Anorexia nervosa is an eating disorder, which is associated with many different medical complications as a result of the weight loss and malnutrition that characterise this illness. It has the highest mortality rate of any psychiatric disorder. A large portion of deaths are attributable to the cardiac abnormalities that ensue as a result of the malnutrition associated with anorexia nervosa. In this review, the cardiac complications of anorexia nervosa will be discussed.

A comprehensive literature review on cardiac changes in anorexia nervosa was carried out.

There are structural, functional, and rhythm-type changes that occur in patients with anorexia nervosa. These become progressively significant as ongoing weight loss occurs.

Cardiac changes are inherent to anorexia nervosa and they become more life-threatening and serious as the anorexia nervosa becomes increasingly severe. Weight restoration and attention to these cardiac changes are crucial for a successful treatment outcome.

The peri-operative mortality of the arterial switch operation in neonates with transposition of the great arteries is considerably low; however, long-term outcomes of translocated coronary arteries still remain one of the most crucial issues.

A total of 110 neonates with transposition of the great arteries after arterial switch operation were evaluated; three (2.7%) late deaths occurred. The remaining 107 patients except for one underwent follow-up angiography. Angiography showed coronary artery stenosis in nine (8.4%), with right coronary artery lesions in two and left main trunk lesions in seven. In two patients, right coronary artery stenosis regressed during follow-up. In left main trunk lesions, the severity of stenosis improved in four, did not change in one, and progressed to total occlusion in two patients. In children with coronary artery stenosis, myocardial scintigraphy showed perfusion defects in five out of six (83%) with left main trunk with ⩾75% stenosis and in four out of four with left main trunk stenosis ⩾90%. In contrast, patients whose coronary artery stenosis disappeared during follow-up had no perfusion defects on scintigraphy.

Regression of ostial stenosis of the transplanted coronary artery on angiogram was observed. The stenosis regressed over time in six patients; two coronary arteries with 99% stenosis and delayed angiographic enhancement of the distal coronary artery resulted in total occlusion within 1 year after the arterial switch operation. Combination of angiography and myocardial scintigraphy could be useful to differentiate deceptive stenosis from progressive stenosis.

This study aimed to describe the frequency of QTc prolongation in children with restrictive eating disorders early in the course of disease admitted for inpatient therapy, to determine the frequency of associated ventricular arrhythmia, and to evaluate the relationship between QTc interval and concomitant electrolyte abnormalities and rate of weight loss.

This was a retrospective cohort study of patients aged 11–25 years with early restrictive eating disorders.

In all, 82 patients met the inclusion criteria (84% female). In total, 9.8% had prolonged QTc interval during hospitalisation. Patients with prolonged QTc had significantly higher resting heart rates (p=0.006), but there was no association with hypokalaemia (p=0.31), hypomagnesaemia (p=0.43), hypophosphataemia (p=1), or rate of weight loss (p=1).

Mild QTc prolongation in patients with restrictive eating disorders is not related to electrolyte abnormalities or rate of weight loss in this population, suggesting that investigation about other potential risk factors of prolonged QTc interval may be warranted.

To describe the motor proficiency of 5-year-old children who underwent early infant cardiac surgery and had atypical infant gross motor development. To identify risk factors for motor dysfunction at 5 years of age.

A total of 33 children (80.5% participation rate) were re-assessed by a physiotherapist blinded to the diagnosis and previous clinical course, using standardised motor assessment tools.

Motor proficiency was categorised as below average or well below average in 41% of the study patients. Approximately 30% of the cohort had balance deficits. Motor abilities at 4 months and 2 years of age were associated with motor proficiency at age 5; however, atypical motor development in infancy was not predictive of below-average or well below-average scores at age 5. Risk factors associated with motor ability at age 5 included respiratory support and intensive care length of stay in the 1st year of life, asymmetrical crawling in infancy, and cyanotic CHD at age 5.

Despite differences from other reported studies in terms of cohort diagnoses and age at surgery, the rate of motor dysfunction was similar, with rates much higher than expected in typical children. Further assessment is needed in later childhood to determine the significance of these findings.

Waist circumference to height ratio, a measure of central obesity, is a better predictor of cardiovascular risk than body mass index in the paediatric population. Increased left ventricular mass secondary to obesity and hypertension increases the risk for death and cardiovascular disease in adults. Similar data on left ventricular mass are lacking among young patients with central obesity, as defined by waist circumference/height. The present study evaluates left ventricular mass in young patients with central obesity as defined by waist circumference/height.

