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1.  Visualization of anomalous origin and course of coronary arteries in 748 consecutive symptomatic patients by 64-slice computed tomography angiography 
Coronary artery anomalies (CAAs) are currently undergoing profound changes in understanding potentially pathophysiological mechanisms of disease. Aim of this study was to investigate the prevalence of anomalous origin and course of coronary arteries in consecutive symptomatic patients, who underwent cardiac 64-slice multidetector-row computed tomography angiography (MDCTA).
Imaging datasets of 748 consecutive symptomatic patients referred for cardiac MDCTA were analyzed and CAAs of origin and further vessel course were grouped according to a recently suggested classification scheme by Angelini et al.
An overall of 17/748 patients (2.3%) showed CAA of origin and further vessel course. According to aforementioned classification scheme no Subgroup 1- (absent left main trunk) and Subgroup 2- (anomalous location of coronary ostium within aortic root or near proper aortic sinus of Valsalva) CAA were found. Subgroup 3 (anomalous location of coronary ostium outside normal "coronary" aortic sinuses) consisted of one patient with high anterior origin of both coronary arteries. The remaining 16 patients showed a coronary ostium at improper sinus (Subgroup 4). Latter group was subdivided into a right coronary artery arising from left anterior sinus with separate ostium (subgroup 4a; n = 7) and common ostium with left main coronary artery (subgroup 4b; n = 1). Subgroup 4c consisted of one patient with a single coronary artery arising from the right anterior sinus (RAS) without left circumflex coronary artery (LCX). In subgroup 4d, LCX arose from RAS (n = 7).
Prevalence of CAA of origin and further vessel course in a symptomatic consecutive patient population was similar to large angiographic series, although these patients do not reflect general population. However, our study supports the use of 64-slice MDCTA for the identification and definition of CAA.
PMCID: PMC2799381  PMID: 20003347
2.  Incidence of anomalous origin of coronary artery in 1879 Chinese adults on dual-source CT angiography 
Netherlands Heart Journal  2010;18(10):466-470.
Background and Objective. Dual-source CT (DSCT) has been used to detect coronary artery anomalies. The purpose of this study was to assess the incidence of anomalous origin of the coronary artery in Chinese adults.
Methods. We summarised all patients who underwent DSCT coronary angiography (CTCA) from December 2006 to February 2008, and data of anomalous origin of the coronary artery in Chinese adults were recorded.
Results. 1879 patients underwent CTCA during that period; 24 patients with an anomalous origin of the coronary artery were detected, giving an incidence of 1.3%. Fifteen patients had an anomalous origin of the right coronary artery (12 from left coronary sinus, 3 high takeoff), eight patients had an anomalous origin of the left coronary artery (LCA from posterior sinus of Valsalva in three cases, LCX from the right coronary sinus, LCX from RCA, high takeoff, LCA from right coronary sinus, and single coronary artery in one case, respectively), and one patient had an anomalous origin of both coronary arteries (high takeoff).
Conclusion. The incidence of anomalous origin of the coronary artery in Chinese adults in this study is 1.3%. DSCT can clearly visualise the anomalous origin and course of the coronary artery and is a useful screening modality. (Neth Heart J 2010;18:466–70.)
PMCID: PMC2954298  PMID: 20978590
Coronary Artery Disease; Coronary Artery Anomaly; Tomography; X-ray Computed; Angiography
3.  Coronary artery anomalies: A diagnostic challenge 
Coronary artery anomalies (CAAs) are found in approximately 1% of all patients undergoing coronary angiography and in 0.3% of patients undergoing autopsy (Roberts, 1986). CAAs may be classified into those of origin and course, intrinsic coronary arterial anatomy, and coronary termination (Angelini et al. 2002). The most common malformation is abnormal origin and course, origin of a coronary artery from a wrong aortic sinus of Valsalva; either the right from the left coronary sinus or the left from the right coronary sinus; these anomalies must be excluded in young adults with typically ischemic- sounding chest pain or syncope. Anomalies coronary artery termination typically presented as coronary artery fistula, commonly the right coronary artery is affected, although, left sided coronary artery fistulae are well documented (Gandy et al. 2004). The anomalies of intrinsic coronary arterial anatomy, such as ostial stenosis, Artesia, and single, absent, or hypoplastic coronary arteries are rare but may have clinical importance.
PMCID: PMC3727461  PMID: 23960633
Coronary artery anomalies; Cardiac CT; Coronary Angiography (AC)
4.  Sudden Cardiac Arrest Associated with Anomalous Origin of the Right Coronary Artery from the Left Main Coronary Artery 
Texas Heart Institute Journal  2011;38(5):539-543.
