Anomalous origin of the right coronary artery from the pulmonary artery.

Three patients with anomalous origin of the right coronary artery from the pulmonary artery are presented together with a review of the 14 patients with this anomaly previously reported. One of the current patients is the youngest to be diagnosed before operation and the youngest to undergo corrective operation. Although the lesion is usually asymptomatic, it may contribute to sudden death. The only suggestive physical finding is a continuous murmur with diastolic accentuation, which is present only in some patients. There are no diagnostic electrocardiographic or chest roentgenographic changes. Diagnosis is made best by selective left coronary arteriography showing retrograde filling of the right coronary artery from collateral vessels. Operative repair should consist either of direct reimplantation of the proximal right coronary artery into the aortic root or of saphenous vein bypass graft to the coronary artery with ligation of its origin. Operation is recommended when the lesion is recognized.     

Anomalous origin of the left main coronary artery from the right anterior aortic sinus.

Anomalous origin of the left main coronary artery from the right sinus of Valsalva is an uncommon problem, occurring in four distinct patterns. When the left main coronary artery passes between the aorta and the pulmonary trunk, acute myocardial ischemia or sudden death may occur. Angiography is required in establishing a diagnosis. Surgical correction or bypass surgery are sometimes indicated. The authors report two cases of anomalous origin of the left main coronary artery and discuss the anatomic variants, clinical significance, and technical considerations in both diagnosis and management.     

Repair of anomalous origin of right coronary artery from the left sinus of Valsalva

Anomalous origin of the right coronary artery from the left sinus of Valsalva is a rare congenital anomaly but a relatively frequent cause of sudden death in the young. The medical records of 4 consecutive patients with this anomaly were retrospectively reviewed. The first 2 patients underwent coronary artery bypass and had early graft failure. The next 2 patients underwent coronary reimplantation and unroofing and have done well. This limited series suggests that bypass procedures used to treat anomalous origin of the right coronary artery from the left sinus of Valsalva are prone to early graft failure.     

Anomalous origin of the left coronary artery

Coronary artery anomalies are a frequent finding in the general population and often result in sudden death. Therefore, rapid diagnosis and surgical correction are essential. Although coronary arteriography ultimately is necessary to determine the exact nature of the anomaly, two-dimensional echocardiography and radionuclide tests may provide valuable information in the initial workup of patients with chest pain, as demonstrated in the case reported here. This patient, who had angina-like symptoms, was found on cardiac angiography to have an anomalous left coronary artery rising from the right coronary sinus of Valsalva. Double coronary artery bypass grafting involving the left anterior descending and circumflex marginal vessels alleviated symptoms and resulted in improved exercise tolerance. The authors discuss theories explaining the spectrum of ischemia, infarction, and sudden death and surgical alternatives that improve survival rates.     

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presenting with ventricular fibrillation in an adult: a case report

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. The usual clinical course is severe left sided heart failure and mitral valve insufficiency presenting during the first months of life. However, in some cases collateral blood supply from the right coronary artery is sufficient and symptoms may be subtle or even absent. Arrhythmias or sudden cardiac death in adult life may be the first clinical presentation in patients with ALCAPA. We report a case, where a 39-year old woman presented with ventricular fibrillation during phycial exertion. Coronary angiography and CT-angiography revealed an anomalous origin of the left coronary artery, and an aortic reimplantation of the left coronary artery was performed followed by ICD implantation. A review of the literature on ALCAPA is presented along with CT images before and after surgery.     

Anomalous origin of the left coronary artery. A twenty-year review of surgical management.