A total of 156 patients, 2- to 20-years old, without evidence of structural heart disease were studied. As the left ventricular mass is related to age and gender, 52 patients with central obesity – waist circumference/height ⩾0.55 – were randomly matched for age and gender with 52 patients at risk for central obesity – waist circumference/height ⩾0.5 to <0.55 – and 52 patients with no central obesity – waist circumference/height <0.5. The left ventricular mass parameters measured by echocardiography were compared across the three groups.

Patients with central obesity had significantly (p<0.05) increased Z scores for left ventricular mass, left ventricular mass/height2.7, and left ventricular mass/height1.7 compared with patients at risk for central obesity and patients with no central obesity. Z scores for left ventricular mass were higher among patients at risk for central obesity compared with patients with no central obesity.

Left ventricular mass data of the present study support maintaining an ideal waist circumference/height <0.5. Aggressive efforts to identify and manage patients with central obesity are warranted.

Although transcatheter closure of perimembranous ventricular septal defect is emerging as an accepted, viable alternative, conduction disturbances still remain a major concern. Although steroid treatment has shown encouraging results with complete recovery, efficacy of prophylactic use of steroids is still speculative. We aim to study the mid-term outcome of perimembranous ventricular septal defect closure in children who received prophylactic oral steroids.

A prospective study was designed and antegrade device closure was attempted in eligible children who met the following inclusion criteria: age 3–18 years and weight >10 kg, defect diameter ⩽12 mm, and symptomatic, haemodynamic changes or history of infective endocarditis. Prophylactic steroid protocol consisted of 2 weeks oral prednisolone (1 mg/kg/day) initiated immediately after the procedure, and in the event of bradyarrhythmia it was escalated to 2 mg/kg. Patients were regularly followed-up at 1, 6, and 12 months and then annually. Patients with post-procedure heart block underwent Holter monitoring after a minimum of 1 year interval.

Between May, 2007 and August, 2012, successful device closure was accomplished in 290/297 patients. Mean age and weight were 9±3.12 years and 21±8.27 kg, respectively. The defect measured 5±1.38 mm on echocardiography. Mean fluoroscopy time was 12.98±8.64 minutes. Eight patients with major complications included one each with device embolisation, haemolysis, severe aortic regurgitation, and five with bradyarrhythmias, including complete atrioventricular block in three, Mobitz II in one, and bifascicular block in one. Patients with complete atrioventricular block responded to high-dose steroid and temporary pacemaker. Minor complications included post-procedure heart block (n=22) and blood loss (n=2). At 18.23±13.15 months follow-up, 8/27 (five major, 22 minor) with arrhythmia had persistent post-procedure heart block of no clinical consequences.

In our patient population, transcatheter device closure of the perimembranous ventricular septal defect with prophylactic oral steroid resulted in excellent closure rate and acceptably low incidence of conduction disturbances at mid-term follow-up.

Hypoplastic left heart syndrome is the most expensive birth defect managed in the United States, with a 5-year survival rate below 70%. Increasing evidence suggests that hospital volumes are inversely associated with mortality for infants with single ventricles undergoing stage 1 surgical palliation. Our aim was to examine the relative effects of surgeon and institutional volumes on outcomes and resource utilisation for these children.

A retrospective study was conducted using the Pediatric Health Information System database to examine the effects of the number of procedures performed per surgeon and per centre on mortality, costs, and post-operative length of stay for infants undergoing Risk Adjustment for Congenital Heart Surgery risk category six operations at tertiary-care paediatric hospitals, from 1 January, 2004 to 31 December, 2013. Multivariable modelling was used, adjusting for patient and institutional characteristics. Gaussian kernel densities were constructed to show the relative distributions of the effects of individual institutions and surgeons, before and after adjusting for the number of cases performed.

A total of 2880 infants from 35 institutions met the inclusion criteria. Mortality was 15.0%. Median post-operative length of stay was 24 days (IQR 14–41). Median standardized inpatient hospital costs were $156,000 (IQR $108,000–$248,000) in 2013 dollars. In the multivariable analyses, higher institutional volume was inversely associated with mortality (p=0.001), post-operative length of stay (p=0.004), and costs (p=0.001). Surgeon volume was associated with none of the measured outcomes. Neither institutional nor surgeon volumes explained much of the wide variation in outcomes and resource utilization observed between institutions and between surgeons.

