The purpose of the study was to determine the prevalence of coronary artery anomalies and to demonstrate in which cases multidetector computed tomography has an additional clinical value compared with the conventional angiography.

A total of 2375 multidetector computed tomography studies were retrospectively reviewed to determine the dominance of the coronary artery anomalies. The classification of coronary artery anomalies was made according to anatomical criteria – origin, course, intrinsic anatomy, and termination – and clinical relevance – benign versus malignant.

The coronary artery system was right dominant in 83.99%, left dominant in 8.0%, and co-dominant in 9.01% of the cases. The incidence of the origin and/or course anomalies was 1.76%, that of fistulas was 0.42%, and that of myocardial bridges was 10.82%. Multidetector computed tomography was performed after conventional angiography in 23 cases and it provided additional information regarding its origin and proximal course, as well as its relationship with the aortic root and main pulmonary trunk in 100% of the cases; eight malignant cases were found. In addition, in all of (100%) the six cases with coronary artery fistulas, conventional angiography failed to detect their terminations, which were clearly depicted by multidetector computed tomography.

Multidetector computed tomographic angiography is superior to conventional angiography in delineating the ostial origin and proximal course of anomalous coronary arteries. Furthermore, it reveals the exact relationship of anomalous coronary arteries with the aorta and the pulmonary artery. Anomalies of the intrinsic anatomy and the termination of coronary arteries are also better visualised with multidetector computed tomography.

Foetal aortic valvuloplasty has been proposed as a strategy to improve left heart growth and function in foetuses with severe aortic stenosis at risk of progression to hypoplastic left heart syndrome. We report our experience with this intervention.

Between 2005 and 2010, five foetuses with aortic stenosis and at risk of progression to hypoplastic left heart syndrome underwent ultrasound-guided percutaneous foetal aortic valvuloplasty. There were no associated maternal complications or foetal demise. In one case, the pregnancy was terminated a couple of weeks after the intervention, one foetus evolved to hypoplastic left heart syndrome, and three did not.

Foetal aortic valvuloplasty seems to be a safe and feasible procedure. It has been reported that it has the potential to prevent progression to hypoplastic left heart syndrome in selected foetuses with severe aortic stenosis. Further investigation regarding physiological and clinical aspects of this disease both prenatally and postnatally will probably allow to improve therapeutic strategies and clinical outcome.

To compare potential risk factors for complications and recurrence after radiofrequency catheter ablation in symptomatic atrioventricular reentrant tachycardia in children and adolescents.

We retrospectively reviewed the data of 213 consecutive patients with symptomatic atrioventricular reentrant tachycardia who underwent both electrophysiological study and radiofrequency catheter ablation, divided these patients into two groups, children (age <12 years) and adolescents (12 ≤ age < 18 years), and compared the location of the accessory pathway, success rate, recurrence rate, complications, presence of congenital heart disease, presence of intermittent ventricular pre-excitation, and presence of Wolff–Parkinson–White syndrome in the two groups.

The position of the accessory pathway was mostly right sided in children (61.3%) and left sided in adolescents (61.5%). Children had significantly more congenital heart disease than adolescents (6.4% versus 0.8%). Univariate analysis showed children or adolescents with right-sided accessory pathways to be 6.84 times and those with accessory pathways on both sides of the septum 25 times more likely to relapse than those with a single accessory pathway. Multivariate analysis indicated that children or adolescents with two accessory pathways were six times, and those with intermittent ventricular pre-excitation nine times more at risk of relapsing following radiofrequency ablation than those with single accessory pathways. All five complications occurred in children.

The findings suggest that the position and number of accessory pathways and presence of intermittent ventricular pre-excitation are related to risks of recurrence of atrioventricular reentrant tachycardia in children and adolescents.

The objective was to report two new patients with the diagnosis of alveolar capillary dysplasia and congenital heart disease, to describe the associated cardiac defects seen in these cases and in the literature, and to consider recent genetic advances concerning the FOX transcription factor gene cluster in chromosome 16q24.1q24.2.

We retrospectively analysed the records of all patients with congenital heart disease and alveolar capillary dysplasia seen in the Pediatric Cardiology Department between 2005 and 2010. We reviewed all literature published in the English language relating to cases of alveolar capillary dysplasia and congenital heart disease.

Two infants with alveolar capillary dysplasia and cardiac malformation were identified: one had an atrioventricular septal defect and a de novo balanced reciprocal translocation t(1;16)(q32;q24), the second infant had a ventricular septal defect. Analysis of 31 cases of the literature including these new cases showed a predominant association of alveolar capillary dysplasia with obstructive left heart disease (35%), as well as an atrioventricular septal defect (29%). FOX gene cluster defects were identified in eight of these patients.

Genetic background of alveolar capillary dysplasia is discussed in the light of the balanced reciprocal translocation t(1;16)(q32;q24) identified in the first child of this report. Alveolar capillary dysplasia should be suspected in neonates with congenital heart disease and unexpectedly elevated pulmonary vascular resistances, especially in cases of obstructive left heart disease or atrioventricular septal defect. Detecting FOX gene cluster defects should be considered in infants with alveolar capillary dysplasia with or without congenital heart disease.

