Left ventricular non-compaction with Ebstein anomaly attributed to a TPM1 mutation.

Left ventricular non-compaction (cardiomyopathy) (LVN(C)) is a rare hereditary cardiac condition, resulting from abnormal embryonic myocardial development. While it mostly occurs as an isolated condition, association with other cardiovascular manifestations such as Ebstein anomaly (EA) has been reported. This congenital heart defect is characterized by downward displacement of the tricuspid valve and leads to diminished ventricular size and function.

Closure of a lateral baffle leak in a Fontan patient with an amplatzer PDA II AS plug.

Cyanosis after Fontan surgery or surgery for total cavopulmonary connection (TCPC), due to different types of communications (fenestration, venovenous collaterals or fistula), is not uncommon. We present the case of an 8-year-old girl presenting with increasing cyanosis during exercise 4 years after an intracardiac TCPC with lateral tunnel. Angiography showed a fistulous trajectory originating at the superior vena cava towards the base of the right atrial appendage.

Catheter interventions in congenital heart disease without regular catheterization laboratory equipment: the chain of hope experience in Rwanda.

This report describes the feasibility and safety of cardiac catheterization in a developing country without access to a regular cardiac catheterization laboratory. The equipment used for imaging consisted of a monoplane conventional C-arm X-ray system and a portable ultrasound machine using the usual guidewires and catheters for cardiovascular access. In this study, 30 patients, including 17 children younger than 2 years and 2 adults, underwent catheterization of the following cardiac anomalies: patent ductus arteriosus (20 patients) and pulmonary valve stenosis (9 patients, including 2 patients with critical stenosis and 3 patients with a secundum atrial septal defect).

Late cardiotoxicity after low dose of anthracycline therapy for acute lymphoblastic leukemia in childhood.

Introduction: Late cardiotoxicity is a known complication of anthracycline therapy but the long-term effects of low cumulative doses are not well documented. We studied late cardiotoxicity in survivors of childhood acute lymphoblastic leukemia (ALL) treated with low anthracycline doses 10 to 20 years earlier.

Methods: Seventy-seven ALL survivors who received a cumulative anthracycline dose <250 mg/m² and were at least 10 years after treatment were evaluated for signs of clinical heart failure.

Pulmonary function in children after surgical and percutaneous closure of atrial septal defect.

This study aimed to study differences in lung function after surgical and percutaneous atrial septal defect (ASD) closure. Several studies have demonstrated abnormalities of pulmonary function in adults and children with ASD. These abnormalities persist even a few years after correction.

Effective cardioverter defibrillator implantation in children without thoracotomy: a valid alternative.

The implantation method for a cardioverter defibrillator in children is poorly standardized because of obvious features related to size and predisposing cardiac disease in children presenting with malignant ventricular arrhythmia. We propose an alternative method of implanting a cardioverter defibrillator without the need for associated thoracotomy, based on the subxiphoidal insertion of an epicardial bipolar ventricular pacing and sensing lead, an active can placed in the abdomen, and a subcutaneous array tunneled along the left thoracic wall as a shock electrode. This technique offers the advantage of an effective and minimally invasive implantable cardioverter defibrillator with wide applicability for children, independent of their size and cardiac status.

Plastic bronchitis in a 5-year-old boy causing asystoly and fatal outcome.

Here we present the case of a 5-year-old boy who experienced a prehospital cardiac arrest after a period of wheezing, upper respiratory tract infection, and diarrhea. After successful resuscitation, ventilation was initially extremely difficult for no obvious reason. Various bronchodilatatory therapies were started with only limited result.

Transthoracic echocardiography guidance of transcatheter atrial septal defect closure in children.

Transesophageal echocardiography (TEE) guidance is part of interventional closure of secundum atrial septal defect (ASD) in children and adults. However, sometimes TEE is impossible for technical or anatomical reasons. If available, intracardiac echocardiography can be used, but especially in children, transthoracic echocardiography (TTE) can be an easy, safe, and cheap alternative.

Pleural effusions, water balance mediators and the influence of lisinopril after completion Fontan procedures.

Objective: To investigate whether the duration of pleural drainage after Fontan completion operations can be influenced by postoperative lisinopril administration or can be related to water balance hormone levels.

Methods: In a prospective trial 21 patients scheduled for total cavopulmonary connection were randomized into two groups, with group I receiving lisinopril postoperatively, and group II receiving no angiotensin converting enzyme inhibitor. Plasma levels of antidiuretic hormone, renin and aldosteron were analyzed preoperatively and at four-time points postoperatively.

Congenital heart disease in 111 225 births in Belgium: birth prevalence, treatment and survival in the 21st century.

Aim: To investigate the birth prevalence, treatment modalities and short-term survival of children with congenital heart disease who were born in 2002.

