Introduction: Current Pediatric Brain Death/Death by Neurologic Criteria (BD/DNC) Consensus Guidelines recommend apnea testing as a part of the brain death exam. Criteria to proceed and conclude apnea testing do not allow for hypoxemia. For patients with single ventricle physiology, and baseline oxygen saturations (SpO2) below 85%, this poses an issue. Description: A 5-month-old female with hypoplastic left heart syndrome (HLHS) and Kabuki syndrome was admitted for management of HLHS. She underwent stage one Norwood procedure at two months of age. Her post-operative course was complicated by chronic respiratory failure secondary to tracheomalacia requiring non-invasive positive pressure ventilation, arrhythmias, and E. faecalis bacteremia. Through this, she remained inpatient and relatively stable, awaiting stage two cardiac palliation. One morning, there were concerns for neurologic changes. Head imaging revealed a large intraparenchymal hemorrhage. Despite emergent decompressive hemicraniectomy, repeat imaging showed progression of herniation and electroencephalogram demonstrated no brain activity. Family eagerly wanted to proceed with organ donation and therefore, brain death testing was needed. Notably, inter-stage cardiac patients have baseline SpO2 of 75-85%, as did she. BD/DNC guidelines state that to proceed with apnea testing, PaO2 must be >200 mmHg and testing must be stopped if SpO2 falls below 85%, which she could never meet. After discussions with neurocritical care, cardiology, and organ procurement, the following was decided: If baseline SpO2 are 75-85% when at her previous neurological baseline, then there is no reason to suspect that being at these saturations now would be contributing to her current exam. Therefore, for the apnea test, we would target SpO2 between 75-85% and would abort the exam if SpO2 dropped below 70%. Testing was performed and SpO2 remained greater than 75%. She ultimately completed two separate brain death examinations that were consistent with BD/DNC. Discussion: Patients with single ventricle physiology typically have a baseline SpO2 lower than the stated goals for apnea testing provided within the BD/DNC guidelines. This case offers a multi-disciplinary agreed upon process to proceed with brain death testing in this population of children.
Dagher et al. (Sun,) studied this question.