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Case Report
Anomalous Origin of All Three Coronary Arteries from Right Sinus of Valsalva in a Patient with Angina Pectoris
January 2011
ABSTRACT: Anomalous origin of coronary arteries is uncommon, and all 3 coronary arteries arising from a single sinus of Valsalva is very rare. The anomaly has been associated with myocardial infarction and sudden cardiac death in particular when the anomalous left coronary artery courses between the great vessels. Imaging modalities have a complementary role to angiography in the diagnosis. Percutaneous interventions are technically difficult. Surgical options like direct repair of anomalous artery or coronary artery bypass graft can be helpful. We report a case of a patient who presented with chest pain and was found to have all 3 coronary arteries arising from the right sinus of Valsalva.
J INVASIVE CARDIOL 2011;23:E240–E242
Key words: anomalous origin of coronary arteries, angina pectoris,
right sinus of Valsalva
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Anomalous origin of coronary arteries is uncommon and all 3 coronary arteries arising from a single sinus of Valsalva is very rare. The anomaly has been associated with myocardial infarction and sudden cardiac death. We report a rare case in which a patient presented with chest pain and was found to have all 3 coronary arteries arising from the right sinus of Valsalva.
Case Report. A 46-year-old Caucasian gentleman without any significant past 
medical history presented with severe substernal chest discomfort. His pain was a pressure-like sensation, 8/10 in severity and radiating to his left arm. His vitals were within normal limits and physical examination was insignificant. An electrocardiogram showed nonspecific ST-T wave abnormality in inferior leads and tall T-waves in precordial leads. The peak troponin level was 15.9 ng/ml. Emergent cardiac catheterization was performed because of persistent chest pain, and revealed that the left anterior descending (LAD) artery originated from the right sinus of Valsalva. It was a 
small-caliber vessel with proximal occlusion. The left circumflex artery (LCX) also originated from the right sinus of Valsalva. The right coronary artery (RCA) was dominant and had mild luminal irregularities. Computed tomographic angiogram (CTA) was done to evaluate the specific course of the vessel and revealed anomalous left coronary artery (LCA) and left circumflex artery originating from a common origin with the right coronary from the right coronary cusp. The LAD was obstructed just after its origin and the course was not visualized. The patient was managed with optimal medical therapy.
Discussion. Coronary arteries with anomalous origin are a rare clinical entity, with the incidence report between 0.6–1.3% in angiographic series1 and 0.3% in autopsy series.2 Anomalous coronary arteries are involved in 12% of sports-related sudden cardiac deaths versus 1.2% non-sports related deaths. Anomalous origin of LCA from the right sinus of Valsalva is even more rare, with a reported prevalence of 
0.017–0.030% according to angiographic studies.3 An anomalous LCX is the most common anatomic variant, and it occurs in approximately 0.32–0.67% of patients who undergo coronary angiography.3 This anomalous LCX artery passes behind the aortic root and has not been associated with death. The most common course of an anomalous RCA arising from the left sinus of Valsalva is between the great arteries and it is associated with sudden cardiac death in up to 30% of patients. The LCA can originate from the right sinus of Valsalva or as a branch from a single coronary artery in 0.09–0.11% of angiographic studies.7 The interarterial course commonly occurs (in up to 75% of patients) where the vessel passes between the great vessels (aortic root and the right ventricular outflow tract), and is associated with sudden cardiac death. The LCA also may travel anterior to the right ventricular outflow tract or posterior to the aortic root, or it may follow a septal course.3
Anomalus origin of the LCA from the right sinus of Valsalva is consistently related to sudden death in 59% of patients, and follows exercise in 81% of event.8 Angeini et al11 used intravascular ultrasonography (IVUS) to clarify the mechanisms and severity of the anomaly. They described that, in cases of symptomatic left anomalous coronary artery originating from the opposite sinus of Valsalva, the proximal segment of the LCA consistently has 1) an intramural course inside the aortic wall; 2) hypoplasia, as determined by its circumference; and 3) a cross-sectional ovaloid deformity (lateral compression) with phasic and exercise-induced worsening of the deformity.11 The course of anomalous artery between 2 great vessels is associated with acute myocardial infarction and sudden cardiac death. The acute angle of the ostium, the stretch of the intramural segment and the compression between the commissure of the right and left coronary cusps increases the risk of sudden cardiac death. Exercise leads to expansion of the aortic root and pulmonary trunk, and may increase the existing angulation of the coronary artery, decreasing the luminal diameter in the proximal portion of the coronary artery. Resting electrocardiograms are usually normal and stress tests are not always positive.4
