Faculty Bibliography

Myxomas are rare tumors in neonates and tend to have a different presentation compared to adults. We present an infant with a left atrial myxoma presenting with episodic tachycardia who underwent successful surgical excision. In addition, we performed a review of the literature, identifying 17 cases of neonatal myxomas. Unlike adults, neonatal myxomas are more common in males and occur more often on the right side of the heart. Constitutional symptoms such as fever or embolism are rare among neonates. Most patients have favorable outcomes following surgical excision, refuting earlier claims that neonatal myxomas are associated with poor outcomes.

Tricuspid atresia with an absent pulmonary valve is a rare congenital cardiac defect. Although extensive pathological reviews have been published in the past, there are only a handful of cases that have been successfully palliated to the stage of Fontan. We hereby describe the successful management of one such case and review the surgical strategies described in the literature.

We present the case of a 4-month-old, former 23-week premature baby who underwent patent ductus arteriosus device closure in the cardiac catheterisation lab with an Amplatzer Piccoloâ„¢ device at 12 weeks of life. This was complicated by late migration of the device into the aorta resulting in severe obstruction and requiring surgical intervention.

Intrapericardial teratomas are rare, predominantly benign tumors that warrant surgical resection in the neonatal period because of their potential detrimental effects on the cardiorespiratory system. Surgical resection can be a challenge when the tumor encases and obscures a coronary artery. Adherence to certain operative principles is necessary to achieve successful outcomes.

We report a case of a 17-year-old healthy male presenting with multisystem hyperinflammatory shock temporally associated with COVID-19. Cardiac involvement was suspected based on evidence of significant cardiac injury (elevated cardiac biomarkers, electrocardiographic and echocardiographic abnormalities). Cardiac magnetic resonance imaging was performed demonstrating global biventricular systolic dysfunction, as well as a small area of T2 hyperintensity and mid-wall late gadolinium enhancement. This case discusses the varied cardiac involvement in pediatric patients with COVID-19 infection and highlights that cardiac injury is not just limited to hyperinflammatory syndrome related global dysfunction but a more focal myocarditis can also be seen.

The Center for Disease Control recommends prompt removal of nonessential central venous catheters (CVCs) to reduce the risk for central line-associated bloodstream infections. Safety checklists have been trialed to reduce nonessential CVC days, but pediatric studies are lacking. Our specific aim was to detect >10% reduction in mean CVC duration after implementation of a safety checklist addressing CVCs in our unit.

PURPOSE/OBJECTIVE:Drug reaction with eosinophilia and systemic symptoms (DRESS) is associated with antiepileptic drug use and is a rare but life-threatening side effect. We present a case of phenobarbital-induced DRESS in a patient who subsequently required phenobarbital and was successfully desensitized. SUMMARY/CONCLUSIONS:A 5-year-old male presented with medically refractory status epilepticus (SE). He had been trialed on several antiepileptic medications without achieving burst suppression. Burst suppression was achieved with a pentobarbital infusion, and thus, phenobarbital was initiated as the pentobarbital was weaned. After five days of phenobarbital, the patient developed signs and symptoms concerning for DRESS; a punch biopsy confirmed the drug reaction. Two months later, he again developed SE unresponsive to antiepileptic infusions. Burst suppression was achieved with pentobarbital, and it was decided to transition the patient to phenobarbital. Due to concerns of phenobarbital-induced DRESS, the patient underwent a phenobarbital desensitization consisting of 6 doses sequentially administered in 10-fold increasing concentrations before achieving therapeutic dosing. Three days later, the patient achieved therapeutic phenobarbital levels, was weaned off of pentobarbital, and remained seizure-free without recurrence of DRESS. CONCLUSIONS:Graded desensitization may be an option to minimize recurrence of DRESS in patients where avoidance of the offending agent is not possible.