Interstage mortality causes are often unknown in infants with shunt-dependent univentricular defects. For 2 years, screening catheterisation was encouraged before neonatal discharge to determine if routine evaluation improved interstage outcomes.

Retrospective single-centre review of home monitoring programme from December, 2010 to June, 2012. Composite scores were created for physical examination/echocardiography risk factors; catheterisation risk factors; and interstage adverse events. Composite scores were compared between usual care and screening catheterisation groups. The ability of each risk factor composite to predict interstage adverse events, individually and in combination, was assessed with sensitivity, specificity, and receiver operating characteristic curves.

There were 27 usual care and 32 screening catheterisation patients. There were no significant differences between groups except rates of catheterisation before discharge (29.6 versus 100%, p < 0.001). Usual care patients who underwent catheterisation for clinical indications had higher intervention rates (37.5 versus 3.1%, p = 0.004). Physical examination/echocardiography risk factor frequency was similar, but usual care patients with catheterisation had a higher catheterisation risk factor frequency. Interstage adverse event frequency was similar (48.2 versus 53.1%, p = 0.7). For interstage adverse event prediction, sensitivity for the physical examination/echocardiography, catheterisation, and either risk factor composites was 53.3, 72, and 80%, respectively; specificity was 59, 60, and 48%. The area under the receiver operating characteristic curve was 0.56, 0.66, and 0.64.

Screening catheterisation evaluation offered slightly increased sensitivity and specificity, but no difference in interstage adverse event frequency. Given this small advantage versus known risks, screening catheterisations are no longer encouraged.

Vitamin B12 deficiency induces hyper-hyperhomocysteinemia by inhibiting intracellular methionine re-methylation. Hyper-hyperhomocysteinemia increases the risk of atherosclerosis. Asymmetric dimethylarginine is an endogenous inhibitor of nitric oxide synthase and its level elevates in cardiovascular diseases. In this study, we aimed to examine the relationship between asymmetric dimethylarginine and arterial stiffness and atherosclerosis in adolescents with vitamin B12 deficiency.

A total of 88 adolescents with age ranging between 11 and 17 years of age were enrolled for this study. Among them, 50 patients had vitamin B12 deficiency <130 pg/ml and 38 healthy controls had B12 >200 pg/ml. In all cases, the levels of asymmetric dimethylarginine were measured with high performance liquid chromatography method. The carotid artery intima media thickness and left ventricular mass index were measured using echocardiography. All these measurements of the study groups were compared.

Both plasma levels of asymmetric dimethylarginine and carotid artery intima media thickness were significantly higher in the vitamin B12 deficiency group than in the control group. Correlation analysis showed significant negative correlation of vitamin B12 with homocysteine, asymmetric dimethylarginine, and carotid artery intima media thickness (p<0.05).

Our results suggest that endothelial dysfunction starts in the early stage of adolescent vitamin B12 deficiency, and vitamin B12-deficient adolescents have increased circulating asymmetric dimethylarginine, showing that endothelial dysfunction and increased carotid artery intima media thickness be related to atherosclerosis.

This study sets out to determine the influence of age at the time of surgery as a risk factor for post-operative length of stay after bidirectional cavopulmonary anastomosis.

All patients undergoing a Glenn procedure between January 2010 and July 2015 were included in this retrospective cohort study. Demographic data were examined. Standard descriptive statistics was used. A univariable analysis was conducted using the appropriate test based on data distribution. A propensity score for balancing the group difference was included in the multi-variable analysis, which was then completed using predictors from the univariable analysis that achieved significance of p<0.1.

Over the study period, 50 patients met the inclusion criteria. Patients were separated into two cohorts of ⩾4 months (28 patients) and <4 months (22 patients). Other than height and weight, the two cohorts were indistinguishable in their pre-operative saturation, medications, catheterisation haemodynamics, atrioventricular valve regurgitation, and ventricular function. After adjusting group differences, younger age was associated with longer post-operative length of hospitalisation – adjusted mean 15 (±2.53) versus 8 (±2.15) days (p=0.03). In a multi-variable regression analysis, in addition to ventricular dysfunction (β coefficient=8.8, p=0.05), Glenn procedures performed before 4 months were independently associated with longer length of stay (β coefficient=−6.9, p=0.03).

We found that Glenn procedures performed after 4 months of age had shorter post-operative length of stay when compared to a younger cohort. These findings suggest that balancing timing of surgery to decrease the inter-stage period should take into consideration differences in post-operative recovery with earlier operations.

Pulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries, and pulmonary arteries hypoplasia are rare and complex congenital defects that require early interventions to relieve cyanosis and enhance the growth of native pulmonary arteries. The treatment of these patients is still controversial. Surgical techniques require cardiopulmonary bypass which is poorly tolerated by small infants. Percutaneous techniques such as radiofrequency perforation can be challenging. The hybrid technique consists of perventricular stenting of the right ventricle outflow tract through medial sternotomy, to restore native pulmonary flow.

