BackgroundSupraventricular tachycardia is a group of rhythm disturbances that affect 1 in 300‐1200 Australian children annually. The differentiation of supraventricular tachycardia (SVT) symptoms and age of onset according to their subtype is not well understood in the pediatric population. Most studies rely on ECG criteria only to characterize the subtype of the SVT, which is not applicable to all subtypes. The purpose of this study was to identify the symptoms and ages of onset of SVT subtypes, and to analyze whether ethnicity or severity correlated with the SVT subtype confirmed in an invasive Electrophysiology (EP) study.MethodsA retrospective analysis and prospective survey evaluated 364 patients who underwent an EP study at The Royal Children's Hospital, Melbourne between 2009 and 2015. Age of onset, symptoms, and ethnicity were collected by phone survey or medical records in addition to EP study diagnostic data, medication status, and follow‐up information about their symptom status following EP procedure. Patients were grouped according to their SVT subtype. Data analysis was performed using chi‐squared, Fisher's exact, and ANOVA statistical tests to determine associations between SVT substrates.ResultsTwo hundred and thirty‐three suitable cases of SVT were identified (131 men, 102 women) aged between 0 and 18 years. Atrioventricular Reentrant Tachycardia (AVRT) (n = 153) was the most common SVT subtype, followed by Atrioventricular Nodal Reentrant Tachycardia (AVNRT) (n = 55), Atrial Tachycardia (AT) (n = 17), and other SVT subtypes (n = 8) which included Atrial Fibrillation, Atrial Flutter, and Junctional Tachycardia. There was a male predominance in all subtypes, except for AVNRT. AVNRT patients had palpitations, dyspnoea, dizziness, and anxiety more than any other group, AVRT patients complained of vomiting most and patients with AT had the most fatigue. The mean age of symptom onset varied among groups, being earlier in AVRT, later in AVNRT with a significant difference between AVRT with unidirectional retrograde accessory pathway (URAP) and AVNRT subtypes (P < 0.01).ConclusionSome specific symptoms were strong discriminators between different SVT subtypes. Ethnicity did not have strong correlations with SVT subtype incidence. This study was able to show clinical differences among children with SVT due to AVRT (URAP) compared to AVNRT, allowing the prognosis and intended management of pediatric SVT to be anticipated by less invasive means.

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guidelines (www.nhlbi.nih.gov/health-pro/guidelines/current/von-willebrandguidelines/full-report/4-management-of-vwd.htm) in which the suggested level for the designation of type 1 VWD was a VWF:Ag or VWF:RCo level of <30 IU/dL and 'low VWF levels' refer to individuals with VWF:Ag levels between 30 and 50 IU/dL. The aim was to reclassify the VWD patient cohort at the National Paediatric Comprehensive Care Centre Our Lady's Children's Hospital Crumlin, Dublin. Methods 315 Case records of children <18 years with VWD or possible VWD over a 10 year period were retrospectively extracted from the Irish National Bleeding Disorder database. These records were 'interpreted' according to NHLBI/NIH diagnostic criteria. The algorithm applied was; VWF level-s<30IU/dl on 2 separate occasions-VWD; VWF levels 30-50IU/dl on 2 separate occasions-Low VWF; VWF not less than 50IU/dl on 2 occasions and multiple testing-not VWD. Blood group was also recorded. Where incomplete laboratory data, patients were recalled to a review clinic for further testing.Results 315 children on the Irish National Bleeding disorder database had been historically diagnosed with VWD or possible VWD.Following the review there was a 81% reduction in the number of patients diagnosed with Type 1 VWD (187 patients in the original cohort were classified as Type 1 which reduced to 36 post -reclassification).Predictably, no significant change in numbers diagnosed with Type 2 (26) and Type 3 (2 patients) VWD, 185 (59% of total population) are now classified as Low VWF with a preponderance of Group O patients. 37 (15% of total population) were deemed to have no form of VWD and reclassified as normal.19 (6% of total population) have not returned for full reclassification and still remain as unspecified or possible. Discussion/Conclusion Our data suggest over-diagnosis of VWD in this population using previous guidelines. Diagnosis, especially for individuals with mildly decreased VWF (30-50IU/dl) requires correlation of clinical assessment and laboratory results. Reclassification has resulted in reallocation of resources to priority patients.This recommendation does not preclude the diagnosis of VWD in individuals with VWF:RCo of 30-50 IU/dl if there is supporting clinical and/or family evidence for VWD or the use of agents to increase VWF levels where VWF:RCo is 30-50IU/dl and may be at risk for bleeding.

We herein describe the successful surgical repair of a very rare combination of an aorta-to-left ventricle tunnel with the right coronary artery arising from it. The neonate presented with signs of heart failure due to significant regurgitation of blood via the tunnel. The closure of the tunnel was feasible during neonatal period without patches.

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