The aims of this case–control study were to (a) compare the caries experience and oral hygiene, and (b) quantify the persistence of a delay in the dental age in children with cardiac disease and a group of healthy children.

The study population comprised a group of 268 3- to 16-year-old children and adolescents with a cardiac disease and a group of 268 age- and sex-matched healthy children and adolescents. Specifically, the decayed, missed, and filled teeth indices, simplified oral hygiene index, and the dental ages of the two groups of children were calculated and then compared.

Although the oral health of the children with either a congenital or an acquired heart disease was the same as that of the healthy children, there were significant differences in the decayed, missed, and filled teeth indices. Dental ages of the children with a congenital heart disease were significantly lower than those of healthy children. The findings showed that complex univentricular heart diseases had the highest negative impact on dental development (−1.1), followed by complex biventricular (−0.9), simple surgical (−0.5), and mild (−0.4) heart disease patients.

Once thorough knowledge of the child's cardiac status is gained, a definitive dental treatment plan for the child with a cardiac disease can be established.

Surgical ligation of patent ductus arteriosus is considered when medical treatment fails or is contraindicated. This study aims to determine the mortality and morbidity of preterm neonates referred for patent ductus arteriosus ligation.

A prospective study was conducted in the East of England to follow the outcome of premature infants under 37 weeks’ gestation undergoing patent ductus arteriosus ligation. A standardised proforma was used to collect information before and after the procedure.

A total of 102 premature infants were recruited, and patent ductus arteriosus ligation was performed in 92. Surgical complications occurred in 8.7% (8/92), which included pneumothorax (5/8), recurrent laryngeal nerve palsy (2/8), and chylothorax (1/8). Morbidity outcome data were not available for all infants. The incidence of chronic lung disease was 88% (88/99); intraventricular haemorrhage was 49% (49/100); necrotising enterocolitis 39% (39/99), and retinopathy of prematurity 42% (41/97). The overall mortality rate in our study was 7.8% (8/102). Mortality rate in infants who had patent ductus arteriosus ligation was 4.3% (4/92). The 30-day survival rate after ligation was 99% (91/92). Beyond 30 days post-ligation, three infants died from other causes that were not directly related to surgery.

Patent ductus arteriosus ligation in premature infants is associated with low mortality and complication rates; however, there is a high incidence of neonatal morbidity. Surgical capacity for patent ductus arteriosus ligation needs to be carefully planned nationally as the duration of “waiting time” and transport to another surgical centre could adversely affect outcomes in this high-risk population.

Delay and impairment of motor development is reported in patients with congenital heart disease. This pilot study addressed the feasibility and effect of a low-dose motor training programme of 60 min once per week on motor ability in preschool children with congenital heart disease.

In all, 14 children – including four girls, in the age group of 4–6 years – with various types of congenital heart disease performed the motor developmental test MOT 4–6 before and after 3 months of a playful exercise programme of 60 min once a week.

At baseline, the motor quotient ranged from normal to slightly impaired (median 92.0; Quartile 1: 83.75; Quartile 3: 101.25). After intervention, motor quotient did not change significantly for the entire group (95.0 (88.0, 102.5); p = 0.141). However, in the subgroup of nine children with retarded motor development at baseline (motor quotient lower 100), seven children had an improved motor quotient after 3 months of intervention. In this subgroup, motor quotient increased significantly (p = 0.020) by 5%.

Overall, a short intervention programme of 60 min only once a week does not improve motor ability in all children with congenital heart disease. However, those with retarded motor development profit significantly from this low-dose intervention.

Recent evidence in adults suggests that left ventricular mass measured as left ventricular mass/height1.7 predicts cardiovascular morbidity and mortality better than the two widely used indices, left ventricular mass/body surface area and left ventricular mass/height2.7. Standards of left ventricular mass/height1.7 have not been reported in children, for whom, owing to lack of significant cardiovascular morbidity and mortality, body mass index has traditionally been used as a potential cardiovascular risk factor.

In this retrospective study, 692 clinically normal children aged 1 day to 18 years underwent detailed echocardiographic assessment to assess whether any of the left ventricular mass indices – left ventricular mass/height1.7, left ventricular mass/body surface area, and left ventricular mass/height2.7 – are associated with obesity as measured by body mass index. Correlations, t-tests, and linear regressions were used for statistical testing.

Left ventricular mass/height1.7 was better correlated (R2 = 0.36) with body mass index than left ventricular mass/body surface area (R2 = 0.179) and left ventricular mass/height2.7 (R2 = 0.006), although all three dependent variables show a significant correlation (p < 0.035). In addition, a higher percentage of obese patients were noted to have elevated left ventricular mass as measured by left ventricular mass/height1.7 than by the other two methods.

Left ventricular mass/height1.7 is a reliable indicator of obesity-associated left ventricular hypertrophy. Left ventricular mass/height1.7 can be used conveniently during transitions from youth to adults for long-term follow-up. These findings support the importance of including left ventricular mass/height1.7 in future studies of cardiovascular risks and preventive strategies in children and adolescents.