Acute coronary syndrome in a patient with a single coronary artery arising from the right sinus of Valsalva

Coronary artery anomalies are usually encountered as coincidental findings during coronary angiography or at autopsy.Life threatening symptoms,such as arrhythmias,syncope,myocardial infarction,or sudden death,can occur in up to 20% of patients.However,the majority of anomalies(80%) are benign and asymptomatic.A single coronary artery(SCA) is one of the most rarely seen coronary anomalies with an incidence of 0.05%.We report the case of a 55-year old male patient who presented with symptoms of chest pain associated with an acute myocardial infarction.Coronary angiography revealed an anomalous left main coronary artery(LMCA) originating from the right coronary ostium,and an occluded distal right coronary artery.The occluded distal right coronary artery was successfully treated by thrombosuction and stenting.In order to confirm the origin and course of the SCA,multi-slice computed tomography(MSCT) of the heart was performed after coronary angiography.MSCT showed that the anomalous LMCA originated from the right coronary artery ostium and then passed the interventricular septum,instead of being intra arterial,and under the right ventricular infundibulum.The anomalous LMCA was classified as R-Ⅱ S subtype according to Lipton’s classification.     

Rare case of coronary to pulmonary vein fistula with coronary steal phenomenon

Coronary artery fistulas are abnormal connections between coronary artery territories and cardiac chambers or major vessels,most of them are congenital.Patients with coronary artery fistula can be asymptomatic or present with different symptoms like angina.Cardiac computed tomography(CT)is one of the best modalities for diagnosis.We present an elderly patient that presented with angina symptoms,non invasive stress test was positive for ischemic heart disease,coronary angiogram could not reveal any obstructive lesions,but an abnormal branch of the left descending coronary artery(LAD),cardiac CT showed fistula that connect left anterior descending coronary artery to left superior pulmonary vein.Our case is extremely rare as most of the reported cases were fistulas between LAD and pulmonary artery,but in our case the fistula between LAD and left superior pulmonary vein.In addition,our patients’symptoms resolved with anti-ischemic medical treatment without any surgical intervention.     

Congenital Coronary Anomalies and Surgical Treatment

Coronary artery anomalies are not uncommon, occurring in 1.3% (range = 0.3–5.6%) of the population, and are often an incidental finding in asymptomatic patients. Approximately 20% of coronary anomalies have potential for life‐threatening complication, including myocardial infarction, arrhythmia, or sudden death early in life or during adulthood. Coronary artery anomalies are composed of a wide variety of disorders. Some, such as anomalous location of a coronary ostium, duplication of coronary arteries, or multiple ostia, become clinically significant only when another cardiac surgical procedure is necessary, and generally, surgical correction is not required in these patients. On the other hand, the diagnosis of anomalous origin of left coronary artery from pulmonary artery or from the opposite sinus with inter‐arterial course is an indication for operation. Some anomalies like coronary artery fistulas, myocardial bridging, and coronary aneurysm require operation only when they cause clinical symptoms. Coronary artery anomalies should be included in the differential diagnosis of anginal symptoms, myocardial infarction, arrhythmia, or heart failure, especially in young patients. Increased awareness of these pathologies will lead to earlier diagnosis and treatment of a potentially life‐threatening condition.     

Anomalias congénitas das artérias coronárias

Congenital coronary artery anomalies are modifications of their origin, course or structure and its incidence varies between 0,2 and 5,6% of the general population. Although the majority is asymptomatic, they are the second leading cause of sudden cardiac death in young athletes.The aim of this study is to highlight the main anomalies with hemodynamic significance, including the anomalous origin of a coronary artery from the opposite sinus and anomalous origin of the left coronary artery from pulmonary artery.The anomalous aortic origin of a coronary artery from the opposite sinus accounts for 14‐16% of all cardiac deaths, that unexpectedly occur in healthy children or young athletes during or immediately after exercise. The mechanism responsible for the compression/occlusion of the coronary artery originating from the opposite sinus is still unclear and there are several proposed mechanisms. The clinical presentation of these patients is variable and physical examination is normal in most individuals. Transthoracic echocardiogram is the diagnostic test most commonly used. Treatment and management are controversial topic.The anomalous origin of the left coronary artery from pulmonary artery is a very rare condition and without surgery, most infants die within the first 12 months of life. The echocardiogram is also the method of choice for diagnostic confirmation. The diagnosis of this congenital anomaly in an infant, usually seriously ill, is an indication for urgent surgery.Because these anomalies produce hemodynamic changes, it is important an early diagnosis and treatment.     

