Thallium-201 myocardial imaging in patients with angina pectoris and anomalous aortic origin of the circumflex coronary artery

Anomalous aortic origin of the circumflex coronary artery is the commonest coronary artery anomaly. In this study, the significance of anomalous aortic origin of the circumflex coronary artery was investigated in seven patients with exertional chest pain using exercise thallium scintigraphy. Five patients with circumflex coronary arterial disease served as controls. Myocardial perfusion was normal in the five patients with a nonstenosed anomalous artery. In these patients, exercise heart rate and blood pressure response were normal. Two patients with a stenosed anomalous circumflex coronary artery and four of the five control patients had posterolateral myocardial perfusion defects. It is concluded that anomalous aortic origin of the circumflex coronary artery does not cause impairment of myocardial perfusion unless it is the site of significant coronary arterial stenosis.     

Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes

OBJECTIVES: The purpose of this study is to characterize the clinical profile and identify clinical markers that would enable the detection during life of anomalous coronary artery origin from the wrong aortic sinus (with course between the aorta and pulmonary trunk) in young competitive athletes. BACKGROUND: Congenital coronary artery anomalies are not uncommonly associated with sudden death in young athletes, the catastrophic event probably provoked by myocardial ischemia. Such coronary anomalies are rarely identified during life, often because of insufficient clinical suspicion. However, since anomalous coronary artery origin is amenable to surgical treatment, timely clinical identification is crucial. METHODS: Because of the paucity of available data characterizing the clinical profile of wrong sinus coronary artery malformations, we reviewed two large registries comprised of young competitive athletes who died suddenly, assembled consecutively in the U.S. and Italy. RESULTS: We reported 27 sudden deaths in young athletes, identified solely at autopsy and due to either left main coronary artery from the right aortic sinus (n = 23) or right coronary artery from the left sinus (n = 4). Each athlete died either during (n = 25) or immediately after (n = 2) intense exertion on the athletic field. Fifteen athletes (55%) had no clinical cardiovascular manifestations or testing during life. However, in the remaining 12 athletes (45%) aged 16 +/- 7, certain clinical data were available. Premonitory symptoms had occurred in 10, including syncope in four (exertional in three and recurrent in two, 3 to 24 months before death) and chest pain in five (exertional in three, all single episodes, < or =24 months before death). All cardiovascular tests were within normal limits, including 12-lead electrocardiogram (ECG) pattern (in 9/9), stress ECG with maximal exercise (in 6/6) and left ventricular wall motion and cardiac dimensions by two-dimensional echocardiography (in 2/2). CONCLUSIONS: With regard to congenital coronary artery anomalies of wrong aortic sinus origin in young competitive athletes, 1) standard testing with ECG under resting or exercise conditions is unlikely to provide clinical evidence of myocardial ischemia and would not be reliable as screening tests in large athletic populations, 2) premonitory cardiac symptoms not uncommonly occurred shortly before sudden death (typically associated with anomalous left main coronary artery), suggesting that a history of exertional syncope or chest pain requires exclusion of this anomaly. These observations have important implications for the preparticipation screening of competitive athletes.     

Clinical evaluation of anomalous aortic origin of a coronary artery (AAOCA)

The clinical evaluation of patients with an anomalous aortic origin of a coronary artery (AAOCA), a congenital abnormality of the origin or course of a coronary artery that arises from the aorta, is challenging given its first presentation being sudden cardiac arrest in about half of the patients. Symptoms of chest pain, shortness of breath and syncope during exertion should be of concern in evaluating young athletes and nonathletes. The lack of abnormal signs on the physical exam and electrocardiogram further adds to the difficulty in establishing the diagnosis. Additional imaging with echocardiography, computed tomography angiography and/or cardiac magnetic resonance imaging (MRI) is often needed and establishes the diagnosis. High‐risk lesions include origin of the coronary artery from the opposite sinus of Valsalva, intramural course and ostial abnormalities (stenosis, hypoplasia). Functional studies should be performed to assess myocardial perfusion at rest and during stress, such as nuclear imaging, stress echocardiography and stress cardiac MRI.     

