A rare case of anomalous left circumflex coronary artery from the left pulmonary artery

The anomalous origin of the coronary artery in children is a rare disease. In particular, an anomalous origin of the left coronary artery from the pulmonary artery is a fatal condition in neonate with myocardial failure. A 46-month-old child with an anomalous origin of the left circumflex artery from the left pulmonary artery was detected due to asymptomatic cardiac murmur and immediately re-implantation of the left circumflex coronary artery was carried out successfully.     

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.     

Anomalous origin of left coronary artery from the right pulmonary artery with coarctation of aorta

The patient had a rare complex of congenital cardiovascular anomalies, namely anomalous origin of left coronary artery from the right pulmonary artery and coarctation of aorta. At the age of 2, the patient underwent coarctation repair. Before the coronary anomaly was detected in adulthood, she developed anginal attacks. Investigation revealed the anomalous origin of left coronary artery from the right pulmonary artery and recoarctation with aneurysm distal to it. Direct implantation of the anomalous coronary artery into the aorta and reconstruction of the right pulmonary artery with a pericardial patch was performed, followed by repair of the recoarctation.     

Anomalous origin of the left coronary artery from the right pulmonary artery associated with complex congenital heart disease

The rare coexistence of anomalous origin of the left coronary artery from the main pulmonary artery with other cardiac malformations prompts us to report an unusual case of anomalous origin of the left coronary artery from the right pulmonary artery associated with complete atrioventricular canal, patent ductus arteriosus, and coarctation of the aorta. It is important to recognize the presence of an anomalous origin of the left coronary artery in complex congenital heart disease since the coronary artery anomaly will increase the morbidity of the associated congenital cardiac malformation. Conversely, correction of the associated congenital cardiac defect may decrease the pulmonary artery pressure resulting in reduced left coronary artery flow, myocardial ischemia, and death.     

Ultrasonic features of anomalous origin of the left coronary artery from the pulmonary artery

Two cases of anomalous origin of the left coronary artery from the pulmonary artery with and without marked dilatation of the right coronary artery were studied by M-mode echocardiography and cross-sectional echocardiography. The M-mode echocardiogram obtained from the case with the dilated right coronary artery demonstrated an abnormal structure anterior to the aortic root. The cross-sectional echocardiogram of the same case visualized the origin of the dilated right coronary artery. In addition, echocardiographic abnormalities suggesting the ischemia of the left ventricle were observed in both cases. Although many of the ultrasonic features are nonspecific, the combination of ultrasonic technique provides useful clues to the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery.     

A rare coronary artery anomaly: anomalous right coronary artery arising from the left anterior descending artery

Anomalous right coronary artery is a rare entity with an incidence of 0.26%. The anomalous origin usually arises from the left sinus valsalva. An anomalous right coronary artery arising from the left anterior descending artery is rare. It is usually known as a benign entity but may have clinical importance due to its course between the aorta and pulmonary artery which may cause myocardial ischemia or sudden cardiac death. Here we present an anomalous coronary artery arising from the left anterior descending artery detected by coronary artery angiogram and confirmed by multislice computed tomography thereafter. 40% retrospective electrocardiographic gating is performed and volume-rendered 3 D and axial MIP (maximum intensity projection) images were reconstructed on a Vitrea post- processing Workstation. The images demonstrated the anomalous origin of the right coronary artery arising from the proximal portion of left anterior descending artery after the first septal perforator and coursing.     

Anomalous origin of the main stem of the left coronary artery from the pulmonary trunk presenting with left ventricular hypertrophy

Patients with anomalous origin of the left coronary artery from the pulmonary trunk may present with paroxysmal angina on exertion, congestive heart failure, dyspnoea, syncope or sudden death. The association of such anomalous origin of the left coronary artery from the pulmonary trunk with a hypertrophic left ventricle is extremely rare. In our cohort of patients with anomalous origin of the left coronary artery from the pulmonary trunk, two presented with a hypertrophic left ventricle. We discuss these cases, accompanied by a review of the English literature describing different morphological anomalies of the coronary arteries associated with left ventricular hypertrophy. Whether the hypertrophy is a result of the evolvement of the collateral coronary system, or due to an additional pathological gene for hypertrophic obstructive cardiomyopathy, remains an enigma. The hypertrophy may have served as a compensatory mechanism accounting for the atypical clinical presentation. We further discuss the possible factors associating the occurrence of anomalous origin of the left coronary artery from the pulmonary trunk and myohypertrophy, supported by the documentation of the regression of hypertrophy following surgical correction of the anomalous arterial origin as seen in our patients.     

Anomalous origin of the right coronary artery from the pulmonary artery

Two patients with an anomalous right coronary artery arising from the pulmonary artery are presented. The first patient was asymptomatic and had a continuous murmur. The correct diagnosis was established by cardiac catheterization and selective angiography. There was a moderate sized shunt from the dilated left anterior descending coronary artery to the right coronary artery, which arose from the main pulmonary artery. The second patient was also asymptomatic but died several days after a sudden cardiorespiratory arrest. On postmortem examination she was demonstrated to have anomalous origin of the right coronary artery from the pulmonary artery. The left coronary artery was completely occluded at the orifice by a thrombus, and microscopic examination demonstrated evidence of both acute and chronic left ventricular ischemia and infarction. The pathophysiologic alterations and potential danger to patients with this anomaly are discussed.     

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

An adolescent boy with anomalous origin of the right coronary artery from the main pulmonary artery is presented. Although he had been asymptomatic, a to and fro heart murmur and slight cardiac enlargement suggested a cardiac malformation. Once the diagnosis was established by cardiac catheterization and angiocardiography, the defect was corrected by transplanting the anomalous coronary vessel to the aorta. This is the first pediatric patient with isolated anomalous right coronary artery from the main pulmonary artery whose condition was diagnosed ante mortem and the first patient to have surgical correction of the malformation.     

