Advocate Children's Hospital

Introduction: Rhabdomyolysis in pediatric patients can be life-threatening, often presenting with acute kidney injury (AKI), and may progress to multisystem organ failure (MSOF). Diagnosis can be challenging, but prompt recognition and initiation of therapies to treat consequences of muscle degradation is key to recovery. Etiologies in pediatrics include viral infection, strenuous exercise, status epilepticus, hemoglobinopathies and metabolic disorders. We present a case of an adolescent presenting with shock due to severe rhabdomyolysis. Description: A 17-year-old male presented in shock after syncope while running track. He reported abdominal and leg pain for 3 weeks prior to presentation. He was in acute renal failure with severe metabolic acidosis and hyperkalemia requiring fluid resuscitation and renal replacement therapy (RRT). He developed compartment syndrome of the right thigh requiring emergent fasciotomy. Dramatically elevated creatinine phosphokinase (CPK) levels and MRI revealing diffuse active myositis of his abdominal musculature confirmed severe rhabdomyolysis. Hemoglobin electrophoresis revealed sickle cell trait. Microbiological studies were negative. High dose methylprednisolone was initiated. Subsequently during hospitalization, he suffered acute hypoxic respiratory failure secondary to pulmonary embolism, requiring venoarterial ECMO for 4 days. While recovering from his MSOF, mobility and neurocognitive function remained delayed, requiring inpatient rehabilitation. Seven months after initial presentation he had complete recovery of pulmonary and cognitive function. His renal function recovered, with secondary hypertension managed with losartan and amlodipine. Mild spasticity in his lower extremities is managed with oral baclofen, and he has full use of all muscle groups. He has not had any recurrence of rhabdomyolysis to date. Discussion: Pediatric rhabdomyolysis is rare, requiring a high index of suspicion, as the triad of myalgia, muscle weakness, and tea-colored urine is only present in approximately 10% of patients. Consideration of this diagnosis in pediatric patients presenting with AKI, particularly in the presence of associated conditions discussed above, including sickle cell trait, may lead to earlier recognition and prevention of MSOF.

Introduction: Functionally univentricular parallel circulation represents one of the highest-risk circulatory physiologies for humans. With the arterial saturation linked to both the pulmonary venous and systemic venous saturations and total cardiac output being the sum of systemic and pulmonary blood flow, this circulation is truly unique. Maintaining adequate oxygen delivery is important, and oxygen extraction has been demonstrated to be associated with outcomes. This study aimed to characterize relationships between various hemodynamic parameters and oxygen extraction in this circulation.Methods: Data from the pre-Glenn catheterization in children with functionally univentricular parallel circulation were utilized to determine relationships between various hemodynamic parameters and oxygen extraction. Oxygen extraction was calculated using the superior caval vein saturation.Results: Data from 45 catheterizations across 45 unique patients were included in these analyses. Higher systemic blood flow, higher total cardiac output, lower pulmonary to systemic blood flow ratio, higher hemoglobin, and lower systemic vascular resistance were found to be associated with lower oxygen extraction.Conclusion: Catheterization data demonstrate that a variety of hemodynamic parameters are associated with oxygen extraction. These may be clinically monitored and utilized to help augment oxygen extraction.

BackgroundLoeys-Dietz syndrome (LDS) is a rare connective tissue disorder (CTD) with musculoskeletal, craniofacial, and cardiovascular features with a prevalence of approximately 1:50,000. Morbidity and mortality often occur earlier in patients with LDS compared to patients with other CTDs.Case SummaryWe present a teenager with subacute heart failure, 4/6 holosystolic murmur with diastolic rumble, facial differences, and arachnodactyly. She had genotype-positive, phenotype-positive LDS including an atrial septal defect, severely dilated great arteries/ventricles, and depressed systolic function, requiring prompt medical and surgical therapy.DiscussionTo our knowledge, this is the first case of LDS with such dramatic atrial and ventricular dilation, likely the result of shunting, valve regurgitation, and underlying CTD.Take-Home MessagesLDS can be aggressive in young patients; screening and diagnosis should be prompt when LDS is suspected. Elective repair of the aorta is indicated at smaller absolute diameters in LDS. Intervention lowers risk of a sudden, potentially life-ending cardiac event.

Multicenter studies describing electrocardiogram (ECG) patterns in children presenting with anomalous left coronary artery from the pulmonary artery (ALCAPA), to our knowledge, have not been published. This study aimed to describe the ECG findings at presentation and over time in a multicenter cohort of children with ALCAPA and assess their association with clinical outcomes. We performed a retrospective ECG analysis of 188 patients with ALCAPA who presented between 1/2009 and 3/2018 at 21 centers affiliated with Collaborative Research for the Pediatric Cardiac Intensive Care Society. ECGs were categorized apriori as Classic (pathologic Q waves in anterolateral leads), non-classic (abnormal but withoutpathologic Q waves), and normal. The primary outcome was major adverse cardiac events (MACE) following ALCAPA repair, defined as extracorporeal support, cardiopulmonary resuscitation, heart transplantation, or death. Classic, non-classic, and normal ECG findings were seen at presentation in 124 (66%), 49 (26%), and 15 (8%) patients, respectively. There was no association between ECG category and MACE. Patients with non-classic or normal ECG findings were significantly more likely to present with normal or mildly diminished left ventricular systolic function and had significantly shorter duration of ventilation and hospital stay. Among 70 patients with ECGs at ~ 3years following surgery, classic, non-classic, and normal ECG patterns were seen in 6 (8%), 29 (41%), and 35 (50%) patients, respectively. In conclusion, one-third of patients with ALCAPA presented with non-Classic or normal ECG patterns, and these patients were more likely to have preserved cardiac function and recover more quickly following surgical repair.

