Authors: Omar El Masri, MD, MA - Stanford University AND Kaitlin M. Flannery, MD, MPH - Stanford University
A 5-day-old, 3.2kg, term neonate with severe aortic coarctation and hypoplastic transverse arch (Z-score = -4) is currently managed on a PGE1 infusion with low lactate and good lower extremity pulses. According to the 2024 Society of Thoracic Surgeons (STS) Clinical Practice Guidelines on the management of neonates and infants with coarctation which is the MOST appropriate next step in management?
EXPLANATION
Coarctation of the aorta accounts for 4-5% of congenital heart disease (CHD). It is the second most common CHD requiring neonatal intervention after patent ductus arteriosus (PDA). In the current era, mortality following surgical repair is low at 0.98%. However, important short and long-term morbidity requiring repeat intervention persists, including re-coarctation and hypertension.1, 2
Despite the frequency of coarctation, management plans remain heterogenous among institutions and surgeons. In 2024, the STS published practice guidelines for the management of neonates and infants with coarctation of the aorta. Experts in the field reviewed the available literature and using a modified Delphi method established guidelines with classification of the recommendations and level of evidence.1
The following are the practice guidelines for neonates and infants with isolated coarctation1:
1. Surgical intervention is recommended in the absence of obvious contraindications. (Class I)
2. In patients with prematurity, low weight, or other risk factors for surgical intervention, medical optimization before intervention or primary operation is reasonable. (Class IIa)
3. In patients without associated arch hypoplasia, repair through a thoracotomy is indicated. (Class I)
4. In patients with associated arch hypoplasia that cannot be adequately addressed through a thoracotomy, repair through a sternotomy is preferable. (Class IIa)
5. In patients with bovine arch anatomy, repair through a sternotomy may be reasonable given the potential increased risk of re-coarctation with bovine arch anatomy repaired through a thoracotomy. (Class IIb)
6. In patients undergoing repair through a sternotomy, antegrade cerebral perfusions or limited duration deep hypothermic circulatory arrest may be reasonable. (Class IIb)
7. In patients undergoing repair through a sternotomy, extended end-to-end, arch advancement, and patch augmentation are all reasonable techniques. (Class IIa)
The patient presented above has no contraindications to surgical intervention and should undergo surgical repair before onset of complications. Given the patient’s significant transverse arch hypoplasia with a Z-score of -4, a sternotomy with repair on CPB is preferable as the risk of re-coarctation would be high with thoracotomy and repair that did not address the hypoplastic transverse arch.
The decision to perform arch repair on CPB with antegrade cerebral perfusion versus coarctation repair via thoracotomy without CPB significantly changes the anesthetic management and postoperative course. However, the literature review to produce these clinical practice guidelines found no significant difference in mortality, global neurologic outcomes, recurrent laryngeal nerve injury, or incidence of chylothorax between the two approaches.1,3
REFERENCES
1. Stephens EH, Feins EN, Karamlou T, et al. The Society of Thoracic Surgeons Clinical Practice Guidelines on the Management of Neonates and Infants with Coarctation.Ann Thorac Surg. 2024 Sep;118(3):527-44.
2. Spaeth JP, Loepke AW. Chapter 22: Anesthesia for left-sided obstructive lesions. In: Andropoulos DB, Stayer S, Mossad EB, Miller-Hance WC, eds. Anesthesia for Congenital Heart Disease. 3rd ed. Wiley Blackwell; 2015: 506-9.
3. Waldman JC, Ing RJ, Stone ML. Current Practice Management Guidelines in Neonates and Infants with Isolated Coarctation of the aorta. J Cardiothorac Vasc Anesth. 2025 Mar;39(3):573-5.
