Coronary artery anomalies in 126,595 patients undergoing. Most coronary artery anomalies are benign and clinically insignificant. Congenital anomalies of the coronary arteries are a cause of sudden cardiac death. The coronary anomalies program at texas childrens hospital is a multidisciplinary program dedicated to the diagnosis and management of patients with congenital coronary anomalies. Cardiovascular computed tomography ct has emerged as the standard of reference for identification and characterization of coronary artery anomalies. It is a rare problem, comprising less than 1% of all congenital heart defects in children. Acaos anomalous coronary arteries originating from the opposite sinus of. Yamanaka and hobbs reported 126,595 patients undergoing coronary arteriography over a 28year period. May 21, 2009 multiple variations of congenital anomalies of the coronary arteries exist that may occur in isolation or in association with other congenital anomalies. We classify these anomalies based on the origin of the anomalous artery that is from the opposite coronary sinus of valsalva.
Congenital anomalies of coronary artery origin in adults. Visibility of the coronary arteries by ct has markedly improved due to high temporal resolution and ecgsynchronized data acquisition. The anomalous aortic origin of the left main coronary artery from the right aortic sinus may be related to scd, usually during or after exhausting exercise. Increasing use of mdct for cardiac imaging of adults requires familiarity with the crosssectional appearance of congenital coronary artery anomalies visualized with noninvasive imaging techniques.
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. Congenital coronary artery anomalies caa are rare and may be broadly classified as abnormalities of coronary artery origin, course, destination, and size or number of vessels. Pdf the anomalous origin of the left coronary artery. The anomalous coronary artery in aortic valve replacement. The anomalous origin of the left coronary artery from the pulmonary artery alcapa is a rare malformation that represents less than 0. The left main coronary artery lmca or left coronary artery lca is one of the two main coronary arteries that supply the heart with oxygenated blood. The classification of primary forms of coronary artery anomalies varies between ectopic origin of the coronary arteries from the aortic sinus, stenosis of the coronary arteries, absence of a coronary artery, to anomalous origin of the left or right coronary artery from the pulmonary artery or congenital coronary artery fistula. Congenital anomalies of the carotid arteries, including the. In this situation, the right coronary artery comes from a different part of the aorta and can become compressed by other structures in the chest, reducing blood flow to the heart muscle. Doctors at the texas heart institutes center for coronary artery anomalies ccaa are working to increase awareness about caas and to develop a screening program to help prevent sudden cardiac death in young athletes with caas.
Direct translocation of the left coronary artery could be difficult due to its intramural course and proximity to the aortic valve. This article, however, is focused on isolated coronary artery anomalies ie, in the absence of other major congenital cardiac defects. Features clear visual and textual guides to the complex morphology that is essential to the understanding of coronary artery disease. Preoperatively, 1 patient had an acute coronary syndrome, whereas the other 2 patients had effort angina. They are often found in patients with other congenital heart diseases. As detailed by mayo clinic adult congenital heart disease expert naser ammash, m.
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. Coronary artery anomalies may also be classified as hemodynamically either significant or. They range from benign incidental findings to an important cause of sudden cardiac death scd. May 31, 2018 coronary artery anomalies are frequently found in association with other major congenital cardiac defects. Congenital coronary artery anomalies are not common, but it is crucial to identify them as in some cases they can cause severe reduction of blood flow to the myocardium ischaemia and lead to chest pain, arrhythmias and sudden cardiac death, and that in themselves can increase the risk of routine procedures. Clinically, anomalous coronary arteries arising from the opposite sinus and anomalous left coronary artery arising from the pulmonary artery are. In this article, we have classified the coronary artery anomalies into anomalies of origin, anomalies of course, and anomalies of termination, with use of a modified version of a classification system developed by greenberg et al, , table. The angiographic appearance of coronary anomalies is not always straightforward, and computed tomographic angiography chapter 56 has become the diagnostic modality best suited to delineate the origin and course of the anomaly fig. Chapter 124 surgery for congenital anomalies of the coronary arteries julie a.