Increased institutional – but not surgeon – volumes are associated with reduced mortality, post-operative length of stay, and costs for infants undergoing stage 1 palliation.

The present study aims to evaluate the epidemiological characteristics of paediatric pulmonary hypertension within the entire Turkish population over a period of 5 years using the registry of the National Health Insurance System.

All individuals aged <18 years who were admitted to a Turkish hospital for the first time between 2009 and 2013 with a discharge diagnosis of idiopathic pulmonary hypertension and secondary pulmonary hypertension were identified.

The overall annual incidence of idiopathic pulmonary hypertension during childhood was 11.7 cases/million, whereas the overall annual incidence of secondary pulmonary hypertension during childhood was 9.5 cases/million. There was a gradual and significant increase in the annual incidence of idiopathic pulmonary hypertension and that of secondary pulmonary hypertension during the 5-year study period (p=0.001 for both). In the years 2012 and 2013, idiopathic pulmonary hypertension was significantly more frequent in children aged <2 years when compared with children aged above 2 years (p=0.002 for both). The male to female ratio was 1.2:1 for idiopathic pulmonary hypertension, whereas the female to male ratio was 1.1:1 for secondary pulmonary hypertension during childhood.

The incidence of paediatric pulmonary hypertension in Turkey is higher than those reported for the Western populations. Moreover, no female dominance could be observed. These discrepancies may be attributed to the differences in the study design, study cohort, timing of the study, and the definitions adopted for pulmonary hypertension classification.

At present, the exclusion for percutaneous pulmonary valve implantation for free pulmonary regurgitation after tetralogy of Fallot repair includes an unfavourably large right ventricular outflow tract.

To report feasibility and early experience with a recently developed transcatheter heart valve, Venus P-valveTM, implanted in six patients with severe pulmonary regurgitation with large right ventricular outflow tracts.

There were two female patients and four male patients. The median age of the patients was 18.5 years, and the mean body weight was 53.8 kg. All the patients were in NYHA class II and had severe pulmonary regurgitation after previous transannular patch repair of tetralogy of Fallot. The median time after the last surgical operation was 13.5 years.

The Venus P-valveTM was successfully implanted in all the patients with implanted valve diameters ranging from 24 to 32 mm. The mean fluoroscopy time was 29.8 minutes. None of the patients had significant outflow tract gradient or pulmonary regurgitation immediately after valve implantation. Only one patient had unexpected mild proximal valve migration to the right ventricular body during withdrawal of the delivery system. It caused mild paravalvar leak and significant tricuspid regurgitation. At 6 months follow-up, the median of right ventricular end-diastolic volume indices decreased from 146 to 108 ml/m2 (p-value=0.046). The Doppler systolic peak gradient across the valve ranged from 4 to 40 mmHg, and there was no evidence of stent fracture on fluoroscopy or structural valve failure.

The Venus P-valveTM can be implanted successfully and effectively in patients with severe pulmonary regurgitation and a large right ventricular outflow tract. The early results with this valve are encouraging.

Adults with high premature ventricular contraction burden can develop left ventricular dilation, dysfunction, and strain, consistent with a cardiomyopathy, which is reversible with radiofrequency ablation of the premature ventricular contractions. Evidence in children with similar ectopy burden is limited. We performed a single-centre retrospective review to examine the prevalence of premature ventricular contraction-induced cardiomyopathy, natural history of ventricular ectopy, and progression to ventricular tachycardia in children with frequent premature ventricular contractions.

Children aged between 6 months and 18 years, with premature ventricular contractions comprising at least 20% of rhythm on 24-hour Holter monitor, were included in our study. Those with significant structural heart disease, ventricular tachycardia greater than 1% of rhythm at the time of premature ventricular contraction diagnosis, or family history of cardiomyopathy – except tachycardia-induced – were excluded. Cardiomyopathy was defined by echocardiographic assessment.

A total of 36 children met the study criteria; seven patients (19.4%, 95% CI 6.2–32.6%) met the criteria for cardiomyopathy, mostly at initial presentation. Ectopy decreased to <10% of beats without intervention in 16.7% (95% CI 4.3–29.1%) of the patients. No patient progressed to having ventricular tachycardia as more than 1% of beats on follow-up Holter. Radiofrequency ablation was performed in three patients without cardiomyopathy.

