Remote monitoring is increasingly used in the follow-up of patients with cardiac implantable electronic devices. Data on paediatric populations are still lacking. The aim of our study was to follow-up young patients both in-hospital and remotely to enhance device surveillance.

This is an observational registry collecting data on consecutive patients followed-up with the CareLink system. Inclusion criteria were a Medtronic device implanted and patient’s willingness to receive CareLink. Patients were stratified according to age and presence of congenital/structural heart defects (CHD).

A total of 221 patients with a device – 200 pacemakers, 19 implantable cardioverter defibrillators, and two loop recorders – were enrolled (median age of 17 years, range 1–40); 58% of patients were younger than 18 years of age and 73% had CHD. During a follow-up of 12 months (range 4–18), 1361 transmissions (8.9% unscheduled) were reviewed by technicians. Time for review was 6±2 minutes (mean±standard deviation). Missed transmissions were 10.1%. Events were documented in 45% of transmissions, with 2.7% yellow alerts and 0.6% red alerts sent by wireless devices. No significant differences were found in transmission results according to age or presence of CHD. Physicians reviewed 6.3% of transmissions, 29 patients were contacted by phone, and 12 patients underwent unscheduled in-hospital visits. The event recognition with remote monitoring occurred 76 days (range 16–150) earlier than the next scheduled in-office follow-up.

Remote follow-up/monitoring with the CareLink system is useful to enhance device surveillance in young patients. The majority of events were not clinically relevant, and the remaining led to timely management of problems.

Parental understanding of their children’s heart disease is inadequate, which may contribute to poor health outcomes. The purpose of this study was to determine what parental knowledge is important in the care of children with heart disease from the perspective of parents, nurses, and physicians.

Focus groups were formed with parents of children with single ventricle congenital heart disease (CHD), biventricular CHD, and heart transplantation, and with nurses and physicians who provide care for these children. A nominal group technique was used to identify and prioritise important parental knowledge items and themes. The voting data for each theme were reported by participant type – parent, nurse, and physician – and patient diagnosis – single ventricle CHD, biventricular CHD, and heart transplantation.

The following three themes were identified as important by all groups: recognition of and response to clinical deterioration, medications, and prognosis and plan. Additional themes that were unique to specific groups included the following: medical team members and interactions (parents), tests and labs (parents), neurodevelopmental outcomes and interventions (physicians), lifelong disease requiring lifelong follow-up (physicians and nurses), and diagnosis, physiology, and interventions (single ventricle and biventricular CHD).

Parents, nurses, and physicians have both common and unique views regarding what parents should know to effectively care for their children with single ventricle CHD, biventricular CHD, or heart transplantation. Specific targeted parental education that incorporates these findings should be provided to each group. Further development of questionnaires regarding parental knowledge with appropriate content validity is warranted.

To report procedural characteristics and adverse events on data collected in the registry.

The IMPACT – IMproving Paediatric and Adult Congenital Treatment – Registry is a catheterisation registry of paediatric and adult patients with CHD undergoing diagnostic and interventional cardiac catheterisation. We are reporting the procedural characteristics and adverse events of patients undergoing diagnostic and interventional catheterisation procedures from January, 2011 to March, 2013.

Demographic, clinical, procedural, and institutional data elements were collected at the participating centres and entered via either a web-based platform or software provided by American College of Cardiology-certified vendors, and were collected in a secure, centralised database. Centre participation was voluntary.

During the time frame of data collection, 19,797 procedures were entered into the IMPACT Registry. Procedures were classified as diagnostic only (35.4%); one of six specific interventions (23.8%); other or multiple interventions (40.7%); and were further broken down into four age groups. Anaesthesia was used in 84.1% of diagnostic procedures and 87.8% of interventional ones. Adverse events occurred in 10.0% of diagnostic and 11.1% of interventional procedures.

The IMPACT Registry is gathering data to set national benchmarks for diagnostic and certain specific interventional procedures. We are seeing little differences in procedural characteristics or adverse events in diagnostic procedures compared with interventional procedures overall, but there is significant variation in adverse events amongst age categories. Risk stratification and patient acuity scores will be required for further analysis of these differences.

To assess foetal electrocardiographic intervals across gestational age among foetuses with and without congenital heart disease, and to investigate differences between groups.

A prospective observational cohort study.

Center for Prenatal Pediatrics, Morgan Stanley Children’s Hospital of NewYork-Presbyterian.

A total of 92 participants with singleton pregnancies, 41 with normal anatomy and 51 with congenital heart disease were included in this study.

Using a maternal abdominal monitor, foetal electrocardiogram was obtained serially from foetuses with and without congenital heart disease at 20–24 weeks (F1), 28–32 weeks (F2), and 34–38 weeks (F3) of gestation. A signal-averaged waveform was calculated, and PR, QRS, and QT intervals were measured. Intervals from controls were compared with gestational age norms. Using Pearson’s correlation coefficient, we analysed the relationship between gestational age and foetal electrocardiographic intervals. Intervals from control and congenital heart disease foetuses were compared by Student’s t-test.

PR (r=0.333, p=0.02) and QRS (r=0.248, p=0.05) intervals correlated with gestational age only among controls. QRS intervals in foetuses with congenital heart disease were significantly longer than controls at F1 (63±6 versus 52±5 ms, p<0.001), F2 (61±8 versus 56±7 ms, p=0.02), and F3 (64±10 versus 56±9 ms, p=0.007).

PR and QRS intervals lengthen across gestational age among foetuses with normal cardiac anatomy but not in foetuses with congenital heart diseases. As early as 20 weeks of gestation, differences between foetuses with and without congenital heart disease are discernible, with congenital heart disease foetuses demonstrating longer QRS intervals compared with controls.

