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1.  The primary anomalies of coronary artery origin and course: A coronary angiographic analysis of 16,573 patients 
BACKGROUND/OBJECTIVES:
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.
METHODS:
Coronary angiographic data of 16,573 patients were analyzed. Anomalous origins and courses of coronary arteries were assessed.
RESULTS:
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.
CONCLUSIONS:
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
2.  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
3.  Primary congenital anomalies of the coronary arteries and relation to atherosclerosis: an angiographic study in Lebanon 
Background
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.
Methods
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.
Results
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.
Conclusion
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.
doi:10.1186/1749-8090-4-58
PMCID: PMC2775738  PMID: 19874587
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.
WEB SITE FEATURE
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.  Visualization of anomalous origin and course of coronary arteries in 748 consecutive symptomatic patients by 64-slice computed tomography angiography 
Background
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).
Methods
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.
Results
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).
Conclusions
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.
doi:10.1186/1471-2261-9-54
PMCID: PMC2799381  PMID: 20003347
6.  Anomalous Left Main Coronary Artery: Case Series of Different Courses and Literature Review 
Background. Congenital anomalies of the coronary arteries are a cause of sudden cardiac death. Of the known anatomic variants, anomalous origination of a coronary artery from an opposite sinus of Valsalva (ACAOS) remains the main focus of debate. Case Series. We present three cases, all presenting to our facility within one week's time, of patients with newly discovered anomalous origination of the left coronary artery from the right sinus of Valsalva (L-ACAOS). All patients underwent cardiac computed tomography for evaluation of coronary anatomy along with other forms of functional testing. Despite the high risk nature of two of the anomalies, the patients are being treated medically without recurrence of symptoms. Summary. After review of the literature, we have found that the risk of sudden cardiac death in patients with congenital coronary anomalies, even among variants considered the highest risk, may be overestimated. In addition, the exact prevalence of coronary anomalies in the general population is currently underestimated. A national coronary artery anomaly registry based on cardiac computed tomography and invasive coronary angiography data would be helpful in advancing our understanding of these cardiac peculiarities. The true prevalence of congenital coronary anomalies and overall risk of sudden cardiac death in this population are not well known. Surgical intervention remains the mainstay of therapy in certain patients though recent investigations into the pathophysiology of these abnormalities have shown that the risk of surgery may outweigh the minimal reduction in risk of sudden cardiac death.
doi:10.1155/2013/380952
PMCID: PMC3877606  PMID: 24455397
7.  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
8.  The Incidence and Pattern of Coronary Artery Anomalies in the North-West of Iran: A Coronary Arteriographic Study 
Korean Circulation Journal  2012;42(11):753-760.
Background and Objectives
Coronary artery anomalies are found in approximately 1% of patients undergoing diagnostic coronary angiography (CAG). Angiographic recognition of these vessels is important because of their clinical significance and importance in patients undergoing coronary angioplasty or cardiac surgery. There are fairly enough reports concerning the incidence of coronary anomalies in different geographic areas, but this is the first study among the Iranian population.
Subjects and Methods
We reviewed the database of the Catheterization Laboratory of Imam Reza and Shahid Madani Hospitals, Tabriz University of Medical Sciences, Iran. Our inquiry included all patients who referred for CAG from other hospitals, between February 2007 and April 2009. Patients with congenital heart diseases, high "take off" of coronary arteries and separate origin of the conus artery from the right coronary sinus (RCS) were excluded. In total, 6065 films were reviewed.
Results
Seventy nine (1.30%) patients were found to have coronary anomalies. Seventy five (1.24%) patients had anomalies of origin and distribution, while four (0.06%) had coronary artery fistulae. Most common anomaly was separate ostia of the left anterior descending artery and left circumflex artery, which was found in 42 patients (53.16%) with angiographic incidence of 0.69%. The next most common anomalies were anomalous circumflex artery from RCS/right coronary artery (RCA) {n=17 (21.51%)}, and anomalous RCA arising from left coronary sinus {n=6 (7.59%)}.
