Anomalous Aortic Origin of a Coronary Artery (AAOCA)

Right and left, child to adult

Artery

Age

High-risk anatomy

By source
Group	Features	Source
Athletes	Interarterial left AAOCA: intramural course, high take-off or slit-like orifice (proposed)	Kim 2025
Athletes	Interarterial right AAOCA: longer intramural length (proposed)	Kim 2025
≤30 y	Ischemia in 38% of AAOLCA vs 10% of AAORCA (known ischemia status); in AAOLCA linked to intramural course, high orifice or slit-like orifice	CHSS 2019
≤30 y	AAORCA with ischemia: longer intramural course	CHSS 2019
Adult	Ostial or proximal stenosis, slit-like orifice, acute angle of takeoff, intramural course	ACHD 2025
Adult	Intramural course; orifice anomalies: slit-like, acute-angle take-off, >1 cm above the sinotubular junction	ESC 2020

Single-centre cohorts

Molossi 2020 (single centre, 163 patients ≤20 y): intramural course (OR 13.2, 95% CI 5.0–34.9) and exertional syncope (OR 15.8, 1.3–185.4) predicted the program’s high-risk group; 82% unrestricted; all alive at a median 1.6–1.8 y.
Doan 2023, left (single centre, 56 AAOLCA <21 y): aborted sudden death in 6/27 interarterial. Juxtacommissural origin between the left and noncoronary sinus: aborted sudden death 1/9 and cardiogenic shock 1/9; 5/9 intramural, including all 4 with an event or inducible ischemia. Intraseptal: no aborted sudden death in 20; inducible ischemia in 6/16 tested.
Doan 2023, right abstract (single centre, 220 AAORCA <21 y): inducible ischemia on stress perfusion in 9% with no or nonexertional symptoms and 18% with exertional chest pain or syncope (P=0.09); intramural length 5 mm with and without ischemia (IQR 4–7 in both; P=0.65); the exercise test was a poor predictor.

How to image: echo, CT, CMR

Echo (ASE 2020): parasternal short axis above the valve; left main normally at about 4 o’clock, right coronary at about 11 o’clock. Color Nyquist about 20–40 cm/s (20–30 to tell right from left sinus origin).
Intramural clues: acute-angled origin with linear diastolic flow along the aortic wall; blue flow in AAOLCA, red in AAORCA from the left sinus. Long axis: a circle just in front of the aortic root; also a high right origin. Subcostal sweep to the pulmonary trunk. Intraconal: in the conal septum below the pulmonary valve.
Pitfalls: dropout mimics a normal origin (never rely on still frames); interarterial vs intramural and ostial stenosis are hard on echo; TEE helps.
CT: acute angle, vessel hugging the aortic wall and abrupt caliber change suggest an intramural course; check for a circular-to-oval lumen; length measurable; “hammock sign” for intraconal (ASE 2020). Preferred for high-risk anatomy (ESC 2020, adult). CMR: course, fibrosis and perfusion.

Evaluation and ischemia testing

Class	Recommendation	Level, source
I	Suspected AAOCA: transthoracic echo for origin and proximal course	B AATS 2017
IIa	CT angiography or cardiac MRI to define and confirm the anatomy	B AATS 2017
I	No ischemic chest pain or aborted sudden death: exercise test with nuclear perfusion or stress echo	B AATS 2017
I	Catheterization if noninvasive imaging cannot define anatomy, and in adults with atherosclerotic risk factors	B AATS 2017
IIa	Before sport: imaging for high-risk patterns and exercise stress test	C ESC 2020 sports, young, <40 y

Adult ACHD 2025 and ESC 2020 (3 rows)

Class	Recommendation	Level, source
1	Suspected AAOCA: coronary CT angiography (MR angiography if CT is contraindicated)	B-NR ACHD 2025
1	From the opposite sinus: risk-stratify for physiological and/or ischemic changes	B-NR ACHD 2025
I	Functional imaging with physical stress (nuclear, echo or CMR) to confirm or exclude ischaemia	C ESC 2020

No class

Children: exercise is the most physiologic stress; no FFR or iFR cut-offs for AAOCA (AHA 2026).
Athletes: test for inducible ischemia; vasodilator stress not recommended (Kim 2025).
A normal stress test is not fully reassuring (AATS 2017; ACHD 2025, adult).

Surgery

Class	Recommendation	Level, source
1	Ischemic chest pain, syncope suspected from ventricular arrhythmia, or aborted sudden death: restrict activity and offer surgery	B AATS 2017
IIb	Ischemic chest pain, syncope suspected from ventricular arrhythmia, or aborted sudden death, with prohibitively high surgical risk: restrict activity; catheter-based intervention may be considered	C AATS 2017
1	No symptoms, left from the right sinus, interarterial: offer surgery	B AATS 2017
I	Repair from the opposite sinus of Valsalva removes the intramural course and ostial narrowing: unroofing, ostioplasty or reimplantation	B AATS 2017
IIb	Moving the pulmonary artery confluence away may be considered, as an adjunct	C AATS 2017

Athletes (Kim 2025, no class): interarterial left, surgery should be considered regardless of presentation or ischemia testing ; interarterial right, if inducible ischemia or ischemic symptoms.

