Editor’s Note: Dr. Elif Ozdogan (she/her) is a resident physician in Pediatrics at The Boston Combined Residency Program at Boston Children's Hospital and Boston Medical Center. She is interested in quality improvement and computational research and hopes to pursue further training in Transplant Medicine. -Rachel Y. Moon, MD, Associate Editor, Digital Media, Pediatrics
The aorta is the big vessel that leaves the heart to supply oxygen and nutrients to the body. Right after the aorta leaves the left ventricle, it forms an arch and settles next to the spine to course downwards. Coarctation (CoA), or congenital narrowing of the aorta, most commonly occurs below where the left subclavian artery branches off.
Although the constriction is present at birth, symptoms of CoA usually do not appear until a few days after birth, when the ductus arteriosus, a fetal vessel that shunts blood from the right side of the heart to the body to bypass the lungs, closes. CoA can result in a significantly decreased blood flow to the rest of body, and this can lead to heart failure and cardiogenic shock.
This is why as pediatricians, we painstakingly feel the femoral pulses of all our patients, but especially our newborn patients, in well-child visits.
In the systematic review entitled “Advances in the Treatment of Neonatal Coarctation of the Aorta” being early released this week in Pediatrics, Ms. Klea Hyosko and colleagues from Hannover Medical School and University Hospital Erlangen provide an in-depth analysis of current approaches to managing isolated neonatal CoA (10.1542/peds.2024-067434).
Key updates in the management of neonatal CoA include:
- Diagnosis: Although advances in prenatal detection have enhanced early intervention and reduced mortality, the sensitivity of fetal echocardiography is just 51%. After birth, echocardiography remains the cornerstone of diagnosis, with the aortic isthmus ratio (the diameter of the coarctation site divided by the diameter of the aorta at the diaphragm) the most useful.
- Transcatheter interventions: In the first few days of life, if a neonate is not stable enough for surgery, transcatheter intervention with balloon angioplasty and/or stent placement is probably the best option. There are reports of successful stenting in preterm weighing as little as 600 grams. Although balloon dilatation alone is less effective, this must be balanced by the risk of having a stent, which must be removed during final repair, and this may complicate surgical correction. Some centers thus try to do the surgical repair within a few weeks after stent placement to minimize vascular growth around the stent. Authors of this review note that there is no definitive evidence that this approach is superior to repair later.
- Surgical correction: CoA repair involves removing the narrowed segment and reconnecting the remaining segments. During the procedure, blood flow to the body is stopped by a technique called cross-clamping. Evolving research focuses on comparing different modalities to minimize the damage that can be caused by this lack of blood flow. More recently, diversion of blood flow to brain and visceral organs through techniques such as selective antegrade continuous perfusion has proven more beneficial than deep hypothermia, which primarily reduces tissue oxygen consumption.
- Long-term outcomes: Neurodevelopmental impacts remain under-researched, and long-term complications like hypertension require ongoing follow-up.
For those seeking updates on neonatal CoA management, I highly recommend this article. While cardiology focused, it offers valuable insights for general pediatricians and serves as a reminder of the importance of physical exam and of advocating for neurodevelopmental follow-up after CoA repair.