ECGs in the ED

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A 2-month-old infant boy presents to the emergency department with a history of episodic cyanosis. The cyanosis is episodic and exacerbated by crying. There has been no respiratory distress or syncope associated with the episodes. He breastfeeds vigorously every 3 to 4 hours, and there is no diaphoresis or other sign of distress during feedings. He was born at 40 weeks gestation following an uneventful pregnancy and delivery. He has had normal growth and development. He was noted to have a systolic murmur during the first week of life. He is due for his 2-month well-child check. He takes a multivitamin with fluoride, but takes no other medications. There are no known drug allergies. The family history is noncontributory for congenital heart disease or premature sudden death. There is a healthy 4-year-old sister.
The physical exam reveals a robust, well-nourished, mildly cyanotic baby in no respiratory distress. Pulse oximetry in room air is 86%. The heart rate is 140 bpm, the respiratory rate is 38 per minute, and the blood pressure is 86/42. The baby is well hydrated with cyanosis notable on the lips and oral mucous membranes. The remainder of the head and neck exam is unremarkable. The chest is clear to auscultation. The cardiac exam is significant for an increased right ventricular impulse, a normal first heart sound and a narrowly spit second heart sound. There is no gallop. There is a 3/6 harsh high-frequency systolic ejection murmur at the left sternal border that radiates to the back and axillae. The pulses are full and equal. There is no hepatomegaly. The extremities are warm despite the cyanosis. The neurologic exam is nonfocal.
An electrocardiogram was performed (Figure 1).
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