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

Abstract

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.

Figure 1. Consort diagram for patient outcomes

Describes the outcome for patients based on initial anatomy and symptoms. ARCA = anomalous right coronary artery off the left sinus; ALCA = anomalous left coronary off the right sinus; Abnl = abnormal; ECG = electrocardiogram; Echo = echocardiogram; AVR = aortic valve replacement; PPS = post-pericardiotomy syndrome. Abnormal echocardiograms include those with abnormal function; all echocardiograms demonstrated an anomalous coronary. All percentages represent the percent of total patients, n=60.

Figure 2. Change in symptoms of ischemia

Bar chart depicts the number of patients with symptoms pre- and post-operatively. Parentheses represent percent change in symptoms. Includes patients in whom both pre- and post-operative data are available (n=59). All patients with post-operative symptoms experience pre-operative symptoms. No asymptomatic patients developed symptoms post-operatively.

Figure 3. Post-operative Aortic Insufficiency

Bar graphs represent the number of patients with any degree of aortic insufficiency on initial post-operative and most recent follow-up echocardiogram. As noted, there were 12 patients without follow-up echocardiogram available, represented by the light gray box. The median time for follow-up echocardiogram was 583 days post-operatively.

Central Picture

Cardiac CT angiogram in patient with aborted sudden cardiac death 6 years after unroofing. Coronal view demonstrating the takeoff of the anomalous left coronary artery.

Video 1

Axial multiplanar reconstruction images from cardiac CT performed after cardiac arrest in this patient who underwent ALCA unroofing demonstrates a ridge of hypoattenuation at the left coronary origin that extends rightward of the intercoronary commissure, with associated orifice narrowing (indicated by the yellow arrow).