in animal and adult human models. Adverse cardiovascular effects include occasional episodes of bradycardia, with rare reports of sinus pause or cardiac arrest. Both hypotension and hypertension also have been reported. The latter is related to peripheral a 2B agonism leading to vasoconstriction. No adverse effects on the pulmonary vasculature have been noted even in patients with preexisting pulmonary hypertension. Although there are no direct effects on myocardial function, decreased cardiac output may result from changes in heart rate or increases in afterload. Although not currently Food and Drug Administration (FDA)-approved for the pediatric population, findings have shown dexmedetomidine to be effective in various clinical scenarios of patients with CHD including

Bacteremia resulting from dental extraction is regarded as an important cause of bacterial endocarditis, and it is therefore recommended that patients undergoing tooth extraction be given prophylactic antibiotics. As dental procedures other than extractions may also cause bacteremias, we studied a variety of dental procedures routinely used in pediatric dentistry. Blood samples for cultures were obtained 30 s after each of 13 dental operative procedures in 735 anesthetized children aged 2–16 years. Four procedures used for conservative dentistry caused bacteremias significantly more often than the baseline value of 9.4%: polishing teeth 24.5%, intraligamental injection 96.6%, rubber dam placement 29.4%, and matrix band with wedge placement 32.1%. In comparison, toothbrushing alone caused a bacteremia on 38.5% of occasions. The organisms isolated were typical of odontogenic bacteremias in that 50% of the isolates were identified as varieties of viridans streptococci. These data show that a wider variety of dental procedures than was previously documented cause bacteremia.

Purpose of review Pediatric brady-dysrhythmias and conduction disorders are uncommon, but timely recognition and evaluation are critical. This review will highlight the key diagnostic and management steps for first, second, and third-degree atrioventricular heart block in pediatric patients. Recent findings There is a breadth of acquired and often reversible causes of atrioventricular block in childhood. Recent advances in diagnostics and pacing therapies have led to improved outcomes. Summary A thorough evaluation is required to determine when atrioventricular block requires treatment. In symptomatic or unstable patients, the management should focus on resuscitative measures, diagnostic testing, potential reversible causes, monitoring for progression, cardiac consultation and evaluating the need for definitive pacemaker placement.

Patients with anomalies of the heart frequently suffer from arrhythmias that either are associated with a congenital heart defect or result from the course of the disease. For most of the bradyarrhythmias, appropriate timing of the initiation of treatment is more challenging than its eventual execution. In the case of tachycardias, technical aspects of treatment require more attention because the often imperative impact such tachycardias have on quality of life, morbidity, and mortality determine intervention timing. Increasingly, interventional electrophysiology is turned to as a potentially definitive and substrate-related treatment because of antiarrhythmic drug therapy's failure to prevent arrhythmia recurrences and the potential detrimental side effects from drug therapy seen in this particular patient population. Using the experience gained during the past 10 years in the treatment of patients with arrhythmias but without associated structural heart disease, several groups reported their results and difficulties with the application of such therapy to patients with congenital heart defects. In this report, we summarize our hospital's experience with transcatheter radiofrequency current application for treatment of various types of tachyarrhythmias in 139 children and adults with congenital heart defects, emphasizing the current limitations of such therapy and addressing the potential benefits expected from future technology. Patient ages ranged from 5 months to 76 years (mean 25.3 ± 17.7 years), including 56 children and adolescents less than 16 years of age. At least one attempt at surgical palliation or correction was made in 93 patients; the remaining 46 patients had no surgical intervention attempts. A total of 225 different tachycardias were found, 93 of which were based on a congenital arrhythmogenic substrate (e.g., an accessory pathway). Acquired substrates (e.g., scars or myocardial fibrosis) gave rise to the remaining 132 tachycardias. Radiofrequency current ablation (183 sessions) successfully treated 121 of 139 patients. Within a follow-up period of 21 months a recurrence of the intrinsically treated tachycardia was seen in 24 patients (10.7%); 13 of the 24 underwent a successful repeat session. There were no significant pro-cedure-related complications. Young and adult patients with congenital heart disease can be safely and successfully treated for tachycardias with the use of radiofrequency current ablation. Because such treatment meets the specific needs of this patient group, early consideration for this therapy is recommended.

In this study, we correlated cardiac malformations in the various types of conjoined twins with the proposed site of embryologic union and evaluated the possibility of survival after surgical separation. We conclude that surgical separation is only very rarely successful in twins with united hearts, whereas survival in twins with separate hearts is more dependent on the malformations of other viscera. This report deals primarily with conjoined twins in whom anomalies of the heart are the major factor in deciding whether surgical separation is feasible.

The aim of this study was to assess the utility of arm and leg oxygen saturation as a candidate screening test for the early detection of ductal-dependent left heart obstructive disease. We measured arm and leg oxygen saturation in 2876 newborns admitted to well baby nurseries and 32 newborns with congenital heart disease. Fifty-seven newborns in the well baby nurseries (0.02%) had an abnormal test (leg saturation less than 92% in room air or 7% lower saturation in the leg than in the arm). Four of the 57 had critical congenital heart disease, including 1 with coarctation of the aorta. Of the 32 newborns with congenital heart disease, 11/13 (85%) with left heart obstructive disease had abnormal oxygen saturation tests, as did 15/19 (79%) with other forms of congenital heart disease. Pulse oximetry deserves further study as a screening test for critical congenital heart disease.