Vaccine hesitancy increased during the COVID-19 pandemic. We evaluated if manifestation of adverse events when caregivers received their vaccine was associated with their willingness to vaccinate their children. A multicenter, cross-sectional, multi-lingual survey of caregivers presenting to 19 pediatric Emergency Departments in the USA, Canada, Israel, and Switzerland early during the early stage of the pandemic, before vaccines for children were available. We asked caregivers if they were vaccinated against COVID-19, to report any side effects and if they would give vaccine to their child. We categorized all reported side effects and report the most common ones. We report willingness to vaccinate based on child's age. Of 4261 caregivers, mean caregiver age was 38.3years, 2893 (68.3%) mothers, 3108 (73.3%) greater than high school education. 43.6% reported side effects, 35.7% reported no side effects and 20.7% were unvaccinated. The lowest rate of caregivers planning to vaccinate their children was the unvaccinated (6.9%) and the highest was among caregivers who did not report any side effects (74.4%). Caregivers with no reported side effects after vaccination were more likely to vaccinate their children compared to those that had some side effects (aOR of 1.34, 1.52 and 2.14 for ages <5, 5-11 and≥12years, respectively). Reporting general illness/feeling unwell, experiencing weakness, and breathing difficulty were associated with caregivers unlikely to plan to vaccinate their children. The OR to vaccinate children for each additional side effect was 0.86, 95%CI=0.78-0.95, p=0.004, representing a decrease of 14% in the odds of planning to vaccinate for each additional side effect. Side effects experienced by caregivers are associated with their intention to vaccinate their children. Building of trust in vaccines, including communicating risks and benefits in the context of caregiver's experience, is important and may help enhance rate of vaccination of children.

As abortion care restrictions increase, a growing population is continuing pregnancies complicated by life-limiting fetal conditions, making it more critical than ever to evaluate the state of the evidence in perinatal palliative care (PPC). Perinatal palliative care provides interdisciplinary, person-centered care, integrating medical management with psychosocial and bereavement support to enable values-driven decision-making. This scoping review evaluates US-based evidence on the safety, effectiveness, acceptability, and equity of PPC, assessing how these findings relate to growing abortion restrictions. Analysis of 13 studies found that US PPC programs are understudied, with limited evidence on maternal health and neonatal comfort outcomes. Studies lacked use rates for all eligible individuals, preventing assessment of overall PPC uptake. Most PPC patients reported high satisfaction, citing compassionate care, emotional support, and parental validation. However, studies lacked diversity. None examined the experience of receiving PPC due to abortion restrictions. Existing PPC evidence is limited, leaving critical gaps in safety, effectiveness, acceptability, and equity-key factors in assessing whether PPC meets its intended goals and serves diverse populations. Our review highlights that evidence is insufficient to determine whether PPC can adequately support the growing, vulnerable patient population now directed into it by policy rather than choice.

Background: Psychosocial factors in childhood, such as emotional and behavioral difficulties, contribute to long-term cardiovascular health (CVH). However, limited research has explored the relationship between these difficulties and childhood CVH. Methods: This cross-sectional analysis includes participants aged 2-18 (N = 6,892) from the Young Hearts cohort with complete behavioral CVH (bCVH) and Strengths and Difficulties Questionnaire (SDQ) data. SDQ scores included the Total Difficulties Score (TDS), measuring emotional and behavioral difficulties, and the Prosocial Behavior Score (PBS), reflecting empathy and cooperation. We calculated bCVH scores using the Life’s Essential 8 metrics (diet, exercise, nicotine exposure, sleep, and BMI). Linear regression was used to assess associations between TDS, PBS, and bCVH, adjusting for age, sex, race/ethnicity, parental education, financial stability, food security, and health insurance status. Results: The sample had a mean age of 8.7, 48% female, 46% non-Hispanic White, 28% Hispanic, and 15% non-Hispanic Black. Higher TDS was associated with lower bCVH (-1.91; 95% CI -2.27, -1.56), while higher PBS was linked to better bCVH (1.75; 95% CI 1.41, 2.1), and physical activity largely contributed to these differences (Figure 1). Conclusion: Psychosocial difficulties and prosocial behaviors have a strong association with physical activity, making it a potential intervention target for improving childhood CV outcomes. Enhancing prosocial behaviors through interventions like social-skills training may further support both psychosocial well-being and cardiovascular health in children.