Successful transition for adolescent survivors of CHD: the Cambridge cardiac transition pathway.

PURPOSE: National standards to ensure effective transition and smooth transfer of adolescents from paediatric to adult services are available but data on successful transition in CHD are limited. The aim of this study is to assess the effectiveness of our transition pathway. METHODS: Adolescents with CHD, aged 15-19 years, who attended the joint cardiac transition clinic between 2009 and 2018 were identified from the Patient Administration Systems. Patient attendance at their first adult CHD service appointment at Royal Papworth Hospital was recorded. RESULTS: 179 adolescents were seen in the joint cardiac transition clinic in the 9-year study period. The median age of the patients when seen was 16 (range 15-19) years. 145 patients were initially planned for transfer to the Royal Papworth Hospital adult CHD service. Three patients were subsequently excluded and the success of the transfer of care in 142 patients were analysed. 112 (78%) attended their first follow-up in the adult CHD clinic as planned, 28 (20%) attended after reminders were sent out with 5/28 requiring multiple reminders, and only 2 (1.4%) failed to attend. Overall, transfer of care was achieved in 140 (98.6%) patients. CONCLUSION: A dedicated joint cardiac transition clinic involving multi-professional medical and nursing teams from paediatric and adult cardiology services appears to achieve high engagement rates with the adult services. This approach allows a 'face' to be put on a named clinician delivering the adult service and should be encouraged.

Clinical outcomes and programming strategies of implantable cardioverter-defibrillator devices in paediatric hypertrophic cardiomyopathy: a UK National Cohort Study.

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.

Patent Ductus Arteriosus in Extreme Prematurity: Role of Echocar-diography and Other Imaging Techniques.

Clinical signs alone are unreliable in the diagnosis of patent ductus arteriosus (PDA) in preterm infants, and therefore echocardiography remains the mainstay of diagnosis of this common condition. Echocardiography also facilitates understanding of the hemodynamic effects of a PDA, and thus aids in management decisions. Several echocardiographic parameters, including duct size, maximum ductal velocity, left atrial: aorta ratio, mitral inflow E:A ratio, and isovolumic relaxation time, have been utilized in the assessment of PDA, but no single measurement can be used in isolation to inform clinical judgement. Therefore, it is important that echocardiographers on the neonatal unit have a comprehensive understanding of available methods and their limitations. Newer echocardiographic techniques, such as 3 Dimensional echocardiography, tissue Doppler imaging and strain imaging, are now providing insights into myocardial function in the adaptation of preterm infants to extra-uterine life, and into the effects of a PDA causing systemic-to-pulmonary artery shunting. Magnetic resonance imaging delivers excellent diagnostic information and accurate hemodynamic evaluation; however this modality is not easily accessible for most preterm infants, in comparison to echocardiography, which is readily available at the cotside in most neonatal units. Further developments in echocardiography may further refine the contribution it makes to individualized clinical decisionmaking in the management of premature infants with PDA.