Children with anomalous origin of the left coronary artery from the pulmonary artery are at risk for myocardial infarction and death. Surgical management of this condition in children has evolved significantly during the past 20 years. Between 1970 and 1990, a total of 20 of these patients underwent surgical intervention at two institutions. Age at operation ranged from 3 weeks to 11 years (mean, 26 months). Twelve patients had congestive heart failure, three were in cardiogenic shock, and two had cardiac murmurs. Operative techniques included ligation (n = 9), subclavian artery anastomosis (n = 5), aortic implantation (n = 3), internal mammary artery anastomosis (n = 1), intrapulmonary tunnel from aortopulmonary window to coronary artery (n = 1), and cardiac transplantation (n = 1). The three deaths in the series occurred at 3 weeks, at 2 months, and at 9 years after ligation. There have been no deaths after establishment of a two coronary artery system or after transplantation. Two of the five patients who had subclavian artery anastomosis to the anomalous coronary artery have severe anastomotic stenosis and collateralization. For patients with anomalous origin of the left coronary artery from the pulmonary artery, we recommend direct aortic implantation of the anomalous coronary artery at the time of diagnosis. Intrapulmonary tunnel from aortopulmonary window to coronary artery, or aorta-coronary bypass with internal mammary artery are recommended for children in whom aortic implantation is not anatomically feasible. Left coronary artery ligation is not indicated for these patients; those who have survived ligation should be considered for elective establishment of a two coronary artery system because of the risk of late death.     

An alternative procedure for correction of anomalous origin of left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiac malformation that is commonly associated with mitral insufficiency. Direct implantation of the anomalous origin of the left coronary artery from the pulmonary artery into the aorta is ideal, but it may not be fit for some patients whose anomalous left coronary arteries arise remotely from the ascending aorta. To solve the same technical problem in a boy with anomalous origin of the left coronary artery from the pulmonary artery in combination with moderate mitral insufficiency, we successfully elongated the anomalous coronary artery by creating a tube-shape graft using part of the pulmonary arterial wall. Simultaneous mitral annuloplasty was performed after a dual-coronary repair.     

Left subclavian-left coronary artery anastomosis for anomalous origin of the left coronary artery. Long-term follow-up

From 1972 to 1987, seven patients, from two to 28 months of age, underwent left subclavian artery-left coronary artery anastomosis for anomalous origin of the left coronary artery from the pulmonary trunk. All of these infants, median age 4 months, had severe congestive heart failure caused by anterolateral myocardial infarctions. There were two hospital deaths (29% mortality rate) with no late deaths after an average 10-year follow-up. All survivors have good exercise tolerance New York Heart Association class I), reduction in heart size, and significant improvement or normalization of ventricular function by echocardiography. Patency of the subclavian-left coronary artery anastomosis has been documented in two of four patients who have undergone catheterization. In contrast to other revascularizing procedures for treatment of an anomalous origin of the left coronary artery, anastomosis of the left subclavian to the left coronary artery may be performed without cardiopulmonary bypass or aortic occlusion. Moreover, this procedure appears to have an acceptable mortality rate with excellent long-term functional results in critically ill infants.     

Aberrant origin of the left main coronary artery arising from the right coronary artery associated with coronary artery disease: a case report

Coronary artery anomalies are not frequent, nevertheless they are associated with increased and potentially lethal cardiac events. Recognition of these anomalies is fundamental in patients undergoing diagnostic or interventional coronary angiography. Most patients presenting with coronary anomalies are asymptomatic, but the risk of myocardial ischemia and sudden death requires the treatment of those patients. Different therapeutic options have been discussed, including surgery, conservative therapy, and interventional approaches. In this report, an aberrant origin of the left main coronary artery arising from the right coronary artery associated with coronary artery atherosclerosis and its surgical correction is described.     

Surgical correction of congenital left coronary artery-main pulmonary artery fistula in association with anomalous right coronary artery.

The association of a left coronary artery-main pulmonary artery fistula and an anomalous right coronary artery originating from the main pulmonary artery is the subject of this report. This unique combination of congenital cardiac anomalies establishes a double coronary steal from the left coronary artery, which hemodynamically represents the sole source of myocardial perfusion. The left coronary artery-main pulmonary artery fistula was closed and the coexisting anomalous right coronary artery reimplanted into the anterior aspect of the ascencing aorta. A dual coronary supply was therefore established and thus eliminated the potential threat of total myocardial ischemia should the left coronary artery become critically compromised. Patency of both the left coronary artery and the transplanted right coronary artery was documented 1 year postoperatively by aortic root angiography.     

