Idiopathic dilatation of the right atrium (IDRA) is a rare anomaly defined as isolated enlargement of the right atrium in the absence of other cardiac lesions known to cause right atrial dilatation. IDRA is a congenital anomaly with unknown pathogenesis and highly variable clinical presentation. Optimal management of severe IDRA is controversial and individualized. Literature reports of long-term follow-up have been limited. We describe a child with IDRA with rapid atrial tachycardia (AT) refractory to both medical and surgical management, and we provide long-term follow-up on our two previously reported cases, both of whom had documented AT. For infants with AT, the clinical course is unpredictable, and medical therapy is the first line of treatment. The decision to proceed with surgical resection of a giant right atrium should be made on an individual basis. Atrial resection along with a modified right atrial MAZE procedure could be considered in infants with life-threatening atrial tachyarrhythmia refractory to medical treatment. Surgical scarring of the right atrium may produce substrate for atrial arrhythmia, which may also be refractory to medical therapy. Histological examination of excised atrial tissue remains inconsistent and not contributory to the determination of the etiology of IDRA. Our three infants with IDRA illustrate unique features of their variable clinical courses, as well as continued difficulties with establishing clear guidelines with regard to surgical management of this unusual disorder.

Postpericardiotomy syndrome (PPS), a potential complication of open heart surgery, has a variable clinical course and severity. This study evaluated the effectiveness of acetylsalicylic acid (ASA) prophylaxis in preventing PPS after surgical closure of atrial septal defects (ASDs) in pediatric patients. A retrospective review was performed for 177 patients who underwent uncomplicated ASD closure from 1986 to 2006. The study group received prophylactic ASA 20 to 50 mg/kg/day for 1 to 6 weeks after surgery, whereas the control group did not. The primary outcome was a diagnosis of PPS based on the presence of two or more of the following symptoms or signs occurring at least 72 h postoperatively: fever (temperature >38 degrees C), pericardial or pleural rub, and worsening or recurring anterior pleuritic chest pain. Consequently, PPS developed in 5 (2.8%) of the 177 children: 2.8% (3/106) in the control group and 2.8% (2/71) in the study group (p = 1.00). The secondary outcomes were frequency of other postoperative complications. Postoperative pericardial effusions experienced by 26.7% of the patients were identified more frequently in the treatment group (p < 0.001). Postoperative prophylaxis ASA at a dose of 20 to 50 mg/kg/day for 1 to 6 weeks after surgical closure of ASD does not decrease the incidence of PPS in pediatric patients.

Key Points Question What are the highest-priority unanswered research questions in pediatric hospital medicine from the perspective of young people, parents/caregivers, and health care professionals? Findings This study, which included 2 surveys and a final consensus meeting using nominal group technique, gathered the perspectives of youths, parents/caregivers, and clinicians. The top 10 questions identified focused on the care of special inpatient populations (eg, children with medical complexity), communication, shared decision-making, support strategies, mental health supports, reducing time in the hospital, and supporting Indigenous families. Meaning The findings of this study suggest that the most important research questions in pediatric hospital medicine focus on processes and models of care, communication, and hospitalization outcomes.

Anti N-methyl-d-aspartate (NMDA) receptor encephalitis in children is associated with psychiatric changes, seizures, and dyskinesias. We present the first report of autistic regression in a toddler caused by this entity. A 33-month-old boy presented with decreased appetite, irritability, and insomnia following an upper respiratory tract infection. Over the next few weeks he lost language and social skills, and abnormal movements of his hand developed. Within a month, this patient came to fit the diagnostic criteria for autistic spectrum disorder. Upon investigation, anti-NMDA receptor antibodies were found in the boy's cerebrospinal fluid. He was treated with intravenous immunoglobulins and steroids, resulting in reacquisition of language and social skills and resolution of movements. Our case emphasizes the significance of suspecting anti-NMDA receptor encephalitis as the cause of autistic regression, even in an age group where the diagnosis of autistic spectrum disorder is typically made, and especially when presentation follows a febrile illness.