Cardiac catheterization is the gold standard for the evaluation of coronary anomalies. Multidetector row-computed tomography scan (MDCT), cardiac magnetic resonance (MR) and multi plane transesophageal echocardiography (TEE) have a complementary role with coronary angiogram for the diagnosis and delineation of the origin with the course of the anomalous coronary arteries. Multiplane TEE can be useful for the delineation of the proximal course and confirming the course between 2 great vessels.6 MDCT is a non-invasive alternative imaging technique to conventional coronary angiography for screening the anomalous vessels of coronary arteries because of its excellent spatial resolution, which is very important for detecting the relationship of anomalous vessels with great arteries and cardiac structures.7
The management of patients with an anomalous coronary artery includes medical treatment or observation, coronary angioplasty with stent deployment or surgical repair. The therapy of the anomalously originating LCA from the RSOV is mainly based on observations and experts opinion, since the literature consists of case reports.6,7 There are 2 surgical approaches that can be used for treatment of ACAOS of the LCA. One is the direct repair of the anomalous origin in the aortic root, and the other is coronary artery bypass surgery. Direct repair of the anomalous proximal coronary segment has been reported, unroofing of the whole intussuscepted segment or the creation of new ostium at the distal end of that segment. Coronary artery bypass surgery is technically more feasible, but there are disadvantages with the grafts. Arterial grafts tend to atrophy or fail to develop when used to bypass coronary lesions that are not severely obstructive at baseline. Vein grafts have limitations on longevity. Percutaneous transluminal coronary angioplasty is technically difficult, and there are only a few reports of PTCA of anomalous coronary arteries including rotational atherectomy and stenting (“rota-stenting”) and laser angioplasty.9,10Conclusion. Anomalous origin of all 3 coronary vessels from a single origin is a rare disease entity. The disease has a fatal outcome when there is an interarterial course of the LCA. Imaging modalities have a complementary role to angiography in the diagnosis of the disease. When diagnosed, the treatment should be provided as soon as possible to prevent a fatal outcome.
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From the Department of Medicine, Advocate Christ Medical Center/University of Illinois at Chicago, Oak Lawn, Illinois.
The authors report no conflicts of interest regarding the content herein.
Manuscript submitted March 3, 2010, provisional acceptance given April 12, 2010, final version accepted May 18, 2010.
Address for correspondence: Harshit Modi, MD, 15613 Plumtree Drive, Orland park, IL 60462. E-mail: modiharshit@hotmail.com
medical history presented with severe substernal chest discomfort. His pain was a pressure-like sensation, 8/10 in severity and radiating to his left arm. His vitals were within normal limits and physical examination was insignificant. An electrocardiogram showed nonspecific ST-T wave abnormality in inferior leads and tall T-waves in precordial leads. The peak troponin level was 15.9 ng/ml. Emergent cardiac catheterization was performed because of persistent chest pain, and revealed that the left anterior descending (LAD) artery originated from the right sinus of Valsalva. It was a 
small-caliber vessel with proximal occlusion. The left circumflex artery (LCX) also originated from the right sinus of Valsalva. The right coronary artery (RCA) was dominant and had mild luminal irregularities. Computed tomographic angiogram (CTA) was done to evaluate the specific course of the vessel and revealed anomalous left coronary artery (LCA) and left circumflex artery originating from a common origin with the right coronary from the right coronary cusp. The LAD was obstructed just after its origin and the course was not visualized. The patient was managed with optimal medical therapy.