Adult ACHD 2025 and ESC 2020 (9 rows)

Class	Recommendation	Level, source
1	Symptoms, or ischemia attributable to the anomalous artery: surgery	B-NR ACHD 2025
2a	No symptoms, left coronary, high-risk anatomy: surgery reasonable	C-EO ACHD 2025
2b	No symptoms, from the opposite sinus, no ischemia or compromised perfusion: benefit of surgery not well established; observation or surgery may be reasonable	B-NR ACHD 2025
I	Typical angina with stress ischaemia in the matching territory or with high-risk anatomy: surgery	C ESC 2020
IIa	No symptoms, ischaemia (right or left)	C ESC 2020
IIa	No symptoms, AAOLCA, no ischaemia, high-risk anatomy	C ESC 2020
IIb	Symptoms without ischaemia or high-risk anatomy	C ESC 2020
IIb	No symptoms, AAOLCA, no ischaemia or high-risk anatomy, young (<35 years)	C ESC 2020
III	No symptoms, AAORCA, no ischaemia or high-risk anatomy: surgery not recommended	C ESC 2020

Where the sources differ

Left, interarterial, no symptoms or ischemia: surgery offered (AATS 2017, 1) or to be considered regardless (Kim 2025) ; Adult with high-risk anatomy (ACHD 2025 2a; ESC 2020 IIa; IIb without it under 35).
Right, no symptoms or ischemia: competitive sport permissible (AATS 2017, IIa) or reasonable (Kim 2025) ; between the great vessels, most moderate- or high-demand competitive sport not recommended (ESC 2020 sports III, <40 y); Adult observation or surgery may be reasonable (ACHD 2025 2b); no surgery without high-risk anatomy (ESC 2020 ACHD III).
Ischemia without symptoms: interarterial right, consider surgery (Kim 2025); no graded AATS 2017 row ; Adult surgery for ischemia attributable to the anomalous artery (ACHD 2025, 1); IIa (ESC 2020).

Sports and return to play

Class	Recommendation	Level, source
1	Unrepaired, left from the right sinus, interarterial, with or without symptoms: no competitive sports	B AATS 2017
IIa	Right from the left sinus, no ischemic symptoms, negative exercise test with imaging, after counselling on sudden-death risk: competitive sports permissible	C AATS 2017
III	Acutely angled take-off or course between the great vessels: most competitive sports with moderate or high demand not recommended	C ESC 2020 sports, young, <40 y
IIb	No symptoms, not between the great vessels, no slit-like orifice with reduced lumen or intramural course, no inducible ischaemia: competition may be considered after counselling	C ESC 2020 sports, young, <40 y
1	AED and trained staff at competition and training	B AATS 2017
After repair
I	No aborted sudden death: competitive or recreational sport after ≥3 months if symptom-free, exercise test without ischemia or concerning arrhythmia	C AATS 2017
IIa	Aborted sudden death: competitive sport after 12 months, same conditions	C AATS 2017
IIa	Aborted sudden death: recreational sport and PE class at 3 months, same conditions	C AATS 2017
IIb	All sports may be considered at the earliest 3 months after surgery, if asymptomatic with no ischaemia or complex arrhythmia on a maximal exercise test	C ESC 2020 sports, young, <40 y

Athletes (Kim 2025 Table 9; no class)
Wording	Consideration
Should not	Interarterial left, unrepaired: compete
Reasonable	Interarterial right: temporarily withhold or limit sport during initial evaluation
Reasonable	Interarterial right, no ischemic symptoms, no inducible ischemia or complex ventricular arrhythmias: compete, with shared decision-making and surveillance
Can	Intraseptal or noncoronary-sinus left, no ischemic symptoms, no inducible ischemia, no complex ventricular arrhythmias: compete
Can	Left prepulmonic or retroaortic, or retroaortic circumflex from the right sinus or right coronary: compete
Can	After repair: resume after complete sternal healing and testing without ischemia or complex ventricular arrhythmias; no interval stated

Follow-up

After repair: AATS 2017 schedule

After repair (AATS 2017 Table 2, suggested protocol)
When	Tests
7–10 d, 4–6 wk	ECG, echo
3 mo	Exercise test with imaging
6 mo	ECG, cardiac MRI if available
Long term, yearly	Visit, ECG, echo; exercise test every 1–3 y (yearly in competitive or high-level recreational sport)
Aspirin	“1 baby aspirin daily indefinitely” (dose in mg not given)

