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Fetal compressive intrapericardial teratoma is a rare and life-threatening condition, qualifying as a high-acuity low-occurrence (HALO) event. To prepare for delivery and immediate neonatal management, specialists from pediatric cardiology, cardiac surgery, maternal-fetal-medicine, neonatology, cardiac anesthesia, critical care, clinical perfusion, obstetrical nursing, and operating room nursing convened. An in situ operating room simulation was used to identify and introduce key team members, derive and practice the anticipated clinical management algorithm, position human and equipment resources strategically, and ensure that each specialist team was familiar with the environment and available equipment. As rehearsed in the simulation, the cesarean delivery of the patient and neonatal cardiac surgery was uncomplicated and yielded a favourable clinical outcome. A patient-specific HALO simulation preparation (PSHSP) can facilitate positive clinical outcomes and improve health care team confidence in HALO scenarios such as the birth of newborns anticipated to have cardiorespiratory instabilty., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: Unexplained cardiac arrest (UCA) is rare in children. Despite investigations, the etiology in up to one-half of patients remains unknown., Objective: The purpose of this study was to assess the management and outcomes of pediatric UCA survivors through the Canadian Pediatric Heart Rhythm Network., Methods: A retrospective case series of children (age 1-19 years) who presented with UCA between January 1, 2004, and November 1, 2017, was conducted. Patients with known heart disease pre-UCA were excluded. UCA details, investigations, genetic test results, treatment, implantable cardioverter-defibrillator (ICD) data, subsequent diagnoses, and family screening data were collected., Results: Forty-six patients (61% male) were survivors of sudden unexpected death and met inclusion criteria at 8 participating sites. Median age at UCA was 13.8 years (interquartile range [IQR] 9-16 years). Baseline retrievable investigations included electrocardiogram (96%), echocardiogram (85%), exercise stress test (73%), and cardiac magnetic resonance imaging (57%). The presumed etiology for the UCA was identified in 24 (52%), mainly long QT syndrome or catecholaminergic polymorphic ventricular tachycardia. Genetic testing was performed in 33 of 46 (72%), with pathogenic/likely pathogenic variants identified in 13 of 33 (39%) and variants of uncertain significance in 8 of 33 (24%). ICDs were implanted in 35 of 46 (76%). Over median follow-up of 36 months (IQR 17-57 months), 8 of 35 had arrhythmia events captured on device interrogation. Families of 26 of 46 patients(57%) underwent screening, leading to a cardiac diagnosis in 6 of 26 families., Conclusion: A cause for UCA was not identified in nearly 50% of patients despite extensive investigations, including cascade screening. A large proportion (75%) of ICD shocks occurred in patients without a diagnosis., (Copyright © 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

We report a case of recurrent strokes in a healthy teenager after complete closure of atrial septal defect with Gore Septal Occluder (W.L. Gore and Associates, Newark, DE) device. The disk of the device produced a friction injury to the left atrial endocardium promoting thrombus formation with subsequent embolization to the brain requiring surgical intervention., (Copyright © 2017 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)