Pediatric Cardiac Emergencies: When to Worry and When to Reassure.

Cardiac symptoms are a frequent reason for pediatric patients to present to the emergency department. As stressful as these visits can be for both parents and inexperienced providers, many of these symptoms may have a benign explanation, and recognition of red flags are of the utmost importance to provide optimal care. In this article, we present four clinical scenarios that have a cardiac etiology and are common to the pediatric emergency department. In addition to highlighting differential diagnoses, we discuss important red flags, key signs, and findings on physical examination that should not be missed. A brief review of important workup and management is also discussed. Lastly, we review common electrocardiogram pearls and pitfalls important for the ordering provider to recognize. In this article, we hope to provide guidance on when to provide reassurance and when to refer to a pediatric cardiologist for evaluation. [Pediatr Ann. 2023;52(4):e128-e134.].

Development of pediatric multidisciplinary extracorporeal membrane oxygenation simulations: A novel educational program to enhance team communication and emergency preparedness.

INTRODUCTION: Pediatric extracorporeal membrane oxygenation (ECMO) is a high risk, low-volume technology. Infrequency of this technology and associated complications may translate to unfamiliarity of identification and management of potentially life-threatening events, which may require knowledge and procedural skills to be performed quickly. Providers involved in managing ECMO must be able to promptly identify and initiate management for such events, particularly when surgical colleagues are not readily available. METHODS: A multidisciplinary ECMO simulation program was implemented in a tertiary children's hospital. Over 18 months, a prospective, observational study was conducted evaluating simulations involving circuit and patient emergencies, teamwork and communication behaviors and technical skills. An on-line survey was sent to participants following sessions to evaluate post-simulation confidence, lessons learned and potential barriers to implementation of necessary skills and behaviors. RESULTS: Ten simulation sessions occurred during implementation. Mean participants per session was 7 (range: 5-11). Eight Pediatric Cardiac Intensive Care Unit attendings, four Advance Practice Nurses, 54 pediatric intensive care unit registered nurses, and 55 pediatric respiratory therapists attended. Tasks with highest self-reported increase in confidence were related to (1) diagnosis (tension pneumothorax, oxygenator failure, and ventricular tachycardia), (2) fluid administration and (3) early and efficient mobilization for ECPR, with less reported confidence increase with technical skills More than 90% of participants provided a task or behavior they would implement if a specific emergency was encountered in real-life following simulation training. Real-life application occurred following simulations with participants reporting direct impact of training on their ability to perform the skill efficiently and correctly. CONCLUSIONS: Implementation of ECMO multidisciplinary simulations provides structured opportunities for the team to learn and practice ECMO skills together in scenarios they may encounter without surgical presence. Ensuring competency of providers through implementation of such a program may improve patient safety through enhanced team communication, knowledge, and hands-on experience.

Heterotaxy Syndrome and Intestinal Rotation Abnormalities.

BACKGROUND: Infants with heterotaxy syndrome (HS) have abnormal lateralization of organs along the right-left body axis. Intestinal rotation abnormalities (IRAs) are a potential source of morbidity and mortality. For this study, our objective was to prospectively observe a cohort of infants with HS and determine the incidence and natural history of IRA. METHODS: Infants ≤6 months of age with HS were enrolled in this prospective observational study. Exclusion criteria were other congenital abnormalities that necessitated abdominal surgery. HS was defined as any arrangement of organs that was not situs solitus or situs inversus along with associated congenital heart disease. The investigation for IRA was at the discretion of each participating center. RESULTS: Infants were recruited from January 2012 to December 2016. Thirty-eight infants from 7 institutions were included; 22 infants had right isomerism and 16 infants had left isomerism. Twenty-nine infants (76%) were evaluated for IRAs; 21 of 29 evaluations (72%) were abnormal. Eight infants were investigated because of symptoms, and 21 infants were evaluated routinely. The median age at symptom presentation was 46 days (range: 5-171 days). Seven infants had a Ladd procedure; 4 were prophylactic, with 3 as part of a combined procedure, and 3 were emergent. No child suffered acute midgut volvulus over a median follow-up of 1.6 years (range: 0.06-4.93 years). CONCLUSIONS: IRAs are common in infants with HS. Infants with symptoms presented by 6 months of age. There was no failure of expectant management resulting in midgut volvulus during a median follow-up of 1.6 years.

Post-cardiotomy extracorporeal cardiopulmonary resuscitation in neonates with complex single ventricle: analysis of outcomes.

OBJECTIVE: Extracorporeal cardiopulmonary resuscitation (ECPR) in children with cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) has been reported with encouraging results. We sought to review outcomes of neonates with functional single ventricle (FSV) receiving post-cardiotomy ECPR. METHODS: Forty-eight patients who required post-cardiotomy extracorporeal membrane oxygenation (ECMO) since the introduction of our ECPR protocol (January 2007-December 2009) were identified. Twenty-seven were neonates. Review of records and survival analysis were conducted. RESULTS: Of 27 neonates receiving post-cardiotomy ECMO 20 had FSV. Fourteen had ECPR. Ten underwent Norwood operation (NO) for hypoplastic left heart syndrome (HLHS). Four had FSV other than HLHS. Three underwent Damus-Kay-Stansel or modified NO with systemic-to-pulmonary shunt (SPS) and one SPS with anomalous pulmonary venous connection repair. Mean age and weight were 7.8 ± 2.9 days and 3.44 ± 1.78 kg, respectively. ECMO median duration was 6 days (interquartile range (IQR) 3-14). Survival to ECMO discontinuation was 79% (11 of 14 patients) and at hospital discharge was 57% (8 of 14 patients). The most common cause of death was multi-organ failure (four of six deaths). At last follow-up (median: 11 months (1-34)) 43% of patients were alive. CPR mean duration for patients with favorable versus unfavorable outcome was 38.6 ± 6.3 versus 42.1 ± 7.7 min (p = 0.12). Previously reported determinants for poorer prognosis in conventional non-rescue ECMO (such as pre-ECMO pH<7.2, renal, neurological or pulmonary hemorrhage complications, and pre- and post-vasoactive inotropic score) did not influence outcome between survivors and non-survivors (p>0.05). CONCLUSIONS: ECMO support in neonates with FSV requiring ECPR can result in favorable outcome in more than half of patients at hospital discharge. Aggressive strategy toward timely application of ECPR is justified. Expeditious ECPR deployment after proper patients' selection, refinement of CPR quality and use of adjunctive neuroprotective interventions, such as induced hypothermia, might further improve outcomes.