Robotic coronary artery bypass for aberrant right coronary artery stenosis

Anomalous coronary arteries that course between the aorta and pulmonary artery are subject to compressive forces and can manifest angina, myocardial infarction and sudden death. The current report presents a young, female patient who presented with a short duration of severe, rapidly progressive angina despite optimal medical therapy. Combined computed tomography and myocardial perfusion scanning identified an anomalous dominant right coronary artery that appeared kinked at its origin between the aorta and main pulmonary artery. A robot-assisted right internal thoracic artery to right coronary artery bypass was performed, which was confirmed to be widely patent (FitzGibbon grade A) on routine intraoperative angiography. The procedure completely resolved the patient's angina symptoms.     

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.     

Anomalous aortic origin of a coronary artery: a universally applicable surgical strategy

The most commonly reported coronary arterial malformation, in accounts of sudden deaths, is anomalous aortic origin of a coronary artery. Anomalous coronary arteries may arise from the left, right, or non-coronary sinuses of Valsalva. Importantly, although the left coronary artery from the right sinus has the worst prognosis, sudden death has been reported in all variants of origin from the various sinuses of Valsalva. This paper describes a technique that addresses all of the problems relating to anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva. This technique includes the transection of the ascending aorta and pulmonary trunk, coronary arterial enlargement with a pericardial patch, and lateral translocation of the pulmonary trunk to the left pulmonary artery. Anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva is a potentially lethal cardiac anomaly that can be corrected in all cases using this simplified surgical technique that addresses the major anatomic and physiological problems.     

Sudden unexpected death as a result of anomalous origin of the right coronary artery from the left sinus of Valsalva

The association of sudden unexpected death with coronary artery anomalies has been limited to cases of anomalous origin of the left main coronary artery, either from the right sinus of Valsalva or from the main pulmonary artery. In contrast, anomalies involving the origin of the right coronary artery have been considered to be benign. Postmortem examination in a patient who died suddenly at 23 years of age disclosed that the right coronary artery originated anomalously from the left sinus of Valsalva. The findings in this patient: (1) constitute the first necropsy documentation of premature sudden unexpected death in a patient whose sole pathologic abnormality was anomalous origin of the right coronary artery; (2) have important implications regarding previously proposed mechanisms of sudden death due to anomalous origin of the left main coronary artery; and (3) support recent suggestions that markedly acute angulation of either coronary artery, even when located in the appropriate sinus of Valsalva, may predispose to sudden unexpected death.     

Anomalies of coronary arteries

Concealed undiagnosed congenital anomalies of coronary arteries (CACA) can cause sudden death of young men. Isolated CACA are detected at 0.6-1.8% of coronary angiographies. Classification of CACA (2002) includes anomalous origin of coronary artery from pulmonary artery, anomalous origin of coronary artery from the aorta, congenital atresia of the left main coronary artery, coronary arteriovenous fistula, coronary artery with myocardial bridge, coronary artery aneurism, coronary artery stenosis. In most cases coronary artery anomalies for long time remain asymptomatic. Clinical picture of anomalous origin of coronary artery from pulmonary artery is often erroneously related to cardiomyopathy or myocarditis because of signs of heart failure. Modern methods of visualization are used for diagnosis of CACA: echocardiography (transthoracic and transesophageal), computer angiotomography (electron beam tomography, multispiral computer tomography), magnetic resonance angiography, thallium stress scintigraphy, single photon positron emission tomography, dobutamine stress echocardiography, endovascular ultrasound study. Coronary angiography is the gold standard for diagnosis of congenital anomalies of coronary arteries. Drug therapy, transluminal balloon angioplasty with stenting or surgical revascularization are indicated to patients with overt clinical picture.     

An unusual combination of possible causes of sudden death imaged by 64-slice computed tomography

Hypertrophic cardiomyopathy and anomalous origin of the coronary arteries are important causes of sudden cardiac death in the young, for which several diagnostic imaging modalities are currently available. We report the case of a young sudden cardiac death survivor in whom the unusual coexistence of hypertrophic cardiomyopathy and anomalous origin of the left circumflex artery was demonstrated using 64-slice computed tomography.     

