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.     

Coronary artery anomalies in 126,595 patients undergoing coronary arteriography

Coronary artery anomalies were found in 1,686 patients (1.3% incidence) undergoing coronary arteriography at the Cleveland Clinic Foundation from 1960 to 1988. Of the 1,686 patients, 1,461 (87%) had anomalies of origin and distribution, and 225 (13%) had coronary artery fistulae. Most coronary anomalies did not result in signs, symptoms, or complications, and usually were discovered as incidental findings at the time of catheterization. Eighty-one percent were "benign" anomalies: 1) separate origin of the left anterior descending and circumflex from the left sinus of Valsalva; 2) ectopic origin of the circumflex from the right sinus of Valsalva; 3) ectopic coronary origin from the posterior sinus of Valsalva; 4) anomalous coronary origin from the ascending aorta; 5) absent circumflex; 6) intercoronary communications; and 7) small coronary artery fistulae. Other anomalies may be associated with potentially serious sequelae such as angina pectoris, myocardial infarction, syncope, cardiac arrhythmias, congestive heart failure, or sudden death. Potentially serious anomalies include: 1) ectopic coronary origin from the pulmonary artery; 2) ectopic coronary origin from the opposite aortic sinus; 3) single coronary artery; and 4) large coronary fistulae. Coronary artery anomalies require accurate recognition, and at times, surgical correction.     

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.     

Myocardial infarction before age 36: Risk factor and arteriographic analysis

One-hundred twenty consecutive patients who were 35 years of age or younger underwent coronary arteriography after clinical myocardial infarction. Ninety-two percent were men. Four distinct subgroups were identified: Ninety-four patients (78 percent) had significant coronary artery disease (greater than 50 percent diameter narrowing of at least one major coronary artery), 20 (17 percent) had normal coronary arteries, 5 (4 percent) had major coronary arterial anomalies and 1 patient had coronary arteritis. Of the patients with obstructive coronary disease, risk factors were smoking in 89 percent, positive family history of coronary artery disease in 48 percent, hypertension in 21 percent and a history of lipid abnormality in 20 percent. Risk factors were distinctly less frequent in the groups without coronary atherosclerosis. In the group with coronary artery disease, the prevalence rate of one, two and three vessel disease was 32, 26 and 42 percent, respectively. Coronary arterial anomalies included anomalous origin of the left coronary artery from the pulmonary artery (three patients) and single right and single left coronary artery (one patient each).It is concluded that myocardial infarction before age 36 is a disease of men who smoke and who often have a family history of premature coronary artery disease. Twenty-two percent of patients will have normal coronary arteries, coronary arterial anomalies or coronary vasculitis. Coronary arteriography should be considered for patients who sustain a myocardial infarction before age 36 for purposes of diagnosis, management and prognosis.     

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.     

Overview of coronary artery variants,aberrations and anomalies

Coronary artery anomalies and variants are relatively uncommon congenital disorders of the coronary artery anatomy and constitute the second most common cause of sudden cardiac death in young competitive athletes. The rapid advancement of imaging techniques, including computed tomography, magnetic resonance imaging, intravascular ultrasound and optical coherence tomography, have provided us with a wealth of new information on the subject. Anomalous origin of a coronary artery from the contralateral sinus is the anomaly most frequently associated with sudden cardiac death, in particular if the anomalous coronary artery has a course between the aorta and the pulmonary artery. However, other coronary anomalies, like anomalous origin of the left coronary artery from the pulmonary artery, atresia of the left main stem and coronary fistulae, have also been implicated in cases of sudden cardiac death. Patients are usually asymptomatic, and in most of the cases, coronary anomalies are discovered incidentally during coronary angiography or on autopsy following sudden cardiac death. However, in some cases, symptoms like angina, syncope, heart failure and myocardial infarction may occur. The aims of this article are to present a brief overview of the diverse coronary variants and anomalies, focusing especially on anatomical features, clinical manifestations, risk of sudden cardiac death and pathophysiologic mechanism of symptoms, as well as to provide valuable information regarding diagnostic workup, follow-up, therapeutic choices and timing of surgical treatment.     

冠状动脉先天性异常患者的冠状动脉造影分析

目的　了解在行冠状动脉造影的人群中冠状动脉先天性异常的发生情况 ,并探讨其临床意义。方法　对北京大学第一医院自 2 0 0 0年 1月至 2 0 0 3年 1 1月行冠状动脉造影的患者中存在冠状动脉先天性异常者的造影结果进行回顾性分析。结果　 2 82 4例冠状动脉造影患者中 ,检出各种类型冠状动脉先天性异常共 6 1例 ,占 2 1 %。其中 84 1 %为冠状动脉起源和分布异常 ,右冠状动脉先天性异常远较左冠状动脉先天性异常多见。 4例未能成功施行右冠状动脉选择性造影。结论　临床上冠状动脉先天性异常并非罕见 ,其中部分类型可引起心肌缺血。冠状动脉起源异常对介入诊疗有一定影响。     

Origem anómala da coronária direita e enfarte agudo do miocárdio: causa ou coincidência?