Anomalous origin of the left anterior descending artery from the pulmonary artery associated with bicuspid aortic valve and aortic coarctation

A 15-year-old boy presented with exertional palpitations and chest pain. Investigation revealed anomalous origin of his left anterior descending coronary artery from his pulmonary trunk causing myocardial ischaemia. He previously had aortic coarctation repair with known aortic root dilation and a bicuspid aortic valve. His left anterior descending artery was implanted into the aortic root using a Gortex interposition conduit. This represents an interesting combination of cardiac abnormalities for which repair required consideration of the requirement of further surgery in the future.     

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.     

Anomalous origin and course of the coronary arteries

In the normal heart, the right and left coronary arteries arise from the aortic valvar sinuses adjacent to the pulmonary trunk. The right coronary artery then directly enters the right atrioventricular groove, whereas the main stem of the left coronary artery runs a short course before dividing to become the anterior interventricular and circumflex arteries. These arteries can have an anomalous origin from either the aorta or pulmonary trunk; their branches can have various anomalous origins relative to arterial pedicles. Other abnormal situations include myocardial bridging, abnormal communications, solitary coronary arteries, and duplicated arteries. Understanding of these variations is key to determining those anomalous patterns associated with sudden cardiac death. In the most common variant of an anomalous origin from the pulmonary trunk, the main stem of the left coronary artery arises from the sinus of the pulmonary trunk adjacent to the anticipated left coronary arterial aortic sinus. The artery can, however, arise from a pulmonary artery, or the right coronary artery can have an anomalous pulmonary origin. The key feature in the anomalous aortic origin is the potential for squeezing of the artery, produced by either the so-called intramural origin from the aorta, or the passage of the abnormal artery between the aortic root and the subpulmonary infundibulum.     

Origem coronária anómala: da suspeita à revascularização cirúrgica

Congenital anomalies of the coronary arteries are uncommon and can present a diagnostic challenge.The authors present the case of a patient with recurrent chest pain during exertion admitted for acute coronary syndrome. Coronary angiography revealed no coronary lesions but showed that the right coronary artery originated from the anterolateral aortic wall, above the sinuses of Valsalva, leading to suspicion of compression by the pulmonary artery, confirmed by CT angiography. The patient underwent surgical revascularization with a good result.The authors highlight the need to consider compression of an anomalous coronary artery by the pulmonary artery in the differential diagnosis of recurrent chest pain on exertion and acute myocardial infarction without significant coronary stenosis.     

CT diagnosis of isolated anomalous origin of the RCA arising from the main pulmonary artery

Anomalies of the coronary arteries can be benign or life threatening. The prevalence of these anomalies is reported to be approximately 0.3% to 1%, however, this may be an underestimation as conventional angiography may not allow correct identification of these abnormalities. Morphologic variations can arise in the origin, course, or termination of coronary arteries. These variations may be related to other congenital abnormalities or isolated. Some anomalies can lead to myocardial ischemia and have been implicated in episodes of sudden death in young adults. Noninvasive imaging modalities such as multidetector computerized tomography provide an efficient method of evaluating coronary artery anomalies by allowing more complete visualization of chest, mediastinal, and vascular structures. We describe a case of anomalous origin of the right coronary artery originating from the main pulmonary trunk demonstrated by computerized tomography in a patient with exertional ischemia. In the past, this diagnosis has only been made by angiography, echocardiography, and at autopsy.     

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.     

Unusual Cause of Myocardial Ischemia in an Infant

Abnormal coronary artery anatomy should be ruled out in any patient with myocardial dysfunction and an abnormal electrocardiogram. The reported etiologies of infantile myocardial ischemia are abnormalities of coronary arteries, perinatal asphyxia, umbilical catheterization, and myocarditis. Generalized arterial calcification of infancy, although rare, should be considered in the differential diagnosis of infantile myocardial ischemia if coronary artery origin is found to be normal on echocardiography.     

Surgical treatment of coronary artery anomalies

In this paper, we review literature from June 1990 through May 1991. We discuss normal anatomy and physiology of the proximal coronary arteries, anomalous origin of the coronary arteries from an inappropriate aortic sinus, and anomalous origin of the left coronary artery from the pulmonary artery. Other topics reviewed include coronary arteriovenous fistulas, coronary anomalies in left-sided heart syndrome and pulmonary atresia with intact interventricular septum, coronary arterial abnormalities in transposition of the great arteries, and iatrogenic coronary anomalies.     

