Outcomes in Anomalous Aortic Origin of a Coronary Artery Following a Prospective Standardized Approach

Outcomes in Anomalous Aortic Origin of a Coronary Artery Following a Prospective Standardized Approach.

Molossi S, Agrawal H, Mery CM, Krishnamurthy R, Masand P, Sexson Tejtel SK, Noel CV, Qureshi AM, Jadhav SP, McKenzie ED, Fraser CD Jr.Circ Cardiovasc Interv. 2020 Feb;13(2):e008445. doi: 10.1161/CIRCINTERVENTIONS.119.008445. Epub 2020 Feb 13.PMID: 32069111


Take Home Points:

  • Surgical unroofing of anomalous aortic origin of a coronary artery cannot be offered to all patients and alternatives are needed.
  • Transection and reimplantation of anomalous aortic origin of a coronary artery offer good results at a 4-year follow-up.
  • After surgical repair, ˜ 90% of patients with anomalous aortic origin of a coronary artery can be cleared for exercise.


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Commentary from Dr. Frederic Jacques (Quebec City, QC, Canada), chief section editor of Congenital Heart Surgery Journal Watch:


Surgical correction of anomalous aortic origin of a coronary artery is usually offered to symptomatic patients and high-risk asymptomatic patients in order to correct or prevent ischemia, and to prevent sudden death. The creation of an unobstructed coronary blood inflow from the appropriate sinus of Valsalva is the goal. Unroofing of the intramural and interarterial course of the coronary artery is the most common surgical procedure performed for such anomaly. However, it is not always possible to perform it due to anatomic variants, and it is not devoted of postoperative risks including coronary events, coronary reinterventions, and aortic insufficiency. Bonilla-Ramirez et al. compared their results with a transection and reimplantation technique in 16 patients to 45 unroofings at Texas Children’s Hospital. Preoperatively, about 45% had exertional symptoms and 30% nonexertional symptoms (leaving another 25% asymptomatic). Objective ischemia was demonstrated by stress cardiac MRI in 46%. Compared to unroofing, the transection and reimplantation technique required longer cardiopulmonary and aortic cross-clamp time by about one third. One patient with the transection and reimplantation technique had recurrent ischemia mandating CABG at a median follow-up of 4 years (compared to none among unroofing). One patient with unroofing had recurrent sudden cardiac arrest due to a previously unrecognized myocardial bridge. This patient had a redo with the transection and reimplantation technique and did well thereafter according to the authors. Over 90% of patients were cleared for exercise in both groups after surgical repair and postoperative non-invasive assessment. The transection and reimplantation led to the demonstration of less postoperative ischemia than the unroofing.



Surgeons dealing with anomalous aortic origin of a coronary artery know that unroofing —although favored— is not always possible. This paper from Bonilla-Ramirez et al. (along others such as Gaillard M. et al. featured in November 2020), is reassuring as it states that other ways of creating an unobstructed coronary inflow in such anomalies can result in good mid-term outcomes. The authors describe their decision not to perform unroofing for 3 categories of patients: 1) the course of the coronary artery is below the commissure, 2) when unroofing does not relocate the ostium to the appropriate sinus, and 3) when unroofing results in compression by the intercoronary pillar. Stating their rationale, they also express that they do not use osteoplasty techniques and that they do not create aortic buttons. The fact that one patient with unroofing and recurrent symptoms was treated by the alternative technique of transection and reimplantation highlights the facts that not all patients have the same baseline anatomy and that likely the repair should be tailored to the individual problem rather than trying to fit every anatomical variant with a standard technique.

Interestingly, the failure of a post-reimplantation technique leading to CABG highlights that surgical manipulation of coronary origins is not devoted of potential complications. It makes us wonder if creating an aortic button may have prevented such complication. As the authors suggest, postoperative assessment by provocative myocardial ischemia testing should be performed before allowing exercise to prevent complications. As most studies have a limited follow-up (about 5 years), further studies with longer follow-up will be needed to better define the freedom from ischemic events among repaired anomalous aortic origin of a coronary artery. No question, we need large multicentric studies with long follow-up to better understand what technique should be applied to what anatomical variant and what should be expected or prevented in these patients down the road.