In pediatric interventional cardiology, cardiologists need to stay closer to the patient than during adult catheterization, and the use of biplane systems increases the scatter radiation. Occupational radiation risk is rather high, and estimation of lens doses becomes necessary. Deriving factors for assessing these doses from the patient doses displayed in catheterization laboratories can help in preserving staff radiation safety. A biplane X-ray system and polymethylmethacrylate plates of 4 to 20 cm to simulate pediatric patients have been used. Patient entrance dose rates, dose-area product, and doses to the eyes of the cardiologists for the typical operation modes have been measured. Correlations between patient and staff doses have been obtained. Scatter dose rates increase by a factor of 92 from low fluoroscopy to cine acquisition when phantom thickness increases from 4 to 20 cm. Scatter doses increase linearly with dose-area product for all the thicknesses. Administration of 1 GyÁcm 2 to the patient involves 7 lSv to the eyes of the cardiologist (without extra protection). In conclusion, the experimental correlation factors found between phantom and scatter doses allow a fairly good estimation of staff doses from the dosimetric patient data.

BACKGROUND:
As survival and outcomes continue to improve in neonates born prematurely, there is an increasing need to promptly identify and treat pulmonary hypertension (PHT) in this population. Several echocardiographic indices have been used to evaluate for PHT. There is no clear consensus on how to utilize these parameters specifically for the evaluation of PHT in infants with chronic lung disease of prematurity.
OBJECTIVES:
Τhe objectives of the study were (1) to identify the different echocardiographic techniques for assessment of PHT in infants with chronic lung disease of prematurity in an evidence-based manner and (2) to establish an echocardiographic screening protocol based on available literature using different echocardiographic techniques.
METHODS:
We conducted a systematic review of the literature regarding use of echocardiographic techniques for evaluation of PHT in infants with bronchopulmonary dysplasia. On the basis of the available evidence, we came up with a screening algorithm using various echocardiographic techniques.
RESULTS:
We identified nine techniques that had been employed for detection of PHT noninvasively using echocardiography in 23 studies. Using these echocardiographic parameters, we came up with a flow chart to diagnose PHT in infants born prematurely, based on presence or absence of tricuspid regurgitation, presence or absence of patent ductus arteriosus, and direction of flow.
CONCLUSIONS:
We have proposed a new screening strategy for assessment of PHT using echocardiography in infants with chronic lung disease of prematurity. Further studies will be necessary to confirm its validity.

Congenital heart defects (CHDs) occur in 1% of live-born infants and frequently are associated with extracardiac malformations. This study aimed to assess the feasibility and accuracy of three-dimensional ultrasonography (3DUS) in fetuses with CHD and to investigate whether 3DUS can add information about the heart and general fetal morphology that shows other congenital malformations or suggests syndromes. For 30 fetuses affected by CHD, 3DUS was performed using a Sonos 7500 ultrasound machine with a cardiac 3D transducer. In 44% of the exams, 3DUS was completely diagnostic for the CHD, providing additional information in 28% of the exams. Furthermore, 3DUS showed 82% of associated malformations, providing the complete diagnosis in 57% of the cases and helping with recognition of syndromes in others. The diagnostic accuracy of 3DUS was superior, with a higher number of acquisitions per exam. Performance was better in fetuses younger than 24 weeks for general morphologic details and in fetuses older than 24 weeks for the heart morphology.

Sudden cardiac death (SCD) is a rare but devastating event among young people. The precise scale of the event remains undefined. This uncommon but catastrophic event usually is proved to be the consequence of varied unsuspected congenital or acquired cardiovascular diseases. Hypertrophic cardiomyopathy and coronary artery anomalies are the two most frequent causes of SCD. Most commonly, SCD resulting from these diseases occurs during or immediately after exercise. This report highlights the prevalence and spectrum of SCD-predisposing diseases and how they are affected by athletic participation. In addition, disease-specific guidelines for sports participation are addressed.