Anomalous origin of the coronary artery from the opposite sinus of Valsalva and a course of that artery between the ascending aorta and the pulmonary artery is a rare congenital anomaly. It can cause myocardial ischemia, syncope, and sudden cardiac death in young people. Herein, we report the case of a 24-year-old man who was brought to our hospital after cardiac arrest due to ventricular fibrillation. Emergent coronary angiography revealed that the left coronary artery was normal; however, the right coronary artery originated at the left sinus of Valsalva. After admission, the patient was treated with mild therapeutic hypothermia for 48 hours and had a favorable neurologic recovery. Subsequent 16-slice multidetector computed tomography revealed that the right coronary artery arose from the left main coronary artery, took an intramural course, and was severely compressed between the ascending aorta and the pulmonary artery. The patient underwent direct implantation of the anomalous artery into the correct aortic sinus. Histologic specimens from the proximal end of the right coronary artery showed an intramural segment with intimal fibrous thickening, fragmentation and random arrangement of the elastic fiber, degeneration of the medial smooth-muscle cells, and an increase in the medial stromal substance. Postoperatively, repeat coronary angiography with provocation testing for coronary spasm revealed no myocardial ischemic change. The patient recovered uneventfully. We found that cardiac multidetector computed tomography was useful in evaluating the cause of the sudden cardiac arrest, identifying the anomalous coronary artery, and helping to guide the surgical decisions.
PMCID: PMC3231532  PMID: 22163129
Coronary angiography; coronary vessel anomalies/diagnosis/physiopathology/surgery; death, sudden, cardiac/prevention & control; replantation; risk assessment; sinus of Valsalva/abnormalities/surgery; treatment outcome
5.  A retrospective study of angiographic ally determined anomalous coronary arteries in 12,844 subjects in Thrace region of Turkey 
Hippokratia  2012;16(3):246-249.
Background: Congenital anomalies of the coronary arteries are rarely encountered in patients undergoing cardiac catheterization. In patients undergoing coronary angioplasty or cardiac surgery, angiographic recognition of coronary anomalies is important for the proper management of these patients.
Method: We retrospectively reviewed the records of 12,844 patients who had previously undergone coronary angiography in the catheterization laboratory of Trakya University Cardiology Department over the past 14 years. We tried to investigate the presence of a variety of coronary anomalies in these patients to determine the prevalence of various types of anomalies and their anatomic variation in a selected population of the European part of Turkey. The potential association between coronary atherosclerosis and congenital coronary anomalies was also investigated.
Results: Among these patients, 95 patients were found to have major coronary anomalies that predominantly comprised anomalous aortic origin of coronary arteries. Among the major anomalies, anomalous aortic origin of the left circumflex (LCX) artery from the right sinus of Valsalva or right coronary artery (RCA) was found to be the the most prevalent (46 out of 95 patients) outnumbering the second most common anomaly that was anomalous aortic origin of the RCA (32 out of 95 patients). In the present study, the incidence of major coronary arterial anomaly was found to be 0.74 %. However, only about one third of the patients (31 out of 95, 32.6%) with major anomaly had significant coronary atherosclerotic lesions among whom nine were found to involve the LCX artery with a posterior course.
Conclusion: The incidence of congenital coronary anomalies in a selected population of the European part of Turkey is similar to those of other populations. Congenital coronary anomalies generally present as isolated anomalies and are not associated with an increased risk of coronary atherosclerosis in this series. Cardiologists and surgeons should be familiar with these entities for the proper management of patients undergoing cardiac surgery or coronary angioplasty.
PMCID: PMC3738732  PMID: 23935292
Congenital coronary anomaly; coronary atherosclerosis; incidental finding
6.  Symptomatic Anomalous Origination of the Left Coronary Artery from the Opposite Sinus of Valsalva 
Texas Heart Institute Journal  2006;33(2):171-179.
Anomalous origination of a coronary artery can have serious, even fatal, consequences. Intravascular ultrasonography has recently provided new insights into anomalous coronary artery origination from the opposite sinus of Valsalva. On the basis of these insights, we describe 3 typical forms of this anomaly with left coronary artery involvement, including clinical presentations, diagnostic methods (particularly intravascular ultrasonography), and details of surgical treatment. In this case series, the left coronary artery originated from the noncoronary sinus in 1 patient and from the right sinus in another patient. In the 3rd patient, both the left and right coronary arteries originated from the ascending aorta above the sinotubular junction. Baseline areas of stenosis ranged from 48.6% to 70.1%. Intravascular ultrasonography was the only method that enabled us to clarify the mechanisms and the severity of the anomaly. Pharmacologic challenge was useful to predict worsening that might have occurred under physiologic conditions.
We found that, in cases of symptomatic left anomalous coronary artery origination from the opposite sinus of Valsalva, the proximal segment of the left coronary artery 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. With regard to surgical treatment, ostioplasty is preferable to coronary bypass.
To establish sound guidelines for managing these anomalies, a larger series should be studied prospectively with quantitative parameters and long-term follow-up.
PMCID: PMC1524694  PMID: 16878619
Anomalous left coronary artery; coronary angiography; coronary artery bypass; coronary vessel anomalies/classification/diagnosis/pathology/surgery/ultrasonography; death, sudden, cardiac/prevention & control; diagnostic imaging/methods; magnetic resonance imaging; myocardial ischemia/mechanism
7.  In vitro study of coronary flow occlusion in transcatheter aortic valve implantation 
Journal of Thoracic Disease  2014;6(12):1772-1777.