Milrinone may be an appropriate adjuvant therapy for infants with persistent pulmonary hypertension of the newborn. We aimed to describe the effect of milrinone administration on right and left ventricular function in infants with persistent pulmonary hypertension not responding to inhaled nitric oxide after 4 hours of administration.

This is a retrospective review of infants born after or at 34 weeks of gestation with persistent pulmonary hypertension who received milrinone treatment. The primary endpoint was the effect of milrinone on myocardial performance and haemodynamics, including right and left ventricular outputs, tissue Doppler velocities, right ventricle and septal strain, and strain rate. Secondary endpoints examined included duration of inhaled nitric oxide and oxygen support.

A total of 17 infants with a mean (standard deviation) gestation and birth weight of 39.8 (2.0) weeks and 3.45 (0.39) kilograms, respectively, were included in the study. The first echocardiogram was performed 15 hours after the commencement of nitric oxide inhalation. Milrinone treatment was started at a median time of 1 hour after the echocardiogram and was associated with an increase in left ventricular output (p=0.04), right ventricular output (p=0.004), right ventricle strain (p=0.01) and strain rate (p=0.002), and left ventricle s` (p<0.001) and a` (p=0.02) waves. There was a reduction in nitric oxide dose and oxygen requirement over the subsequent 72 hours (all p<0.05).

The use of milrinone as an adjunct to nitric oxide is worth further exploration, with preliminary evidence suggesting an improvement in both oxygenation and myocardial performance in this group of infants.

Despite an increasing prevalence of adults living with a CHD, little is known about the psychosocial impact of CHD. We sought to investigate the relative impact of disease severity and patients’ perceptions about their condition on depression, anxiety, and quality of life over a period of a year.

A total of 110 patients aged over 16 years completed an initial questionnaire containing measures for anxiety, depression, quality of life, and illness perceptions when they attended the Adult Congenital Heart Disease Clinic. Cardiologists rated the patients’ disease severity and illness course. A year later, patients were invited to complete the same measures. Regression analyses were performed to determine the relative impact of illness perceptions and disease severity on psychological outcomes a year later.

At baseline, 23% of the study population had depressive symptoms and 30% had elevated trait anxiety. After controlling for associations with disease-related variables, illness perceptions explained 28% of the variance in depression, 40% anxiety, and 27% overall quality of life at baseline. Baseline illness perceptions bivariately predicted quality of life, cardiac anxiety, and depression 1 year later, and regression analyses controlling for other factors showed that they were significant predictors of outcomes 1 year later.

Symptoms of depression and anxiety are common among adults with CHD. Patients’ illness perceptions are related to psychological outcomes, especially cross-sectionally. Future research could investigate whether an intervention to discuss patients’ perceptions about their CHD can improve mental health and quality of life.

The physiological importance of the lateral tunnel stenosis in the Fontan pathway for children with single ventricle physiology can be difficult to determine. The impact of the stenosis and stent implantation on total cavopulmonary connection resistance has not been characteriszed, and there are no clear guidelines for intervention.

A computational framework for haemodynamic assessment of stent implantation in patients with lateral tunnel stenosis was developed. Cardiac magnetic resonances images were reconstructed to obtain total cavopulmonary connection anatomies before stent implantation. Stents with 2-mm diameter increments were virtually implanted in each patient to understand the impact of stent diameter. Numerical simulations were performed in all geometries with patient-specific flow rates. Exercise conditions were simulated by doubling and tripling the lateral tunnel flow rate. The resulting total cavopulmonary connection vascular resistances were computed. A total of six patients (age: 14.4±3.1 years) with lateral tunnel stenosis were included for preliminary analysis. The mean baseline resistance was 1.54±1.08 WU·m2 and dependent on the stenosis diameter. It was further exacerbated during exercise. It was observed that utilising a stent with a larger diameter lowered the resistance, but the resistance reduction diminished at larger diameters.

Using a computational framework to assess the severity of lateral tunnel stenosis and the haemodynamic impact of stent implantation, it was observed that stenosis in the lateral tunnel pathway was associated with higher total cavopulmonary connection resistance than unobstructed pathways, which was exacerbated during exercise. Stent implantation could reduce the resistance, but the improvement was specific to the minimum diameter.

Discordant atrioventricular with concordant ventriculo-arterial connections is a rare cardiac defect. When isolated, the haemodynamics resemble transposition of the great arteries. In complex heart defects such as heterotaxy, haemodynamics guide the surgical approach.

To report a series of eight patients with discordant atrioventricular and concordant ventriculo-arterial connections focussing on anatomical and diagnostic difficulties, surgical management, and follow-up.

A retrospective review was carried out from 1983 to 2013. Anatomical description was based on segmental analysis. Emphasis was placed on the venoatrial connections.

Segmental arrangement was {I, D, S} in six patients, all with spiralling great vessels. There were two patients with parallel great vessels of whom one had {S, L, D} and the other had {S, L, A} arrangement. Of eight patients, five had heterotaxy syndrome. Median age at repair surgery was 1.4 years (with a range from 1.1 months to 8.1 years). The repair surgery finally performed was the atrial switch procedure in seven out of eight patients. The main post-operative complications were two cases of baffle obstruction and one sick sinus syndrome needing pacemaker implantation. There were two early post-operative deaths and six late survivors. Median follow-up was 4.2 years (with a range from 3.9 to 26.7 years) with good functional status in all survivors.

Diagnosing discordant atrioventricular with concordant ventriculo-arterial connections remains challenging. There are ongoing controversies about the definition of atrial morphology and heterotaxy syndrome animating the anatomic discussion of these complex heart defects. Haemodynamically, the atrial switch procedure is the surgical method of choice with an encouraging long-term follow-up despite rhythm disturbances and baffle obstruction.