Obesity trends in children,adolescents, and young adults with congenital heart disease

Objectives: To determine the prevalence, age of onset, and risk factors for overweight and obesity in children with congenital heart disease (CHD).
Study Design: Children with CHD who were seen at our institution from 1996 to 2017 were studied. Patients were full-time residents of the United States and were receiving all cardiac care at our institution. Patients were categorized by age and CHD diagnosis. The date of last normal weight for age and the date of first recorded weight in the range of overweight and obese were documented.
Results: Nine hundred sixty-eight patients with CHD were included. The prevalence of overweight and obesity was 31.5% and 16.4%, respectively. For patients who became overweight or obese, the last recorded normal weight was between 6 and 10 years of age. Electrophysiologic disease and older age were risk factors for obesity.
Conclusions: Children with CHD have an increasing risk of becoming overweight and obese in early childhood. This study provides important information and identifies critical period to implement preventative measures and counsel families about the risk of obesity in CHD.     

Interventional therapy for neonates with critical congenital heart disease

Transcatheter interventional therapies for children continue to evolve at a remarkable rate. Perhaps no where are these procedures more important than in the critically ill newborn. In this core curriculum review article several of the most commonly performed interventions performed in critically ill newborns are discussed with regards to technique and outcomes. © 2008 Wiley‐Liss, Inc.     

Radiofrequency-assisted atrial septoplasty for an intact atrial septum in complex congenital heart disease.

Septoplasty of the atrial septum was performed with sequential balloon dilation following radiofrequency-assisted perforation of an intact atrial septum in two newborn infants with hypoplastic left heart syndrome and one with double-outlet right ventricle.     

Coronary artery disease in patients with congenital heart defects

The prognosis of patients with congenital heart defects has improved significantly: more and more patients reach adulthood and old age. At the same time, the possibility of cardiovascular morbidity increases. The conventional risk factors for coronary artery disease are at least as high or even higher in patients than in the general population. Obesity and sedentary life style are more common in adults with congenital heart defect (ACHD) than in general population. In some patients, for example those with coarctation of the aorta or patients with operated coronary arteries in the infancy, the incidence of coronary artery disease (CAD) is clearly increased. In some patients with cyanotic heart defects (e.g. Fontan), the incidence of CAD might be lower, but it usually returns to the average level or higher after correction of the defect. Coronary artery disease is one of the most important reasons for mortality also in ACHD patients, and the consequences of a coronary event might be more fateful in a patient with a corrected congenital heart defect than in her/his peer. There should be a paradigm shift from operative mortality and short‐term outcome to long‐term morbidity and prevention of cardiovascular disease – a task that often has been forgotten during follow‐up visits.     

Radiofrequency perforation in the treatment of congenital heart disease.

Catheter-directed perforation of cardiac tissue with radiofrequency (RF) energy has expanded the horizon of the interventional cardiologist dealing with congenital heart disorders. The focus of the following discussion will be to detail the biophysical basis behind RF perforation and review its application in the management of congenital heart lesions.     

Catheter-based decompression of the left atrium in patients with hypoplastic left heart syndrome and restrictive atrial septum is safe and effective.

Infants with hypoplastic left heart syndrome (HLHS) and restrictive or intact atrial septum (rAS) present with cyanosis, pulmonary edema, and are critically ill. A previous report from our institution on emergent Norwood for HLHS with rAS showed 10% survival. We hypothesized that transcatheter left atrial (LA) decompression in HLHS with rAS would safely and effectively relieve LA hypertension, improve oxygenation, and improve Norwood survival. Between 1996 and 2004, 30 patients with HLHS and rAS underwent cardiac catheterization for pre-Norwood intervention. Twenty-eight atrial septostomies were performed: 23 static balloon dilations, 4 Rashkind septostomies, and 1 intra-atrial stent. Two procedures were aborted due to perforation (n = 1) or inability to enter the LA (n = 1). Eight total patients required surgical septectomy, for a failure rate of 27%. There were no catheter-related mortalities, although two patients died within 36 hr of the procedure after surgical septectomy. Major complications occurred in three patients (10%)--atrial perforations requiring intervention. Mean atrial septal defect gradient fell from 16.7 +/- 4.9 to 6.3 +/- 3.4 mm Hg (P < 0.001; n = 18). Mean LA pressure dropped from 21.8 +/- 5.5 to 13.1 +/- 6.5 mm Hg (P < 0.001; n = 16). Mean PaO(2) rose from 29.5 +/- 9.1 to 36.5 +/- 5.1 torr (P < 0.001; n = 23). Seventeen of 30 patients (57%) survived to discharge from Norwood. Thirteen have undergone hemi-Fontan and nine Fontan. Sixteen of 22 successful decompressions (73%) survived to discharge. Transcatheter decompression of the LA for patients with HLHS and rAS can be performed safely, reduces the transatrial gradient, and improves oxygenation. Catheter intervention improves survival compared to historical controls undergoing emergent Norwood.     