Catastrophic myocardial ischemia resulting from a left coronary artery anomaly with an origin in the right sinus of Valsalva

A congenital left coronary artery anomaly originating from the right aortic sinus is a rare congenital defect associated with the risk of sudden death in young individuals. In most cases, the proximal portion of the anomalous left coronary artery exists between the ascending aorta and pulmonary trunk, and it has an intramural aortic course; this could critically impair the left coronary flow owing to compression of the anomalous left main trunk between the great vessels during exercise. Herein, we report a 14-year-old boy who experienced cardiac collapse due to an acute myocardial infarction after long-distance running. After resuscitation using percutaneous cardiopulmonary support, computed tomography and coronary angiography revealed an anomalous origin of the left main coronary artery in the right sinus of Valsalva and a proximal course between the aorta and pulmonary trunk. The patient was successfully treated using an unroofing procedure of the intramural left coronary artery.     

Internal thoracic artery grafting for congenital coronary malformations.

We report 2 patients with congenital coronary anomalies (atresia of left main coronary artery and anomalous origin of the left coronary artery from the pulmonary artery) successfully treated with single or double internal thoracic artery grafting. Because the internal thoracic artery has a potential for circumferential as well as longitudinal development, and because of the uncertainty of ultimate vein graft function, we believe that the internal thoracic artery is the best graft material for the treatment of congenital coronary malformations requiring bypass operation in children, adolescents, or even in adults.     

Anomalous origin of left coronary artery from pulmonary artery in association with intracardiac lesions. Report of two cases.

Two cases are presented to illustrate the diagnostic and therapeutic dilemma when an anomalous origin of the left coronary artery from the pulmonary artery occurs with a hemodynamically significant intracardiac lesion. Hemodynamic alterations resulting from the associated intracardiac lesion may obscure the clinical features of the coronary arterial abnormality, and the appearance of normal coronary arterial distribution with left ventricular or aortic root angiography may create a false impression of a normal origin of the coronary arterial supply. A high index of suspicion may be the only lead to the detection of an anomalous origin of the left coronary artery from the pulmonary artery in association with an intracardiac lesion. In addition to the intracardiac lesion known to be associated with coronary arterial abnormalities, the question of anomalous coronary supply should be raised by the finding of poor left ventricular function or mitral insufficiency after operation (unexplainable by other causes), by the development of poor myocardial function during cardiac catheterization or anesthesia, or by the significant enlargement of a coronary artery seen during left ventriculography or supravalvular aortography.     

Anomalous origin of left coronary artery from pulmonary artery. Case report and review of literature concerning teen-agers and adults.

An adult with angina was found to have anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Review of the literature regarding this anomaly in teen-agers and adults disclosed only 25 cases diagnosed during life. Eighteen additional cases of ALCAPA in this age group have been diagnosed post mortem. In this report, we will review the management of teen-agers and adults in whom ALCAPA was correctly diagnosed during life. We shall also describe the eighth case of successful aorta-left coronary artery grafting with the saphenous vein in this age group. This case brings the total in the literature to 44. Of those patients offered surgical therapy, 13 underwent successful ligation of the anomalous artery. Saphenous vein grafts were employed in 8. Five did not undergo ligation or saphenous vein grafting. There was one death. It would appear that saphenous vein grafting is the definitive means of surgically correcting ALCAPA, because it restores the dual coronary circulation.     

Surgical therapy of an aberrant origin of the left anterior descending coronary artery from the right sinus of valsalva: a case report

Anomalous aortic origin of a coronary artery from the incorrect coronary sinus of Valsalva is a rare congenital cardiac defect that is associated with an increased risk of sudden death. In most of these defects, the anomalous coronary artery takes an intramural course between the great arteries and leaves the aortic wall from the appropriate coronary sinus of Valsalva. Many times this anomalous artery shares a common orifice with the other coronary artery. We report on a previously healthy 15-year-old boy who presented with signs of an anterolateral myocardial infarction after physical activity (soccer game). Transthoracic echocardiography revealed an aberrant origin of the left anterior descending coronary artery from the right sinus of Valsalva. Repair was accomplished by unroofing the intramural segment. Because the intramural segment was below the commissure, detachment of the intercoronary commissure was not necessary. To conclude, transthoracic echocardiography can accurately depict this rare anomaly, and in symptomatic patients surgical therapy is indicated.     