Congenitally corrected transposition of the great arteries (ccTGA) is a rare disorder with reduced survival that is in¯uenced by the presence of associated anomalies, tricuspid regurgitation (TR), and right ventricular (RV) function. The double switch procedure has been proposed as an aggressive surgical approach in selected patients. We sought to review our experience with conventional repair to determine if a change in surgical strategy was warranted. Clinical records of 121 patients with ccTGA and two adequate-sized ventricles were retrospectively reviewed. Median length of follow-up was 9.3 years; 5-, 10-, and 20-year survival rates were 92%, 91%, and 75%, respectively. Surgery was performed in 86 patients, including conventional biventricular repair in 47 patients. Risk factors for mortality by univariate analysis included age at biventricular repair (p = 0.04), complete atrioventricular (AV) canal defect (p = 0.02), dextrocardia (p = 0.05), moderate or severe TR (p = 0.05), and poor RV function (p = 0.001). By multivariate analysis, complete AV canal defect (p = 0.006) and poor RV function (p = 0.002) remained signi®cant as risk factors for mortality. Risk factors for the development of signi®cant TR included conventional biventricular repair (p = 0.03) and complete AV block (p = 0.04). Risk factors for progressive RV dysfunction included conventional biventricular repair (p = 0.02), complete AV block (p = 0.001), and moderate or severe TR (p < 0.001). This is the largest nonselected cohort of patients with ccTGA followed at a single center. Our results con-®rm that signi®cant TR and poor RV function are risk factors for poor outcome and provide convincing evidence that patients undergoing conventional biventricular repair are at higher risk for deterioration of tricuspid valve and right ventricular function compared to palliated or unoperated patients. We support a move toward an alternative surgical approach (double switch procedure) in carefully selected patients.

Sports camps for children with cardiac anomalies have existed for many years. However, no formal evaluation of the benefits of attending such camps has been undertaken heretofore. We assessed potential changes in the self-perceived health of children with congenital heart disease who attended a special sports camp. Thirty-one children with cardiac anomalies attended a 3-day multisports camp. Sixteen children, all of whom were 10 years or older, literate, and Dutch-or French-speaking, completed the Child Health Questionnaire (CHQ-CF87) before and after attending the camp. The scores of the children were compared with those of healthy peers by calculating mean standardized differences. After attendance at the sports camp, the children achieved significant improvements in the self-perception of their physical functioning, role functioning due to emotional problems, role functioning due to behavioral problems, mental health, and general behavior. The childrenÕs self-esteem and general behavior after the camp were significantly better than that of their healthy counterparts. We conclude that children with congenital heart disease who participate in activities at special sports camps may reap benefits in terms of their subjective health status. Although further research is needed, we recommend the participation in sport activities by children with heart defects, and more specifically their participation in sports camps.

The Bruce treadmill protocol was used to evaluate the exercise endurance in a pediatric urban population. The sample population consisted of 525 healthy children 4 to 18 years of age (303 males and 222 females). Endurance time increased with age in males and increased up to 10 to 12 years of age in females, thereafter leveling o. Results were compared with those of a widely used standard reference of normal for treadmill exercise testing in children. Mean endurance times were signi®cantly shorter compared to those of the reference study (p < 0.03). When the subjects were grouped in percentiles based on the reference study, 61% of males and 81% of females performed below the 25th percentile. There was a strong negative correlation between body mass index and endurance time (p < 0.0001), suggesting that obesity is a major contributor to decreased physical ®tness. Compared to subjects in the reference study, children from a large U.S. metropolitan area have markedly diminished exercise endurance times, suggesting poor physical ®tness. Inactivity with consequent lack of ®tness, if unchecked, are likely to lead to cardiovascular disorders in adulthood. Eorts should be made to encourage physical ®tness in children. Key words: Exercise Ð Bruce treadmill protocol Ð Physical ®tness Ð Obesity Cumming et al.'s [8] study, the children were not allowed to hold onto the guardrails because this can decrease the metabolic cost of work

A case of biopsy-proven myocarditis in a 7year-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%).

Recent studies have suggested that survival after the modified Norwood procedure is influenced by anatomy and is worse for patients with hypoplastic left heart syndrome (HLHS) than for patients with other forms of functional single ventricle. This study aimed to compare the outcomes of the modified Norwood procedure for these two groups at a single center. A retrospective chart review compared modified Norwood procedure outcomes between children with HLHS (n = 40) and children with other single-ventricle malformations (n = 19) from January 2002 to August 2012. The HLHS patients had a lower mean age (10.0 ± 13.2 days) at the time of operation than the non-HLHS patients (16.5 ± 18.5 days). The difference was not significant. The number of patients with a Sano modification was significantly higher in the HLHS group (HLHS 85 % vs non-HLHS 32 %; p \ 0.001). The followup data did not differ between the groups (HLHS 3.6 ± 3.2 years vs non-HLHS 4.8 ± 3.3 years, nonsignificant difference). The overall operative survival was 85 %: 83 % for the patients with HLHS and 89 % for the patients with non-HLHS (nonsignificant difference). The Kaplan-Meier transplant-free survival estimate at 10 years was 73 % and did not differ between the HLHS patients (67 %) and the non-HLHS patients (84 %, nonsignificant difference). The timing of stages 2 and 3 palliation (bidirectional Glenn and external conduit Fontan) did not differ significantly (HLHS, 7.0 ± 2.8 months and 3.0 ± 0.5 years vs non-HLHS 6.1 ± 1.7 months and 2.8 ± 0.8 years). The two groups were similar with respect to the need for post-Norwood extracorporeal membrane oxygenation support, aortic arch reinterventions, atrioventricular valve repair, pulmonary artery reconstruction, pacemaker implantation, and progression to bidirectional Glenn and Fontan completion. The modified Norwood reconstruction provides good palliation for HLHS as well as other forms of functional single-ventricle anatomy. Survival, postoperative morbidity, and progression to Glenn and Fontan are not significantly different.