Transcatheter aortic valve implantation (TAVI) has been developed recently for patients with high morbidities and who are believed to be not tolerate standard surgical aortic valve replacement. Nevertheless, the TAVI is associated with complications such as potential obstruction of coronary ostia, mitral valve insufficiency, and stent migration although it seems promising. Impairment of the coronary blood flow after TAVI is catastrophic and it was believed to be associated with the close position of the coronary orifice and the aortic leaflets and valve stent. However, few data was available as to the anatomic relationship between valve stent and aortic root anatomic structures including the coronary arterial ostia, aortic leaflets.
The aortic roots were observed in 40 hearts specimens. The width of aortic leaflet, height of aortic sinus annulus to the sinutubular junction (STJ), distance between aortic sinus annulus to its corresponding coronary ostia, and coronary arterial ostia to its corresponding STJ level were measured. Moreover, the relationships of valve stent, aortic leaflets and coronary ostia before/post stent implantation and after the open of aorta were evaluated respectively.
Approximate three quarters of the coronary ostia were located below the STJ level. The mean distances from left, right and posterior aortic sinus annulus to the related STJ level was comparable, which was 18.5±2.7, 18.9±2.6, 18.7±2.6 mm, respectively. Meanwhile, the height of left and right aortic sinus annulus to its corresponding coronary ostia was 16.6±2.8 and 17.2±3.1 mm for left and right side respectively.
Most of the coronary ostia were located below the STJ level and could be covered by the leaflets. This highlights the need of modified stents to prevent occlusion of coronary flow after TAVI.
PMCID: PMC4283334  PMID: 25589972
Aortic valve; coronary ostia; transcatheter aortic valve implantation (TAVI); stent
8.  Anatomy of the coronary arteries in transposition of the great arteries and methods for their transfer in anatomical correction. 
Thorax  1978;33(4):418-424.
For the success of anatomical correction of transposition of the great arteries (TGA) it is essential to transfer the coronary ostia to the posterior vessel without undue tension, torsion, or kinking of the proximal coronary arteries or their early branches. This requires thorough understanding of the different modes of origin and early branching of the coronary arteries in TGA. Based on observations made during anatomical correction, a classification of the coronary arteries in TGA is suggested. In type A the right and left coronary ostia arise from the middle of the right and left posterior aortic sinuses and curve forwards to reach the right atrioventricular groove or anterior interventricular groove respectively. In type B both coronary arteries arise by a single ostium, while in type C the two coronary ostia are situated posteriorly, very close to each other, in a position similar to that in type B. The origin of the coronary arteries in type D is similar to that of type A. However, the right coronary artery gives origin to the circumflex coronary artery that curves round the posterior (pulmonary) vessel to reach the atrioventricular groove. In type E the right coronary artery arises in common with the left anterior descending artery from the left posterior sinus, while the circumflex artery arises separately from the right posterior sinus. Three techniques for the transfer of the different types of coronary arteries during anatomical correction are described.
PMCID: PMC470907  PMID: 694795
9.  Kugel's Artery 
Texas Heart Institute Journal  2004;31(3):267-270.
In this study, we tried to resolve the confusion in the literature regarding the existence and course of Kugel's artery.
With the aid of a new technique, we studied 100 human hearts ex vivo by radiography and by direct observation through dissection, to demonstrate anatomical and postmortem angiographic findings of Kugel's artery.
Kugel's artery was found in only 6 hearts out of 100 (6%). It originated from the proximal left circumflex artery and ended in the right coronary artery in 2 cases; from the right coronary artery and ended in the same artery in 2 cases; from the left circumflex artery and ended in the same artery in 1 case; and from the right coronary artery through the sinus node artery, ending in the left circumflex artery, in 1 case. In all 100 hearts, an anastomotic network of small atrial branches was found in the same area (lower portion of the interatrial septum), connecting the large vessels indirectly. Branches of the sinus node artery in all hearts, and of the atrioventricular node artery in 66 hearts, participated in this network. Our procedure showed the detailed course of Kugel's artery and its course independent from the atrioventricular node artery and from the anastomotic network.
In conclusion, in all cases an anastomotic network of small atrial branches courses through the lower interatrial septum and connects indirectly the proximal and distal ends of the larger coronary arteries. Kugel's artery provides an additional direct arterial anastomosis in the same area in 6% of the hearts.
PMCID: PMC521768  PMID: 15562847
Angiocardiography; atrioventricular node/anatomy & histology; autopsy; cadaver; collateral circulation; coronary angiography; coronary circulation; coronary vessels/anatomy & histology
10.  Unusual congenital coronary anomaly and myocardial ischaemia 
Heart  1999;82(5):e7.