Conclusion
In general, the incidence and pattern of coronary anomalies in our study was similar to earlier reports from different parts of the world.
doi:10.4070/kcj.2012.42.11.753
PMCID: PMC3518709  PMID: 23236327
Coronary arteries; Accept; Angiography
9.  Right coronary artery originating from left anterior descending artery: a case report 
Right Coronary Artery (RCA) originating from left anterior descending artery is a very rare congenital coronary artery anomaly. A 66-year-old man presented with hypertension and complaints of exertional chest pain. The angiography was performed. Aortic root angiography showed no coronary ostium orginating from the right sinus of valsalva. Right coronary artery was vizualized as anomalously originating from the midportion of left anterior descending artery. Severe stenosis were seen in ostium of anomalous right coronary artery, in midportion of left anterior descending and in midportion of circumflex artery. The patient was referred for coronary artery bypass grafting. The patient underwent coronary artery bypass surgery for three vessels. He was discharged home on postoperative day 7 without any complication. His echocardiogram on follow-up visit revealed good biventricular function.
doi:10.1186/1749-8090-5-49
PMCID: PMC2890517  PMID: 20529357
10.  Surgery for Anomalous Origin of the Left Main Coronary Artery from the Right Sinus of Valsalva, in Association with Left Main Stenosis 
Texas Heart Institute Journal  2009;36(4):309-312.
Most medical literature regarding the anomalous origin of a coronary artery from the opposite sinus of Valsalva pertains to sudden death in the young. The surgical treatment of anomalous origin of the left main coronary artery from the right sinus of Valsalva is not particularly well codified, and when an anomalous left main coronary artery is associated with an extramural stenosis, treatment becomes more technically challenging. Herein, we describe a unique approach toward treating this combination of conditions.
A 15-year-old adolescent boy was diagnosed with anomalous origin of the left main coronary artery from the right sinus of Valsalva. The condition was accompanied by a slit ostium and an extramural stenosis of the left main coronary artery. Surgery was offered to the patient in view of his young age and the uncertain prospective course of the disease. Coronary unroofing was not indicated, due to the extramural location of the stenosis. With the patient under cardiopulmonary bypass, the stenotic segment of the left main coronary artery was transected outside the aorta, repaired by vein patch augmentation, and reimplanted directly into the left coronary sinus. The patient recovered uneventfully. A postoperative computed tomographic angiogram showed good patency of the reconstructed artery. We expect excellent longevity of the directly reimplanted coronary artery.
PMCID: PMC2720304  PMID: 19693304
Adolescent; cardiac surgical procedures; chest pain/etiology; coronary angiography; coronary vessel anomalies/classification/diagnosis/surgery; death, sudden/prevention & control; prognosis; replantation; sinus of Valsalva/abnormalities
11.  A Case of Acute Myocardial Infarction with the Anomalous Origin of the Right Coronary Artery from the Ascending Aorta above the Left Sinus of Valsalva and Left Coronary Artery from the Posterior Sinus of Valsalva 
Yonsei Medical Journal  2009;50(1):164-168.
Coronary anomalies are rare angiographic findings. Moreover, there are few reports of cases of an anomalous origin of the right coronary artery from the left sinus of Valsalva and of the left coronary artery from the posterior sinus of Valsalva. Here, we report a case with an anomalous origin of the right coronary artery from the ascending aorta above the left sinus of Valsalva and the left coronary artery from the posterior sinus of Valsalva. This was observed in a patient who was treated for a myocardial infarction of the inferior wall caused by a thrombus in the proximal right coronary artery. The patient was treated successfully with the implantation of a stent in the anomalous origin of the right coronary artery using a 6Fr Amplatz left 1 catheter.
doi:10.3349/ymj.2009.50.1.164
PMCID: PMC2649847  PMID: 19259366
Anomalous origin of coronary artery; myocardial infarction
12.  Cardiac arrest associated with sildenafil ingestion in a patient with an abnormal origin of the left coronary artery: case report 
Background
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.
Conclusion
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.
doi:10.1186/1471-2261-11-49
PMCID: PMC3166914  PMID: 21824399
13.  Isolated single coronary artery (RII-B type) presenting as an inferior wall myocardial infarction: A rare clinical entity 
Indian Heart Journal  2014;66(5):553-554.
Isolated single coronary artery without other congenital cardiac anomalies is very rare among the different variations of anomalous coronary patterns. The prognosis in patients with single coronary varies according to the anatomic distribution and associated coronary atherosclerosis. If the left main coronary artery travels between the aorta and pulmonary arteries, it may be a cause of sudden cardiac death. We present multimodality images of a single coronary artery, in which the whole coronary system originated by a single trunk from the right sinus of Valsalva with inter-arterial course of left main coronary artery. This rare type of single coronary artery was classified as RII-B type according to Lipton's scheme of classification. A significant flow-limiting lesions were found in the right coronary artery that was successfully treated with percutaneous coronary intervention.
doi:10.1016/j.ihj.2014.05.021
PMCID: PMC4223188  PMID: 25443613
14.  Successful Primary Percutaneous Coronary Intervention in a Patient With Acute Myocardial Infarction and Single Coronary Artery Ostium 
Korean Circulation Journal  2012;42(4):284-287.
