Patients with anomalous aortic origin of the coronary artery remain at risk after surgical repair

J Thorac Cardiovasc Surg. 2018 Jun;155(6):2554-2564.e3. doi: 10.1016/j.jtcvs.2017.12.134. Epub 2018 Feb 8.


Objectives: Anomalous aortic origin of a coronary artery (AAOCA) from the opposite sinus of Valsalva is a rare cardiac anomaly associated with sudden cardiac death (SCD). Single-center studies describe surgical repair as safe, although medium- and long-term effects on symptoms and risk of SCD remain unknown. We sought to describe outcomes of surgical repair of AAOCA.

Methods: We reviewed institutional records for patients who underwent AAOCA repair, from 2001 to 2016, at 2 affiliated institutions. Patients with associated heart disease were excluded.

Results: In total, 60 patients underwent AAOCA repair. Half of the patients (n = 30) had an anomalous left coronary artery arising from the right sinus of Valsalva and half had an anomalous right. Median age at surgery was 15.4 years (interquartile range, 11.9-17.9 years; range, 4 months to 68 years). The most common presenting symptoms were chest pain (n = 38; 63%) and shortness of breath (n = 17; 28%); aborted SCD was the presenting symptom in 4 patients (7%). Follow-up data were available for 54 patients (90%) over a median of 1.6 years. Of 53 patients with symptoms at presentation, 34 (64%) had complete resolution postoperatively. Postoperative mild or greater aortic insufficiency was present in 8 patients (17%) and moderate supravalvar aortic stenosis in 1 (2%). One patient required aortic valve replacement for aortic insufficiency. Two patients required reoperation for coronary stenosis at 3 months and 6 years postoperatively.

Conclusions: Surgical repair of AAOCA is generally safe and adverse events are rare. Restenosis, and even sudden cardiac events, can occur and long-term surveillance is critical. Multi-institutional collaboration is vital to identify at-risk subpopulations and refine current recommendations for long-term management.

Keywords: anomalous coronary artery; congenital heart disease; congenital heart surgery; pediatrics; sudden death.

MeSH terms

  • Adolescent
  • Adult
  • Aortic Valve Stenosis
  • Child
  • Coronary Vessel Anomalies / diagnostic imaging
  • Coronary Vessel Anomalies / epidemiology*
  • Coronary Vessel Anomalies / surgery*
  • Female
  • Humans
  • Male
  • Postoperative Complications / epidemiology*
  • Retrospective Studies
  • Treatment Outcome
  • Young Adult