Angiography was used to diagnose a rare congenital coronary anomaly with myocardial ischaemia in a woman with typical angina. All three coronary arteries arose from a solitary coronary ostium in the right aortic sinus; the left anterior descending coronary artery followed a septal course, the circumflex coronary artery ran behind the ascending aorta, and the right coronary artery followed a normal course. No significant coronary lumen narrowing was found. Transoesophageal echocardiography confirmed the anomalous origin and course of the aberrant coronary arteries. An exercise test reproduced angina, and ECG changes and myocardial perfusion study showed an anterior reversible defect. In contrast to previous reports, myocardial ischaemia was associated with the septal (intramuscular) course of the left anterior descending coronary artery; there was no other significant coronary artery disease.

Keywords: congenital heart defects; myocardial ischaemia; angiography; echocardiography
PMCID: PMC1760764  PMID: 10525527
11.  Primary congenital anomalies of the coronary arteries and relation to atherosclerosis: an angiographic study in Lebanon 
Most coronary artery anomalies are congenital in origin. This study angiographically determined the prevalence of different forms of anomalous aortic origins of coronary anomalies and their anatomic variation in a selected adult Lebanese population. Correlation between these anomalies and stenotic coronary atherosclerotic disease was also investigated.
4650 coronary angiographies were analyzed for anomalous aortic origin. These anomalies were clustered in four main groups: anomalous left circumflex (LCX) coronary artery, anomalous right coronary artery, anomalous left main coronary artery and anomalous left anterior descending coronary artery.
Thirty four patients had anomalous aortic origin of coronary arteries. Of these, anomalous LCX coronary artery was the most common (19 of 34 patients). The second most common anomaly was anomalous RCA origin (9 of 34 patients.) The incidence of coronary stenosis in non-anomalous vessels was 50%. However, a significantly smaller percentage (17.46%; 6 of 34 patients) of anomalous vessels exhibited significant stenosis, reminiscent of atherosclerotic disease. Of these six vessels, five were LCX coronary artery arising from right coronary sinus or from early branch of right coronary artery. The sixth was right coronary artery arising from left coronary sinus.
The incidence of congenital coronary anomalies in Lebanon is similar to other populations where the most common is the LCX coronary artery. Isolated congenital coronary anomalies do not increase the risk of developing coronary stenosis or atherosclerosis. Angiographic detection of these anomalies is clinically important for coronary angioplasty or cardiac surgery.
PMCID: PMC2775738  PMID: 19874587
12.  Coronary arterial pattern in superoinferior ventricular heart. Implications on significance of morphogenesis of this anomaly. 
British Heart Journal  1981;46(5):559-561.
The coronary arterial pattern was studied in two patients with superoinferior ventricular heart and haemodynamically complete transposition. In one of them the aorta was dextro-positioned and in the other laevo-positioned. In both the right coronary artery arose from the posterior aortic sinus and the left coronary artery from the left aortic sinus. This pattern is similar to the common coronary arterial pattern in complete transposition of the great arteries. The similarity supports the theory that in the superoinferior ventricular heart the connections between the ventricles and the great arteries (concordant or discordant) area those of the basic condition--complete transposition or corrected transposition. The angiocardiographic findings indicate that relations are not always as expected for a given connection.
PMCID: PMC482696  PMID: 7317222
13.  Left Main Coronary Artery Originating from the Proper Sinus but with Acute Angulation and an Intramural Course, Leading to Critical Stenosis 
Texas Heart Institute Journal  2010;37(2):221-225.
Because of the variety of their anatomy and clinical implications, coronary anomalies tend to confuse many observers. Recently, our group and other investigators have proposed that only 1 specific type of anomaly, by means of a specific mechanism, is able to cause both symptoms of myocardial ischemia and sudden death. This anomaly is known as anomalous origin of a coronary artery from the opposite sinus of Valsalva, with intramural course (ACAOS). Its defining pathophysiologic feature is that the proximal section of the ectopic artery has an intramural course, which leads to variable degrees of functional obstruction. Herein, we describe an unusual, previously unreported coronary anomaly: a “normal origin” of the left main coronary artery from the left sinus of Valsalva that resulted in progressive, critical ischemia. The proximal few millimeters of this artery were intramural, embedded into the aortic-sinus wall, and laterally compressed. Therefore, this anomaly may be regarded also as “ACAOS of the left coronary artery without an ectopic origin.” Angiography and intravascular ultrasonography revealed a variable degree of obstruction without intimal thickening and, likely, without spasm. Surgical repair, including ostioplasty, completely relieved the patient's clinical symptoms.
PMCID: PMC2851442  PMID: 20401300
Angina pectoris/etiology/pathology; coronary angiography/methods; coronary stenosis/surgery; coronary vessel anomalies/complications/diagnosis/epidemiology/physiopathology/surgery; coronary vessels/anatomy & histology; myocardial ischemia/epidemiology/pathology; sinus of Valsalva; ultrasonography, interventional
14.  Percutaneous revascularization in a patient with anomalous origin of left main coronary artery 
World Journal of Cardiology  2011;3(9):311-314.