One of the single anomalous origins of coronary artery that has rarely been reported is a congenital anomaly of coronary circulation that occurs in the left coronary artery originating from the right coronary sinus of valsalva. We report a 49-year-old male patient with non-ST segment elevated myocardial infarction that was identified to have an anomalous origin of the left coronary artery from the right coronary artery (RCA) with thrombotic total occlusion of RCA by coronary angiography and cardiac computed tomography. The patient underwent successful percutaneous coronary intervention in total occlusion of the RCA and was discharged after uneventful recovery.
doi:10.4070/kcj.2012.42.4.284
PMCID: PMC3341428  PMID: 22563344
Coronary vessel anomalies; Myocardial infarction; Percutaneous transluminal coronary angioplasty
15.  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.
doi:10.1016/j.jsha.2010.07.008
PMCID: PMC3727461  PMID: 23960633
Coronary artery anomalies; Cardiac CT; Coronary Angiography (AC)
16.  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.
doi:10.14503/THIJ-12-2867
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
17.  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
18.  Strenuous Exercise Induced Syncope Due to Coronary Artery Anomaly 
Coronary artery anomalies are among the neglected topics in cardiology. Anomalous origin of the left main coronary artery from the right sinus of valsalva is a rare coronary anomaly observed in 0.15% of patients. During exercise, the distended aorta and pulmonary artery with increased blood flow may squeeze the Left Main Coronary Artery (LMCA) between them. Even though arrhythmias are common causes of syncope, one should also think about aberrant coronary artery in the patients with syncope of unexplained origin. Patients experiencing exercise induced syncope accompanied by symptoms of coronary ischemia (typically: chest pain, ischemic findings on ECG, and raised cardiac markers) should be referred to diagnostic coronary angiography.
PMCID: PMC4109038  PMID: 25177677
Syncope; Coronary Anomaly; Coronary Angiography
19.  Single Coronary Artery with Anomalous Origin of the Right Coronary Artery from the Distal Portion of Left Circumflex Artery: A Very Rare Case 
Congenital anomalies of coronary arteries, albeit rare, may be significant contributors to angina pectoris, hemodynamic abnormalities, and sudden cardiac death. A 47-year-old man referred to us with atypical chest pain. Electrocardiography demonstrated no significant ischemic changes, but cardiac troponin I test was positive. The patient underwent coronary angiography, which revealed a single coronary artery from the left Valsalva sinus. In addition, the left anterior descending (LAD) and the left circumflex (LCx) arteries were in normal position with significant stenosis in the mid-portion of the LAD and the distal portion of the LCx. A large branch originated from the distal portion of the LCx and tapered toward the proximal portion as the right coronary artery (RCA). This is a rare coronary anomaly that has no ischemic result. Coronary lesions were the cause of the patient’s angina pectoris. Angioplasty and stenting of the LAD and LCx was done, and medical therapy (Clopidogrel, Aspirin, Atorvastatin, and Metoprolol) was continued. The patient was asymptomatic at 8 months’ follow-up.
PMCID: PMC3874377  PMID: 24396367
Coronary angiography, Coronary vessel anomalies; Angina pectoris
20.  Anomaly of the Left Anterior Descending Coronary Artery Arising from the Right Sinus of Valsalva and Ventricular Septal Defect in Adult: A Rare Case 
Yonsei Medical Journal  2005;46(5):729-732.
Anomaly of the left anterior descending (LAD) coronary artery arising from the right sinus of valsalva is frequently seen with tetralogy of Fallot (TOF). The association of the LAD coronary artery with ventricular septal defect (VSD) is uncommon. We described an anomalous origin of the LAD coronary artery from the right sinus of valsalva with ventricular septal defect in a 38-year-old male patient suffering from atypical angina. The LAD coronary artery arose from the right sinus of valsalva, just next to the right coronary artery. There was a single opening in the membranous part of the interventricular septum. From this case, we suggest that angiography is useful for both documenting anomalies of the LAD coronary artery associated with VSD and for determining the safest surgical procedures.
doi:10.3349/ymj.2005.46.5.729
PMCID: PMC2810584  PMID: 16259076
Coronary vessels; ventricular septal defects; congenital abnormalities
21.  Unusual cause of exercise-induced ventricular fibrillation in a well-trained adult endurance athlete: a case report 
Introduction
The diseases responsible for sudden deaths in athletes differ considerably with regard to age. In young athletes, congenital malformations of the heart and/or vascular system cause the majority of deaths and can only be detected noninvasively by complex diagnostics. In contrast, in older athletes who die suddenly, atherosclerotic disease of the coronary arteries is mostly found. Reports of congenital coronary anomalies as a cause of sudden death in older athletes are rare.
