Aims Sudden cardiac death (SCD) is the most common mode of death in paediatric hypertrophic cardiomyopathy (HCM). This study describes the implant and programming strategies with clinical outcomes following implantable cardioverter-defibrillator (ICD) insertion in a well-characterized national paediatric HCM cohort. Methods and results Data from 90 patients undergoing ICD insertion at a median age 13 (±3.5) for primary (n = 67, 74%) or secondary prevention (n = 23, 26%) were collected from a retrospective, longitudinal multi-centre cohort of children (<16 years) with HCM from the UK. Seventy-six (84%) had an endovascular system [14 (18%) dual coil], 3 (3%) epicardial, and 11 (12%) subcutaneous system. Defibrillation threshold (DFT) testing was performed at implant in 68 (76%). Inadequate DFT in four led to implant adjustment in three patients. Over a median follow-up of 54 months (interquartile range 28–111), 25 (28%) patients had 53 appropriate therapies [ICD shock n = 45, anti-tachycardia pacing (ATP) n = 8], incidence rate 4.7 per 100 patient years (95% CI 2.9–7.6). Eight inappropriate therapies occurred in 7 (8%) patients (ICD shock n = 4, ATP n = 4), incidence rate 1.1/100 patient years (95% CI 0.4–2.5). Three patients (3%) died following arrhythmic events, despite a functioning device. Other device complications were seen in 28 patients (31%), including lead-related complications (n = 15) and infection (n = 10). No clinical, device, or programming characteristics predicted time to inappropriate therapy or lead complication. Conclusion In a large national cohort of paediatric HCM patients with an ICD, device and programming strategies varied widely. No particular strategy was associated with inappropriate therapies, missed/delayed therapies, or lead complications.

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There is growing interest in the use of digital medicine to reduce the need for traditional outpatient follow-up. Remote interrogation of pacemakers and implantable cardioverter defibrillators is now possible with most devices. The aim of our study was to evaluate the safety and efficacy of virtual pacing clinics in following up children with pacemakers and implantable cardioverter defibrillators, including epicardial systems.Methods:The study was retrospective over 8 years (2010–2017), with review of patient records and analysis of downloads from the implantable cardiac devices to the virtual clinics.Results:A total of 75 patients were set up for virtual clinic follow-up during the study period, 94.5% with a pacemaker and 5.5% an implantable cardioverter defibrillator. The majority (76.8%) had an epicardial system. Data on lead impedance, battery longevity, programmed parameters, detected arrhythmias, percentage pacing and delivered defibrillator therapies were obtainable by download. Lead threshold measurements were obtainable via download in 83.7% of the devices, including epicardial systems. No concerning device issue was missed. In 15% of patients a major issue was detected remotely, including three patients with lead fractures. The virtual clinics resulted in fewer hospital attendances while enhancing monitoring and enabling more frequent device checks. The vast majority (91.4%) of families who responded to a questionnaire were satisfied with the virtual clinic follow-up.Conclusions:Virtual clinics allow safe and effective follow-up of children with pacemakers and implantable cardioverter defibrillators, including those with epicardial systems and are associated with high levels of parent satisfaction.

Proteus syndrome is an extremely rare overgrowth condition caused by a somatic variant of the AKT1 gene. It can involve multiple organ systems though rarely is there symptomatic cardiac involvement. Fatty infiltration of the myocardium has been described but has not been reported to cause functional or conduction abnormalities. We present an individual with Proteus syndrome who suffered a sudden cardiac arrest.

Objective: Pacing in a univentricular circulation has been associated with worsened outcomes. We investigated the long-term outcomes of pacing in children with a univentricular circulation compared to a complex biventricular circulation. We also identified predictors of adverse outcomes.Methods: A retrospective study of all children with major congenital heart disease who underwent pacemaker implantation under the age of 18 years between November 1994 and October 2017.Results: Eighty-nine patients were included; 19 with a univentricular and 70 with a complex biventricular circulation. A total of 96% of pacemaker systems were epicardial. Median follow up was 8.3 years. The incidence of adverse outcome was similar between the two groups. Five (5.6%) patients died and two (2.2%) underwent heart transplantation. Most adverse events occurred within the first 8 years after pacemaker implantation. Univariate analysis identified five predictors of adverse outcomes in the patients in the biventricular but none in the univentricular group. The predictors of adverse outcome in the biventricular circulation were a right morphologic ventricle as the systemic ventricle, age at first congenital heart disease (CHD) operation, number of CHD operations, and female gender. The nonapical lead position was associated with a much higher risk of an adverse outcome. Conclusions:Children with a pacemaker and a complex biventricular circulation have similar survival to the ones with a pacemaker and a univentricular circulation. The only modifiable predictor was the epicardial lead position on the paced ventricle, emphasizing the importance of apical placement of the ventricular lead.

BackgroundNICE guidelines on antibiotic prophylaxis against infective endocarditis (IE) were published in 2008 resulting in a change in practice. There are recommendations for ongoing audit to monitor the incidence of IE, particularly in the high risk congenital heart disease (CHD) population.MethodsThis is a retrospective review of cases of infective endocarditis in paediatric patients with CHD in Scotland. Patients were identified from the cardiac unit database. The current 30-month period was compared to two previous audits that covered the period immediately before and after the guideline release.ResultsThe period prior to the guidelines only identified 2 cases but this is most likely due to under-reporting. The period following the guidelines identified 8 cases of IE compared with 15 cases in the current study period. All patients had a history of cardiac surgery, median age was 5 years, median weight 19 kg and 4/15(26.7%) had DiGeorge syndrome. Only 1/15(6.7%) had a dental procedure and another 4/15(26.7%) had a cardiac surgical procedure within 3-months of diagnosis. Therefore the majority (10/15(66.7%)) had no invasive procedure within 3-months of diagnosis. Of note, in 9/15(60%) the site of infection was a right ventricle-pulmonary artery conduit. 7/9 (77.8%) were a Contegra conduit.SummaryOngoing surveillance of IE is vitally important and our study has revealed an increase in the incidence in our population. The majority of the cases were spontaneous infections. Further analysis will be required to determine if this is an ongoing trend and the causes are likely multifactorial.