Coronary artery anomalies rsna publications online. Surgery for congenital anomalies of the coronary arteries. Figure 2 from coronary artery anomalies and clinically. The angiographic appearance of coronary anomalies is not always straightforward, and computed tomographic angiography chapter 56 has become the diagnostic modality best suited to delineate the origin and course of the anomaly. Congenital heart diseases associated with coronary artery.
Identifying, characterizing, and classifying congenital. Ive downloaded your program because my dad has been suffering from diabetes for a very long time but now, thanks to your help, his blood sugar level is well within normal range and all his symptoms are gone. Anomaol us orgi n oi f the rgi ht coronary artery from the puml onary artery arcapa is a very rare congenital heart defect. Recommendations for multimodality assessment of congenital. Coronary artery anomalies range in prevalence from 0. 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. Diagnostic methods 203 imaging of coronary artery anomalies. Congenital abnormalities of the coronary arteries are extremely variable and include anomalies of their origin atresia, anomalies of origin from the aorta or from the pulmonary artery, the course of the epicardiac coronary branches intramural, myocardial bridge and distal connections coronary cardiac chamber fistulae. The aim of this article is to describe and illustrate a comprehensive array for the. A coronary artery anomaly caa may relate to the origin where the artery springs up in the heart or the location of the coronary artery. Dual left anterior descending lad artery circulation is an uncommon coronary anomaly. Congenital coronary artery anomalies in young patients jacc.
A right coronary artery rca arising from the left coronary sinus is far more common than the left coronary artery lca from the right sinus. Symptoms of anomalous coronary artery aca stanford health. William gaynor anomalous origin of a coronary artery from the pulmonary artery anatomy pathophysiology clinical presentation diagnostic imaging surgical management indications for surgery surgical techniques postoperative management results special. Coronary artery anomalies may be present in patients with chest pain syndromes or in young individuals who have survived sudden death. The left coronary artery lca, which carries blood to the heart muscle, begins from the pulmonary artery instead of the aorta. Coronary artery anomalies university of florida health. Of the known anatomic variants, anomalous origination of a coronary artery from an opposite sinus of valsalva acaos remains the main focus of debate. Anomalous origin of the left main coronary artery lmca fig 1, the left anterior descending or circumflex branch from the proximal pulmonary artery or more distally to the proximal right pulmonary artery is a rare congenital anomaly occurring in 0. Conclusion ct coronary angiography is a useful method to show the anomalous origin of the coronary artery in children with anomalous origin of the left coronary artery from the pulmonary artery. Anomalous course of a coronary artery is a type of congenital coronary artery anomaly. Coronary computed tomography angiography and magnetic resonance imaging have been deemed appropriate for the evaluation of coronary anomalies by accurately allowing the noninvasive depiction of coronary artery anomalies of origin, course, and termination. The true prevalence of congenital coronary anomalies and overall risk of sudden cardiac death in this population are not well known. Although congenital coronary artery anomalies are relatively uncommon, they are the second most common cause of sudden cardiac death among young.
Congenital coronary artery anomalies, both in isolation and associated with other forms of congenital heart disease, have been recognized as important lesions. They can be both hemodynamically significant and insignificant. Anomalous origin of coronary arteries from pulmonary. This is really an amazing lifesaving book, its a blessing beyond words. Coronary artery anomalies were found in 1,686 patients 1. A very rare case of single coronary artery anomaly, with left anterior descending and left circumflex arteries originating separatelyfrom proximalright coronary artery. Coronary artery anomalies are present in up to 1% of the population. In fact, coronary anomalies are the second leading cause of scd in athletes and are responsible for. Patent ductus arteriosus pda is the failure of the fetal ductus arteriosus to. The classification of primary forms of coronary artery anomalies varies between ectopic origin of the coronary arteries from the aortic sinus, stenosis of the coronary arteries, absence of a coronary artery, to anomalous origin of the left or right coronary artery from the pulmonary artery or. Coronary artery anomalies represent a disease spectrum from incidental to lifethreatening. We present 3 cases of anomalous origin of the right coronary artery treated with coronary bypass surgery using a free right internal thoracic artery and ligation of the proximal native coronary artery to avoid competitive flow. Coronary anomaly can be defined as any change in the anatomical pattern, such as the number of ostium, proximal course, distal bed, and it is rarely found in the general population. Congenital coronary anomalies are the second most common cause of sudden cardiac death in children and young adults.