Congenital coronary artery anomalies are one of the causes of myocardial ischemia and sudden death in the young, mainly during sports. Origin of the right coronary artery from the left anterior descending artery is very rare, with a prevalence of 0.015%, corresponding to 1.2% of all coronary artery anomalies. The authors present the case of a 22-year-old man, with a history of cocaine use, admitted to hospital with a non-ST elevation acute myocardial infarction. Coronary angiography revealed the presence of this rare coronary anomaly and the absence of atherosclerotic luminal stenosis, and so it was assumed to be a type II infarction caused by cocaine-induced vasospasm of the anomalous vessel.     

Right coronary artery emerging as a septal branch from the left anterior descending artery

Coronary artery anomalies are detected on approximately 1.3% of coronary angiograms. Single coronary artery anomaly (SCA) is defined as the coronary artery arising from a single coronary ostium, nourishing the entire heart. SCA anomalies are usually benign; however, serious complications such as sudden cardiac death and myocardial infarction resulting from these anomalies are also reported in the literature. We report the anomalous origin of the right coronary artery (RCA) as a continuum of the septal branch from the left anterior descending (LAD) artery, which is a very rare variation of a single coronary artery.     

Myocardial infarction in childhood: clinical analysis of 17 cases and medium term follow up of survivors

Between 1979 and 1989 17 patients aged two months to 12 years with acute myocardial infarction of any cause (other than after cardiac surgery) were seen at a children's hospital. Eight died from three days to three years after diagnosis (overall mortality 47%). The nine survivors, now aged 2-17 years, have been followed for one to 10 years (mean follow up five years) after infarction. The commonest causes of myocardial infarction in this series were anomalous origin of left coronary artery from the pulmonary artery (six patients (35%] and Kawasaki disease (five patients (27%]. The main symptoms of acute myocardial infarction were dyspnoea, vomiting, and difficulty feeding. Diagnosis was made in all patients by electrocardiography and confirmed by echocardiography, cardiac catheterisation, or at operation. All survivors were symptom free with excellent exercise capacity. The left ventricular ejection fraction in survivors ranged from 21% to 66%, and only one child was on regular cardiac medications. There were no cases of late sudden death. Twenty four hour Holter monitoring performed on survivors was normal (seven) or showed minor abnormalities only (one), suggesting that serious arrhythmia is rare after paediatric myocardial infarction. Myocardial infarction in children had a high early mortality; however, the incidence of serious arrhythmia was low in the survivors, who had a good exercise tolerance even when the left ventricular ejection fraction was low.     

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.     

成人冠状动脉起源异常10例

目的 :了解冠状动脉起源异常 (coronaryarteryoriginanomalies,CAOA)的情况。 方法 :回顾性分析我院从 1998至 2 0 0 3年 838例冠状动脉造影资料。结果 :838例中CAOA 10例 ( 1.19% ) ,其中右冠状动脉起源于左乏氏窦 4例、无冠窦 1例 ,右冠状动脉高位开口 2例 ,左前降支和左回旋支分别独立起源于左乏氏窦 2例 ,单支冠状动脉 1例。结论 :最常见的类型是右冠状动脉起源于左乏氏窦 ,大多数异常类型并不导致严重的临床症状 ,只有少数需要外科手术治疗。     

A 72 year old woman with ALCAPA

ALCAPA syndrome (anomalous origin of the left coronary artery from the pulmonary artery), which causes the left coronary artery to grow with an anomalous origin from the pulmonary artery, is a rare disease which may result in myocardial infarction, congestive heart failure, and sometimes death during the early infantile period. A 72 year old woman with ALCAPA syndrome is presented. The asymptomatic patient presented with a cardiac murmur which was discovered during a routine check up for a gynaecological intervention. Coronary cineangiography established the diagnosis. Although surgical correction is the usual treatment for such cases, medical treatment was preferred for this patient because she was asymptomatic without clinical signs of heart failure.


Keywords: coronary vessel anomalies; ALCAPA syndrome     

Myocardial infarction in childhood: clinical analysis of 17 cases and medium term follow up of survivors.

Between 1979 and 1989 17 patients aged two months to 12 years with acute myocardial infarction of any cause (other than after cardiac surgery) were seen at a children's hospital. Eight died from three days to three years after diagnosis (overall mortality 47%). The nine survivors, now aged 2-17 years, have been followed for one to 10 years (mean follow up five years) after infarction. The commonest causes of myocardial infarction in this series were anomalous origin of left coronary artery from the pulmonary artery (six patients (35%] and Kawasaki disease (five patients (27%]. The main symptoms of acute myocardial infarction were dyspnoea, vomiting, and difficulty feeding. Diagnosis was made in all patients by electrocardiography and confirmed by echocardiography, cardiac catheterisation, or at operation. All survivors were symptom free with excellent exercise capacity. The left ventricular ejection fraction in survivors ranged from 21% to 66%, and only one child was on regular cardiac medications. There were no cases of late sudden death. Twenty four hour Holter monitoring performed on survivors was normal (seven) or showed minor abnormalities only (one), suggesting that serious arrhythmia is rare after paediatric myocardial infarction. Myocardial infarction in children had a high early mortality; however, the incidence of serious arrhythmia was low in the survivors, who had a good exercise tolerance even when the left ventricular ejection fraction was low.     