Adult ACHD 2025 routine intervals

Adult Routine intervals, months (ACHD 2025 Table 45)
Test	Stage A / B / C / D
ACHD cardiologist, ECG	24–36 / 12 / 12 / 12
Echo	24–36 / 24 / 12 / 12

Key points

Echo first, then CT or CMR (AATS 2017, ASE 2020); adults: CT angiography (ACHD 2025).
Interarterial AAOLCA: surgery offered without symptoms; no competitive sport unrepaired (AATS 2017).
AAORCA, symptom-free, no inducible ischemia: competitive sport after counselling (AATS 2017); Kim 2025 adds no complex ventricular arrhythmias, shared decision-making and surveillance.
Between the great vessels or acute-angle take-off: most moderate- or high-demand competitive sport not recommended (ESC 2020 sports, III, <40 y).
After repair: at least 3 months (12 after aborted sudden death), symptom-free, exercise test without ischemia or concerning arrhythmia (AATS 2017).

Sources and how this page was checked

Full text read

Brothers JA, Frommelt MA, Jaquiss RDB, Myerburg RJ, Fraser CD Jr, Tweddell JS. Expert consensus guidelines: anomalous aortic origin of a coronary artery. J Thorac Cardiovasc Surg 2017;153(6):1440-1457. doi:10.1016/j.jtcvs.2016.06.066. Archived publisher PDF. American Association for Thoracic Surgery (AATS) expert consensus, 15 recommendations, all shown; written for individuals of any age, with separate wording where it means adults.
Kim JH, Baggish AL, Levine BD, et al. Clinical considerations for competitive sports participation for athletes with cardiovascular abnormalities: a scientific statement from the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2025;85(10):1059-1108; Circulation 2025;151(11):e716-e761. doi:10.1016/j.jacc.2024.12.025. Author manuscript (PubMed Central) and the archived, corrected Circulation version (doi:10.1161/CIR.0000000000001297); Table 9 is identical in both. Defined wording, no class. It updates the 2015 AHA/ACC statement, whose rows are not shown. Its correction notice (doi:10.1161/CIR.0000000000001326) was not read.
Olivieri LJ, Lee S, Chen MH, et al. Multimodality approach to coronary ischemic testing in pediatric patients: a scientific statement from the American Heart Association. J Am Heart Assoc 2026;15(8):e047790. doi:10.1161/JAHA.125.047790. Open access. No classes.
Frommelt P, Lopez L, Dimas VV, et al. Recommendations for multimodality assessment of congenital coronary anomalies: a guide from the American Society of Echocardiography. J Am Soc Echocardiogr 2020;33(3):259-294. doi:10.1016/j.echo.2019.10.011. ASE-hosted PDF. No classes.
Gurvitz M, Krieger EV, Fuller S, et al. 2025 ACC/AHA/HRS/ISACHD/SCAI guideline for the management of adults with congenital heart disease. Circulation 2026;153(8):e115-e251 (also J Am Coll Cardiol 2026;87(7):822-976). doi:10.1161/CIR.0000000000001402. Archived publisher copy with the recommendation-table and Figure 7 images. Adults only (not for under 18); replaces the 2018 guideline. All 5 AAOCA rows shown. Its correction (doi:10.1161/CIR.0000000000001432) concerns the byline only.
Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J 2021;42(6):563-645. doi:10.1093/eurheartj/ehaa554. Archived publisher copy with the recommendation-table images. Adult. The 6 AAOCA rows and the general functional-imaging row are shown; the 3 rows on coronary arteries from the pulmonary artery are not.
Pelliccia A, Sharma S, Gati S, et al. 2020 ESC Guidelines on sports cardiology and exercise in patients with cardiovascular disease. Eur Heart J 2021;42(1):17-96. doi:10.1093/eurheartj/ehaa605. Archived publisher copy; all 4 rows of its table for young individuals and athletes with anomalous origin of coronary arteries (its term AOCA). Its text gives no exercise or sport recommendations for older patients (>40 years) with AOCA, for lack of studies, and the Class III footnote says under 40 years, so all four rows are shown as “young, <40 y”. No lower age is printed, so they are not labelled Adult.
Jegatheeswaran A, Devlin PJ, McCrindle BW, et al. Features associated with myocardial ischemia in anomalous aortic origin of a coronary artery: a Congenital Heart Surgeons' Society study. J Thorac Cardiovasc Surg 2019;158(3):822-834.e3. doi:10.1016/j.jtcvs.2019.02.122. Archived publisher PDF. Multicentre registry (CHSS 2019).
Molossi S, Agrawal H, Mery CM, et al. Outcomes in anomalous aortic origin of a coronary artery following a prospective standardized approach. Circ Cardiovasc Interv 2020;13(2):e008445. doi:10.1161/CIRCINTERVENTIONS.119.008445. Archived publisher copy. Single centre.
Doan TT, Wilkes JK, Reaves O'Neal DL, et al. Clinical presentation and medium-term outcomes of children with anomalous aortic origin of the left coronary artery: high-risk features beyond interarterial course. Circ Cardiovasc Interv 2023;16(5):e012635. doi:10.1161/CIRCINTERVENTIONS.122.012635. Archived publisher copy. Single centre.