A rare but potentially lethal condition is the anomalous origin of the left coronary artery from the pulmonary artery. Coronary artery anomalies are most often classified by their origin, course, termination, or aberrant intrinsic anatomy table 16. In patients with congenital heart disease, coronary artery anomalies are common and have different clinical importance from individuals with structurally normal hearts. Exercise testing, in combination with stressimaging modalities, can be of value in the assessment of some of these patients. Anomalous origin of the right coronary artery from the left sinus one such anomaly is the anomalous origin of the right coronary artery from the left sinus. Coronary artery anomalies, coronary angiography, multidetector computed tomography background the anomalous origin of the coronary artery is a rare congenital condition with an incidence ranging from 0. Major coronary artery anomalies in a pediatric population. The anomalous aortic origin of the left main coronary artery aaolca or right coronary artery aaorca can course in front of the pulmonary artery prepulmonic or posterior to the aorta posteriorretroaortic. Late diagnosis of anomalous aortic origin of a coronary.
It is important to recognize these anomalies to avoid errors in diagnosis, decrease technical difficulties in angiography, and prevent inadvertent injury in surgery. Pdf coronary artery anomalies jorge monge academia. Coronary artery anomalies are best described in the dog, hamster, and cow though reports also exist in the horse and pig. Anomalous aortic origin of a coronary artery from the. The common clinical feature is congestive heart failure. For these reasons, cct has recently emerged as the test of choice for diagnosing coronary anomalies.
Specifically, the wrong sinus coronary artery origin, that is, the left main artery arising from the right anterior sinus almca, and the right. Anomalous origin of right coronary artery from left. Coronary artery anomalies are commonly associated with congenital heart disease. The clinical importance of anomalous coronary artery anatomy during aortic valve replacement has previously been highlighted by misawa and colleagues 3, who reported 3 patients with an anomalous coronary artery undergoing aortic valve replacement, with an accidental transection of the aberrant artery occurring in 1 patient during aortotomy. Anomalies of the coronary arteries are uncommon, but sometimes can be clinically significant. In many types of coronary anomalies, the risk of sudden cardiac death scd is largely unknown, as the anomalies are quite rare. Anomalous left coronary artery from the pulmonary artery alcapa is a heart defect.
Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. Congenital heart disease congenital anomalies of the. This is one of the most serious ca anomalies, with an estimated prevalence of one in 3 00 000 live births. Clinically, anomalous coronary arteries arising from the opposite sinus and.
Anomalous left coronary artery from the pulmonary artery. Thirtyone consecutive children with anomalous left coronary artery underwent direct aortic reimplantation of the anomalous artery without an associated procedure. Of the 1,686 patients, 1,461 87% had anomalies of origin and distribution, and 225 % had coronary artery fistulae. Multidetector row ct is superior to conventional angiography in delineating the ostial origin and proximal path of an anomalous coronary artery. Nevertheless, congenital coronary artery anomalies ccaa are frequently found in about 20% of cases, and they represent the second most frequent disease responsible for athletic field deaths.
Some variants are insignificant, some variants may or may not be symptomatic, and some variants commonly result in serious cardiovascular sequelae. Anomalous left coronary artery alca occurs when the left coronary artery, which carries blood to the heart, is connected to the pulmonary artery instead of the aorta. Congenital heart disease congenital anomalies of the coronary. Anomalies of coronary artery origin and course are wellrecognized in human medicine, but have received limited attention in veterinary medicine. Many of these anomalies are benign, but a small number are associated with myocardial ischemia and sudden death. The malformation is congenital present at birth and is most often related to the origin or location of the coronary artery. Clinical presentation depends on the specific anomaly. Aug 03, 2016 congenital anomalies of the coronary circulation 6.
Dec 30, 2019 basso c, maron bj, corrado d, thiene g. An acute angle of origin of the anomalous artery with respect to the ostium may form a slitlike opening that could become further compressed or occluded during systole 3,25. The clinical manifestations of coronary artery anomalies vary in severity, with some anomalies causing severe symptoms and cardiovascular sequelae and others being benign. The subject of caas is undergoing profound evolutionary changes related to the definition, morphogenesis, clinical presentation, diagnostic workup, prognosis, and treatment of these anomalies.