Anomalous coronary arteries coursing between the aorta and pulmonary trunk: clinical indications for coronary artery bypass

Coronary arteries of anomalous origin with subsequent coursing between the aorta and pulmonary trunk can cause ischaemia, infarction or sudden death. However, reports of surgical correction are sparse due to the rarity of ante-mortem diagnosis. We report two cases in which symptoms were related to anomalous origin of a non-atherosclerotic coronary artery. Surgical repair was performed to prevent sudden death or recurrent ischaemia.     

Noninvasive imaging of anomalous coronary arteries

Anomalous coronary arteries are a rare but recognized cause of myocardial ischemia and sudden death. Most patients with coronary anomalies remain asymptomatic because the anomaly either does not produce any symptoms during life or the first manifestation is sudden death. Noninvasive imaging plays crucial role in diagnosis of the potentially life threatening coronary anomalies.     

The Management of the Older Adult Patient with Anomalous Left Coronary Artery from the Pulmonary Artery Syndrome: A Presentation of Two Cases and Review of the Literature

ALCAPA (anomalous left coronary artery from pulmonary artery) syndrome is a rare congenital abnormality that involves an anomalous insertion of the left coronary artery into the pulmonary artery. Ninety percent of patients present in the first year of life with signs and symptoms of heart failure or sudden cardiac death secondary to chronic myocardial ischemia. There have been an increasing number of reports of ALCAPA patients surviving to adulthood. There seems, however, to be a tendency to die suddenly in the third decade of life. Adult survivors are either asymptomatic or present with mitral regurgitation, cardiomyopathy, myocardial ischemia, or malignant arrhythmias. The management of the older patient presenting with symptoms resulting from ischemia and progressive left ventricular dysfunction remains a challenge. Treatment is largely based on guidelines for adult congenital heart disease management and an extrapolation of evidence from heart failure practice. Currently, surgical reimplantation of the anomalous coronary onto the aorta is the mainstay of treatment. The management of heart failure, sudden cardiac death, and ventricular arrhythmia present problems that are not addressed by reimplantation of the anomalous vessel alone. In this report, we present two cases with different modes of presentation and discuss treatment options.     

The role of coronary bypass operation on children with Kawasaki disease

BACKGROUND: Kawasaki disease, initially called mucocutaneous lymph node syndrome was reported 35 years ago as a new inflammatory disease in infants and children and is characterized by a variety of symptoms and signs resulted from systemic vasculitis. Although the etiology of the disease remains unknown, its serious coronary complications have been proved to cause ischemic heart disease in children, and are now the most common cause of pediatric coronary disease in the world. The incidence of serious coronary sequelae is fortunately low (2-3% of patients with Kawasaki disease), but once myocardial infarction occurs in children, the mortality is quite high (22% at the first infarction). Development of surgical treatment for the disease was essential in preventing premature death and improving the quality of life of children. METHODS AND RESULTS: Coronary revascularization surgery was attempted following careful evaluation of characteristic patters of coronary aneurysms and obstructions secondary to Kawasaki disease, although the surgical efficacy was initially questioned because the disease is inflammatory vasculitis in origin. The operation utilizing the pedicled internal thoracic artery has been demonstrated quite successful and now established as a reliable treatment for inflammatory coronary obstructions due to Kawasaki disease (the Kitamura Operation). There is valid evidence for the internal thoracic artery graft being a viable structure, accommodating in length and diameter for the growth of children. Results of the surgery and long-term prognosis are favorable and postoperative quality of life is markedly improved. CONCLUSIONS: Coronary bypass operation utilizing the pedicled internal thoracic artery is a safe and reliable surgical modality for coronary artery sequelae in children due to Kawasaki disease. Long-term follow-up results up to 20-years are quite satisfactory.     

Acute myocardial infarction caused by "malignant" anomalous right coronary artery detected by multidetector row computed tomography.

Anomalous coronary arteries are usually identified incidentally by angiography or autopsy, but some "malignant" coronary anomalies are associated with a high incidence of syncope, arrhythmia, myocardial infarction, and sudden death. So far, the pathogenesis of the coronary events in such cases has only been revealed by autopsy. In the present case report, a patient with anomalous origin of the right coronary artery from the left sinus of Valsalva developed acute myocardial infarction, and visualization of the anomaly and assessment of the culprit plaque in the artery were done by multidetector row computed tomography and intravascular ultrasound.     

Coronary artery anomalies: Anomalous origin of the left coronary artery and circumflex branch in two patients

Coronary anomalies may be isolated defects or accompany congenital malformations of the heart. The determination of these anomalies is important in the treatment approach and the surgical procedure in bypass and valve surgery. The present article reports on clinical and angiographic findings in two patients with coronary artery anomalies -one patient with an anomalous origin of the left coronary artery from the right aortic sinus, and another patient with an anomalous origin of the circumflex branch of the left coronary artery from the right coronary artery.