Anomalous Origin of the Left Coronary Artery from the Right Pulmonary Artery Presenting Following Relief of Left Heart Obstruction: A Distinct and Predictable Clinico-Pathological Syndrome

Introduction. Pre-operative recognition of significant abnormalities of the coronary arteries is important in a variety of congenital cardiac conditions. Failure to diagnose anomalous origin of the coronary artery from the pulmonary artery during repair of other anomalies is important because reduction in pulmonary artery pressure will reduce myocardial perfusion pressure. Patients. We report two cases of the rare association of anomalous origin of the left coronary artery from the right pulmonary artery, aortic coarctation, and mitral stenosis. Conclusions. Definitive imaging of coronary artery anatomy by echocardiography or other modalities should form a routine part of diagnostic assessment in all congenital heart disease patients but particularly those with left heart obstruction.     

Anomalous origin of the circumflex coronary artery--two case reports.

As shown in many series, congenital coronary artery anomalies are found in 0.6 to 1.5% of patients undergoing coronary angiography. Various types of coronary anomalies have been described, many involving the circumflex artery. The second most common anomaly is of the circumflex arising from the right sinus of Valsalva, while origin in the right coronary artery is also frequent. The most common anomaly is absence of the left main coronary artery, the anterior descending and circumflex arteries originating separately in the left coronary sinus. Such anomalies are usually benign, although earlier and more aggressive atherosclerosis is more likely than in normal coronaries and myocardial ischemia can result. Although rare, this can manifest as sudden death. Conventional coronary angiography may be unable to determine the three-dimensional course of the anomalous vessel. The development of multislice computed tomography and its application to cardiac imaging mean that it is now possible to visualize the coronary arteries non-invasively and to obtain more accurate information on their proximal course. We present two cases of congenital anomaly of the circumflex coronary artery diagnosed with the aid of multislice computed tomography.     

Coronary Artery Anomalies: Current Recognition and Treatment Strategies. Update on Recent Progress

Anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva is a relatively rare congenital cardiac defect. While the majority of these anomalies are benign, there are certain coronary anomalies that increase the risk of sudden cardiac death in the young. Patients may present with cardiovascular complaints while others may be asymptomatic at diagnosis. Transthoracic echocardiography is useful for the initial diagnosis. Often, further non-invasive imaging, such as cardiac MRI or CT scan, is used to confirm and further delineate the coronary anatomy. Treatment is dependent on the anomalous coronary artery course as well as patient presentation. There is controversy regarding the best treatment for those anomalous coronary arteries that course between the aorta and pulmonary artery, especially in the asymptomatic patient with anomalous right coronary artery from the left sinus of Valsalva. Future directions should focus on better methods to risk stratify these pediatric patients.     

Echocardiographic imaging of anomalous origin of the coronary arteries

In the past, coronary arterial anomalies have been difficult to diagnose by non-invasive methods. Identification of coronary arterial origins is now a routine part of the standard paediatric echocardiogram. Anomalous origin of a coronary artery from the pulmonary trunk is an extremely important diagnosis to make. Many echocardiographic features are not directly related to the visualisation of the coronary arterial origin. Left ventricular dilation and abnormal ventricular performance are common, along with mitral regurgitation and evidence of collateralisation of the flow from the coronary artery that has an aortic origin. In some cases, the anomalous coronary artery can be seen to arise directly from the pulmonary trunk. Congenital atresia of the main stem of the left coronary artery has a similar echocardiographic presentation, except that its aortic origin is not determined. Anomalous aortic origin of the coronary artery has important implications, as the first presenting symptom can be sudden death. With meticulous attention to the origins of the coronary arteries, echocardiographic diagnosis can also be achieved. In contrast to the anomalous origin of a coronary artery from the pulmonary trunk, ventricular performance is usually normal. Whenever there is doubt as to the definition of the origin of the coronary arteries and, indeed, when there is serious clinical concern that a coronary artery has an anomalous origin, other testing, such as cine-computed tomography, magnetic resonance imaging, or cardiac catheterisation may be indicated for confirmation or to provide greater anatomic detail.     