We retrospectively reviewed the cardiovascular database of our centre in order to analyse our experience in hybrid procedure. We detected six patients who underwent hybrid first approach between November 2007 and December 2015. We report our early results and mid-term outcomes.

Median age at the procedure was 26 days, median weight was 3150 g, and median Nakata index was 52 mm2/m2. All procedures were successful except for one: this patient underwent a surgical shunt. No immediate and early deaths or major complications occurred and oxygen saturation levels increased in all the patients. Patients were followed up for a period of 12–103 months, and four of them underwent a procedure of unifocalisation at the mean age of 12.5 months.

We reported data from the largest series of patients who underwent this hybrid procedure. Our experience demonstrated encouraging results to expand the use of this approach to bridge high-risk patients with diminutive pulmonary arteries to a second step of surgical repair.

Cardiopulmonary exercise testing has been used to measure functional capacity in children who have undergone a heart transplant. Cardiopulmonary exercise testing results have not been compared between children transplanted for a primary diagnosis of CHD and those with a primary diagnosis of cardiomyopathy despite differences in outcomes. This study is aimed to compare cardiopulmonary exercise testing performance between these two groups.

Patients who underwent heart transplant with subsequent cardiopulmonary exercise testing at least 6 months after transplant at our institution were identified. They were then divided into two groups based on primary cardiac diagnosis: CHD or cardiomyopathy. Patient characteristics, echocardiograms, cardiac catheterisations, outcomes, and cardiopulmonary exercise test results were compared between the two groups.

From the total of 35 patients, 15 (43%) had CHD and 20 (57%) had cardiomyopathy. Age at transplant, kidney disease, lung disease, previous rejection, coronary vasculopathy, catheterisation, and echocardiographic data were similar between the groups. Mean time from transplant to cardiopulmonary exercise testing, exercise duration, and maximum oxygen consumption were similar in both groups. There was a difference in heart rate response with CHD heart rate response of 63 beats per minute compared to cardiomyopathy group of 78 (p = 0.028). Patients with CHD had more chronotropic incompetence than those with cardiomyopathy (p = 0.036).

Primary diagnosis of CHD is associated with abnormal heart rate response and more chronotropic incompetence compared to those transplanted for cardiomyopathy.

It has become increasingly apparent that only the truly effective humanitarian work emphasises empowering local practitioners. One problem, though, is that we are often seen as the “experts” who have come to “save” the children. This perception may adversely affect the confidence in the country’s own providers.

Non-profit organisations performing paediatric heart surgery in developing countries were identified from two sources: the CTSnet “volunteerism” web page and an Internet search using the term “Pediatric Heart Surgery Medical Mission.” The website of each organisation was reviewed, seeking a “purpose” or “mission” statement or summary of the organisation’s work. A separate Internet search of news articles was performed. The top five articles were analysed for each organisation, and the findings are then analysed using the Principlist and Utilitarian ethical systems.

A total of 10 separate non-profit organisations were identified. The websites of eight (80%) placed significant emphasis on the educational aspects of their work and/or on interaction with local professionals. However, of 43 news articles reviewed, reporters mentioned education of, or interaction with, local professionals in only 14 (33%), and four out of 10 organisations studied had no mention of the local providers in any article.

Although non-profit organisations emphasise the teaching and programme-building aspects of their efforts, media reports largely focus on simpler and more emotional stories such as patient successes or large donations. Acknowledgement of the clinical and financial contributions of the host countries is both a duty following from the principle of justice and an important factor in long-term programme building.

Myocardial strain measurements are increasingly used to detect complications following heart transplantation. However, the temporal association of these changes with allograft rejection is not well defined. The aim of this study was to describe the evolution of strain measurements prior to the diagnosis of rejection in paediatric heart transplant recipients.

All paediatric heart transplant recipients (2004–2015) with at least one episode of acute rejection were identified. Longitudinal and circumferential strain measurements were assessed at the time of rejection and retrospectively on all echocardiograms until the most recent negative biopsy. Smoothing technique (LOESS) was used to visualise the changes of each variable over time and estimate the time preceding rejection at which alterations are first detectable.

A total of 58 rejection episodes were included from 37 unique patients. In the presence of rejection, there were decrements from baseline in global longitudinal strain (−18.2 versus −14.1), global circumferential strain (−24.1 versus −19.6), longitudinal strain rate (−1 versus −0.8), circumferential strain rate (−1.3 versus −1.1), peak longitudinal early diastolic strain rate (1.3 versus 1), and peak circumferential early diastolic strain rate (1.5 versus 1.3) (p<0.01 for all). The earliest detectable changes occurred 45 days prior to rejection with simultaneous alterations in myocardial strain and ejection fraction.

Changes in graft function can be detected non-invasively prior to the diagnosis of rejection. However, changes in strain occur concurrently with a decline in ejection fraction. Strain measurements aid in the non-invasive detection of rejection, but may not facilitate earlier diagnosis compared to more traditional measures of ventricular function.