Abstract only

Doan TT, Sachdeva S, Bonilla-Ramirez C, et al. Ischemia in anomalous aortic origin of a right coronary artery: large pediatric cohort medium-term outcomes. Circ Cardiovasc Interv 2023;16(4):e012631. doi:10.1161/CIRCINTERVENTIONS.122.012631. Single centre.

Not yet read in full, not shown

Mery CM, Nathan M, Gräni C, et al. Proposed standardized nomenclature for anomalous aortic origin of a coronary artery: an international multidisciplinary initiative. JACC Cardiovasc Imaging 2026;19(8):983-1001. doi:10.1016/j.jcmg.2026.02.005. A Delphi consensus on terms; this page keeps each guideline’s own terms.
Jegatheeswaran A, Devlin PJ, Williams WG, et al. Outcomes after anomalous aortic origin of a coronary artery repair: a Congenital Heart Surgeons' Society study. J Thorac Cardiovasc Surg 2020;160(3):757-771.e5. doi:10.1016/j.jtcvs.2020.01.114. Surgical complications after repair.
Mery CM, De León LE, Molossi S, et al. Outcomes of surgical intervention for anomalous aortic origin of a coronary artery: a large contemporary prospective cohort study. J Thorac Cardiovasc Surg 2018;155(1):305-319.e4. doi:10.1016/j.jtcvs.2017.08.116. Single-centre surgical cohort, 2 to 18 years.
Mery CM, Lawrence SM, Krishnamurthy R, et al. Anomalous aortic origin of a coronary artery: toward a standardized approach. Semin Thorac Cardiovasc Surg 2014;26(2):110-122. doi:10.1053/j.semtcvs.2014.08.001. The original standardized algorithm.
Lorber R, Srivastava S, Wilder TJ, et al. Anomalous aortic origin of coronary arteries in the young: echocardiographic evaluation with surgical correlation. JACC Cardiovasc Imaging 2015;8(11):1239-1249. doi:10.1016/j.jcmg.2015.04.027. Registry echo protocol.
Fuss C, McCallum R, Ghoshhajra BB, et al. ACR Appropriateness Criteria: evaluation of coronary artery anomalies. J Am Coll Radiol 2025;22(5):S234-S242. doi:10.1016/j.jacr.2025.02.030.
Maestrini V, Gati S, D’Ascenzi F, et al. Indications, protocols, and interpretation of cardiovascular imaging for the evaluation and management of athletes. A clinical consensus statement of the European Association of Preventive Cardiology (EAPC) and the European Association of Cardiovascular Imaging (EACVI) of the European Society of Cardiology (ESC). Part 2: cardiovascular magnetic resonance, cardiac CT and nuclear imaging. Eur Heart J Cardiovasc Imaging 2026;27(8):1469-1497. doi:10.1093/ehjci/jeag060.
Van Hare GF, Ackerman MJ, Evangelista JK, et al. Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 4: congenital heart disease. Circulation 2015;132(22):e281-e291. doi:10.1161/CIR.0000000000000240. Previous AHA/ACC version, updated by Kim 2025.

How it was checked

Classes, levels and numbers are copied from the sources (November 2026); wording is condensed. Classes appear as printed: ACHD 2025 uses 1, 2a, 2b; ESC 2020 I, IIa, IIb, III; AATS 2017 prints both “Class I” and “Class 1” (recommendations 5, 7, 8 and 15) for the same class, shown as printed. Kim 2025 wording (should, reasonable, can) is shown in grey chips; it is not a class.
Terms follow each source: AAOCA, AAOLCA and AAORCA (AATS 2017, ESC 2020, ASE 2020, CHSS 2019); “right” and “left AAOCA” (Kim 2025); “anomalous origin of the left coronary artery” (ACHD 2025); AOCA (ESC 2020 sports).
As printed: ACHD 2025 Figure 7 shows surgery 2a for the left coronary from the right sinus without ischemia and does not restate the high-risk-anatomy condition of its recommendation. Kim 2025 text says surgery “should be performed” before competition for interarterial left AAOCA; its Table 9 says “should be considered”. AATS 2017 Table 4 (sports for those restricted) is not shown.
Not covered: anomalous coronary artery from the pulmonary artery, coronary fistula and myocardial bridging.