We found right-to-left shunts through the cardiac veins postoperatively in 2 patients who had undergone the Fontan procedure. In one of the patients, channels were present through the cardiac veins independent of the coronary sinus. In the other patient, an atretic orifice for the coronary sinus, coupled with a persistent left-sided superior caval vein, complicated the postoperative course.

Objective: To evaluate heart rate variability by Holter monitoring in type 1 diabetic children compared with a healthy control group and determine the factors modifying heart rate variability. Methods: This was designed as a prospective study comparing 28 patients, diagnosed with type 1 diabetes and under follow-up, with 27 healthy control group subjects. Results: The patients were aged 9.9 ± 4.2 years in the diabetic group, including 13 (46.5%) girls and 15 (53.5%) boys. The healthy control group comprised 20 (74%) girls and seven boys (26%) with an average age of 8.6 ± 3.7 years. The search for factors modifying heart rate variability yielded the following correlations: for the time-dependent variables, negative between age and both average and maximal heart rate (r = −0.263 and −0.460, respectively), negative between haemoglobin A1c and percentage of differences between adjacent RR intervals >50 ms, positive between diabetes duration and square root of the mean of the sum of squares of differences between adjacent NN intervals. The average heart rate and percentage of differences between adjacent RR intervals >50 ms was significantly higher in the girls than the boys in all groups. With regard to the frequency-dependent factors affecting heart rate variability, correlations were found between haemoglobin A1c level and both total power and very low frequency (r = −0.751 and −0.644) and between very low frequency and diabetes duration. Conclusion: A reduction in heart rate variability parameters was observed in type 1 diabetes mellitus patients who had a long disease duration or were poorly controlled, as compared with healthy controls.

Loeys–Dietz syndrome is a newly described entity characterised by a constellation of arterial tortuosity, cranial malformations, and hypertelorism. We report a case of a 7-year old boy with confirmed Loeys–Dietz syndrome and discuss magnetic resonance imaging as a complete technique for assessment and follow-up of aggressive vascular pathology in the brain, thorax, and abdomen, which may dictate early surgical intervention.

When there is obstruction of the left ventricular outflow tract, there are several surgical approaches to the repair of discordant ventriculo-arterial connections in the setting of concordant atrioventricular connections. Choosing the optimal technique demands not only knowledge of the different surgical procedures, but also the understanding of the particular anatomic features present in a specific patient. These requisites are then essential to plan the operation, to foresee some difficult situations, and to avoid post-operative complications. In this review, we assess all these surgical and anatomic aspects, focussing on their relative importance in clinical assessment.

We report a case of severe hypertriglyceridemia associated with an everolimus drug-eluting stent in an infant with pulmonary vein stenosis. We review from current literature the mechanisms by which everolimus may cause dyslipidaemia, pharmacokinetics of everolimus in drug-eluting stents, and treatments of hypertriglyceridemia. This case demonstrates the need to closely monitor serum triglyceride levels after everolimus drug-eluting stent placement in infants.

Aims: Advances in medical treatment have resulted in increased life expectancy in congenital heart disease. Consequently, the focus of management has shifted from reducing mortality to reducing long-term morbidity with the goal of improving quality of life. A predictor of quality of life might be N-terminal pro-brain natriuretic peptide, a well-established marker for heart failure. We aimed to determine the association between N-terminal pro-brain natriuretic peptide and quality of life in patients with congenital heart disease. Methods: We collected blood samples from consecutive patients who were initially operated between 1968 and 1980 (47.8% women; mean age 40.2±5.4 years). The 36-item Short-Form Health Survey was completed to assess subjective health status as a measure of quality of life. Analysis was performed for the entire group and for subgroups defined as simple versus complex congenital heart diseases. Median N-terminal pro-brain natriuretic peptide level was 15.2 pmol/L (overall range 1.3–299.3 pmol/L). N-terminal pro-brain natriuretic peptide levels were associated with the subdomain physical functioning (β=−0.074, p=0.031). This association remained significant after adjustment for age and sex (β=−0.071, p=0.038) and after adjustment for age, sex, body mass index, left ventricular function, and renal function (β=−0.069, p=0.048). In complex congenital heart disease, the association between N-terminal pro-brain natriuretic peptide and physical functioning remained significant in multivariable analysis (β=−0.076, p=0.046). No associations were found in the simple congenital heart disease group or on the other health status subdomains. Conclusion: In adults operated for congenital heart disease, N-terminal pro-brain natriuretic peptide is associated with the subdomain physical, primarily in the complex subgroup.

In this observational case report, we share our experience of achieving >40% LDL cholesterol reduction in four Chinese homozygous familial hypercholesterolaemia children below 8 years of age with a triple combination of atorvastatin, probucol, and ezetimibe for >6 years. Within a follow-up duration of 6–13 years, this triple therapy achieved significant reduction of LDL cholesterol as well as an impressive regression of xanthomas in all paediatric cases. All the children remained free from treatment-related adverse responses and cardiovascular events throughout follow-up.