Anomalous origin of the coronary artery from opposite coronary sinus is infrequently observed during coronary angiography. Percutaneous coronary intervention (PCI) of anomalous coronary artery is technically difficult and challenging. It requires appropriate selection of guide catheters for adequate stability, coaxial alignment and backup support during the intervention. We hereby report a rare case of anomalous origin of left main coronary artery (LM) from the right coronary sinus, having a retro-aortic course to the left side before its bifurcation into left anterior descending (LAD) and circumflex artery. The 59-year-old man had successful PCI of atherosclerotic LAD lesions. A 64-slice Multi-Detector Computed Tomography (MDCT) performed at 4 years of follow-up demonstrated patency of coronary stents and also delineated the origin and course of the anomalous LM. The case illustrates the rarity of anomalous LM, and describes technical issues during PCI and the role of MDCT in coronary anomaly imaging.
PMCID: PMC3176899  PMID: 21949573
Anomalous left main coronary artery; Atherosclerosis; Coronary artery anomalies; Drug-eluting stent; Multi-detector computed tomography; Percutaneous coronary intervention; Retro-aortic course
15.  Hearts with concordant ventriculoarterial connections but parallel arterial trunks 
Heart  2006;93(1):100-106.
To determine the characteristic morphological features of hearts with concordant ventriculoarterial connections and parallel arterial trunks, and to provide unequivocally a method to describe their anatomy.
Design, methods and patients
The entire cardiac database and cardiac pathological archive at the Hospital for Sick Children, Toronto, Ontario, Canada, was interrogated to identify all patients with concordant ventriculoarterial connections and parallel arterial trunks. The clinical records, autopsy reports and actual cardiac specimens of those who underwent autopsy, were reviewed.
8 cases meeting our criteria were identified. The infundibular anatomy was variable, including four hearts with bilateral infundibulums, three with subpulmonary infundibulums and one with bilaterally absent infundibulums. Considerable variability was also found in the type of atrial arrangement, along with the morphology of the atrioventricular junctions. The most common findings were the usual atrial arrangement (n = 5), left juxtaposition of the right atrial appendages (n = 3), an atrial septal defect (n = 6), univentricular atrioventricular connection (n = 5), ventricular septal defect (n = 8) and pulmonary obstruction (n = 4). In addition, five specimens had either a single coronary artery or two coronary arteries arising from the anticipated right coronary aortic sinus.
Concordant ventriculoarterial connections with parallel arterial trunks can be found in a variety of segmental combinations. An accurate diagnosis of these rare hearts can be achieved by detailed analysis of not only the ventriculoarterial connections but also the infundibular anatomy and the spatial relationship of the arterial trunks. Particular attention to the coronary arteries is warranted.
PMCID: PMC1861342  PMID: 16807270
16.  Coronary Ostial Stenosis after Aortic Valve Replacement 
Texas Heart Institute Journal  2010;37(4):465-468.
Coronary ostial stenosis is a rare but potentially serious sequela after aortic valve replacement. It occurs in the left main or right coronary artery after 1% to 5% of aortic valve replacement procedures. The clinical symptoms are usually severe and may appear from 1 to 6 months postoperatively. Although the typical treatment is coronary artery bypass grafting, patients have been successfully treated by means of percutaneous coronary intervention.
Herein, we present the cases of 2 patients in whom coronary ostial stenosis developed after aortic valve replacement. In the 1st case, a 72-year-old man underwent aortic valve replacement and bypass grafting of the saphenous vein to the left anterior descending coronary artery. Six months later, he experienced a non-ST-segment-elevation myocardial infarction. Coronary angiography revealed a critical stenosis of the right coronary artery ostium. In the 2nd case, a 78-year-old woman underwent aortic valve replacement and grafting of the saphenous vein to an occluded right coronary artery. Four months later, she experienced unstable angina. Coronary angiography showed a critical left main coronary artery ostial stenosis and occlusion of the right coronary artery venous graft. In each patient, we performed percutaneous coronary intervention and deployed a drug-eluting stent. Both patients were asymptomatic on 6-to 12-month follow-up. We attribute the coronary ostial stenosis to the selective ostial administration of cardioplegic solution during surgery. We conclude that retrograde administration of cardioplegic solution through the coronary sinus may reduce the incidence of postoperative coronary ostial stenosis, and that stenting may be an efficient treatment option.
PMCID: PMC2929858  PMID: 20844624
Angioplasty, transluminal, percutaneous coronary; aortic valve/surgery; cardiac surgical procedures/adverse effects; coronary artery disease/etiology/prevention & control; coronary stenosis/diagnosis/etiology/therapy; heart valve prosthesis implantation/adverse effects; iatrogenic disease/prevention & control; perfusion/adverse effects/instrumentation; postoperative complications/therapy; treatment outcome
17.  Coronary Artery Anomalies 
Texas Heart Institute Journal  1988;15(3):166-173.