Prediction of behavioural and emotional problems in children and adolescents with operated congenital heart disease

Aims The aim of the present study was to determine which medicalvariables were predictors of long-term behavioural/emotionaloutcome after surgical correction for congenital heart diseasein infancy and childhood. Methods The Child Behavior Checklist (CBCL) was used to predict parent-reportedbehavioural/emotional problems in 125 10–15 year-old congenitalheart disease children from: (1) biographical status (2) medicalhistory (3) heart surgery (4) short-term post-operative courseand (5) number of heart operations and (6) extra cardiac concomitantanomalies. Results Higher CBCL total problem scores at follow-up were associatedwith a greater number of heart oper-ations and deep hypothermiccirculatory arrest (<22°). ‘Internalizing problems’were associated with a greater number of heart operations, deephypothermic circulatory arrest, a short gestational age, lowsystemic oxygen saturation, and older age at surgical repair.‘Externalizing problems’ were associated with agreater number of heart operations only. Conclusion Several medical variables were significant predictors and canbe used to identify those congenital heart disease childrenwho are at risk of long-term behavioural/emotional maladjustment.     

先天性心脏病患儿围术期呼吸功能的保护

目的探讨婴幼儿体外循环手术围术期影响呼吸功能的因素及呼吸功能保护策略。方法本文以婴幼儿呼吸道解剖学与病理生理学特点为基础,对不同先天性心脏病及麻醉与体外循环对该类患儿呼吸功能的影响进行全面系统分析,提出心脏手术后呼吸管理策略与方法,如术中的肺保护措施、呼氧末正压（PEEP）的应用、适当过度通气、大心脏患儿采用俯卧位通气、重度低氧血症患儿及时应用外源性肺表面活性物质、肺泡复张手法的应用、气管镜吸痰等策略及方法。结果通过上述策略与方法的实施,我科婴幼儿术后呼吸道管理与早期相比,有了显著进步,机械通气时间由以往的（19.2±6.4）h,减少到（11.9±2.5）h（P<0.01）,ICU时间由（7.2±3.9）d,下降到（4.1±2.6）d（P<0.01）,手术死亡率由5.6%（44/790）下降到3.9%（23/584,P<0.05）。结论围术期有多种因素影响先天性心脏病患儿呼吸功能,早期就开始注意肺保护功能,将会收到明显效果。     

Trajectories of care in congenital heart disease – the long arm of disease in the womb

Our understanding of the development of congenital heart disease (CHD) across the lifespan has evolved. These include the evidence for the change in demographics of CHD, the observations that lifelong complications of CHD result in CHD as a lifespan disease, and the concept of long windows of exposure to risk that start in foetal life and magnify the expression of risk in adulthood. These observations set the stage for trajectories as an emerging construct to target health‐service interventions. The lifelong cardiovascular and systemic complications of CHD make the long‐term care of these patients challenging for cardiologists and internists alike. A life‐course approach is thus required to facilitate our understanding of the natural history and to orient our clinical efforts. Three specific examples are illustrated: neurocognition; cancer resulting from exposure to low‐dose ionizing radiation; and cardiovascular disease acquired in ageing adults. As patients grow, they do not just want to live longer, they want to live well. With the need to move beyond the mortality outcome, a shift in paradigm is needed. A life‐course health development framework is developed for CHD. Trajectories are used as a complex construct to illustrate the patient’s healthcare journey. There is a need to define disease trajectories, wellness trajectories and ageing trajectories in this population. Disease trajectories for repaired tetralogy of Fallot, transposition of the great arteries and the Fontan operation are hypothetically constructed. For clinicians, the life‐course horizon helps to frame the patient’s history and plan for the future. For researchers, life‐course epidemiology offers a framework that will help increase the relevance of clinical enquiry and improve study design and analyses. A health‐service policy framework is proposed for a growing number of conditions that start in the before birth and extend as long as contemporary survival now permits. Ultimately, the goal is the precision delivery of health services that enables lifelong health management, organization of developmental health services, and integration of vertical and horizontal health‐service delivery.     