Introduction to anomalous aortic origin of a coronary artery

Anomalous aortic origin of a coronary artery (AAOCA) occurs when both coronary arteries arise from the same aortic sinus from a single ostium or two separate ostia. While most coronary anomalies are benign, the two most common subtypes that predispose to sudden cardiac death in the young are interarterial anomalous right coronary artery and interarterial anomalous left coronary artery. Practitioners face many challenges with AAOCA. Diagnosing patients may be difficult because children and adolescents are often asymptomatic and first presentation may be sudden death or sudden cardiac arrest. Risk stratification is also challenging as determining which unique characteristics place the child at highest risk of sudden death has not been adequately delineated and ischemic testing may give false negative results. Last, there is significant variability in decision making regarding management of youth with AAOCA. Future research is needed to help determine the best way to identify at‐risk children and which treatment is the safest and most efficacious.     

Ectopic origin of the right coronary artery--case report.

Coronary artery anomalies can occur in isolation or in association with other congenital heart defects. Clinical presentation ranges from asymptomatic forms to sudden cardiac death. The authors report a case of anomalous origin of the right coronary artery from the left sinus of Valsalva, diagnosed with multidetector computed tomography coronary angiography.     

Single coronary artery from the right aortic sinus of Valsalva with anomalous prepulmonic course of the left coronary artery

Cardiac computed tomography allows for improved, noninvasive and accurate visualization of coronary artery anomalies. The case of a single coronary artery with origin from a single ostium in the right sinus of Valsalva with an anomalous course of the left coronary artery anterior to the pulmonary trunk is presented. The unusual distal reconstitution of a normal anatomical course at the junction of the mid and distal left anterior descending artery with occlusion of the proximal circumflex artery has not, to the authors’ knowledge, been previously described.     

Intravascular ultrasound and pharmacological stress test to evaluate the anomalous origin of the right coronary artery

The anomalous origin of the right coronary artery is a common finding. With its origin in the left coronary sinus, the right coronary artery can have a route between the aorta and pulmonary artery trunk and can cause myocardial ischemia and sudden death. The anomalous origin of the artery and its route may be diagnosed by coronary angiography or multislice computed tomography. Intravascular ultrasound provides high-resolution images and a precise evaluation of coronary anomalies. The role of intravascular ultrasound was recently demonstrated in the diagnosis of extrinsic compression of the anomalous right coronary artery. We describe 3 cases of anomalous right coronary artery originating in the left coronary sinus. The intravascular ultrasound detected a reduction of the coronary lumen from anomalous course, even when the luminal reduction was not evident by angiography. We suggest that a pharmacological stress test should be used, with a vasoactive drug that simulates physical effort, to determine the reduction of the arterial lumen.     

Diagnosis of anomalous origin of the right coronary artery using multislice computed tomography: evaluation of possible causes of myocardial ischemia

Anomalous origin of the right coronary artery (RCA) is a rare condition, but may cause myocardial ischemia and sudden death. Multislice computed tomography, which allows three-dimensional visualization of the coronary artery with high spatial resolution, may be the most promising imaging modality for diagnosing this anomaly. We describe a patient with anomalous origin of the RCA arising from the left sinus of Valsalva. Volume rendering, and axial and curved multiplanar images showed stenosis in the proximal portion of the RCA that coursed between the aorta and the pulmonary artery, and an acute angled take-off of the RCA from the aorta. Three-dimensional virtual angioscopic images showed a hypoplastic RCA orifice and luminal narrowing in the proximal portion of the RCA. Multislice computed tomography was thought to be useful for detecting anomalous origin of the RCA and for evaluating possible causes of myocardial ischemia.     

Native whole-heart coronary imaging for the identification of anomalous origin of the coronary arteries

Congenital abnormalities of the coronary arteries are an uncommon cause of chest pain and in some cases may cause sudden cardiac death. We examined with magnetic resonance (MR) a 52 year-old woman presenting with effort angina, and positive treadmill stress test. Whole-heart coronary imaging performed without paramagnetic contrast agents revealed an anomalous origin of a single coronary artery from the right sinus of Valsalva. This is one of the first reports describing whole-heart coronary MR finding of an anomalous origin of the coronary arteries, obtained without contrast agents.