We reviewed the records of 10,661 patients who had undergone coronary angiography at the Clayton Foundation Cardiovascular Laboratories between 1 June 1974 and 15 March 1986, and identified major coronary artery anomalies in 83 adults. In addition, we included in our review 9 adults and 2 adolescents who had been referred for evaluation of anomalies documented elsewhere. Here we present the clinical and angiographic data for all 94 patients (76 men and 18 women). Most patients were men who presented with chest pain. The most common anomaly, found in 38 patients, was origin of left circumflex coronary artery from right coronary artery or right aortic sinus. In contrast to other studies, which have not shown increased incidence of coronary atherosclerosis in the anomalous circumflex artery, 71% of our patients with this anomaly had significant coronary atherosclerosis in the proximal portion of the anomalous vessel. The posterior course of the anomalous circumflex coronary artery may predispose this vessel to atherosclerosis in patients with coronary disease. The overall incidence of atherosclerotic disease in coronary arteries was 68% (64 of 94 patients) in the present study. (Texas Heart Institute Journal 1988;15:166-173)
PMCID: PMC324820  PMID: 15227247
Coronary vessel anomalies; coronary vessels/abnormalities; heart defects, congenital; cross sectional studies; coronary vessels/radiography; coronary arteriosclerosis; cineangiography; angina pectoris; arteriovenous fistula; coronary disease
18.  Peritruncal Coronary Endothelial Cells Contribute to Proximal Coronary Artery Stems and Their Aortic Orifices in the Mouse Heart 
PLoS ONE  2013;8(11):e80857.
Avian embryo experiments proved an ingrowth model for the coronary artery connections with the aorta. However, whether a similar mechanism applies to the mammalian heart still remains unclear. Here we analyzed how the main coronary arteries and their orifices form during murine heart development. Apelin (Apln) is expressed in coronary vascular endothelial cells including peritruncal endothelial cells. By immunostaining, however, we did not find Apln expression in endothelial cells of the aorta during the period of coronary vessel development (E10.5 to E15.5). As a result of this unique expression difference, AplnCreERT2/+ genetically labels nascent coronary vessels forming on the heart, but not the aorta endothelium when pulse activated by tamoxifen injection at E10.5. This allowed us to define the temporal contribution of these distinct endothelial cell populations to formation of the murine coronary artery orifice. We found that the peritruncal endothelial cells were recruited to form the coronary artery orifices. These cells penetrate the wall of aorta and take up residence in the aortic sinus of valsalva. In conclusion, main coronary arteries and their orifices form through the recruitment and vascular remodeling of peritruncal endothelial cells in mammalian heart.
PMCID: PMC3836754  PMID: 24278332
19.  Coronary artery disease in congenital single coronary artery in adults: A Dutch case series 
World Journal of Cardiology  2014;6(4):196-204.
AIM: To assess the current diagnostic and therapeutic management and the clinical implications of congenital single coronary artery (SCA) in adults.
METHODS: We identified 15 patients with a SCA detected from four Dutch angiography centers in the period between 2010 and 2013. Symptomatic patients who underwent routine diagnostic coronary angiography (CAG) for suspected coronary artery disease and who incidentally were found to have isolated SCA were analyzed.
RESULTS: Fifteen (7 females) with a mean age of 58.5 ± 13.78 years (range 43-86) had a SCA. Conventional CAG demonstrated congenital isolated SCA originating as a single ostium from the right sinus of Valsalva in 6 patients and originating from the left in 9 patients. Minimal to moderate coronary atherosclerotic changes were found in 4, and severe stenotic lesions in another 4 patients. Seven patients were free of coronary atherosclerosis. Runs of non-sustained ventricular tachycardia were documented in 2 patients, one of whom demonstrated transmural ischemic changes on presentation. Myocardial perfusion scintigraphic evidence of transmural myocardial ischemia was found in 1 patient due to kinking and squeezing of the SCA with an interarterial course between the aorta and pulmonary artery. Multi-slice computed tomography (MSCT) was helpful to delineate the course of the anomalous artery relative to the aorta and pulmonary artery. Percutaneous coronary intervention was successfully performed in 3 patients. Eight patients were managed medically. Arterial bypass graft was performed in 4 patients with the squeezed SCA.
CONCLUSION: SCA may be associated with transient transmural myocardial ischemia and aborted sudden death in the absence of coronary atherosclerosis. The availability and sophistication of MSCT facilitates the delineation of the course of a SCA. We present a Dutch case series and review of the literature.