Our study demonstrates a higher prevalence of cardiomyopathy among children with high premature ventricular contraction burden than that previously shown. Ectopy tended to persist throughout follow-up. These trends suggest the need for a multi-centre study on frequent premature ventricular contractions in children. In the interim, regular follow-up with imaging to evaluate for cardiomyopathy is warranted.

Aneurysmal dilation of the right ventricular outflow tract complicates assessment of right ventricular function in patients with repaired tetralogy of Fallot. Tricuspid annular plane systolic excursion is commonly used to estimate ejection fraction. We hypothesised that tricuspid annular plane systolic excursion measured by cardiac MRI approximates global and segmental right ventricular function, specifically right ventricular sinus ejection fraction, in children with repaired tetralogy of Fallot.

Tricuspid annular plane systolic excursion was measured retrospectively on cardiac MRIs in 54 patients with repaired tetralogy of Fallot. Values were compared with right ventricular global, sinus, and infundibular ejection fractions. Tricuspid annular plane systolic excursion was indexed to body surface area, converted into a fractional value, and converted into published paediatric Z-scores.

Tricuspid annular plane systolic excursion measurements had good agreement between observers. Right ventricular ejection fraction did not correlate with the absolute or indexed tricuspid annular plane systolic excursion and correlated weakly with fractional tricuspid annular plane systolic excursion (r=0.41 and p=0.002). Segmental right ventricular function did not appreciably improve correlation with any of the tricuspid annular plane systolic excursion measures. Paediatric Z-scores were unable to differentiate patients with normal and abnormal right ventricular function.

Tricuspid annular plane systolic excursion measured by cardiac MRI correlates poorly with global and segmental right ventricular ejection fraction in children with repaired tetralogy of Fallot. Tricuspid annular plane systolic excursion is an unreliable approximation of right ventricular function in this patient population.

Heterotaxy is a unique clinical entity in which lateralisation of the thoraco-abdominal organs is abnormal, typically with isomerism of the bronchial tree and atrial appendages. This study was carried out to determine whether routine clinical imaging such as chest radiographs, angiographic images, and CT/MRI can determine bronchial isomerism, and how sidedness of bronchial isomerism correlates with overall features anticipated in hearts with isomeric atrial appendages.

We identified 73 patients with heterotaxy, in whom imaging clearly demonstrated the bronchial tree, seen at our institution since 1998. We calculated bronchial angles and lengths using all the available imaging modalities to determine the presence and sidedness of bronchial isomerism. This was then compared with the anticipated presence of isomeric atrial appendages based on the overall clinical findings, as the appendages themselves had not specifically been imaged.

The ratio of bronchial lengths revealed bronchial isomerism in all patients, with bronchial angles permitting distinction of right as opposed to left isomerism. We noted discordances between the identified bronchial isomerism and the presumed arrangement of the atrial appendages in nearly 20% of the patients in our cohort.

Routine clinical imaging with chest radiographs, angiographic imaging, and CT/MRI can determine the presence of bronchial isomerism in patients with so-called heterotaxy. Right as opposed to left isomerism can be distinguished based on bronchial angles. The finding of bronchial isomerism correlates well, but not totally, with the presumed isomerism of the atrial appendages as predicted from the identified intra-cardiac morphology.

This study describes the incidence and course of children with small coronary artery fistulae over a period of 6 years who presented at a paediatric tertiary-care centre.

Age at diagnosis, mode of presentation, location (origin and drainage), and association with a cardiac defect were documented and analysed. All patients obtained an electrocardiogram, and older patients were further evaluated with an exercise treadmill test.

A total of 31 patients were diagnosed with coronary artery fistula via transthoracic echocardiogram and comprised 0.43% of our entire patient group. Mean age was 6.14 years (standard deviation 5.4); 16 patients (52%) had associated cardiac defects. In the remaining 15 patients, the coronary artery fistula was discovered incidentally during diagnostic work-up for heart murmur or chest pain. Among all, 26 patients (84%) had left-sided and five patients (16%) had right-sided coronary artery fistulae. All right coronary artery fistula patients had associated cardiac defects; this was true for 42% of the patients with left coronary artery fistulae. None of the patients required any intervention due to the fistula, and spontaneous closure occurred in 12 patients (39%).