Recent studies suggest good prospective accuracy for two-dimensional echocardiographic imaging of ventricular septal defects (VSD). We obtained two-dimensional images with high-frequency, high-resolution scanners from 36 patients proved by cardiac catheterization to have perimembranous VSD. In 20 patients, the VSD was an isolated lesion and in 16 it was associated with other forms of heart disease. VSDs were imaged in long-axis, apical four-chamber and subcostal echocardiographic views. The smallest VSD im-aged was 2 mm in diameter on echo; the largest, 23 mm. The imaged size of VSDs was larger at end-diastole than at end-systole by paired t test on all views (all p < 0.005). VSD size also varied between views, with no predictive relationship except between apical and subcostal four-chamber views in diastole (r = 0.71, p < 0.005). This agreed with qualitative direct observations of an ellipsoid or irregularly shaped VSD in operated patients. Echocardiographically measured VSD size normalized for either aortic root size or for patient weight could be used to separate isolated VSDs with large shunts (Qp/Qs > 2:1) from those with small shunts. Review of 250 two-dimensional echocardiographic studies from patients proved not to have a VSD revealed 28 planes of imaging with false-positive VSD. None of the false-positive VSDs was imaged consistently on all views. Additionally, a "T" artifact (broadening of septal edges around a VSD) has been found to be a reliable marker of true VSD imaging. To best quantify VSD size and to avoid false-positive diagnoses, it is necessary to use multiple views and to consider the marked changes in VSD size that occur between diastole and systole. VENTRICULAR SEPTAL DEFECT is one of the most common congenital lesions, occurring alone or in association with other cardiovascular malforma-tions. Although the clinical diagnosis of isolated VSD is not difficult, evaluating the size of a VSD and detecting a VSD when it is associated with other lesions is quite important in planning catheterization and surgery. Until recently, the firm diagnosis of VSD was confined to cardiac catheterization and angiog-raphy. M-mode echocardiography can provide indirect estimates of left ventricular volume overload, but frequently has poor sensitivity and specificity for VSD.1 Nuclear cardiology appears to allow an estimate of shunt size, but is not useful for estimating VSD associated with other congenital malforma-tions.2 Doppler aids detection of VSDs, but offers no possibilities for estimating the size of the defect.3 Recent studies suggest that two-dimensional echo-cardiography can be used to detect, locate, and evaluate the size of VSD.4 5 We performed two-dimensional echocardiography in a group of patients with proved VSD to identify and analyze factors that affect VSD imaging. From the

Background: A case of sudden unexpected death of an 11-year-old child with tonsillitis illustrates acute myocarditis and the difficulty in diagnosing it.
Aims: Consider myocarditis among acute pediatric conditions, suggesting this disease should be included in the differential diagnosis for children who are toxemic critically ill, particularly when they have respiratory symptoms.
Methods: A critical review of studies published from 1987 to 2018 was conducted. Relevant studies addressed children with deterioration of a condition initially considered benign and discussed clinical aspects suggesting various pathologies such that the presence of myocarditis was “masked.” Papers on myocarditis and sudden death in children were selected by searching electronic databases (Pubmed, Scielo, Semantic Scholar, Google Scholar, Springer Link, Science Direct, NCBI, Research Gate, and others). Articles on immunology, pathology, pneumology and radiology were also included that addressed medical reasoning and might assist in early diagnosis.
Results: Many cases of myocarditis remain undiagnosed due to nonspecific symptoms. Therefore, the true prevalence is unknown. The literature reports a high prevalence of histologically proven myocarditis on post-mortem examination in pediatric cases of sudden death based on the presence of a lymphocytic infiltrate necrosis of myocytes.
Conclusion: Early diagnosis and treatment of myocarditis might save children who would otherwise die or develop chronic dilated cardiomyopathy. Information presented in this review may assist in better recognition of this life-threatening disease.

VACTERL association is a useful acronym for a condition characterized by the sporadic, non-random
association of specific birth defects of multiple organ systems. It includes vertebral anomalies (V), anal anomalies (A),
cardiac anomalies(C), trachea-esophageal fistula (TE), R-renal anomalies or radial bone anomalies(R), L-limb defect (L).
Association is defined by presence of at least three of above malformation. In addition to these core component features,
patient may also have other congenital anomalies. We are reporting one such case which had congenital abnormalities of
cardiac system, trachea-esophageal fistula and vertebral anomalies. Purpose of this case report is to highlight the
distinctive presentation of VACTERL association, to enable earlier detection, to discuss the treatment option.
Keywords: VACTERL, anomalies, malformation