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.     

The sinister course of an intramural right coronary artery

We report the case of an adolescent who was presented with long-standing exertional symptoms, and was diagnosed with an anomalous right coronary arterial origin arising above the commissural junction between the left and right aortic sinus, with inter-arterial and intramural compression. The precise origin of this lesion outside the aortic sinuses is unusual, and multi-detector computed tomography gave excellent definition and spatial resolution of the anomalous origin and course. It is crucial to have a high index of suspicion of exertional symptoms, as sudden death may be the first manifestation of an anomalous coronary artery.     

Anomalous origin of all three coronary arteries from right sinus of Valsalva in a patient with angina pectoris

Anomalous origin of coronary arteries is uncommon, and all 3 coronary arteries arising from a single sinus of Valsalva is very rare. The anomaly has been associated with myocardial infarction and sudden cardiac death in particular when the anomalous left coronary artery courses between the great vessels. Imaging modalities have a complementary role to angiography in the diagnosis. Percutaneous interventions are technically difficult. Surgical options like direct repair of anomalous artery or coronary artery bypass graft can be helpful. We report a case of a patient who presented with chest pain and was found to have all 3 coronary arteries arising from the right sinus of Valsalva.     

Sudden death associated with anomalous coronary origin and obstructive coronary disease in the young

Sudden cardiac death in a young patient is a catastrophic occurrence. Anomalous coronary origin (ACO) is a significant cause of sudden cardiac death among individuals under the age of 35 years. We sought to define the premortem clinical and postmortem histopathologic findings in victims of sudden cardiac death resulting from either ACO or obstructive atherosclerotic coronary artery disease (CAD) among U.S. military recruits (ages 17-35 years). The autopsy records of all sudden cardiac deaths occurring among recruits during their basic military training period from 1977 through 2001 were reviewed. Twenty-one deaths were associated with ACO and 10 with CAD. Recruits with ACO were more likely to have prodromal symptoms of exertional syncope and/or chest pain (48% vs. 0%, P = 0.011). All sudden cardiac deaths resulting from ACO involved a left main coronary artery takeoff from the right coronary sinus with a course between the aorta and the right ventricular outflow tract and an otherwise normal distribution of the major epicardial coronary arteries. Myocardial fibrosis was seen equally in those with both CAD and ACO (30% vs. 20%, P = 0.66), but the finding of necrosis tended to be more common among recruits with CAD (50% vs. 15%, P = 0.08). In conclusion, review of autopsy data of sudden cardiac deaths among U.S. military recruits reveals myocardial fibrosis or necrosis occurred in 70% of cases with CAD and 35% of cases with ACO. Sudden cardiac deaths resulting from ACO were more likely to be associated with premortem exertional chest discomfort and/or syncope compared with deaths resulting from CAD.     

Hypertrophic cardiomyopathy and myocardial bridging.

Two patients are reported in whom hypertrophic cardiomyopathy was associated with myocardial bridging of the left anterior descending coronary artery. One had a history of angina and recurrent syncope, the other had chest pain and exertional dyspnoea. In each case the diagnosis was made by cross-sectional echocardiography and coronary angiography.     

Coronary Arterial Development: A Review of Normal and Congenitally Anomalous Patterns

Coronary artery development is a delicate, complex, and finely tuned process that includes multiple interactions among many pathways, especially in the pericardium and the developing myocardium. There still exists some controversy on the exact origin of certain cellular components. Nevertheless, an understanding of this extremely important developmental process is paramount in identifying some of the causes of anomalous coronary development. There are different patterns of anomalous coronary arteries, with variable risk of myocardial ischemia, malignant arrhythmias, and sudden cardiac death. These anomalies can be broadly categorized into 2 basic anatomic subsets: those with origin of the anomalous coronary artery from the opposite aortic sinus, and those with origin of the anomalous coronary artery from the pulmonary artery. Diagnosis and management of such patterns continues to be challenging. A good knowledge of the normal and abnormal coronary artery development could potentially help us explore new avenues in the treatment of ischemic heart disease as well as anomalous coronary arteries.     

