ABSTRACT: A 52-year-old female with exertional angina of class II severity was referred to us. Her coronary angiogram revealed an anomalous single coronary artery from left coronary sinus. Its circumflex branch continued in the right AV groove to supply the right coronary artery territory. Significant stenoses were found in the left anterior descending and circumflex arteries. In view of her persistent angina despite optimal antianginals, percutaneous intervention of the stenosed arteries was done, after which the symptoms abated. Single coronary artery is a rare coronary anomaly, with reported prevalence of 0.024%-0.066%. It is usually asymptomatic. Coronary angioplasty of such rare anomalous vessels for atherosclerotic disease has been reported previously. We describe stenting of both the major branches of an anomalous single coronary artery in a patient with atherosclerotic coronary artery disease with a brief review and discuss the issues with intervention on single coronary artery.
J INVASIVE CARDIOL 2013;25(7):E147-E150
Key words: anomalous single coronary artery, double-vessel disease
Isolated single coronary artery is a rare coronary anomaly1 in which only one coronary artery arises from the aortic trunk by a single ostium and supplies the entire myocardium. It has a prevalence of 0.024%-0.066% in the general population.1 Approximately 20 possible variations of single coronary artery have been described.2 There have been previous reports of percutaneous interventions on coronaries with this rare anomaly.3-11
Case Report. A 52-year-old female was referred to us with exertional angina of class II severity lasting 6 months. She was hypertensive and dyslipidemic. Her mother had a history of premature coronary artery disease (CAD), having undergone coronary artery bypass graft surgery (CABG) at age 46. The patient’s physical examination was unremarkable. Electrocardiography did not reveal any ST-T change. After her consent, we performed a coronary angiogram. To our surprise, it revealed a single coronary artery from left coronary sinus, while the right coronary sinus did not give rise to any artery (Figure 1). Instead, the circumflex (CX) branch of the single coronary artery continued in the atrioventricular (AV) groove along the course of a normal right coronary artery (RCA) supplying its territory (Figure 2). The other possibility could have been an occluded RCA at the ostium receiving collaterals from the CX. Computed tomography angiography indeed showed the right coronary sinus devoid of any ostium and CX artery winding around in the AV groove (Figure 3) across the crux, falling short of the right coronary sinus (Figure 4). An interarterial course of the vessel was also ruled out, the significance of which is discussed later.
Significant discrete tubular stenosis in the mid-left anterior descending (LAD) artery distal to the first diagonal and diffuse AV groove lesion in the CX distal to the first obtuse marginal origin were found. Since the patient continued to have disabling angina despite optimized antianginals, revascularization of the coronaries was performed. The left coronary artery (LCA) was hooked with a Judkins Left 3.5 guiding catheter. The LAD lesion was crossed with a BMW wire and directly stented with a 3.5 x 18 mm Taxus stent. An extra back-up (EBU) guide was used for CX intervention since a Judkins Left could not provide adequate support. The lesion was crossed with a BHW wire, predilated with a 2 mm balloon, and stented with 3 x 24 mm Taxus stent. The patient was relieved of her angina after the intervention.
Discussion. Single coronary arteries have multiple patterns of distribution. In 1979, Lipton et al3 proposed a classification for this anomaly later modified by Yamanaka and Hobbs.13 The aberrant artery is first designated as R or L depending on the sinus of origin (Figure 5). It is further classified into 3 types: type I = the vessel follows the course of normal left or right coronary artery with a continuation into the missing artery’s territory; type II = an anomalous artery arises from the proximal part of the other normal artery and courses the base of the heart before taking the native course; and type III = LAD and CX arteries originating from the proximal part of the RCA. Type III anomalies are exceedingly rare.2 Our patient had type LI anomaly.
Among the type II anomalies, the aberrant artery could take one of four pathways to reach its vascular territory. These pathways are designated as: type A (anterior to the right ventricular outflow tract); type B (between the aorta and pulmonary trunk); type C (cristal, coursing through the crista supraventricularis portion of the septum); and type D (dorsal or posterior to the aorta) (Figure 6).14 The course of the aberrant branch may have significant clinical implications.
Embryologic considerations. Most cases of single coronary artery are thought to be caused by one of two developmental anomalies. Either it is a congenital absence of one coronary artery anlage with the remaining vessel giving compensatory branches in missing artery territory, or the misplacement of one coronary artery anlage fuses with the first portion of the other normal vessel.15,16
Single coronary artery has been associated with some congenital heart diseases, like truncus arteriosus, bicuspid aortic valve, mitral valve prolapse, and ventricular septal defect. Although there are isolated reports of some particular coronary anomalies occurring in families, no definitive coronary inheritance pattern has been found in humans.17
Clinical course and diagnosis. Most patients are asymptomatic and have a benign clinical course. They are detected incidentally during coronary angiograms or investigation of other congenital heart defects. However, some patients with interarterial course of the anomalous artery may become symptomatic with syncope, angina pectoris, myocardial infarction, ventricular arrhythmias, and even sudden death without evidence of coronary atherosclerosis.18 They may be associated with ischemia and arrhythmias during exercise which may be due to the compression of the vessel between the dilated great arteries.19,20 A higher risk of coronary atherosclerosis has also been suggested with single coronary artery anomaly in a study,21 but that report has not been substantiated.