First reported use of drug‐eluting bioabsorbable vascular scaffold in congenital heart disease

The aim of catheter intervention for vascular stenosis is the restoration of lumen area and optimization of distal blood flow. In pediatric practice, this has traditionally been a compromise between less effective balloon angioplasty and bare metal stent insertion with its attendant limitations of size. Bioabsorbable stents offer short‐term relief of stenosis, radial support of the healing lesion, return of endothelial function and crucially, in children, the potential for long‐term growth. Initial experience, in pediatric practice, with metal‐based bioabsorbable stents was relatively disappointing with frequent restenosis secondary to early reabsorption. Design modifications resulting in polymer‐based, drug eluting, bioabsorbable vascular scaffolds (BVS) have reportedly overcome some of these faults. We describe the first reported use of a drug eluting BVS in three patients with: (1) A newborn with severe right pulmonary artery (RPA) stensosis post repair of type two common arterial trunk. (2) A child with pulmonary atresia/ventricular septal defect (VSD) and major aorto‐pulmonary collateral arteries (MAPCAs), and (3) An infant with severe left pulmonary artery (LPA) stenosis in the setting of an LPA sling. © 2015 Wiley Periodicals, Inc.     

Stent implantation to create interatrial communications in patients with complex congenital heart disease

A restrictive interatrial communication can complicate the management of complex congenital heart disease. The purpose of this report is to present a new technique to achieve a patent and reliable interatrial communication by using an endovascular stent. A stent was successfully implanted across a fenestrated extracardiac conduit in two patients with low cardiac output after Fontan operations and across the interatrial septum in a patient with double inlet left ventricle and severe left atrioventricular stenosis. The procedures were uncomplicated and all patients showed immediate hemodynamic improvement. Cathet. Cardiovasc. Intervent. 47:310–313, 1999. © 1999 Wiley-Liss, Inc.     

Catheter management of aortic valve stenosis in neonates and children.

Therapy for severe aortic valve stenosis in infants and children has shifted from the operating suite to the catheterization laboratory and even to the bedside as a direct result of improved catheter technology, evolving techniques, and comparable results to conventional surgical intervention. This review summarizes the brief history pertaining to the various techniques and outcomes of transcatheter balloon valvuloplasty in infants and children with critical or severe aortic valve stenosis.     

Vancomycin dosing required to achieve a therapeutic level in children post-surgical correction of congenital heart disease

The vancomycin dosing range for safe and effective treatment remains uncertain for children who had corrective surgery for a congenital heart disease (CHD). We aimed to determine the vancomycin dosing requirements for this subgroup of patients.This prospective cohort study included children younger than 14 years old with CHD who received intravenous vancomycin for at least 3 days at the Pediatric Cardiology section of King Abdulaziz Medical City, Riyadh.In total, 140 pediatric patients with CHD were included with a median age of 0.57 years (interquartile range 0.21–2.2). The mean vancomycin total daily dose (TDD), 37.71 ± 6.8 mg/kg/day, was required to achieve a therapeutic trough concentration of 7–20 mg/L. The patient’s age group and the care setting were significant predictors of the vancomycin dosing needs. Neonates required significantly lower doses of 34 ± 6.03 mg/kg/day (P = .002), and young children higher doses of 43.97 ± 9.4 mg/kg/day (P = .003). The dosage requirements were independent of the type of cardiac lesion, cardiopulmonary surgery exposure, sex, and BMI percentile. However, the patients in the pediatric cardiac ward required higher doses of vancomycin 41.08 ± 7.06 mg/kg/day (P = .039). After the treatment, 11 (8.5%) patients had an elevated S_cr, and 3 (2.3%) patients developed AKI; however, none of the patients’ sociodemographic factors or clinical variables, or vancomycin therapy characteristics was significantly associated with the renal dysfunction.Overall, the vancomycin TDD requirements are lower in pediatric post-cardiac surgery compared to non-cardiac patients and are modulated by several factors.     

The Adult Congenital and Pediatric Cardiology Section: increasing the opportunities for the congenital heart disease community within the American College of Cardiology

The Adult Congenital and Pediatric Cardiology (AC/PC) Section was established to develop a clear voice within the American College of Cardiology and address the myriad issues facing the congenital heart disease profession. The Section is governed by the AC/PC Council, which includes pediatric cardiologists, adult congenital cardiologists, a cardiac care associate, and a fellow-in-training member. The Council is responsible for bidirectional communication between the College's Board of Trustees and the AC/PC Section members. Since its founding in 2004, Section objectives have been defined by the College's mission: to advocate for quality cardiovascular care through education, research promotion, and the development and application of standards and guidelines and to influence health care policy. The pillars of the College-advocacy, quality, education, and member engagement-serve as the defining template for the Section's strategy. The Section has developed work groups in advocacy, clinical practice, education and training, quality, and publications. A separate leadership group has been developed for adult congenital heart disease. Work groups are open to all Section members. Recognition of the importance of lifelong care in congenital heart disease led Section leaders to incorporate pediatric cardiology and adult congenital heart disease content into each of the work groups. There are more than 1,200 Section members, with nearly 400 members actively contributing to Section activities. This article outlines Section efforts to date and highlights significant successes to date.     