The identification of gestational diabetes (GDM) through appropriate screening and its subsequent treatment have not been demonstrated to limit neonatal malformations to date. This study aimed to detect congenital heart diseases in newborns of mothers with GDM by evaluating the existence of a correlation with maternal glycemic control. This observational prospective study investigated newborns of mothers with GDM enrolled during a period of 9 months. Four subgroups were considered according to the type of maternal glucidic alteration during pregnancy and the home treatment: impaired glucose tolerance, insulin-dependent gestational diabetes mellitus (IDDM), non-insulin-dependent gestational diabetes mellitus (NIDDM), and gestational diabetes not controlled (NC: untreated diabetes). Student's t test was used to compare the subgroups. The study enrolled 65 newborns (30 boys) born to 82 of mothers with impaired glucidic metabolism. Patent ductus arteriosus was observed in 11 patients (16.9 %), pulmonary stenosis of mild grade in 4 patients ( 6.2 %), and hypertrophy of the ventricular septum in 22 patients (33.8 %). A total of 14 patients had increased thickness in the left ventricle posterior wall, and 17 patients had an abnormal electrocardiogram. Hyperglycemia can influence the development of the fetal heart, affecting both its structure and its function. A treatment with insulin for women with GDM is supported by the study data.

Most contemporary diagnostic and treatment strategies for pediatric patients with cardiovascular disease are not supported by evidence from clinical trials but instead are based on expert opinion, single-institution observational studies, or extrapolated from adult cardiovascular medicine. In response to this concern, the National Heart, Lung, and Blood Institute established the Pediatric Heart Disease Clinical Research Network (PHN) in 2001. The purposes of this article are to describe the initiation, structure, and function of the PHN; to review the ongoing studies; and to address current and future challenges. To date, four randomized clinical trials and two observational studies have been launched. Design and conduct of complex, multicenter studies in children with congenital and acquired heart disease must address numerous challenges, including identification of an appropriate clinically relevant primary endpoint, lack of preliminary data on which to base sample size calculations, and recruitment of an adequate number of subjects. The infrastructure is now well developed and capable of implementing complex, multicenter protocols efficiently and recruiting subjects effectively. The PHN is uniquely positioned to contribute to providing evidence-based medicine for and improving the outcomes of pediatric patients with cardiovascular disease.

A random sample of 457 neonates was prospectively studied in order to identify the incidence, common types, and risk factors for arrhythmias in the neonatal intensive care unit (NICU). A 12-lead EKG was studied in all neonates (n = 457). A total of 139 Holter studies was done in every fourth baby with a normal EKG (n = 100) and in all babies with an abnormal EKG (n = 39). Of the 100 infants who were thought to be arrhythmia-free by EKG, nine infants demonstrated an arrhythmia on Holter studies. When we correlated screening results with maternal, obstetrical, and neonatal risk factors; arrhythmias were significantly associated with male gender, more mature gestational age, lower glucose levels, maternal smoking, high umbilical artery lines, and the use of the nebulized b-2 adrenergic treatment, whereas umbilical venous lines and dopamine infusion did not relate to arrhythmia. We conclude that arrhythmias are more common in the NICU than in the general neonatal population. Compared to Holter monitoring, the sensitivity of the EKG was only 89%.

Double-outlet right ventricle (DORV)/Taussig-Bing (TB) anomaly is the second most common type of DORV. This study evaluates our experience and outcomes of total correction of DORV-TB anomaly at King Abdulaziz Cardiac Center. We conducted a retrospective study for all cases of TB anomaly repaired between June 2001 and April 2009. Patients were divided into two groups: Group A included patients repaired with arterial switch operation, and group (B) included patients repaired with Rastelli procedure. Thirteen patients with TB anomaly underwent total correction. There were 5 male (38%) and 8 female (62%) patients. Mean age and weight at surgery were 6.8 ± 6 weeks and 3.6 ± 0.7 kg, respectively. Of the 13 patients, 9 (69%) were in group A, and 4 (31%) were in group B. Aortic arch abnormalities were present in 9 patients (69%); abnormal coronary artery patterns were present in 7 patients (54%); side-by-side great arteries were present in 5 patients (38%); dextrotransposition of the great arteries was present in 7 patients (54%); and levo-malposition of the great arteries was present in 1 patient (8%). At postoperative follow-up, 4 patients (31%) had developed either left-or right-ventricular outflow tract (VOT) obstruction requiring surgical and/or catheter intervention. There was no early mortality, but there was 1 late mortality caused by left-ventricle dysfunction. DORV-TB is often associated with other congenital cardiac anomalies. In general, total repair is feasible in the majority of patients with satisfactory results and improved outcome. Residual lesion and development of VOT obstruction can occur, requiring close follow-up and intervention for residual lesion.