However, the term caa can be used to describe any defects in a coronary artery, such as an abnormal size or shape. Anomalous coronary artery found in the syncopal workup of. Based on the functional relevance of each abnormality, caas can be classified as. Left main coronary artery radiology reference article. Coronary artery anomalies caas are a diverse group of congenital disorders whose manifestations and pathophysiological mechanisms are highly variable. May 15, 2015 coronary artery anomalies range in prevalence from 0. Congenital coronary artery anomalies radiology reference. Congenital anomalies of the coronary circulation coronary artery anomalies are defined as those angiographic findings in which the number, origin, course, and termination of these arteries are rarely encountered in the general population. Multiple variations of congenital anomalies of the coronary arteries exist that may occur in isolation or in association with other congenital anomalies. Anyone interested in cerebral and cervical arteriography would benefit from reading and studying this book. Congenital coronary artery anomalies can be of four types. The symptoms of an anomalous coronary artery may resemble other medical conditions or heart problems. Coronary artery anomalies caa are a diverse group of congenital disorders, and the pathophysiological mechanisms and manifestations are highly variable.
Anomalous coronary artery angiography made simple cath. Portman, md, facc seattle, washington objectives we sought to prospectively determine the incidence and clinical signi. Familiarity with the ct appearances of various coronary artery anomalies and an understanding of the clinical signi. The most important finding to look for is the malignant course of anomalous coronary artery, i. Both chest pain and heart failure symptoms serve as early warning signs in adults that the heart muscle is no longer receiving sufficient blood supply from the coronary artery circulation, which may have been adequate during infancy and childhood. The course the anomalous coronary artery takes appears to have an impact on its risk of scd. 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. An anomalous coronary artery aca is a coronary artery that has an abnormality or malformation. Coronary anomalies program texas childrens hospital.
Moreover, wellknown experts on coronary anomalies are consulted preferentially. Coronary artery anomalies encompass a broad spectrum. Modern era, incidental diagnosis by echocardiography, ct or mri few studies have screened patients for aaoca in. The prevalence of coronary artery anomalies is less than 0. Gross anatomy origin it is a branch of the ascending aorta, with its normal origin in the. Coronary bypass with the free internal thoracic artery to. Prevalence and characteristics of coronary artery anomalies. Anomalous high takeoff of the right coronary artery evaluated by coronary ct angiography.
Imaging of congenital anomalies of the coronary arteries. Transthoracic echocardiography in a patient with anomalous rigin of the left coronary artery from the right sinus of valsalva and an ntramyocardial course of the anomalous coronary from a shortaxis plane. The pediatric and congenital cardiologist may encounter a variety of congenital and acquired coronary artery anomalies. The authors report the first 3 cases of alcapa diagnosed in senegal in infants under the age of 1 year old. Anomalous origin of either the right or left main coronary artery from the aorta with subsequent coursing between aorta and pulmonary trunk. It may represent a benign and incidental finding, but can also be a malignant course predisposing patients to lifethreatening myocardial ischemia or arrhythmias, depending on where the artery.
Congenital coronary artery anomalies ccaas are not common, found only in 1% range 0. Ectopic coronary artery origin, coronary artery aneurysm, coronary artery fistulae, and the most common, coronary artery bridge. The incidence and pattern of coronary artery anomalies in the northwest of iran. Increased pulsations during or following exercise can sometimes compress. Coronary artery anomalies texas heart institute the. Coronary artery anomalies and associated radiologic.
Coronary artery anomaly an overview sciencedirect topics. Coronary artery anomalies in 126,595 patients undergoing coronary. Surgical repair for anomalous origin of the right coronary. The implications of pathological studies at referral centers were summarized by maron and roberts. Sudden cardiac death occurs most frequently when the anomalous vessel courses between the aorta and pulmonary trunk 3,26,27. In addition to presenting common and uncommon anomalies of the carotid and vertebral arteries, the author has used embryology extensively to explain and understand how these interesting.
1465 1035 794 1109 731 1445 1343 499 1392 848 1076 1233 459 1361 592 1237 640 97 1443 535 1329 419 1277 737 1250 1288 205 1138 309 825 1038