Case presentation
A 48-year-old man who was a well-trained, long-distance runner collapsed at the finish of a half marathon because of a myocardial infarction with ventricular fibrillation. Coronary angiography showed an anomalous origin of the right coronary artery from the left sinus of Valsalva with minimal wall alterations. Multislice computed tomography of the coronary arteries confirmed these findings. Cardiomagnetic resonance imaging demonstrated a mild hypokinesia of the basal right- and left-ventricular posterior wall. An electrophysiological study showed an inducible temporary polymorphic ventricular tachycardia and an inducible ventricular fibrillation. The athlete was subsequently treated by acetylsalicylic acid 100 mg (0-1-0), bisoprolol 2.5 mg (1-0-0) and atorvastatin 10 mg (0-0-1) and was instructed to keep his training intensity under the 'individual anaerobic threshold'. Intense and long-lasting exercise under extreme environmental conditions, particularly heat, should also be avoided.
Conclusion
This case report presents a coronary anomaly as the most likely reason for an exercise-induced myocardial infarction with ventricular fibrillation in a well-trained 48-year-old endurance athlete. Therefore, coronary anomalies have also to be considered as a possible cause of cardiac problems in older athletes.
doi:10.1186/1752-1947-2-120
PMCID: PMC2365969  PMID: 18433498
22.  Percutaneous coronary intervention of a single coronary artery arising from the right sinus of Valsalva 
Anomalous origin of the left main coronary artery from the right sinus of Valsalva is extremely rare when not associated with other congenital cardiac anomalies. In this report we present a patient with a single coronary ostium, with both the left and right coronary artery systems arising from it. The right coronary artery was found to contain a significant flow-limiting lesion that was successfully treated with percutaneous coronary intervention.
doi:10.2147/VHRM.S13381
PMCID: PMC3004510  PMID: 21191427
single coronary artery; percutaneous coronary intervention
23.  The distribution and prognosis of anomalous coronary arteries identified by cardiovascular magnetic resonance: 15 year experience from two tertiary centres 
Background
Aberrant coronary arteries represent a diverse group of congenital disorders. Post-mortem studies reveal a high risk of exercise-related sudden cardiac death in those with an anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) with an inter-arterial course. There is little documentation of lifetime history and long-term follow-up of patients with coronary artery anomalies.
Methods
Patients with anomalous coronary arteries undergoing cardiovascular magnetic resonance over a 15-year period were identified and classified by anatomy and course. Medical records were reviewed for major adverse cardiovascular events (MACE). Revascularisation or myocardial infarction counted only if occurring in the distribution of the anomalous artery.
Results
Consecutive patients with coronary artery anomalies were retrospectively identified (n = 172). Median follow-up time was 4.3 years (IQR 2.5–7.8, maximum 15.6). 116 patients had ACAOS of which 64 (55%) had an inter-arterial course (IAC) and 52 (45%) did not. During follow up 110 ACAOS patients were alive, 5 died and 1 lost to follow-up.
ACAOS patients experienced 58 MACE events (5 cardiovascular deaths, 5 PCI, 24 CABG and 24 had myocardial infarction). 47 MACE events occurred in ACAOS with IAC and 11 in those without (p < 0.0001), the statistical difference driven by surgical revascularisation and myocardial infarction.
Conclusions
In life, patients with an anomalous coronary artery originating from the opposite sinus of Valsalva taking an IAC have higher rates of both myocardial infarction and surgical revascularisation during long-term follow up, compared to those without IAC.
doi:10.1186/1532-429X-16-34
PMCID: PMC4038832  PMID: 24886614
Coronary vessel anomalies; Cardiovascular magnetic resonance; Prognosis
24.  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
25.  Coronary artery anomalies detected by MSCT-coronary angiography in the adult 
Netherlands Heart Journal  2008;16(11):369-375.
Background
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.
Methods
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.
Results
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.
Conclusion
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

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