A 9 day old infant is brought to the ED with difficulty breathing and poor feeding for one day. Pt is found to be tachypneic, mottled, with peripheral cyanosis and no palpable LE pulses. What’s your diagnosis? What should you do?
Coarctation of the aorta: a ductal dependent lesion.
Ductal (PDA) Dependent heart lesions typically present in the first 24hours to 2weeks of life with tachypnea, respiratory distress, poor feeding or cyanosis.
Lesions which depend on flow via the ductus to maintain systemic circulation (heart failure).
• Coarctation of the aorta
• Critical aortic stenosis
• Hypoplastic left heart syndrome
Lesions which depend on flow via the ductus to maintain pulmonary circulation (cyanotic).
• Pulmonary atresia
• Critical pulmonary stenosis
• Tricuspid atresia
• Tetralogy of fallot
Lesions where the systemic and pulmonary circulations are separated
• Transposition of the great arteries
If a ductal-dependent congenital heart lesion is suspected, give prostaglandin E (alprostadil) at an initial dose is 0.05 µg/kg per minute while evaluation for a definitive diagnosis proceeds. Improvement should occur within 15 minutes.
The prostaglandin E1 infusion has the side effects of apnea, and hypotension so all the appropriate airway equipment required for intubation, as well as secondary vascular access should be prepared.
References:
Artman M, Mahony L, Teitel DF. Chapter 5. Initial Evaluation of the Newborn with Suspected Cardiovascular Disease. In: Artman M, Mahony L, Teitel DF, eds. Neonatal Cardiology. 2nd ed. New York: McGraw-Hill; 2011
Sacchetti A. Chapter 18. Neonatal Emergencies. In: Strange GR, Schafermeyer WR, Kestner RW, Wiebe RA, eds. Pediatric Emergency Medicine. 3rd ed. New York: McGraw-Hill; 2009.
