The objectives of this study were to compare the quality-of-life scores of Malaysian children with CHD and their healthy siblings, to determine the level of agreement between proxy-reports and child self-reports, and to examine variables that have an impact on quality of life in those with CHD.

Parental-proxy scores of the Pediatric Quality of Life Inventory 4.0 core scales were obtained for 179 children with CHD and 172 siblings. Intra-class coefficients were derived to determine the levels of proxy–child agreement in 66 children aged 8–18 years. Multiple regression analysis was used to determine factors that impacted Pediatric Quality of Life Inventory scores.

Proxy scores were lower in children with CHD than siblings for all scales except physical health. Maximum differences were noted in children aged 5–7 years, whereas there were no significant differences in the 2–4 and 13–18 years age groups. Good levels of proxy–child agreement were found in children aged 8–12 years for total, psychosocial health, social, and school functioning scales (correlation coefficients 0.7–0.8). In children aged 13–18 years, the level of agreement was poor to fair for emotional and social functioning. The need for future surgery and severity of symptoms were associated with lower scores.

Differences in proxy perception of quality of life appear to be age related. The level of proxy–child agreement was higher compared with other reported studies, with lower levels of agreement in teenagers. Facilitating access to surgery and optimising control of symptoms may improve quality of life in this group of children.

Establishing stable and adequate flow of blood to the lungs using a systemic-to-pulmonary arterial shunt in infants with low birth weight may involve significant morbidity and mortality. We reviewed our experience with this procedure in patients weighing less than 3 kilograms.

Between June, 2002, and June, 2007, we placed systemic-to-pulmonary arterial shunts in 32 infants weighing less than 3 kilograms, the range being 1.8 to 2.86 kg, with a median of 2.5 kg. The median age at placement of the shunt was 8 days, with a range from 2 to 70 days. In 17 patients (53%), the anatomic defects had produced a functionally univentricular heart, while 15 (47%) had defects which permitted staging to biventricular repair. Patients staged to univentricular palliation were much more likely to have a circulation dependent on the arterial duct as compared with those staged to biventricular palliation (p < 0.001). The latter patients tended to have smaller pulmonary arteries, significantly the left pulmonary artery, which has a median diameter of 3.6 versus 2.0 mm, p = 0.01. In all patients a saphenous venous homograft was used as the conduit, its size ranging in diameter from 2.5 to 4 mm, with a median of 3.0 mm.

The overall hospital mortality rate for the entire cohort was 6.25%, with 2 patients dying. There was no significant difference between the two groups with regard to length of stay in intensive care or hospital. Follow-up has ranged from 3 months to 4.7 years, with a mean of 2.1 years). Of those with functionally univentricular hearts, 3 have subsequently died, along with 1 patient having a biventricular circulation (p = 0.3). All deaths occurred before takedown of the shunt. A trend toward longer survival was noted in those with biventricular as compared to functionally univentricular circulations (p = 0.06).

Systemic-to-pulmonary arterial shunts can be constructed safely in infants with biventricular physiology born with low weight. Those having functionally univentricular circulations carry an increased rate of mortality for the period of shunting. Using the saphenous venous homograft permits use of smaller grafts, which does not significantly increase the risk for thrombosis or survival when compared to previous studies using polytetrafluoroethylene grafts.

The aim of the study was to evaluate the early myocardial dysfunction detected by strain echocardiography in children with multisystem inflammatory syndrome related to SARS-CoV-2 infection.

This cross-sectional study was conducted with 47 patients diagnosed with MIS-C and 32 healthy age- and gender-matched children. All patients underwent two-dimensional, colour, pulsed, and tissue Doppler, and 2D speckle tracking echocardiography examination at admission, 2 weeks, and 2 months after discharge. The MIS-C patient group was compared with the control group. Echocardiographic changes in MIS-C patients during follow-up were evaluated.

Of 47 patients, 30 (63.8%) were male and 17 (36.2%) were female. The mean age at diagnosis was 9.1 ± 4.3 (1.25–17) years. At admission, 25 patients had abnormal findings on conventional echocardiography. Among them, eight patients had left ventricular systolic dysfunction. Ejection fraction and fractional shortening were significantly lower in the patient group at admission compared to controls (p = 0.013, p = 0.010, respectively). While the ejection fraction was <55% in eight patients, and global longitudinal strain was lower than −2SD in 29 patients at admission. Global longitudinal strain z-score <−2SD persisted in 13 patients at 2-month follow-up. Ejection fraction increased above 55% in 3.42 ± 0.53 days in 7 of 8 patients with left ventricular systolic dysfunction, ejection fraction was 51% at discharge in one patient, and left ventricular systolic dysfunction persisted at the 6-month of follow-up.

These results confirmed that speckle tracking echocardiography is more likely to detect subclinical myocardial damage compared to conventional echocardiography. In addition, it is a valuable method for follow-up in this patient group.

Left ventricular rotation is physiologically affected by acute changes in preload. We investigated the acute effect of preload changes in chronically underloaded and overloaded left ventricles in children with shunt lesions.

A total of 15 patients with atrial septal defects (Group A: 7.4 ± 4.7 years, 11 females) and 14 patients with patent arterial ducts (Group B: 2.7 ± 3.1 years, 10 females) were investigated using 2D speckle-tracking echocardiography before and after interventional catheterisation. The rotational parameters of the patient group were compared with those of 29 matched healthy children (Group C).