PMCID: PMC3999339  PMID: 24772259
Congenital heart disease; Coronary artery anomaly; Coronary angiography; Single coronary artery; Coronary artery disease; Multi-slice computed tomography
20.  Aberrant right coronary artery origin from the left aortic sinus with interarterial course manifesting acute myocardial infarction 
A 43-year-old patient was admitted to hospital because of an inferior-posterior myocardial infarction. The admission electrocardiogram was suggestive of a right coronary artery (RCA) culprit lesion. Coronary angiography following successful thrombolysis revealed a normal left system and mild intraluminal disease of the dominant RCA, which arose from the left aortic sinus and travelled an interarterial course; the latter was depicted in a subsequent computed tomographic angiogram. The lack of ST segment elevation in V4R and the absence of right ventricular wall motion abnormalities on echocardiography precluded the proximal ectopic vessel from being the culprit. The patient was managed medically; one year following discharge, he is asymptomatic. In cases of aberrant anomalous origin of a coronary artery from the opposite sinus with interarterial course, the proximal ectopic vessel is intussuscepted within the aortic wall, potentially leading to ischemia. The present article highlights that, although medical treatment in cases of such an aberrant RCA without apparent ischemia-driven sequelae may be valid, the need for interventional treatment could be substantiated following investigation of the anatomofunctional features of the intussuscepted proximal ectopic segment with intravascular ultrasound.
PMCID: PMC2827213  PMID: 20198200
Anomalous coronary course; Anomalous right coronary artery; Coronary angiography; CT coronary angiography; Inferior myocardial infarction
21.  The primary anomalies of coronary artery origin and course: A coronary angiographic analysis of 16,573 patients 
Coronary artery anomalies are present at birth, but relatively few are symptomatic. The majority are discovered incidentally. In the present study, coronary angiograms performed in the authors’ centre (Ondokuz Mayis University Hospital, Samsun, Turkey) were analyzed to determine the prevalence and types of coronary artery origin and course anomalies.
Coronary angiographic data of 16,573 patients were analyzed. Anomalous origins and courses of coronary arteries were assessed.
Anomalous coronary arteries were detected in 48 (0.29%) of 16,573 patients. The origin of the circumflex (Cx) artery from the right coronary artery (RCA) or right sinus of Valsalva was the most common anomaly (28 patients [58.3%]). An anomalous RCA originating from the left anterior descending artery (LAD) or Cx artery was observed in six patients (12.5%). The left coronary artery originated from the right sinus of Valsalva in five patients, and the LAD originated from the RCA or the right sinus of Valsalva in five patients. The RCA originated from the left sinus of Valsalva in three patients and from an ectopic ostium in the ascending aorta in one patient.
The most frequent anomaly observed in the present study was related to the Cx artery, which is consistent with previous reports. Although coronary artery anomalies are rare, they may cause difficulties during coronary interventions or cardiac surgery and may occasionally result in sudden cardiac death. Therefore, the recognition and diagnosis of these anomalies is important and requires specialization in coronary angiographic techniques and other imaging modalities.
PMCID: PMC3718591  PMID: 23940436
Coronary angiography; Coronary artery anomaly; Sinus of Valsalva
22.  Cardiac arrest associated with sildenafil ingestion in a patient with an abnormal origin of the left coronary artery: case report 
Left coronary artery arising from the right sinus of Valsalva is an uncommon congenital coro-nary anomaly that seems to be associated with sudden death in young patients.
Case presentation
We report a case of cardiac arrest in a 59-year-old patient after sexual intercourse and Silde-nafil ingestion. A coronary arteriography and an angiographic computed tomography scan subsequently revealed a LCA origin from the right aortic sinus along with an intramural course of the left main stem. In addition a distal stenosis of the right coronary artery was detected. After successful resuscitation without neurological deficits coronary artery bypass surgery was performed.
To our knowledge, this is the first report demonstrating sudden cardiac arrest associated with Sildenafil ingestion in a patient with this type of coronary anomaly. The question arises, whether a cardiac screening is necessary before a Sildenafil therapy is initiated.
PMCID: PMC3166914  PMID: 21824399
23.  Sudden Cardiac Arrest at the Finish Line: In Coronary Ectopia, the Cause of Ischemia Is from Intramural Course, Not Ostial Location 
Texas Heart Institute Journal  2014;41(2):212-216.
A 26-year-old woman, a well-trained runner, had a sudden cardiac arrest just before crossing the finish line of a marathon. She was rapidly resuscitated and was later found to have an ectopic origin of the left coronary artery. This anomaly was surgically repaired by translocating the ostium from the right to the left sinus of Valsalva. Her difficult postoperative course prompted further coronary evaluation, which revealed severe stenosis of the neoostium. The patient underwent a second operation: this time, the stenosis was bypassed via a left internal mammary artery-to-left anterior descending coronary artery (LAD) graft. Hypoplasia of the LAD and spasm during manipulation caused the graft to fail, necessitating double-stent angioplasty of the left main ostium and the LAD 2 months later. At the patient's 6-month follow-up examination, she had no further evidence of functional ischemia, and she resumed jogging.
Because the mode and mechanism of the patient's condition and events were documented in unusual detail, this case furthers our understanding of sudden cardiac arrest in athletes who have rare coronary anomalies. We conclude that ectopia of a coronary artery does not itself cause potentially fatal ischemia. Rather, these events are due to the ectopic artery's intramural proximal course within the aortic media, which might result in critical stenosis by means of hypoplasia or lateral compression of the artery.