Small coronary artery fistulae in children are frequently an incidental finding, and many will close spontaneously. Our data are supportive of a conservative, observant approach in asymptomatic patients with small coronary artery fistula in the paediatric population.

This is the first autopsy study in the United Kingdom to analyse the demographic and pathological characteristics of atheroma associated with sudden cardiac death in young people.

An observational retrospective study of referred cases of sudden cardiac death in the young (⩽35 years) associated with premature atheroma was carried out.

In total, 46 cases were referred, with a median age of 30 years (27, 32); 72% of the referred cases were male, with a mean body mass index of 30 kg/m2. Circumstances of death were as follows: at rest (n=21), exertion (n=7), in bed (n=7), related to drugs/alcohol (n=4), and unknown (n=7). A previous cardiac history was provided in 10 cases. A history of class A/B drug use was found in eight cases. There was macroscopic evidence of infarction in 10 cases (acute, n=3 and chronic, n=7). Microscopically, 10 cases demonstrated contraction band necrosis, 11 acute infarction, and 11 chronic infarction. Single-vessel disease predominated (n=28). The left anterior descending coronary artery was involved in 39/46 cases. Thrombosis was seen in 16 cases, mainly due to erosion; one case showed dual pathology with arrhythmogenic right ventricular cardiomyopathy and another showed left ventricular hypertrophy.

This study highlights premature atheroma mainly in a single vessel in young people with or without evidence of ischaemic damage in the ventricle. Dual pathology may occur. The role of arrhythmias and channelopathies are important considerations. Premature atheroma should prompt investigation for dyslipidaemias in family members.

The global prevalence of obesity in school-age children and adolescents has increased in recent decades. Obesity modifies some aspects of the cardiovascular system in order to preserve the body homoeostasis. Echocardiography to study ventricular function plays an important role in the evaluation of pathological re-modelling associated with left ventricular dysfunction. The aim of this study was to evaluate the left ventricle function and structure with conventional echocardiography and to analyse the longitudinal deformity of the left ventricle using myocardial-tracking signals in a group of severely obese adolescents.

We carried out a descriptive cross-sectional study. We describe the evaluation of the left ventricle using conventional bi-dimensional echocardiography and the myocardial-tracking signals in severely obese adolescents. There were 34 severely obese adolescents included in our study; 52% had a left ventricular ejection fraction<55%, the left ventricular end-diastolic diameter was increased in 70.5% of patients, and 32.3% had an increase in left ventricular mass. On average, 78.9% had abnormal values of left ventricle longitudinal deformations. The number of segments affected per patient was, on average, 5.8, with the anterior apical segment being the most commonly affected. There was a decrease in global longitudinal deformity in 79.4% of the cases.

More than half of this group of asymptomatic severely obese adolescents showed abnormalities in left ventricular structure and function evaluated using traditional echocardiographic methods, but 100% of the cases showed abnormalities in longitudinal deformation in at least one of the 17 left ventricle segments evaluated using myocardial-tracking signals.

Most long QT syndrome patients are associated with genetic mutations. We aimed to investigate the clinical and biochemical characteristics and look for genotype-based preventive implications in Chinese long QT syndrome patients.

We identified two missense mutations of the KCNQ1 gene in two independent Chinese families, including a previously reported mutation R380S in the C-terminus and a novel mutation W305L in the P-loop domain of the Kv7.1 channel, respectively. The proband with R380S was an 11-year-old girl who suffered a prolonged corrected QT interval of 660 ms, recurrent syncope, and sudden cardiac death, whose father was an asymptomatic carrier. The mutation W305L was detected in a 36-year-old woman with long QT syndrome and her immediate family members including the proband’s younger sister with an unexplained syncope, her son, and her elder daughter without symptoms. Metoprolol appeared to be effective in preventing arrhythmias and syncope in long QT syndrome patients with mutation W305L. Both R380S and W305L mutations led to “loss-of-function” of the Kv7.1 channel accounting for the clinical phenotypes.

We first show two missense KCNQ1 mutations – R380S and W305L – in Chinese long QT syndrome patients, resulting in the loss of protein function. Mutation W305L in the P-loop domain of the Kv7.1 may derive a pronounced benefit from β-blocker therapy in symptomatic long QT syndrome patients, whereas mutation R380S located in the C-terminus may be associated with a high risk of sudden cardiac death.