A Prospective Evaluation of Arrhythmias in a Large Tertiary Neonatal Intensive Care Unit.

Arrhythmias in the neonatal period are common and can be classified as bradyarhythmias and tachyarrhythmias and as benign or non-benign. Neonatal arrhythmias are further differentiated between those with abnormalities in generation (non-sinus) and those with abnormalities in propagation. Because the neonatal myocardium is immature and operates at the peak of the Starling curve, significant changes in heart rate can result in a decline in cardiac output and compromise end-organ perfusion. This is especially true for premature neonates, those critically ill, or those with concomitant congenital heart disease. While sustained arrhythmias are frequently witnessed and recorded in tertiary neonatal intensive care units (NICU) very little data exist on the observance of non-sustained brady- or tachyarrhythmias in this cohort. No prospective study has been performed on all neonates admitted to a large tertiary NICU throughout their entire stay. The purpose of this study was to prospectively evaluate the prevalence and type of arrhythmias in a large NICU population from admission to discharge. All neonates admitted to the NICU at Inova Children's Hospital at Inova Fairfax Medical Campus between January 1, 2021 and April 1, 2021 were prospectively evaluated from admission to hospital discharge via continuous bedside monitoring reviewed every 24 h. Concerning telemetry strips were reviewed by two team members as well as the senior electrophysiologist. Two-hundred and one neonates (mean gestational age = 344/7 weeks) were enrolled in the study. Admission length ranged from 1 to 195 days (total of 5624 patient days, median 16 days). Overall, 68% (N = 137) of admissions had one or more arrhythmias, the most common of which was sinus tachycardia (65%, N = 130), followed by sinus bradycardia (30%, N = 60). Clinically relevant arrhythmias were diagnosed in 6.5% of neonates. During the study period there were four deaths, none of which were directly attributable to a primary arrhythmia. Approximately 68% of neonates exhibited at least one arrhythmia. Although the vast majority of these arrhythmias were benign, clinically relevant arrhythmias were observed in 6.5%. Patients admitted to the NICU appear to have a relatively high burden of benign arrhythmias, but a relatively low burden of pathologic arrhythmias.

The impact of socioeconomic status and race on the outcomes of congenital heart disease.

PURPOSE OF REVIEW: Studying the outcomes of congenital heart disease and their associations allows paediatric cardiologists and intensivists to improve the care and health equity of their patients. This review presents the most recent literature discussing the socioeconomic and racial disparities that pervade the outcomes of patients with congenital heart disease in every facet of treatment. The outcomes of congenital heart disease discussed are prenatal detection, maintenance of care, quality of life, neurodevelopment and mortality. RECENT FINDINGS: Historically, it has been documented that patients with congenital heart disease who are of racial and ethnic minorities disproportionately experience poor outcomes. Recently, the association between racial minorities and mortality has been traced to underlying socioeconomic disparities emphasizing that race and ethnicity are not independent determinants of health. SUMMARY: The effect of socioeconomic status on the outcomes of congenital heart disease is profound and reaches beyond the association with racial and ethnic minorities. Changes to address these disparities in outcomes must be made at the individual, institutional, community and system levels.

DICER1 Mutation Detected in an Infant Guides Accurate Diagnosis of Auto-Amputated Embryonal Rhabdomyosarcoma.

BACKGROUND: The DICER1 mutation is a pathogenic, germline mutation that predisposes patients to uncommon malignancies at a young age. CASE: A 6-month-old female infant presented with vaginal bleeding and a protruding vaginal mass of unclear pathogenesis. Chemotherapy was initially targeted toward a germ cell tumor; after pathologic testing and auto-amputation of the tumor, the patient was diagnosed with a rare DICER1-associated embryonal rhabdomyosarcoma. Subsequently, her treatment course was restructured and family genetic surveillance instituted. SUMMARY AND CONCLUSION: Consideration for DICER1 mutation in tumors with complex pathology and unique presentation is critical to aid in diagnosis and management, and direct future comprehensive surveillance.