Cardiac catheterizations are among the X-ray procedures with the highest patient radiation dose and therefore are of great concern in pediatric settings. This study aimed to evaluate factors that influence variability of X-ray exposure in children with congenital heart diseases during cardiac catheterization. The study included 107 children who underwent either diagnostic (n = 46) or interventional (n = 61) procedures. A custom-made sheet for patient and procedural characteristics was designed. Data were collected, and different correlations were applied to determine factors that influence variability of X-ray exposure. The flouroscopy time (FT) differed significantly between the diagnostic (8.9 ± 6.3 min) and intervention (12.8 ± 9.98 min) groups (P = 0.032). The mean dose–area product (DAP) differed significantly between the two groups (3.775 ± 2.5 Gy/cm2 vs. 13.239 ± 15.4 Gy/cm2; P = 0.003). The highest DAP was during left anterior oblique (LAO) cranial 30° angulation (2.8 Gy/cm2/4 s cine). The mean cumulative dose (CD) was 0.053 Gy in diagnostic cases and 0.48 Gy in intervention cases. The effective dose was 5.97 ± 7.05 mSv for theraputic procedures compared with 3.42 ± 3.64 mSv for diagnostic procedures. The FT correlated significantly with both the DAP (r = 0.718; P < 0.001) and the CD (r = 0.701; P < 0.001). Other correlations were reported. An increasing number of therapeutic catheterization procedures are being performed for children. The justification for these procedures is evident because they avoid complicated surgery. However, the complexity of these procedures results in higher radiation exposures.

Platelets play a critical role in normal physiologic processes such as hemostasis, wound healing, inflammation, and innate immunity. However, they also play a role in the pathologic process of thrombosis. Although antiplatelet therapy is most commonly used to prevent thrombotic events for adults with atherosclerotic vascular disease, children with certain types of congenital heart disease, stroke, and Kawasaki disease also are at risk for thrombosis and may benefit from antiplatelet therapy. Unfortunately, very little data on the efficacy and safety of antiplatelet therapy for pediatric patients are available. As a consequence, consistent clinical practice among pediatric practitioners is lacking. Furthermore, much of what is practiced results from extrapolation from adult studies, which may be problematic because many aspects of platelet biology differ between children and adults. This review discusses aspects of antiplatelet therapy for pediatric patients.

Background: Venous thromboembolism (VTE) causes major morbidity in adults after trauma, occurring in up to 50% of patients without prophylaxis. The incidence of VTE after trauma is lower in children. No study has measured the incidence of and risk factors for VTE in critically ill children after trauma. Methods: Nested case-control study of children, younger than 18 years, admitted to the pediatric intensive care unit at a level I trauma center. Three controls were selected for each identified VTE case. Results: Nine of 144 children admitted to the pediatric intensive care unit after trauma developed VTE (incidence 6.2%, 95% confidence interval [CI] 2.3-10.2), with a median age of 8.6 years (range, 2.3-17.9). VTE was diagnosed at a median of 9 days after admission, with 67% of VTE located at the site of previous or existing central venous line (CVL). Significant risk factors for thrombosis included parenteral nutrition (odds ratio [OR] 20, 95% CI 1.9 -227), CVL (OR 19, 95% CI 2-178), deep sedation (OR 13, 95% CI 1.6 -48), neuromuscular blockade (OR 10, 95% CI 1.4 -70), inotropic support (OR 10, 95% CI 1.7-59), and recombinant factor VIIa administration (p ϭ 0.012, OR not calculable). Logistic analysis found a 7.9-fold increase in the odds of developing VTE for each additional CVL (p ϭ 0.005), a threefold increase with each additional risk factor present (p ϭ 0.009), and a 1.3-fold increase for an increase in injury severity (p ϭ 0.03). VTE was not associated with sepsis, spinal cord injury, fracture, or elevated D-dimer level. Conclusions: VTE is not a rare event in critically ill children after trauma. Most patients developing thrombosis have multiple risk factors, including poor perfusion, immobility, and presence of a CVL.

This report describes a transient hypertrophic cardiomyopathy with right-ventricle outlet tract obstruction (RVOTO) induced by perinatal stress due to a major surgical procedure in a female newborn with congenital abnormalities. On day 10, she presented with heart failure, abnormal B-type natriuretic peptide (BNP), and an echocardiogram showing normal wall thickness. An in-hospital follow-up echocardiogram showed biventricular hypertrophy and RVOTO. At discharge, the infant was asymptomatic, with a normal echocardiogram and BNP. Transient RVOTO triggered by surgical stress and abnormal BNP have not been reported previously. Pathophysiology, the role of BNP, and clinical characteristics are discussed.

Gastrointestinal and feeding complications after the Norwood procedure in infants with hypoplastic left heart syndrome increases morbidity and mortality. These problems are the result of intraoperative challenges, shunt-dependent physiology, and the absence of bestpractice guidelines. In response, a systematic review of feeding-related complications and management strategies was performed. A literature search from 1950 to March 2010 identified 21 primary research articles and 4 reviews. Dysphagia, necrotizing enterocolitis (NEC), and poor nutritional status are significant feeding-related complications. Three studies directly compared the modified Blalock-Taussig shunt with the right ventricle-to-pulmonary artery conduit (RV-PA). Patients palliated with either shunt had impaired mesenteric blood flow. Mortality did not differ between shunt types. Three studies demonstrated improved outcomes, e.g., increased survival, decreased incidence of NEC, and decreased median time to recommended daily allowance of calories, with a postoperative feeding algorithm. Two studies showed increased survival between stage I and II surgical palliation after implementation of a home-monitoring system consisting of daily weight and systemic oxygen saturation measurements. The RV-PA shunt does not significantly alter mortality or increase mesenteric blood flow. A postoperative feeding algorithm and a home-monitoring system may improve outcomes and decrease average hospital length of stay (LOS). Additional studies are needed to determine which interventions, as part of a standardized protocol, improve survival and decrease complications.