Simplified technique for correction of anomalous origin of left coronary artery from the anterior aortic sinus

Anomalous origin of the left main coronary artery from the right anterior coronary sinus has been associated with high incidence of sudden death in young adults. We describe a simplified approach to this rare congenital anomaly, which avoids the need for commissural post resuspension or relocation of the coronary button.     

The need for follow-up after surgical correction of anomalous left coronary artery arising from the pulmonary artery

Four patients are described with an anomalous origin of the left coronary artery from the pulmonary artery. Three were treated by ligation of the abnormal left coronary artery at its anomalous origin; one of them died during surgery. One patient was treated with reimplantation of the left coronary artery into the aorta. Of the survivors 2 are in normal health, but 1 developed aortic valve incompetence after reimplantation of the left coronary artery into the aorta and 1 had persistent left ventricular wall motion abnormalities and developed mitral valve prolapse. The third survivor has impaired health with diffuse left ventricular wall motion abnormality. Careful follow-up after surgery for an anomalous left coronary artery arising from the pulmonary artery is necessary for secondary prevention of problems due to incomplete recovery of myocardium or problems as a consequence of surgical therapy.     

法洛四联症伴冠状动脉畸形的外科治疗

目的：总结法洛四联症伴冠状动脉畸形手术治疗经验,探讨冠状动脉畸形在法洛四联症纠治手术中的意义。方法：自1994年1月至1999年12月,手术治疗法洛四联症伴冠状动脉畸形15例,年龄43d-8岁,其中右冠状动脉起源于左冠状动脉或左前降支6例,单支左冠状动脉和左前降支起源于右冠状动脉各3例。双前降支,单支右冠状动脉和右冠状动脉肺动脉瘘各1例。一期根治手术13例,姑息手术2例。结果：姑息手术无死亡,根治手术早期死亡1例;无冠状动脉手术意外损伤;随访结果满意。结论：根据肺血管条件和畸形冠状动脉走行特点,选择适当的手术方式和右室流出道重建方法可提高手术效果;冠状动脉畸形不再是法洛四联症纠治手术中的风险因素。     

Isolated anomalous origin of right coronary artery from the main pulmonary artery

The anomalous origin of the right coronary artery (ARCA) from the main pulmonary artery (MPA) is a rare congenital cardiac malformation and usually associated with other cardiac anomalies. Most patients with isolated ARCA from MPA remain asymptomatic, but they may develop myocardial ischemia and even sudden death. We reported an asymptomatic 7-year-old boy referred for evaluation of a heart murmur. Isolation of ARCA from MPA was diagnosed by echocardiography and then confirmed by cardiac catheterization and angiography. The right coronary artery was re-implanted into the ascending aorta. A preoperative thallium-201 myocardial perfusion showed a myocardial ischemia pattern in the anterolateral septal area after a dipyridamole stress test; the ischemia was completely resolved after surgery.     

Two cases of anomalous origin of LAD from right coronary artery requiring coronary artery bypass

We experienced two cases with anomalous origin of the left anterior descending artery (LAD) from the proximal right coronary artery requiring coronary artery bypass grafting. A 66-yr old female with a long history of angina and a positive stress test had the anomalous artery coursing anterior to the right ventricular outflow tract. A 42-yr old male with worsening angina after an anteroseptal myocardial infarction had the anomalous artery running between the great vessels. Both patients underwent left internal mammary artery-to-LAD bypass on the beating heart with complete resolution of ischemic symptoms.Isolated coronary artery anomaly is an uncommon disease (0.6-1.2%) in patients undergoing cardiac catheterization. An anomalous origin of the LAD from the proximal right coronary artery (RCA) or the right sinus of Valsalva (RSV) is very rare, found in 1.2-6.1% of all coronary anomalies. This coronary anomaly has been considered potentially serious but functionally unimportant. We report two cases of anomalous LAD from the proximal RCA resulting in anterior wall ischemia which was effectively treated by coronary artery bypass surgery.