Sept. 27, 2024
The Society of Thoracic Surgeons released the first clinical practice guidelines for managing neonates and infants with isolated coarctation. The new guidelines, published in The Annals of Thoracic Surgery, offer recommendations to be applied based on surgical experience and clinical institution.
"Coarctation of the aorta is a common congenital heart defect, occurring in 4% to 5% of patients with congenital heart disease," says Elizabeth H. Stephens, M.D., Ph.D., a cardiovascular surgeon at Mayo Clinic in Rochester, Minnesota, specializing in congenital cardiac surgery, and lead author of the guidelines. It's the second most common congenital heart disease requiring neonatal intervention. "Despite this being a relatively common condition, there were no consensus statements or clinical guidelines regarding the management of these patients. There remains significant surgeon and institutional variation in management. These clinical practice guidelines are an important step forward in that regard," says Dr. Stephens.
Created by a panel of congenital cardiac surgeons, cardiologists and intensivists, the guidelines were developed with key questions using the PICO Framework. PICO stands for patients/population, intervention, comparison/control and outcome. A modified Delphi method was used for classification of recommendation and level of evidence.
Determining the right approach
In these guidelines, isolated coarctation refers to coarctation of the aorta that may have associated arch hypoplasia, but no other congenital heart defects or major anomalies. The standard of care for managing isolated coarctation in neonates and infants is surgery. Arch hypoplasia, depending on degree and location, may require a sternotomy approach rather than a thoracotomy approach.
Thoracotomy is indicated when arch hypoplasia is not present. Sternotomy is considered when arch hypoplasia is present that cannot be addressed with a thoracotomy or when there is a bovine aortic arch.
"One of the hardest decisions, and one where we see significant practice variation, is when arch hypoplasia should be addressed via a sternotomy versus a thoracotomy. These guidelines review the data on that and aid in this decision making," says Dr. Stephens.
Making an impact
In the current era, the operative mortality for repair of coarctation is relatively low. The Society of Thoracic Surgeons Congenital Heart Surgery Database reported an operative mortality of 0.98% for off-bypass repair of coarctation from July 1, 2016, to June 30, 2020.
Still, important short-term and long-term morbidities are prevalent, such as recoarctation and systemic hypertension, that need to be improved upon.
The guidelines emphasize that long-term outcomes such as health-related quality of life, hypertension and neurodevelopment should be the focus over the short-term outcomes and perioperative complications.
The guidelines are based on available data and continue to evolve. "Through the process of creating these guidelines, it became apparent how much we still have to learn regarding the management of these patients, including the sternotomy approach versus the thoracotomy approach," says Dr. Stephens.
For more information
Stephens EH, et al. The Society of Thoracic Surgeons Clinical Practice Guidelines on the Management of Neonates and Infants with Coarctation. The Annals of Thoracic Surgery. 2024;118:527.
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