Methods: We undertook a retrospective review of medical records of all patients who were born in 2002, and were diagnosed, treated and/or followed-up in one of the seven-paediatric cardiology programmes in Belgium.

Results: In 111 225 births, 921 children with congenital heart disease were detected, yielding a birth prevalence of 8.

Unsuccessful resuscitation of a preterm infant due to a pneumothorax and a masked tension pneumopericardium.

Pneumopericardium is the least common form of air leak in infants. A tension pneumopericardium is even more infrequent but associated with a very high mortality rate. We describe the case of an unsuccessful resuscitation in a preterm infant due to a pneumothorax and tension pneumopericardium.

Orthodeoxia-platypnea syndrome presenting as paradoxical peripheral embolism.

A paradoxical embolus associated with orthodeoxia-platypnea syndrome and intracardiac shunting is extremely uncommon. We present a patient who was found to have a positional change in desaturation after a right pneumonectomy who suffered from gangrene of the right foot and simultaneous deep venous thrombosis of the left arm. Workup revealed a patent foramen ovale as a cause for both the right-to-left shunt and the paradoxical emboli.

Early cardiac abnormalities in obese children: importance of obesity per se versus associated cardiovascular risk factors.

We investigated whether obese children and adolescents have early echocardiographic signs of subclinical cardiac dysfunction and evaluated the respective influence of obesity per se versus parameters of carbohydrate and lipid metabolism that are frequently abnormal in obese subjects. The role of tissue Doppler imaging as a screening tool for these abnormalities was explored. Blood pressure and echocardiographic parameters, including tissue Doppler measurements of the septal mitral annulus were evaluated in 49 obese children and adolescents and 45 age and sex matched controls.

Midterm assessment of the reconstructed arteries after the arterial switch operation.

Background: The arterial switch operation is the preferred treatment for transposition of the great arteries (TGA), but there are concerns on the fate of the neoarterial trunks.

Methods: Ninety-three children were reviewed for functional and morphologic assessment of both reconstructed arteries after the arterial switch operation. Longitudinal analysis focused on neoaortic valve function, neoaortic obstruction, and neopulmonary stenosis as well as on the time-related size changes of both roots, with its clinical implications.

Impact of oxidative stress on the endothelial dysfunction of children and adolescents with type 1 diabetes mellitus: protection by superoxide dismutase?

Diabetes mellitus is associated with endothelial dysfunction and oxidative stress (OS). We investigated whether these abnormalities are interrelated in children and adolescents with type 1 diabetes mellitus (T1DM) and if early OS markers predictive of vascular dysfunction can be identified. Thirty-five T1DM patients were matched for sex, age, height, and weight with nondiabetic subjects as healthy controls (CO).

Self-reported physical activities in patients after the Mustard or Senning operation: comparison with healthy control subjects.

Background: Some decades ago, the Mustard or Senning operation was used to treat patients who were born with Transposition of the Great Arteries (TGA). Although life expectancy is good, previous studies have demonstrated limitations in exercise capacity. To what extent these limitations affect day-to-day functioning of the patients is not yet known.

Sleep-disordered breathing in overweight and obese children and adolescents: prevalence, characteristics and the role of fat distribution.

Aims: To determine the prevalence of sleep-disordered breathing (SDB) in a clinical sample of overweight and obese children and adolescents, and to examine the contribution of fat distribution.

Methods: Consecutive subjects without chronic lung disease, neuromuscular disease, laryngomalacia, or any genetic or craniofacial syndrome were recruited. All underwent measurements of neck and waist circumference, waist-to-hip ratio, % fat mass and polysomnography.

Improved perceived health status persists three months after a special sports camp for children with congenital heart disease.

Introduction: Although summer and sports camps for children with congenital heart disease are organized in many countries and regions, empirical data on the effects of such camps is limited.

Objectives: The aim of the present study was to investigate changes in the perceived health status and habitual physical activities in children attending a special sports camp.

Materials And Methods: In this longitudinal study, 25 children with congenital heart disease who participated in a three-day multi-sports camp were included.

Female children and adolescents with type 1 diabetes have more pronounced early echocardiographic signs of diabetic cardiomyopathy.

Objective: This study was designed to assess whether children and adolescents with type 1 diabetes have early echocardiographic signs of subclinical cardiac dysfunction and whether sex, state of metabolic control, and diabetes duration are of influence.

Research Design And Methods: Systolic and diastolic blood pressure in supine and upright positions and echocardiographic parameters, including tissue Doppler measurements of the septal mitral annulus, were evaluated in 80 children and adolescents with stable type 1 diabetes and 52 age- and sex-matched control subjects. A possible correlation was examined for age, sex, HbA(1c), and diabetes duration with univariate and multivariate regression analysis.