Maximal torsion (A: 2.45 ± 0.9°/cm versus C: 1.8 ± 0.8°/cm, p < 0.05), apical peak systolic rotation (A: 12.6 ± 5.7° versus C: 8.7 ± 3.5°, p < 0.05), and the peak diastolic torsion rate (A: −147 ± 48°/second versus C: −110 ± 31°/second, p < 0.05) were elevated in Group A and dropped immediately to normal values after intervention (maximal torsion 1.5 ± 1.1°/cm, p < 0.05, apical peak systolic rotation 7.2 ± 4.1°, p < 0.05, and peak diastolic torsion rate −106 ± 35°/second, p < 0.05). Patients in Group B had decreased maximal torsion (B: 1.8 ± 1.1°/cm versus C: 3.8 ± 1.4°/cm, p < 0.05) and apical peak systolic rotation (B: 8.3 ± 6.1° versus C: 13.9 ± 4.3°, p < 0.05). Defect closure was followed by an increase in maximal torsion (B: 2.7 ± 1.4°/cm, p < 0.05) and the peak diastolic torsion rate (B: −133 ± 66°/second versus −176 ± 84°/second, p < 0.05).

Patients with chronically underloaded left ventricles compensate with an enhanced apical peak systolic rotation, maximal torsion, and quicker diastolic untwisting to facilitate diastolic filling. In patients with left ventricular dilatation by volume overload, the peak systolic apical rotation and the maximal torsion are decreased. After normalisation of the preload, they immediately return to normal and diastolic untwisting rebounds. These mechanisms are important for understanding the remodelling processes.

To describe the cardiac lesions seen in children with trisomy 21, the outcome of these children and rates of access to corrective surgery at the Bustamante Hospital for Children.

A 10-year retrospective review of the records of trisomy 21 patients with cardiac lesions referred to the Bustamante Hospital for Children was conducted.

A total of 76 patients were enrolled in the study, 30 (40%) males and 46 (60%) females; among these 110 cardiac lesions were detected. A total of 20 (26%) patients died, 48 (63%) survived, and for 8 (11%) the status was unknown. The most common lesion was the atrioventricular septal defect, which accounted for 41 (37%) of all the lesions and occurred as a single defect in 24 (53%) patients. At presentation, 33 (46%) patients had one or more medical complication; 30 (91%) had cardiac failure, 10 (30%) had pneumonia and 5 (15%) had evidence of systemic pulmonary arterial pressures. Cardiac catheterisation was recommended for 43 (56%) patients but only 10 (23%) had the procedure done. Surgery was recommended for 60 (79%) patients; of these 6 (10%) patients had the procedure done. The median time of survival was 88 months (7.3 years). The age of presentation was not found to significantly affect outcome.

Trisomy 21 patients with cardiac lesions have high morbidity and mortality. This morbidity and mortality could be reduced if surgical intervention was offered routinely.

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.

Outcome analyses in large administrative databases are ideal for rare diseases such as Becker and Duchenne muscular dystrophy. Unfortunately, Becker and Duchenne do not yet have specific International Classification of Disease-9/-10 codes. We hypothesised that an algorithm could accurately identify these patients within administrative data and improve assessment of cardiovascular morbidity.

Hospital discharges (n=13,189) for patients with muscular dystrophy classified by International Classification of Disease-9 code: 359.1 were identified from the Pediatric Health Information System database. An identification algorithm was created and then validated at three institutions. Multi-variable generalised linear mixed-effects models were used to estimate the associations of length of stay, hospitalisation cost, and 14-day readmission with age, encounter severity, and respiratory disease accounting for clustering within the hospital.

The identification algorithm improved identification of patients with Becker and Duchenne from 55% (code 359.1 alone) to 77%. On bi-variate analysis, left ventricular dysfunction and arrhythmia were associated with increased cost of hospitalisation, length of stay, and mortality (p<0.001). After adjustment, Becker and Duchenne patients with left ventricular dysfunction and arrhythmia had increased length of stay with rate ratio 1.4 and 1.2 (p<0.001 and p=0.004) and increased cost of hospitalization with rate ratio 1.4 and 1.4 (both p<0.001).

Our algorithm accurately identifies patients with Becker and Duchenne and can be used for future analysis of administrative data. Our analysis demonstrates the significant effects of cardiovascular disease on length of stay and hospitalisation cost in patients with Becker and Duchenne. Better recognition of the contribution of cardiovascular disease during hospitalisation with earlier more intensive evaluation and therapy may help improve outcomes in this patient population.

Multimedia programmes relating to education and consents may be useful for decreasing anxiety during catheter-based repair in patients with congenitally malformed hearts.

Our study was aimed at evaluating the impact of multimedia protocols for education of a population of consecutive patients with congenitally malformed hearts prior to transcatheter repair.

Between September, 2006, and May, 2008, we enrolled 100 consecutive patients, with a mean age of 45 ± 19 years, of whom 69 were female, for catheter-based repair of their congenitally malformed hearts. In the first 50 patients, we used a written form for informed consent sent to the patients 15 days before the procedure, coupled with a personal interview of 30 minutes. In the subsequent 50 patients, we used multimedia protocol for education, comprising a booklet of 4 pages containing a simple and brief explanation of the intervention, and a digital film of 4 minutes showing the transcatheter procedure with a commentary provided by the referring physician, prior to obtaining the signature for informed consent. We then compared the scores for anxiety, the pre-operative heart rate, the frequency of vaso-vagal episodes, and the need for conscious sedation between the two groups.

Patients who underwent preconditioning using the multimedia programme were significantly less anxious, and had significantly lower heart rates. Vaso-vagal episodes were also significantly less in this group, with no episodes compared to 14% in those providing standard informed consent. Conscious sedation was needed more frequently in those providing standard informed consent.

Our brief study suggests that a comprehensive multimedia programme of preparation increases the tolerability, and decrease the emotional state, of adults about to undergo catheter-based interventions for congenital cardiac disease.