The significance of symptoms before and after surgery for anomalous aortic origin of coronary arteries in adolescents and adults

The significance of symptoms before and after surgery for anomalous aortic origin of coronary arteries in adolescents and adults.

Meijer FMM, Egorova AD, Jongbloed MRM, Koppel C, Habib G, Hazekamp MG, Vliegen HW, Kies P.Interact Cardiovasc Thorac Surg. 2020 Nov 22:ivaa234. doi: 10.1093/icvts/ivaa234. Online ahead of print.PMID: 33221843


Take Home Points:

  • Most symptomatic patients with anomalous origin of coronary artery have atypical symptoms.
  • Surgical management of anomalous origin of coronary artery disease resolves symptoms in about 60% of patients.
  • Major adverse cardiovascular events are at least 7.5% at a median follow-up of 3 years.

Dr. Frederic Jacques


Commentary from Dr. Frederic Jacques (Quebec City, QC, Canada), chief section editor of Congenital Heart Surgery Journal Watch: Meijer et al. published a study in the Interactive CardioVascular and Thoracic Surgery trying to answer: What is the significance of symptoms among adult and teenage patients with anomalous origin of coronary arteries in the preoperative and in the postoperative period? These patients were mainly adults with a mean age 44±15 years. Their follow up, described as mid-term, has a median time of 3 years after surgical correction. They included all their 53 patients referred for surgery of a coronary artery originating from the opposite sinus of Valsalva from 2001 to 2018. Follow up data was available for about two thirds of them. Ninety-six percent of patients had some sort of symptoms before the operation. Almost 80% had suspicion of myocardial ischemia but only 35% of patients had typical angina pectoris prior to surgical correction. The remaining symptomatic patients had aborted sudden cardiac death (6%). For About 15% of patients, the coronary problem was either found during a familial screening or incidentally. Three quarter of the patients had a functional diagnostic test. From a surgical standpoint, almost 75% of patients had an unroofing procedure. Reimplantation, osteoplasty and CABG were also performed. Eight percent of patients had a combination of coronary procedures. Concomitant procedures were performed in 28%. To provide mid-term outcomes, the authors disregarded events occurring immediately after surgery. Only one death was reported. The proportion of symptom-free patient was 59%, which is significantly higher than before surgery. Three patients had typical de novo angina pectoris at follow up which required another intervention.


This is an interesting paper focusing on symptoms related to anomalous coronary origin. As the authors states, anomalous origin of the coronary arteries is a rare disease that may lead to major complications or sudden death in some. However, as the actual denominator of patients living with this condition is unknown and the available corrective strategies are not devoted of complications, no clear consensus was ever established to clinically follow those patients or to surgically treat them. The paper does not answer any question about this denominator. However, it depicts the clinical trajectory of patients who have reached surgical management. It was already known that complications of this surgical management may range from recurrence of symptoms to sudden death. When no symptoms are identified preoperatively, the surgery would be considered prophylactic. As such, to be fully in line with ethics principles, surgeons and cardiologists should prove that intervening on these patients is safer than doing nothing. Here, the question is how successful the surgical management in correcting symptoms of coronary ischemia (96% of patients had some evidence of symptoms (not necessarily typical of myocardial ischemia)) among mostly symptomatic patients. Surgeons and cardiologists should be reassured that intervening on symptomatic patients is likely to help in most of them (59%). This is in line with previous publications on this matter. Having said that, if the success rate of coronary artery bypass grafting were only 60% in the adult acquired coronary disease, it would be considered a failure. It is worth noting that the comparison of pre- and post-operative symptoms was available for only 64% of patients. In other words, we cannot evaluate the resolution or the increase of symptoms in about one third of these patients. This is a major limitation of this study. Regardless, we learn that among those we know about the follow up, at least 1 patient died, and 3 other patients required another intervention. This is concerning because even though the study does not report any stroke or neurological complications and does not report immediate postoperative complication, it already shows a significant complication rate. In truth, from the available data, we know that the classical 3-point major adverse cardiovascular events is at least 7.5%. It is de facto underestimated but again beware that this complication rate that would be considered unacceptable in most contemporary acquired coronary artery disease revascularization trials. This is even more clinically significant as the mean age of the patients of the present cohort would be considered an exceptionally low risk subgroup for acquired coronary artery disease management. Overall, a significant proportion of patients with anomalous origin of coronary artery was helped, but our congenital community should work together to make surgeries and overall management of these patients more sound and safer.