Discussion. Coronary arteries with anomalous origin are a rare clinical entity, with the incidence report between 0.6–1.3% in angiographic series1 and 0.3% in autopsy series.2 Anomalous coronary arteries are involved in 12% of sports-related sudden cardiac deaths versus 1.2% non-sports related deaths. Anomalous origin of LCA from the right sinus of Valsalva is even more rare, with a reported prevalence of 
0.017–0.030% according to angiographic studies.3 An anomalous LCX is the most common anatomic variant, and it occurs in approximately 0.32–0.67% of patients who undergo coronary angiography.3 This anomalous LCX artery passes behind the aortic root and has not been associated with death. The most common course of an anomalous RCA arising from the left sinus of Valsalva is between the great arteries and it is associated with sudden cardiac death in up to 30% of patients. The LCA can originate from the right sinus of Valsalva or as a branch from a single coronary artery in 0.09–0.11% of angiographic studies.7 The interarterial course commonly occurs (in up to 75% of patients) where the vessel passes between the great vessels (aortic root and the right ventricular outflow tract), and is associated with sudden cardiac death. The LCA also may travel anterior to the right ventricular outflow tract or posterior to the aortic root, or it may follow a septal course.3
Anomalus origin of the LCA from the right sinus of Valsalva is consistently related to sudden death in 59% of patients, and follows exercise in 81% of event.8 Angeini et al11 used intravascular ultrasonography (IVUS) to clarify the mechanisms and severity of the anomaly. They described that, in cases of symptomatic left anomalous coronary artery originating from the opposite sinus of Valsalva, the proximal segment of the LCA consistently has 1) an intramural course inside the aortic wall; 2) hypoplasia, as determined by its circumference; and 3) a cross-sectional ovaloid deformity (lateral compression) with phasic and exercise-induced worsening of the deformity.11 The course of anomalous artery between 2 great vessels is associated with acute myocardial infarction and sudden cardiac death. The acute angle of the ostium, the stretch of the intramural segment and the compression between the commissure of the right and left coronary cusps increases the risk of sudden cardiac death. Exercise leads to expansion of the aortic root and pulmonary trunk, and may increase the existing angulation of the coronary artery, decreasing the luminal diameter in the proximal portion of the coronary artery. Resting electrocardiograms are usually normal and stress tests are not always positive.4
Cardiac catheterization is the gold standard for the evaluation of coronary anomalies. Multidetector row-computed tomography scan (MDCT), cardiac magnetic resonance (MR) and multi plane transesophageal echocardiography (TEE) have a complementary role with coronary angiogram for the diagnosis and delineation of the origin with the course of the anomalous coronary arteries. Multiplane TEE can be useful for the delineation of the proximal course and confirming the course between 2 great vessels.6 MDCT is a non-invasive alternative imaging technique to conventional coronary angiography for screening the anomalous vessels of coronary arteries because of its excellent spatial resolution, which is very important for detecting the relationship of anomalous vessels with great arteries and cardiac structures.7
The management of patients with an anomalous coronary artery includes medical treatment or observation, coronary angioplasty with stent deployment or surgical repair. The therapy of the anomalously originating LCA from the RSOV is mainly based on observations and experts opinion, since the literature consists of case reports.6,7 There are 2 surgical approaches that can be used for treatment of ACAOS of the LCA. One is the direct repair of the anomalous origin in the aortic root, and the other is coronary artery bypass surgery. Direct repair of the anomalous proximal coronary segment has been reported, unroofing of the whole intussuscepted segment or the creation of new ostium at the distal end of that segment. Coronary artery bypass surgery is technically more feasible, but there are disadvantages with the grafts. Arterial grafts tend to atrophy or fail to develop when used to bypass coronary lesions that are not severely obstructive at baseline. Vein grafts have limitations on longevity. Percutaneous transluminal coronary angioplasty is technically difficult, and there are only a few reports of PTCA of anomalous coronary arteries including rotational atherectomy and stenting (“rota-stenting”) and laser angioplasty.9,10Conclusion. Anomalous origin of all 3 coronary vessels from a single origin is a rare disease entity. The disease has a fatal outcome when there is an interarterial course of the LCA. Imaging modalities have a complementary role to angiography in the diagnosis of the disease. When diagnosed, the treatment should be provided as soon as possible to prevent a fatal outcome.
References
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8. Kacmaz F, Ozbulbul NI, Alyan O. Imaging of coronary artery anomalies: The role of multidetector computed tomography. Coron Artery Dis 2008;19:203–209. 9. Topaz O, Di Sciascio G, Goudreau E, et al. Coronary angioplasty of anomalous coronary arteries. Notes on technical aspects. Cathet Cardiovasc Diagn 1990;21:106–111. 10. Panduranga P, Riyami A. Separate origin of major coronary arteries from the right sinus with angioplasty and stenting of anomalous left circumflex and left anterior descending arteries. J Invasive Cardiol 2009;21:E33–E36. 11. Angelini P, Walmsley RP, Libreros A, Ott DA. Symptomatic anomalous origination of the left coronary artery from the opposite sinus of Valsalva. Clinical presentations, diagnosis and surgical repair. Tex Heart Inst J 2006;33:171–179.