PMCID: PMC4004477  PMID: 24808787
Cardiac surgical procedures; coronary stenosis/etiology; coronary vessel anomalies/classification/complications/diagnosis/surgery; coronary vessels/ultrasonography; death, sudden, cardiac/etiology/pathology/prevention & control; sinus of Valsalva/abnormalities; treatment outcome; ultrasonography, interventional
24.  Coronary artery anomalies detected by MSCT-coronary angiography in the adult 
Netherlands Heart Journal  2008;16(11):369-375.
Before coronary evaluation by modern imaging techniques was feasible, premorbid diagnoses of coronary artery anomalies (CAAs) were usually made fortuitously by invasive coronary angiography (ICA). However, this technique is limited by its invasive and projectional nature. Coronary magnetic resonance angiography (CMRA) and multi-slice computed tomography (MSCT) broadened clinical information by enabling visualisation of the coronary arteries in their anatomical environment.
This case series visualises and reviews anomalous coronary artery from the opposite sinus (ACAOS) and coronary artery fistulae. All CAAs were detected by means of 64-slice dual source computed tomography after 1000 cardiac scans at the Erasmus MC, Rotterdam, the Netherlands.
Eight ACAOS cases, one anomalous left coronary artery from the pulmonary artery (ALCAPA) and one congenital aneurysm of an aortic sinus were found. Seven out often detected CAAs were considered malignant whereas three CAAs of the ACAOS type (retroaortic path) were considered benign. Significant coronary artery disease was found in three out of eight ACAOS cases. In one of the ACAOS cases complete evaluation of the anomalous coronary artery was limited by motion artifacts. All five cases of right ACAOS were referred for MSCT because the right coronary artery could not be located by invasive angiography.
All CAAs were easy to diagnose because of 3D imaging and high temporal and spatial resolution. High resolution made it possible to not only depict coronary artery abnormalities, but also to quantify luminal and vessel properties such as stenosis grade, aspects of plaque, anomalous vessel length, luminal area ratio and the asymmetry ratio. Because of its comprehensiveness, MSCT can be an effective imaging modality in patients suspected of coronary artery abnormalities caused by coronary artery disease, CAAs, or a combination of both. (Neth Heart J 2008;16:369-75.)
PMCID: PMC2584765  PMID: 19065275
coronary anomalies; anomalous coronary artery from the opposite sinus; coronary artery fistulae; computed tomography; invasive coronary angiography
25.  Arterial Switch Operation in Patients with Intramural Coronary Artery: Early and Mid-term Results 
The intramural coronary artery has been known as a risk factor for early death after an arterial switch operation (ASO). We reviewed the morphological characteristics and evaluated the early and mid-term results of ASO for patients with an intramural coronary artery.
Materials and Methods
From March 1994 to September 15th 2010, 158 patients underwent ASO at Dong-A and Pusan National University Hospitals for repair of transposition of the great arteries and double outlet right ventricle. Among these patients, 14 patients (8.9%) had an intramural coronary artery. Mean age at operation was 13.4±10.2 days (4 to 39 days) and mean body weight was 3.48±0.33 kg (2.88 to 3.88 kg). All patients except one were male. Eight patients had TGA/IVS and 4 patients had an aortic arch anomaly. Two patients (14.3%) had side-by-side great artery relation, of whom one had an intramural right coronary artery and the other had an intramural left anterior descending coronary artery. Twelve patients had anterior-posterior relation, all of whom had an intramural left coronary artery (LCA). The aortocoronary flap technique was used in coronary transfer in 8 patients, of whom one patient required a switch to the individual coronary button technique 2 days after operation because of myocardial ischemia. An individual coronary button implantation technique was adopted in 6, of whom 2 patients required left subclavian artery free graft to LCA during the same operation due to LCA injury during coronary button mobilization and LCA torsion.
There was 1 operative death (7.1%), which occurred in the first patient in our series. This patient underwent an aortocoronary flap procedure for coronary transfer combining aortic arch repair. Overall operative mortality for 144 patients without an intramural coronary artery was 13.2% (19/144). There was no statistical difference in operative mortality between the patients with and without an intramural coronary artery (p>0.1). There was no late death. The mean follow-up duration was 52.1±43.0 months (0.5 to 132 months). One patient who had a subclavian artery free graft required LCA stenting 6.5 years after surgery for LCA anastomotic site stenosis. No other surviving patient needed any intervention for coronary problems. All patients had normal ventricular function at latest echocardiography and were in NYHA class 1.
The arterial switch operation in Transposition of Great Arteries or Double Outlet Right Ventricle patients with intramural coronary can be performed with low mortality; however, there is a high incidence of intraoperative or postoperative coronary problems, which can be managed with conversion to the individual coronary button technique and a bypass procedure using a left subclavian free graft. Both aortocoronary flap and individual coronary button implantation techniques for coronary transfer have excellent mid-term results.
PMCID: PMC3249286  PMID: 22263137
Congenital heart disease; Transposition of the great arteries; Arterial switch operation; Coronary artery; Coronary artery anatomy

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