Fatal Arrhythmia in a 2-day-old Full-term Infant.

Abstract

1. Wendy Si, MD* 2. Hoda Karbalivand, MD*,† 3. Tomas Havranek, MD*,† 1. *Department of Pediatrics and 2. †Division of Neonatal-Perinatal Medicine, Children’s Hospital at Montefiore, Bronx, NY 1. Address correspondence to Wendy Si, MD, Department of Pediatrics, Children’s Hospital at Montefiore, 3415 Bainbridge Ave, Bronx NY, 10467. E-mail: wsi{at}montefiore.org A term male infant is born via elective cesarean delivery with a birthweight of 3.2 kg to a Gravida 2 Para 1 mother. The prenatal course was unremarkable, and there is a consanguineous relationship between the parents (first cousins). The infant’s Apgar scores are 9 and 9 at 1 and 5 minutes of life, respectively. The initial newborn examination is normal. The infant is allowed to room in with his mother and is exclusively breastfed. At 27 hours of age, the infant is brought to the nursery for poor feeding. On repeat examination, he is found to be hypotonic and apneic with poor perfusion. Vital signs show temperature of 92.5°F (33.6°C), heart rate of 40 beats/min, and oxygen saturations of ∼80% on room air. Point of care blood glucose is less than the detectable range. Initial blood gas reveals severe metabolic acidosis with pH of 7.1, base deficit of 22.5 mEq/L (22.5 mmol/L), and immeasurably high lactate level. Positive pressure ventilation is initiated, but no improvement in tone or respiratory effort is seen. The infant is subsequently intubated. Chest radiograph demonstrates clear lungs and a normal cardiothymic silhouette. Over the next 2 hours, the infant receives multiple rounds of chest compressions, multiple doses of epinephrine, a dose of sodium bicarbonate, and several fluid and dextrose boluses. Sepsis evaluation is performed, and antibiotics are started for presumed sepsis. Before transfer to a regional NICU, he is placed on epinephrine and dopamine infusions for hypotension. His hypoglycemia improves on intravenous 10% dextrose solution. Upon arrival to the regional NICU, the infant is noted to be arousable with reactive pupils, weak …