The success of extracorporeal support in providing cardiopulmonary support for a variety of patients has led to use of Extracorporeal Life Support, also known as ECLS, as a rescue for patients failing conventional resuscitation. The use of Extracorporeal Life Support in circumstances of cardiac arrest has come to be termed “Extracorporeal Life Support during Cardiopulmonary Resuscitation” or “ECPR”. Although Extracorporeal Life Support during Cardiopulmonary Resuscitation was originally described in patients following repair of congenital cardiac defects who suffered a sudden arrest, it has now been used in a variety of circumstances for patients both with and without primary cardiac disease. Multiple centres have reported successful use of Extracorporeal Life Support during Cardiopulmonary Resuscitation in adults and children. However, because of the cost, the complexity of the technique, and the resources required, Extracorporeal Life Support during Cardiopulmonary Resuscitation is not offered in all centres for paediatric patients with refractory cardiac arrest. The increasing success and availability of Extracorporeal Life Support during Cardiopulmonary Resuscitation in post-operative cardiac patients, coupled with the fact that patients undergoing the Norwood (Stage 1) operation can have rapid, unpredictable cardiac deterioration and arrest, has led to a steady increase in the use of Extracorporeal Life Support during Cardiopulmonary Resuscitation in this population. For Extracorporeal Life Support during Cardiopulmonary Resuscitation to be most successful, it must be deployed rapidly while the patient is undergoing excellent cardiopulmonary resuscitation. Early activation of the team that will perform cannulation could possibly shorten the duration of cardiopulmonary resuscitation and might improve survival and outcome. More research needs to be done to refine the populations and circumstances that offer the best outcome with Extracorporeal Life Support during Cardiopulmonary Resuscitation, to evaluate the ratios of cost to benefit, and establish the long-term neurodevelopmental outcomes in survivors.

A family is described where the father has the many skeletal, but none of the cardiac abnormalities associated with the Holt-Oram syndrome. His two daughters have similar skeletal anomalies, but with identical cardiac lesions, as does another patient, raising the possibility of an associated genetic marker.

Williams syndrome is a rare genetic disease that affects elastin production, leading to medium and large vessel stenoses and other abnormalities. Cardiac manifestations of Williams syndrome are the most life-threatening, occurring in 80% of children. Children with Williams syndrome are known to be at risk for sudden cardiac death. These tragic events are often precipitated by diagnostic or therapeutic procedures requiring anaesthesia or sedation, such as cardiac catheterisation. We present the case of a 3-month-old infant with Williams syndrome who suffered sudden cardiac arrest during cardiac catheterisation and subsequent arrest approximately 48 hours after the procedure. We also review the current literature focused on children with Williams syndrome who have suffered sudden cardiac arrest during or after cardiac catheterisation procedures.

Outcome following the treatment of congenital heart disease continues to improve and interest has focused on reducing morbidity as well as mortality. One important aspect of this is the psychological effect of congenital heart disease and its treatment on both children and their parents. This review addresses the extent and nature of the psychological morbidity associated with this disease, in particular the impact of the diagnosis on the management of patient and family. The etiology of the high level of psychosocial morbidity remains poorly understood. Data from the 1960s concerned with pre-school and school-age children is now of little value given the dramatic changes in the treatment of congenital heart disease, which is now predominantly in the neonatal period. While theory from developmental and pediatric psychology may be usefully applied to this area, further research is required to define effective psychological care for patients and their families.

Our aim was to analyze left ventricular fractional shortening during extracorporeal membrane oxygenation under the influence of changing volume loading conditions induced by a ductal left-to-right shunt. In all patients, the fractional shortening was observed using echocardiography before, during, and after bypass, irrespective of the presence or absence of the ductal left-to-right shunt. During membrane oxygenation, there was a significant decrease in fractional shortening (p less than 0.001), with no difference before and after membrane oxygenation. A greater decrease in fractional shortening was observed in the group with a ductal left-to-right shunt when compared to patients lacking the ductal shunt (p less than 0.006). The diastolic diameter of the left ventricle also increased significantly during the membrane oxygenation in those patients with left-to-right ductal shunting. Moreover, the patients with left-to-right shunting showed a very severe decreased fractional shortening, lower than 10 per cent, with significantly greater frequency (p less than 0.05) during the course of membrane oxygenation. Conclusion: An important decrease in left ventricular fractional shortening is observed during veno-arterial extracorporeal membrane oxygenation. Left-to-right shunting during bypass, as seen in the patients with patency of the arterial duct, increases the loading conditions on the left ventricle, and produces a significant increase in left ventricular diastolic dimensions. Despite the effects of volume loading produced by the ductal shunt during bypass, the decrease in fractional shortening is significantly more pronounced for these patients. Therefore, during membrane oxygenation the volume loading produced by the ductal shunt is unable to prevent a decrease in left ventricular fractional shortening.

Double-chambered left ventricle is a rare cardiac anomaly. We report a case of double-chambered left ventricle in a one-and-half-year-old asymptomatic boy. We depict the use of three-dimensional echocardiography in the demonstration and diagnosis of the condition.

We have now achieved transcatheter occlusion of the patent arterial duct as a day-case procedure in 26 patients. In all but two, who were judged not to be appropriate for the device, implantation was successful. Most of the patients arrived in the hospital at 8:00 a.m. and had to wait for a mean time of 154 minutes until they were taken for cardiac catheterization. The procedure, performed under sedation in 25 patients and under general anesthesia in two, took a mean of 91 minutes. The children were then monitored for two hours in the recovery room. The interval From the time of arrival in the Unit until waking was a mean of 196 minutes. Then, from the time of waking, a mean of 123 minutes elapsed prior to discharge. Admission of the patient was necessary in three instances (11.1%). Two patients vomited and remained too drowsy for safe discharge. A third patient had either occlusion or thrombosis of the femoral artery and received an infusion of heparin which necessitated admission overnight. All three patients were discharged the following morning. Patients from outside Riyadh stayed overnight in Riyadh before they went home. One patient complained of non-cardiac chest pains a few hours after discharge and was subsequently sent home. Total occlusion, as judged by mean follow-up of 13 months (5–26 months) using color flow Doppler was achieved in 19 of the 24 patients (79!) in whom devices were inserted. All patients are well and there are no complaints concerning the procedure done on a day-case basis. We conclude that the day-case protocol for transcatheter occlusion of the patent arterial duct is a safe and cost-effective option for selected patients.