Origen «maligno» de arteria coronaria izquierda,reporte de caso

Anomalous origin of the coronary arteries are very infrequent, however their diagnosis has been increasing due to the increase in the use of coronary computer tomography angiography (CCTA) within the algorithm of patients with suspected coronary disease; We present a case of a patient with acute on chronic chest pain in whom an anomalous origin was diagnosed with an interarterial “malignant” course of the left coronary artery, who was taken to surgery with complete improvement of symptoms and quality of life.Key words: Congenital heart disease, Chest pain, Coronary arteries, Left coronary     

Surgery and critical care for anomalous coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiac malformation that accounts for 0.25-0.50% of children with congenital cardiac disease and can cause myocardial dysfunction in young infants. In any infant presenting with ventricular dysfunction, the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery must be suspected and the origin of the coronary arteries must be confirmed. The diagnosis of anomalous origin of the left coronary artery from the pulmonary artery is an indication for surgical repair. A two-coronary arterial system is the goal and is almost always achievable. The goal of surgical therapy is the creation of a two-coronary arterial system, which appears to provide better long-term survival and protection from left ventricular dysfunction and mitral valvar regurgitation than does simple ligation of the anomalous coronary artery. Direct reimplantation of the anomalous coronary artery is the procedure of choice. It is straightforward and borrows from well-practised techniques commonly used in other procedures such as the arterial switch operation. For the rare patient in whom direct reimplantation is not possible, strategies to lengthen the anomalous coronary artery, or baffle it within the pulmonary root, are available. Mitral valvar regurgitation is common at presentation, but following the establishment of a two-coronary arterial system and satisfactory myocardial perfusion, regurgitation of the mitral valve resolves in the vast majority. Therefore, mitral valvuloplasty at the time of initial surgery for anomalous origin of the left coronary artery from the pulmonary artery is not indicated. Post-operative care requires careful manipulation of inotropic support and reduction of afterload. Mechanical support, with either extracorporeal membrane oxygenation or left ventricular assist device, should be available for use if necessary.     

Angina pectoris and coronary artery disease in patients with severe aortic valvular disease

We studied the clinical, hemodynamic, and angiographic findings of 90 consecutive patients with significant symptomatic aortic valve disease, 40 years of age or older, to evaluate the prevalence of angina pectoris in relation to coronary artery disease and the effect upon cardiac function.The prevalence of chest pain was 66% (typical angina, 39%; atypical chest pain, 27%), and the prevalence of coronary artery disease was 39%. The prevalence of coronary artery disease in patients with typical angina was 77%, in contrast to 25% in patients with atypical chest pain $\text{(}\text{P}\text{= 0.001)}$. Only two of the 35 patients (6%) with coronary artery disease were free of chest pain. Although the incidence of coronary artery disease in patients with aortic stenosis was slightly higher than in patients with aortic regurgitation or aortic stenosis-aortic regurgitation, it was not statistically significant.Patients with aortic regurgitation and coronary artery disease had significantly lower ejection fraction than patients with aortic stenosis and coronary artery disease. There were no significant differences between ejection fraction in patients without coronary artery disease in the different groups. Patients with aortic stenosis and coronary artery disease tend to have lower mean pressure gradients than those without coronary artery disease. Patients with coronary artery disease in aortic regurgitation and aortic stenosis-aortic regurgitation tend to have higher left ventricular end-diastolic pressure.This study indicates that although patients with aortic valve disease and typical angina are most likely to have associated coronary artery disease, it is not possible to predict this disorder with accuracy by means of clinical or hemodynamic findings.Since the presence or absence of coronary artery disease in patients undergoing aortic valve replacement has prognostic and therapeutic significance, we recommend that coronary arteriography be performed in all patients with significant aortic valve disease undergoing cardiac catheterization when they present with any form of chest pain, or in patients over the age of 40 years even if no chest pain is present. Coronary arteriography would also rule out anomalous aortic origin of the coronary arteries.