The aim of the study was to evaluate the trends in respiratory syncytial virus-related hospitalisations and associated outcomes in children with haemodynamically significant heart disease in the United States of America.

The Kids’ Inpatient Databases (1997–2012) were used to estimate the incidence of respiratory syncytial virus hospitalisation among children ⩽24 months with or without haemodynamically significant heart disease. Weighted multivariable logistic regression and chi-square tests were used to evaluate the trends over time and factors associated with hospitalisation, comparing eras before and after publication of the 2003 American Academy of Pediatrics palivizumab immunoprophylaxis guidelines. Secondary outcomes included in-hospital mortality, morbidity, length of stay, and cost.

Overall, 549,265 respiratory syncytial virus-related hospitalisations were evaluated, including 2518 (0.5%) in children with haemodynamically significant heart disease. The incidence of respiratory syncytial virus hospitalisation in children with haemodynamically significant heart disease decreased by 36% when comparing pre- and post-palivizumab guideline eras versus an 8% decline in children without haemodynamically significant heart disease (p<0.001). Children with haemodynamically significant heart disease had higher rates of respiratory syncytial virus-associated mortality (4.9 versus 0.1%, p<0.001) and morbidity (31.5 versus 3.5%, p<0.001) and longer hospital length of stay (17.9 versus 3.9 days, p<0.001) compared with children without haemodynamically significant heart disease. The mean cost of respiratory syncytial virus hospitalisation in 2009 was $58,166 (95% CI:$46,017, $70,315).

These data provide stakeholders with a means to evaluate the cost–utility of various immunoprophylaxis strategies.

Chronic paediatric heart disease is often associated with residual symptoms, persisting functional restrictions, and late sequelae for psychosocial development. It is, therefore, increasingly important to evaluate the health-related quality of life of children and adolescents with chronic heart disease. The aim of this study was to determine medical and socio-demographic variables affecting health-related quality of life in school-aged children and adolescents with chronic heart disease.

The Pediatric Cardiac Quality of Life Inventory was administered to 375 children and adolescents and 386 parental caregivers. Medical information was obtained from the charts. The socio-demographic information was provided by the patients and caregivers.

Greater disease severity, low school attendance, current cardiac medication, current parental employment, uncertain or limited prognosis, history of connection to a heart–lung machine, number of nights spent in a hospital, and need for treatment in a paediatric aftercare clinic independently contributed to lower health-related quality of life (self-report: R2=0.41; proxy-report: R2=0.46). High correlations between self-reports and parent-proxy reports indicated concordance regarding the evaluation of a child’s health-related quality of life.

Beyond medical treatment, integration into school is important to increase health-related quality of life in children and adolescents surviving with chronic heart disease. Regular screening of health-related quality of life is recommended to identify patients with special needs.

The pathophysiological entity of a persisting left-sided superior caval vein draining into the roof of the left atrium represents an extreme form of coronary sinus de-roofing. This is an uncommon, but well-documented condition associated with systemic desaturation due to a right-to-left shunt. Depending on the size of the coronary ostium, the defect may also present with right-sided volume loading. We describe two patients, both of whom presented with desaturation, and highlight the important anatomical features underscoring management.

Both patients were managed interventionally with previous assessment of the size of the coronary sinus ostium through cross-sectional imaging. This revealed a restrictive interatrial communication at the right atrial mouth of the coronary sinus in both patients, which permitted an interventional approach, as the residual left-to-right shunt subsequent to closure of the aberrant vessel would be negligible. At intervention, test occlusion of the left superior caval vein allowed assessment of decompressing vessels before successful occlusion using an Amplatzer Vascular Plug.

Persistence of a left superior caval vein draining to the left atrium may be associated with an interatrial communication at the mouth of the unroofed coronary sinus. The ostium of the de-roofed coronary sinus can be atretic, restrictive, normally sized, or enlarged. Careful assessment of the size of this defect is required before treatment. In view of its importance, which has received little attention in the literature to date, we suggest an additional consideration to the classification of unroofed coronary sinus.

Carbon monoxide poisoning may cause myocardial toxicity and cardiac autonomic dysfunction, which may contribute to the development of life-threatening arrhythmias. We investigated the potential association between acute carbon monoxide exposure and cardiac autonomic function measured by heart rate variability.