We aimed to assess the frequency of chromosomal abnormalities among infants with congenital heart defects (CHDs) in an analysis of population-based surveillance data. We reviewed data from the Metropolitan Atlanta Congenital Defects Program, a population-based birth-defects surveillance system, to assess the frequency of chromosomal abnormalities among live-born infants and fetal deaths with CHDs delivered from January 1, 1994, to December 31, 2005. Among 4430 infants with CHDs, 547 (12.3%) had a chromosomal abnormality. CHDs most likely to be associated with a chromosomal abnormality were interrupted aortic arch (type B and not otherwise specified; 69.2%), atrioventricular septal defect (67.2%), and double-outlet right ventricle (33.3%). The most common chromosomal abnormalities observed were trisomy 21 (52.8%), trisomy 18 (12.8%), 22q11.2 deletion (12.2%), and trisomy 13 (5.7%). In conclusion, in our study, approximately 1 in 8 infants with a CHD had a chromosomal abnormality. Clinicians should have a low threshold at which to obtain testing for chromosomal abnormalities in infants with CHDs, especially those with certain types of CHDs. Use of new technologies that have become recently available (e.g., chromosomal microarray) may increase the identified contribution of chromosomal abnormalities even further.

The success of the radiofrequency catheter ablation procedure for most types of supraventricular and ventricular tachycardia, particularly in young patients, largely eliminated the role of surgical therapy of arrhythmias. However, there remains a subset of arrhythmia patients in whom the catheter approach has not been successful and types of arrhythmias with high recurrence rates following initially successful catheter ablation procedures where surgery can provide more definitive therapy. In addition, the concepts of ablation therapy can be successfully incorporated into the concomitant repair of complex congenital heart disease, resulting in singlestage therapy for structural and rhythm abnormalities. Prospectively, knowledge of the role of anatomic barriers as substrates for future reentrant arrhythmia circuits provides the opportunity to alter these circuits prophylactically at the time of initial surgical repair of congenital heart disease in an attempt to avoid the late development of tachycardia. This article describes our experience during the past decade with 71 patients undergoing arrhythmia surgery using this approach.

Hypertension after repair of coarctation of the aorta (CoA) is the outcome variable most closely associated with adverse long-term events such as stroke and myocardial infarction. This study sought to evaluate the outpatient management of casual blood pressure (BP) measurements in young children after early repair of CoA. A retrospective analysis was performed of clinical findings, echocardiographic data, casual BP recordings, and subsequent BP management of 114 children with CoA repair aged 1–13 years during 338 outpatient visits managed at two congenital cardiac centers. Children with associated significant congenital heart disease or corrective surgery after the age of 6 months were excluded from the study. Blood pressure was documented at 233 clinic visits (69%), and systolic BP (SBP) was above 95th percentile for age and sex in 45 instances (19%). This represented an elevated SBP recording for 31 children (27%), with two or more successive elevated recordings for 11 children (10%). Of 12 subjects receiving antihypertensive medication, three had inadequate BP control. Blood pressure is not documented at approximately 30% of outpatient visits of children with repaired CoA. When elevated BP is documented, in all cases no recorded action was taken. This may have significant implications for cardiovascular outcomes in this cohort of patients.

The objective of this study was to determine the prevalence of postoperative hyperglycemia in pediatric patients following surgery for congenital cardiac defects and its impact on morbidity and mortality. It was designed as a retrospective cohort study in a pediatric intensive care unit of a university-affiliated free-standing children’s hospital. A cohort of 213 patients who underwent 237 surgical procedures for repair or palliation of congenital cardiac defects comprised the study. Postoperative blood glucose measurements and all clinical and laboratory data were compiled for the first 10 days after surgery. The intensity and duration of hyperglycemia were analyzed for association with hospital morbidities and mortality. Mild and severe hyperglycemia were highly prevalent in our cohort (97% and 78%, respectively). Survivors had significantly lower peak (289.7 ± 180.77 mg/dl vs. 386 ± 147.95 mg/dl), mean (110.13 ± 36.22 mg/dl vs. 146.75 ± 57.12 mg/dl), and duration (2.59 ± 2.3 days vs. 5.35 ± 2.8 days) of hyperglycemia compared to nonsurvivors. Duration of hyperglycemia was independently associated with morbidity [odds ratio (OR): 1.95; p p = 0.03) by multivariate logistic regression. Hyperglycemia is common in children following surgical repair or palliation of congenital cardiac defects. Postoperative hyperglycemia is associated with increased morbidity and mortality in these patients.

