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J Thorac Cardiovasc Surg 2008;136:788-789
© 2008 The American Association for Thoracic Surgery
Brief Communication |
a Department of Pediatric Cardiology, Cliniques Universitaires St Luc, Université Catholique de Louvain, Brussels, Belgium
b Department of Cardiac Surgery, Cliniques Universitaires St Luc, Université Catholique de Louvain, Brussels, Belgium
Received for publication October 8, 2007; accepted for publication February 1, 2008. * Address for reprints: Stéphane Moniotte, MD, PhD, Department of Pediatric Cardiology, Université Catholique de Louvain, 10 avenue Hippocrate, B1200 Brussels, Belgium. (Email: stephane.moniotte{at}uclouvain.be).
Among the clinically relevant congenital coronary malformations, origin from the wrong aortic sinus seems to be the most common in young trained athletes who die suddenly. The reported incidence is 0.92% for anomalous origin of the right coronary artery (RCA) from the left sinus and 0.15% for the left coronary artery (LCA) from the right sinus.1
However, these data likely underestimate the true incidence of these abnormalities because these angiographic studies were presumably performed for other clinical indications. In a recent study of 6.3 million US Army recruits, Eckart and colleagues2 reviewed the medical records of 277 nontraumatic deaths and found that 34% were the result of coronary anomalies. No coronary anomaly other than anomalous origin of the LCA from the right sinus of Valsalva was associated with cardiac death.
Patient 1
A previously healthy 2-year-old girl presented for evaluation of a murmur. Physical examination revealed stable vital signs, normal heart sounds, and a continuous murmur at the left sternal border. Electrocardiographic, radiographic, and laboratory findings were normal. Transthoracic echocardiography showed a patent ductus arteriosus, and an aberrant LCA from the right sinus of Valsalva was suspected.
Cardiac catheterization demonstrated a single coronary ostium from the right sinus of Valsalva, with the left main coronary artery (LMCA) coursing between the aortic root and the main pulmonary artery (
Figure 1). Because of the inherent risk of sudden death associated with this malformation, surgery was proposed. The LMCA was found to be extramural, precluding coronary unroofing. The patent ductus arteriosus was clipped, and given the small coronary artery diameter and single coronary ostium, a coronary bypass operation was proposed at a later stage. At the 1-year follow-up, the patient was asymptomatic.
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The LMCA had an intramural course between the aorta and the main pulmonary artery, as confirmed by magnetic resonance coronary angiography (
Figure 2). Unroofing of the intramural segment of the LMCA was performed. The unobstructed origin of the LMCA was demonstrated by coronarography 1 year after its reimplantation. At the 5-year follow-up, the patient was free of symptoms (Figure E2).
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With the sophistication of noninvasive imaging techniques, the number of coronary artery anomalies identified is increasing. In a recent prospective series of 1950 patients studied by angiography, Angelini1 reported an incidence of 5.64% of coronary anomalies, with an LMCA arising from the right sinus in only 0.15% of the cases.
Although sudden death was frequently the first symptom, premonitory manifestations occurred in a substantial proportion of individuals with wrong sinus coronary artery origin. Basso and colleagues3 reported syncope or chest pain in 7 of 27 athletes within 24 months of sudden death. These symptoms were limited to anomalous LMCA origin, whereas anomalous origin of the RCA proved to be clinically silent. Other authors have reported patients with anomalous RCA who experienced angina, arrhythmia, or syncope before death.4,5
These observations emphasize the need to raise the index of clinical suspicion to identify wrong aortic sinus coronary artery origins in young, physically active individuals. They should systematically be excluded from participation in competitive sports until their specific coronary anatomy is delineated. A specific subgroup of patients have an intramural course of the ectopic coronary artery, often entailing a worse prognosis and requiring more aggressive treatment.5,6
The current approaches available for repair are 1) the reimplantation of the LMCA in the left sinus of Valsalva; 2) a coronary bypass procedure; and 3) an unroofing procedure resulting in relocation of the coronary artery into the appropriate sinus of Valsalva, with modified orifice shape and full patency after reconstruction.
The surgical approach may also vary depending on the patient's age and specific LMCA anatomy. At the Université Catholique de Louvain, the favored surgical technique is based on the specific anatomy of the LMCA (presence or absence of an intramural course between the ascending aorta and the main pulmonary artery) and the patient's age and symptoms (Table E1).
Although further multicentric studies are necessary to design the optimal management algorithm in patients with this rare entity, the unroofing procedure is preferred in the presence of an intramural segment.7 For technical reasons, surgery will be proposed later in life in young asymptomatic patients with an extramural course. Although surgical correction is warranted regardless of the presence of symptoms in the pediatric population, the optimal management of asymptomatic adults is still a matter of debate. Patients aged more than 30 years should probably undergo corrective surgery exclusively in the presence of symptoms.
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Suggested surgical approach based on the patient's age, symptoms, and aberrant left main coronary artery anatomy
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LMCA, Left main coronary artery.
Supplementary data
Supplementary data associated with this article can be found in the online version, at doi:10.1016/j.jtcvs.2008.02.007
References
This article has been cited by other articles:
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  J. Weber Potential for Using 3D Technology to Image the Origins of the Coronary Arteries Journal of Diagnostic Medical Sonography, May 1, 2011; 27(3): 114 - 121. [Abstract] [PDF]
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