Basics
Epidemiology
Prevalence
- Rightcoronary artery (RCA) from the left sinus, 0.17%
- Left coronaryartery (LCA) from the right sinus, 0.047%
Pathophysiology
- LMCA from right sinus of Valsalva: 1-3% of all major coronary anomalies
- RCA from left sinus of Valsalva: 30% of all major coronary anomalies
- Left circumflex coronary from RCA: 30% of all major coronary anomalies
- Single coronary artery arising from a single ostium: 5-20% of all coronary anomalies
Etiology
Congenital anomaly
Associated Conditions
Usually isolated
Diagnosis
- Anomalous origins of the LCA (ALCA) or RCA (ARCA) from the opposite sinus are infrequently diagnosed in children and adolescents:
- Many are discovered as incidental findings during ECG for innocent heart murmurs or screening for exercise participation.
- Sudden death, especially in young asymptomatic athletic individuals, may frequently be the initial manifestation, especially the LCA from the right sinus. Anomalous coronaries represent a relatively frequent cause of athletic-field deaths.
- Patients in whom the anomalous artery courses between the aorta and pulmonary artery (53%) are at greatest risk for exercise-induced sudden death or arrhythmias due to myocardial ischemia, but this is more likely due to the slitlike ostium than to the compression of the coronary artery between the aortic and pulmonary roots. The risk is significantly higher if there is a separate ostium of the LCA from the right sinus of Valsalva. An interarterial course of an ARCA is also associated with risk, since there is a separate ostial origin from the left sinus. However, the incidence of sudden death in the more common ARCA is much lower than that of ALCA. A single ostium is unlikely to be associated with sudden death. An intraseptal course for the ALCA is less likely to be associated with a coronary event.
- Premonitory symptoms such as chest pain and/or syncope or presyncope are reported in <30% patients. Symptoms are more likely to be reported by subjects with anomalous origin of the LMCA, and they usually occur in association with exercise.
- Single coronary artery: Mostly asymptomatic, except with atherosclerotic occlusion of the artery
History
Usually asymptomatic. Symptoms of angina may be described. Sudden unexpected death, often during exercise, occurs most commonly with ALCA.
Physical Exam
The physical exam is almost invariably normal.
Tests
Lab
- No pathognomonic blood tests for diagnosis, but peripheral markers of myocardial ischemia may aid in diagnosis
- Cardiac troponin, creatine phosphokinase, creatine kinase MB fraction levels may be elevated during episodes of ischemia
- EKG is most often within normal limits.
- Resting EKG may be abnormal in symptomatic patients with evidence of myocardial ischemia (ST-T changes)
Imaging
- CXR usually is not helpful.
- Transthoracic echo:
- Echo can diagnose most cases. Coronary anatomy is best visualized in the parasternal long, short, and high short-axis views.
- Echo identifies the origin of the coronary arteries (>1 view should be obtained to avoid problems due to lateral dropout).
Surgery
- In general, standard testing with echo is unlikely to provide clinical evidence of myocardial ischemia and is not reliable as a screening test in large athletic populations. A history of exertional syncope or chest pain requires exclusion of a coronary anomaly in athletes. Echo and stress imaging are indicated in suspected cases.
- Cardiac angiography may be utilized to obtain detailed coronary anatomy.
Differential Diagnosis
Other forms of congenital or acquired coronary disease.
Treatment
Additional Treatment
General Measures
Avoidance of vigorous exercise in most cases who have been clinically identified until surgical repair or a decision is made regarding medical management.
Surgery
- ALCA, which is associated with significant morbidity and mortality should be repaired once diagnosis is made regardless of symptoms.
- The management of an asymptomatic patient with ARCA from the opposite sinus with no evidence of ischemia remains controversial.
- Slit-like ostium, acute angle take-off, intramural aortic segment, and interarterial course are considered high-risk features.
- Many centers advocate surgical treatment when the coronary artery takes an interarterial course due to the risk of ischemia and sudden death. This can usually be accomplished by an unroofing procedure. However, in the absence of predictors of sudden death, the potential benefit from surgery must be balanced with possible early surgical morbidity and mortality, as well as the long-term risk of coronary arterial manipulation.
- In general, the risk of sudden death in unoperated patients with ARCA is very low compared to patients with ALCA. Furthermore, there is a significant incidence of subclinical changes indicative of myocardial ischemia in postoperative patients with ARCA after surgical unroofing procedures.
- Taken together, while it is reasonable to offer surgery to asymptomatic patients with ALCA at diagnosis, the risk-benefit analysis does not support elective surgery in asymptomatic patients with ARCA who have no evidence of stress-induced ischemia or arrhythmias.
- Anomalous LCA or RCA:
- When surgery is carried out, an unroofing procedure to move the coronary artery orifice to the appropriate sinus is the procedure of choice, especially in the presence of a slitlike ostium.
Ongoing Care
Follow-Up Recommendations
Patient Monitoring
Both operated and unoperated patients require ongoing medical follow-up and reevaluation when indicated by their clinical course.
Patient Education
- AHA/ACC guidelines do not differentiate between ARCA and ALCA and recommend exercise restriction. Given the differences in risk between ALCA vs. ARCA, it is important to restrict competitive sports for patients with ALCA prior to surgical repair.
- Exercise restriction in an asymptomatic patient with ARCA needs to be considered on an individual basis given the very low risk of sudden death vs. quality-of-life issues related to excessive exercise restriction. Avoid competitive sports for patients with residual coronary artery abnormalities.
Prognosis
In patients with anomalous origin of coronary arteries from the contralateral sinus and coronary AV fistulas, surgical repair is usually successful. Whereas short-term results have been encouraging, mid- and long-term prevention of sudden death is unknown. A single-center study of 24 children after repair of anomalous coronaries, notably ARCA, revealed subclinical changes of ischemia on stress ECG and imaging despite patent neo-coronary ostia after 15-month follow-up. The impact of these findings on subsequent risk of sudden death is unknown. These findings suggest the need for continued follow-up in patients even after repair of anomalous coronaries.
Additional Reading
1Brothers JA, McBride MG, Seliem MA. Evaluation of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients. J Am Coll Cardiol. 2007;50(21):2078-2082. [View Abstract]2Gersony WM. Management of anomalous coronary artery from the contralateral coronary sinus. J Am Coll Cardiol. 2007;50(21):2083-2084. [View Abstract]3Gowda RM, Chamakura SR, Dogan OM. Origin of left main and right coronary arteries from right aortic sinus of Valsalva. Int J Cardiol. 2003;92:305-306. [View Abstract]4Mirchandani S, Phoon C Management of anomalous coronary arteries from the contralateral sinus. Int J Cardiol. 2005;102:383-389. [View Abstract]5Varghese A, Keegan J, Pennell DJ Cardiovascular magnetic resonance of anomalous coronary arteries. Coron Artery Dis. 2005;16:35-64. [View Abstract]
Codes
ICD9
746.85 Coronary artery anomaly, congenital
SNOMED
28574005 congenital anomaly of coronary artery (disorder)