The term ''Third World'' loosely encompasses a group of middle-and low-income countries. Considerable differences exist in health care delivery and health indices among these countries. The vast majority of children in the Third World do not have health insurance for congential heart disease (CHD). Catheter interventions for CHD are expensive because of installation costs of expensive biplane equipment, the requirement of dedicated personnel, and the need to stock a large inventory of expensive hardware. As a result, many catheter intervention procedures are beyond the reach of the average patient in the developing world. The following cost-effective strategies have evolved in selected institutions that have attempted to perform catheter interventions for CHD at an affordable cost: sharing of space, equipment, and support personnel with a busy adult cardiology program; use of single plane equipment; the development of sedation protocols to reduce the need for anesthesiologists; strategies to reduce procedure time; reuse of hardware through ethylene oxide sterilization; improvisations to use adult hardware items for CHD interventions; judicious case selection; and improvised alternatives to occlusive devices. These strategies may help reduce costs and allow a larger proportion of patients in developing countries with CHD to undergo interventions. However, the safety of these strategies and the cost savings need to be carefully evaluated prospectively.

The pentalogy of Cantrell is a rare midline developmental field complex often with a dismal outcome. We present a newborn qualifying for Cantrell's pentalogy with an abdominal wall defect, a diaphragmatic defect and a heart defect, a ventricular septal defect (VSD) and a left ventricular diverticulum. Early operative correction following accurate diagnostic work-up was prompted at the age of 11 weeks because of progressive heart failure. Beside correction of the VSD and the abdominal and diaphragmatic defects, resection of the distal part of the diverticulum was possible without damage to the LAD and preserving good contractility. Two years postoperative follow-up is uneventful.

Background Controversies exist regarding the use of pulse oximetry for routine screening of newborns. This study aimed to evaluate current practices and opinions of pediatric cardiologists in relation to newborn screening for congenital heart disease (CHD) using pulse oximetry. Methods Email invitations were sent to 1,045 pediatric cardiologists in North America. The survey was Internet based and included multiple-choice questions. Two repeat email reminders were sent after the initial invitation. Results A total of 363 responses (35%) were returned. In terms of experience, 40% of the respondents had more than 20 years, 32% had 10 to 20 years, 21% had 5 to 10 years, and 6% had less than 5 years of experience. More than 90% agreed that an early diagnosis of CHD for newborns prevents morbidity and mortality. In terms of practice, 96% reported that all newborns are examined by a clinician before discharge, 29% reported that newborns get a pulse oximetry reading, and 1.4% (n = 5) reported the use of electrocardiogram. Only 58% of respondents thought that current practice is adequate for detecting significant CHD. With regard to their experience with pulse oximetry, 26% reported ''too many false-positives,'' 21% described it as ''prone to noise and artifact,'' and 30% viewed it as ''very operator dependent.'' The overall support for mandated pulse oximetry screening was 55%. The support for mandate decreased with years of experience, with 76% of the supporters having less than 5 years, 58% of those having 5 to 10 years, 53% of those having 10 to 20 years, and 51% of those having more than 20 years of experience.

The objective of this study was to determine the initial safety and results of unrestricted multi-institution routine community use of the Amplatzer Septal Occluder (ASO) for atrial septal defect (ASD) closure. A multicenter, nonrandomized prospective study was performed in 13 pediatric cardiology centers from November 2004 to September 2007. Data were collected at the time of cardiac catheterization and 1 day postimplant. Four hundred seventy-eight patients underwent cardiac catheterization for ASO device closure of an ASD. The median age was 6 years (range, \1-83 years), and the mean weight was 37.2 kg (range, 2.6-148 kg). Procedural success was 96% (458/478 patients), with deficient rims being the major single reason for failed implantation (9/20). Major and minor complication rates were 1.1% and 4.8%, respectively, and were not different between simple and complex ASD groups. Success at 24 h was 99.4% (333/335) in the simple ASD group and 100% (120/120) in the complex ASD group. The presence of large defects, the presence of multiple defects, the use of multiple devices, and a weight \8 kg were significantly associated with a residual shunt (small to moderate) at 24 h. In conclusion, the ASO device in routine clinical practice for simple and complex ASD closure has an immediate safety and effectiveness profile equal to that reported in the initial pivotal FDA trial for simple ASDs. Based on the evolution in care posed by the ASO and the lack of consensus on patient selection in complex ASDs, this study points out the need to redefine the optimal patient and possibly broaden the indications for device closure of ASDs.

It is generally considered that the development of secundum atrial septal defect (ASDII) or oval fossa defect is the result of excessive resorption of the embryological atrial septum primum, but this does not seem to explain all defects. We investigated 58 postmortem hearts with an ASDII and 22 normal hearts from patients ranging in age from 1 day to 49 years. The different structures of the oval fossa were examined. In 86% of the specimens, the defects were the result of a malformation of the valvula foraminis ovalis or embryological atrial septum primum, and in 14% an absent superior limbus (septum secundum) was the cause of the interatrial communication. The ''septum primum'' ASDs were divided into four subgroups based on the degree of deficiency of the septum primum and position of the ostium secundum within the septum primum. We conclude that the morphogenesis of ASDII is variable and both septum primum and septum secundum defects occur, which may be relevant in view of genetic studies that may lead to further differentiation of patients with and without genetically determined ASDIIs.