The present study included 40 children aged 1–17 years who were admitted to the Pediatric Intensive Care Unit with acute carbon monoxide poisoning and 40 healthy age- and sex-matched controls. Carboxyhaemoglobin and cardiac enzymes were measured at admission. Electrocardiography was performed on admission and discharge, and 24-hour Holter electrocardiography was digitally recorded. Heart rate variability was analysed at both time points – 24-hour recordings – and frequency domains – from the first 5 minutes of intensive care unit admission.

Time domain and frequency indices such as high-frequency spectral power and low-frequency spectral power were similar between patient and control groups (p>0.05). The ratio of low-frequency spectral power to high-frequency spectral power was significantly lower in the carbon monoxide poisoning group (p<0.001) and was negatively correlated with carboxyhaemoglobin levels (r=−0.351, p<0.05). The mean heart rate, QT dispersion, corrected QT dispersion, and P dispersion values were higher in the carbon monoxide poisoning group (p<0.05) on admission. The QT dispersion and corrected QT dispersion remained longer in the carbon monoxide poisoning group compared with controls on discharge (p<0.05).

The frequency domain indices, especially the ratio of low-frequency spectral power to high-frequency spectral power, are useful for the evaluation of the cardiac autonomic function. The decreased low-frequency spectral power-to-high-frequency spectral power ratio reflects a balance of the autonomic nervous system, which shifted to parasympathetic components.

An association of heart disease and its treatment with biliary calculi is popularly accepted. We sought determine the prevalence and risk factors of paediatric gallstone disease in the presence of CHD and analyse the treatment options. We evaluated the role of open-heart surgery in the development of gallstones in patients with CHD.

In a 10-year, retrospective, chart review (2005–2014), patients with CHD and cholelithiasis were identified and reviewed.

In all, 19 of 4729 children with CHD had cholelithiasis (0.4%); eight patients underwent cardiac surgery before diagnosis of cholelithiasis (group 1), whereas 11 of them had not (group 2). The prevalence was 0.3% in group 1 and 0.5% in group 2.

In nine asymptomatic patients, gallstones were found incidentally. Children with cholecystolithiasis (n=17) received ursodeoxycholic acid. A resolution of gallstones was found in four cases; two patients underwent biliary surgery, and the others (15/17) were successfully managed non-operatively.

Despite an accumulation of risk factors, prevalence of gallstones is not as high as expected in children with CHD. Open-heart surgery with a heart–lung machine plays a minor role as an aetiological factor. In about half of the cases, cholelithiasis is an incidental finding and patients stay asymptomatic. Prophylactic administration of ursodeoxycholic acid is not indicated in children undergoing open-heart surgery for CHDs. Biliary surgery is reserved for patients with recurrent symptoms or cholestasis.

In children with CHD, cholelithiasis is a minor and manageable co-morbid condition.

It is unclear whether cryoablation or radiofrequency ablation offers better value for treating atrioventricular nodal re-entrant tachycardia in children. We aimed to compare the value of these procedures for treating atrioventricular nodal re-entrant tachycardia in children, with value being outcomes relative to costs.

We performed a retrospective cohort study of all atrioventricular nodal re-entrant tachycardia ablations for children (age⩽18 years) from July, 2009 to June, 2011 at our institution. Costs included fixed costs, miscellaneous hospital costs, and labour costs, and key outcomes were acute and long-term success (6 months) of the ablations. We conducted T-tests and regression analyses to investigate the associations between the ablation procedure type and the cost and success of the ablations.

Of 96 unique cases performed by three paediatric electrophysiologists, 48 were cryoablation only, 42 radiofrequency ablation only, and six were a combination. Acute success was 100% for the cryoablation only and radiofrequency ablation only cases and 83% for the combination cases. There were no notable adverse events. The average total cost was $9636 for cryoablation cases, $9708 for radiofrequency ablation cases, and $10,967 for combination cases (p=0.51 for cryoablation only versus radiofrequency ablation only). The long-term success rate was 79.1% for cryoablation only, 92.8% for radiofrequency ablation only, and 66.7% for the combination (p=0.01 for cryoablation only versus radiofrequency ablation only), but long-term success varied notably by provider.

Cryoablation and radiofrequency ablation offer similar value in the short term for the treatment of atrioventricular nodal re-entrant tachycardia in children. Differences in long-term success may vary substantially by physician, and thus may lead to differences in long-term value.

