A 1 week old male born at full term presents to the ED with difficulty breathing and a weak cry. Mom also notes that he appears “sweaty” during feeds today and has had fewer wet diapers. History is significant for scant prenatal care however delivery was uncomplicated. On exam he is cyanotic with oxygen saturation 70% on 5L NC O2. Vital signs are as follows: T 36C, HR 180, RR 65, BP 60/30. Which should you order first?

A. 20 cc/kg of crystalloid bolus
B. Dexamethasone
C. Furosemide
D. Prostaglandin

D. Prostaglandin. This patient likely has a ductal dependent congenital cardiac defect as evidenced by acute onset cyanosis, diaphoresis with feeds, and FTT around the time the ductus arteriosus closes after birth. Prostaglandin is a dilator of the ductus arteriosus and may delay closure, therefore it should be given as early as possible. Prostaglandin E1 is given as an infusion at 0.05-0.1 mcg/kg/min. Prepare to intubate as 12% of these patients can become apneic! Your differential for cyanotic heart disease includes the “5 Terrible Ts”: tetralogy of Fallot, transposition of the great vessels, total anomalous pulmonary venous return, tricuspid atresia, and truncus arteriosus. Further testing should include a hyperoxia test (where a PaO2 < 100mmHg suggests cyanotic cardiac heart disease after several minutes of 100% O2 administration), CXR, EKG, and echo.

Pediatrics. “Acyanotic vs Cyanotic Congenital Heart Disease.” Sept 27, 2017. <https://forum.facmedicine.com/threads/acyanotic-vs-cyanotic-congenital-h... . (11/7/19).