Transcatheter closure of perimembranous ventricular septal defects using amplatzer asymmetric ventricular septal defect occluder: preliminary experience with 18-month follow up.

BACKGROUND: This study reports our experience in the nonsurgical closure of perimembranous ventricular septal defects in children and adolescents with the Amplatzer asymmetric ventricular septal defect occluder and the outcome of an 18-month follow up. METHODS AND RESULTS: Twenty patients (median age:10 years; median weight:32 kg) with perimembranous ventricular septal defect were selected for transcatheter closure with the Amplatzer device. The prosthesis diameter chosen was 1-2 mm larger than the largest measured diameter of the defect on transesophageal echo (TEE). All patients were put on oral aspirin (5 mg/kg/day in children and 150 mg/day in adults) five days prior to and for six months after closure. Follow-up evaluation at 48 hr and 1, 6, 12 and 18 months included clinical examination, electrocardiogram, and a transthoracic echocardiogram. The mean defect diameter on color flow mapping on TEE was 7.1 +/- 2.3 mm. The device diameter ranged from 6-14 mm (median = 8 mm). One patient developed an anaphylactic reaction to contrast. The procedure was successful in 17 out of 19 patients where it was attempted (89.4%). In two patients with associated significant aortic valve prolapse and mild aortic regurgitation the device could not be successfully deployed. A trivial residual shunt observed during postdeployment left ventricular angiogram in 7 of 17 patients (41.2%) completely disappeared at one month follow-up. Three patients had right bundle branch block (2 complete and 1 incomplete) whereas one developed junctional escape rhythm with a right bundle branch block morphology. One patient had clinically silent thromboembolism to the left vertebral artery and another patient had hemolysis which resolved spontaneously within 48 hr. Follow-up at 13.5 +/- 5.3 months (range 1-18 months) revealed no residual shunt. The left ventricular internal dimension in diastole decreased significantly from 45 +/- 6 mm to 40 +/- 6 mm (P < 0.01) at the time of the last follow up. The baseline tricuspid regurgitation (n = 4) and aortic regurgitation (n = 3) remained unchanged during the follow up period. None of the patients developed left ventricular outflow tract obstruction or new aortic or tricuspid regurgitation. There were no other device related complications such as device migration, systemic thromboembolism, infective endocarditis, pericardial effusion or delayed conduction disturbances. CONCLUSIONS: In carefully selected children and young adults, the Amplatzer asymmetric ventricular septal defect occluder is a promising device for transcatheter closure of perimembranous ventricular septal defect with encouraging results on short term follow up.     

Interventional treatment for congenital heart disease and its severe complications

Background To approach the incidence, cause and possible treatment of severe complications induced during or after congenital heart disease interventions. Methods Interventional procedures of congenital heart disease were performed in 654 patients from January 2003 to October 2009, which were divided into four groups, i.e. patent ductus arteriosus （PDA）, atrial septum defect （ASD）, pulmonary stenosis（PS）, ventricular septum defect（VSD）, among them 32 patients with severe complications were retrospectively analyzed. Results A total of 654 cases accepted the treatment of intrusion. There was one death in all the patients, ten patients failed in the operation. The overall severe complication rate was 4.89% （32/654）, in which 2.29%（4/175） in PDA group, 5.26%（10/190） in ASD group, 5.77% （3/52） in PS group, 6.33%（15/237） in VSD group, respectively. Conclusions The severe complication rates of interventional therapy for congenital heart disease are low, it is a relatively safe interventional method, and careful supervision is necessary during or after procedure.     

The pediatric cardiologist and adolescents with congenital heart disease

The Cardiology Division of The Children's Hospital of Pittsburgh has designed a format for dealing with adolescent patients with ongoing cardiac disease. At this stage, when the patients are neither children nor adults, the integration of ongoing disease is particularly difficult. They are already more vulnerable because of the complex psychosocial demands of normal adolescent growth and development. The issues of understanding and addressing all of their needs are of upmost importance if they are to be given total medical care.