The improved survival of patients born with CHD has led to increasing interest in research on quality of life of adult survivors. We report the findings of the first study in Malta carried out to investigate quality of life in adults with CHD under follow-up.

A self-reporting questionnaire modelled on the basis of the European Health Interview Survey 2008, including questions on mental health and vitality, was administered to consecutive adult CHD outpatients, aged 16 years and over, between May, 2013 and May, 2014. Foreigners and patients with learning difficulties or cognitive impairment were excluded. Quality-of-life data were compared with that from 371 age- and sex-matched 2008 survey responders – general population cohort. The impact of congenital lesion complexity, hospitalisation in the preceding 12 months, arrhythmias, co-morbidities, and cardiac medication use on quality of life of the CHD cohort was also investigated.

There were a total of 120 patient responders (63 males; mean age 30.53, SD 12.77 years). Overall, there were no significant differences in mental health and vitality between patient and general population cohorts, although older patients had better mental health scores compared with age-matched controls. Within the adult CHD cohort, hospitalisation in the preceding 12 months was the only factor associated with a poorer quality of life.

Overall, CHD has no negative impact on mental health and vitality in Maltese adult patients under follow-up. Patients needing frequent hospitalisations might warrant closer attention by clinical psychologists.

It is still thought by some that a common wall is to be found in the normal heart between the attachments of the caval and pulmonary veins, with absence of this wall underscoring the presence of sinus venosus defects. Recent findings using episcopic microscopy in developing mice have shown the deficiencies of this notion. Understanding that the superior rim of the oval fossa is a fold, rather than a true septum, which can be distorted in the presence of partially anomalous pulmonary venous drainage, has provided an alternative explanation for the morphogenesis of sinus venosus defects.

We reviewed our experience with patients suspected of having a sinus venosus defect from August, 2011, through October, 2015, analysing the findings in light of the current hypotheses used to explain the development of the defects, along with correlations made by inspection of autopsy specimens.

We evaluated findings from 16 patients, with a mean age of 7.7 years, ranging from 2.7 to 15 years. Of the group, 13 were ultimately diagnosed with a superior sinus venosus defect, two with an inferior defect, and one with isolated anomalous pulmonary venous connection in the absence of an interatrial communication. Initially, two patients were thought to have oval fossa defects, one from each subtype, but were correctly diagnosed following cardiac magnetic resonance interrogation. Anomalous pulmonary venous connections were present in all cases.

Appreciation of the changes occurring during normal cardiac development helps in understanding the anatomical substrate underscoring the spectrum of sinus venosus defects. The lesions are veno-venous connections due to partially anomalous pulmonary venous connections, producing interatrial communications outside the confines of the interatrial septum.

In patients with large patent arterial ducts and severe pulmonary hypertension, the natural history of progression of pulmonary hypertension is very variable. Whether to close or not to close is often a difficult decision, as there are no established haemodynamic parameters predicting reversibility.

The objectives of this study were to evaluate the results of device closure of large patent arterial ducts with severe pulmonary hypertension after 2 years of age and to determine haemodynamic variables associated with its regression during long-term follow-up.

A total of 45 patients, with median age of 10 (2–27) years, with large patent arterial ducts and severe pulmonary hypertension, were considered. Haemodynamic variables were assessed in air, oxygen, and after occlusion. The follow-up was performed to assess regression of pulmonary hypertension.

Device closure was successful in 43 (96%) patients. Pulmonary artery systolic and mean pressures decreased from 79 to 67 mmHg and from 59 to 50 mmHg, respectively (p<0.001). At a median follow-up of 80 (41–151) months, severe pulmonary hypertension persisted in four (9.7%) patients. Multivariate analysis showed pulmonary vascular resistance index ⩽6 WU m2 and pulmonary artery systolic and mean pressures ⩽75 and ⩽55 mmHg (all in oxygen), having 97.8% predictive value for regression of pulmonary hypertension (p<0.001) in the long term. In 24 patients with catheterisation-based criteria, regression of pulmonary hypertension was associated with pulmonary vascular resistance index <8 WU m2 (p=0.001) and its fall of >25% (both in oxygen) (p=0.007).

Device closure of large patent arterial ducts with severe pulmonary hypertension is safe and effective. Pulmonary vascular resistance index and systolic and mean pulmonary artery pressures in oxygen are the key prognostic variables predicting regression of pulmonary hypertension.