Computed tomography coronary angiography is the leading modality to confirm the diagnosis of single coronary artery. It can assist in delineating the proximal course of the artery.22
Elective percutaneous transluminal coronary angioplasty (PTCA) of a single coronary artery was first reported by Stauffer et al in 1991 followed by Gibbs et al.3,4 Raddino et al performed the PTCA and stenting of a branch of an anomalous single coronary artery in a patient presenting with unstable angina due to atherosclerotic coronary artery disease.10 Successful primary angioplasty involving arteries with this anomaly have also been reported.9-11 However, to the best of the our knowledge, this is the first report of stenting of both the major branches of an anomalous single coronary artery for atherosclerotic disease.
Guide selection is of paramount importance in interventions on a single coronary artery. An ostial or proximal dissection in such an artery might have disastrous consequences, since the whole myocardium is subtended by it. Soft-tip atraumatic guiding catheters should be preferred wherever possible. We had to use an extra back-up guide for the circumflex intervention to get the required support. Floppy wires should be preferred over stiffer wires for the same reason. Since the lesions were in the LAD and the proximal part of the CX, their intervention was not different from routine. However, an intervention in the distal CX in the RCA territory might have been more difficult due to poor support from the guide. A similar problem may be faced in Lipton type II and III anomalies. One must be very cautious while manipulating the guiding catheter within the ostium of such an artery in search of extra support for the reasons already cited. Using guiding catheters of appropriate shape considering the take-off and subsequent course of the target branch could be crucial to the successful intervention of such anomalous vessels.
Finally, if associated with malignant interarterial course, prompt surgical repair should be done. The options include coronary artery bypass graft with internal mammary graft preferred for anomalous left main artery, unroofing, or marsupialization23 of the coronary artery. Intracoronary stenting is also an alternative and has been performed to protect the vessel from extrinsic compression.24
Conclusion. Single coronary artery is a rare, usually asymptomatic congenital anomaly. It may be detected incidentally during angiography. Computed tomography coronary angiography is the modality of choice to confirm the diagnosis and delineate the course of the single coronary artery. Symptomatic coronary stenoses in such anomalous arteries are amenable to conventional percutaneous coronary angioplasty and stenting. It is also imperative to rule out an interarterial course and vascular compression in symptomatic individuals with this anomaly (in which case surgical treatment is justified) before planning coronary revascularization. In the absence of ischemia, this anomaly is benign.
From the 1Department of Cardiology and 2Department of Cardiac Radiology, All India Institute of Medical Sciences, New Delhi, India.Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest.The authors report no conflicts of interest re- garding the content herein. Manuscript submitted November 26, 2012, provisional acceptance given December 21, 2012, final version accepted January 11, 2013.
- Desmet W, Vanhaecke J, Vrolix M, et al. Isolated single coronary artery: a review of 50,000 consecutive coronary angiographies. Eur Heart J. 1992;13(12):1637-1640.
- Shirani J, Roberts WC. Solitary coronary ostium in the aorta in the absence of other major congenital cardiovascular anomalies. J Am Coll Cardiol. 1993;21(1):137-143.
- Stauffer JC, Sigward U, Vogt P, et al. Transluminal angioplasty of single coronary artery. Am Heart J. 1991;122(2):569-571.
- Gibbs HH, Spokojny AM, Molloy TJ, et al. Percutaneous transluminal coronary angioplasty of a right coronary artery arising from the left main coronary artery. Cathet Cardiovasc Diagn. 1993;30(1):37-39.
- Grenadier E, Beyar R, Amikan S, et al. Two-vessel PTCA of single anomalous coronary artery. Am Heart J. 1992;123(1):220-222.
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- Sasao H, Hasegawa T, Endo A, et al. Coronary stent implantation in patients with a single coronary artery: a report of 3 cases. Angiology. 2003;54(1):131-135.
- Lipton MJ, Barry WH, Obrez I, et al. Isolated single coronary artery: diagnosis, angiographic classification, and clinical significance. Radiology. 1979;130(1):39-47.
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- Roberts WC, Shirani J. The four subtypes of anomalous origin of the left main coronary artery from the right aortic sinus (or from the right coronary artery). Am J Cardiol. 1992;70(1):119-121.
- Spejr FD. Anomalous origin and course of left coronary artery in adult. New York Homeopathic Medical College and Flower Hospital. 1938:1:201.
- Roberts JT, Loube SD. Congenital single coronary artery in man. Report of nine new cases, one having thrombosis with right ventricular and atrial (auricular) infarction. Am Heart J. 1947;34(2):188.
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- Click RL, Holmes DR, Vliestra RE, et al. Anomalous coronary arteries: location, degree of atherosclerosis and effect on survival — a report from the Coronary Artery Surgery Study. J Am Coll Cardiol. 1989:13(3):531-537.
- Shittu A, Kadakia J, Budoff M. Single coronary artery syndrome: cardiac computed tomography angiography as a leading imaging modality. Catheter Cardiovasc Interv. 2011;78(5):764-769.
- Romp RL, Herlong JR, Landolfo CK, et al. Outcome of unroofing procedure for repair of anomalous aortic origin of left or right coronary artery. Ann Thorac Surg. 2003;76(2):589-595.
- Doorey AJ, Pasquale MJ, Lally JF, et al. Six-month success of intracoronary stenting for anomalous coronary arteries associated with myocardial ischemia. Am J Cardiol. 2000;86(5):580.
From the 1Department of Cardiology and 2Department of Cardiac Radiology, All India Institute of Medical Sciences, New Delhi, India.
Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no conflicts of interest regarding the content herein.
Manuscript submitted November 26, 2012, provisional acceptance given December 21, 2012, final version accepted January 11, 2013.
Address for correspondence: Dr Gautam Sharma, Additional Professor, Department of Cardiology, All India Institute of Medical Sciences, New Delhi 110029 India. Email: firstname.lastname@example.org