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Anomalous Aortic Origin of Coronary Arteries: A Single-Center Experience
Assunta Fabozzo1, Matthew DiOrio1, Jane W Newburger2
1Department of Cardiac Surgery, Boston Children's Hospital, Boston, Massachusetts; Department of Surgery, Harvard Medical School, Boston, Massachusetts.
Insights
Surgical repair for anomalous aortic origin of coronary arteries (AAOCA) is not associated with mortality. Surgery is recommended for anomalous left coronary artery (ALCA) with interarterial course, but long-term follow-up is crucial due to rare complications.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Pediatric Cardiac Surgery
Background:
- Anomalous aortic origin of coronary arteries (AAOCA) is a rare congenital heart defect.
- Understanding the clinical course and outcomes of AAOCA, especially after surgical intervention, is critical for patient management.
Purpose of the Study:
- To determine the clinical course and outcomes of patients with anomalous aortic origin of coronary arteries (AAOCA).
- To evaluate the effectiveness and safety of surgical repair for AAOCA.
Main Methods:
- Retrospective review of 155 patients diagnosed with AAOCA between 1996 and 2014 at a single center.
- Analysis of patient demographics, AAOCA course (interarterial/intraconal), coronary artery involvement, and surgical interventions.
- Comparison of outcomes between surgically treated and observed patients, including mortality and perioperative complications.
Main Results:
- Most patients (97%) had an interarterial (IA or IM) course of AAOCA.
- Surgical repair was more common in anomalous left coronary artery (ALCA) and in older patients with symptoms.
- No deaths occurred in the surgical group; 2 deaths in the observed group were due to noncardiac comorbidities. Major perioperative complications occurred in 6% of surgical cases.
Conclusions:
- Surgery for AAOCA was not associated with mortality in this cohort.
- Surgical intervention is recommended for patients with ALCA and an interarterial course.
- Long-term follow-up is essential to monitor for rare but serious complications and inform management guidelines.
Abstract:
The aim of this article is to determine the clinical course and outcomes in subjects with anomalous aortic origin of coronary arteries (AAOCA), particularly after surgical repair. A single-center, retrospective review of patients with AAOCA with right or left interarterial or IM (IA or IM) or intraconal course from 1996-2014. Among 155 patients, median age at diagnosis was 8.5 (range: 0.1-50) years, and 65% were male. The AAOCA course was IA or IM in 151 (97%) and intraconal in 4 (3%). Anomalous right coronary artery (CA) was present in 127 (82%), of whom 52 (42%) had repair. Anomalous left CA (ALCA) was present in 28 (18%), of whom 20 (71%) had repair. In the surgical group, 70 (97%) had IA or IM CAs; CA unroofing was performed in 62 (86%). In univariable analysis, surgical management was associated with ALCA (28% vs 10%, P = 0.003), age > 10 years (median 11 vs 6 years, P < 0.001), symptoms (63% vs 13%, P < 0.001), and exercise restriction at the time of diagnosis (47% vs 13%, P < 0.001). In multivariable modeling, surgery was associated with chest pain or syncope (P < 0.001) and older age (P = 0.03). Major perioperative complications occurred in 4 cases (6%) and 1 patient had late aortic valve repair. In the surgical group, no patients died; in the observed group, 2 patients with anomalous right CA (2.3%) died of severe noncardiac comorbidities. In our center, surgery for AAOCA was not associated with mortality, and surgery was recommended in patients with ALCA with IA or IM course. Rare but serious surgical complications highlight the importance of long-term follow up of patients with AAOCA to develop evidence-based management guidelines.