Echocardiographic diagnosis of anomalous origin of the left coronary artery from the pulmonary artery.

We found increased systolic coronary flow in transthoracic pulsed wave (PW) Doppler in a 42-year-old patient with anomalous origin of left main coronary artery from the pulmonary artery. This is a characteristic echocardiographic finding in this anomaly in the presence of collateral circulation and coronary L-R shunt. In comparison with so far used echocardiographic criteria this parameter when present allows quick recognition of anomalous origin of left coronary artery from the pulmonary artery, and its differentiation from other potentially lethal coronary anomalies.     

冠状动脉起源异常的检出率与冠状动脉狭窄

目的　评价国人冠状动脉(冠脉)起源异常的冠脉造影检出率,并探讨其与冠脉狭窄之间的关系。方法　回顾性分析2001年1月至2004年6月在我院接受选择性冠脉造影的患者,观察并记录冠脉起源异常的检出频度和类型,以及合并冠脉狭窄的情况。结果　4094例患者中,共检出冠脉起源异常者32例,检出率为0 78%,其中右冠脉异常起源最为多见,占总数的65 6%;合并冠脉狭窄者共11例(占34 3% ), 其中仅5例(占15 6% )累及起源异常的冠脉,且无1例冠脉狭窄单独累及起源异常的冠脉。结论　国人冠脉起源异常的检出率与国外文献报道结果相似,其中以右冠脉起源异常最为多见,冠脉起源异常与冠脉狭窄不具有相关性。     

Off-pump reimplantation of anomalous origin of the right coronary artery

A 30-year-old man was diagnosed to have an anomalous origin of the right coronary from the pulmonary artery. Through a mini-sternotomy, without extracorporeal circulation, reimplantation of the anomalous origin of the right coronary artery from the pulmonary artery to the aorta was successfully performed. This is a successful case of off-pump cardiac correction of this type of isolated anomaly, using a minimally invasive approach.     

Anomalous origin of the left coronary artery from the right pulmonary artery: Report of a case

Summary A 1-year-old boy was referred to our hospital for further investigation of mitral regurgitation. Color-Doppler echocardiography revealed the site of the anomalous origin of the left coronary artery which was confirmed by a cineangiographic study. This case is interesting for several reasons: the first is that the anomalous left coronary artery originated from the right pulmonary artery; the second is that two-dimensional echocardiographic findings can be misleading, i.e., a vessel-like structure which seemed to be the left coronary artery arising normally from the aorta was visualized; and the last is that a two-dimensional color-Doppler echocardiogram was useful in the diagnosis of the anomalous origin of the left coronary artery. The patient underwent implantation of the anomalous left coronary artery as well as Kay's plasty of the mitral valve, and has been doing well since then.     

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.     

Distinguishing between anomalous origin of the left coronary artery from the pulmonary trunk and dilated cardiomyopathy: role of echocardiographic measurement of the right coronary artery diameter

Patients with anomalous origin of the left coronary artery from the pulmonary trunk usually have a large right coronary artery. This study examines the diagnostic value of measuring the diameter of the right coronary artery by echocardiography in distinguishing between this lesion and other causes of dilated cardiomyopathy. The diameter of the right coronary artery and the right coronary artery/aorta ratio were measured in the parasternal short axis view in 40 controls, 11 patients with dilated cardiomyopathy, and 10 with anomalous origin of the left coronary artery from the pulmonary trunk. In the controls, the diameter of the right coronary artery increased with age, but the right coronary artery/aorta ratio remained constant. In the control group the 95% upper limits of prediction for right coronary artery diameter were 1.6 mm for one month of age, 1.8 mm for three months, 2.0 mm for one year, 2.2 mm for two years, 2.4 mm for three years, 2.6 mm for four years, 2.7 mm for six years, 3.0 mm for eight years, and 3.2 mm for 10 years; and for right coronary/aorta ratios the limits were 0.17 for one month to one year, 0.18 for one to six years, 0.19 for six to 10 years, and 0.20 for more than 10 years. All patients with dilated cardiomyopathy had normal right coronary artery diameters and right coronary artery/aorta ratios (0.10-0.13). Those patients with anomalous origin of the left coronary artery from the pulmonary trunk had larger than normal right coronary artery diameter and a significant increase in the right coronary artery/aorta ratio (0.21-0.29). The presence of an anomalous left coronary artery was likely if the diameter of the right coronary artery or the right coronary artery/aorta ratio was larger than the normal 95% limits of prediction.     

Distinguishing between anomalous origin of the left coronary artery from the pulmonary trunk and dilated cardiomyopathy: role of echocardiographic measurement of the right coronary artery diameter.

Patients with anomalous origin of the left coronary artery from the pulmonary trunk usually have a large right coronary artery. This study examines the diagnostic value of measuring the diameter of the right coronary artery by echocardiography in distinguishing between this lesion and other causes of dilated cardiomyopathy. The diameter of the right coronary artery and the right coronary artery/aorta ratio were measured in the parasternal short axis view in 40 controls, 11 patients with dilated cardiomyopathy, and 10 with anomalous origin of the left coronary artery from the pulmonary trunk. In the controls, the diameter of the right coronary artery increased with age, but the right coronary artery/aorta ratio remained constant. In the control group the 95% upper limits of prediction for right coronary artery diameter were 1.6 mm for one month of age, 1.8 mm for three months, 2.0 mm for one year, 2.2 mm for two years, 2.4 mm for three years, 2.6 mm for four years, 2.7 mm for six years, 3.0 mm for eight years, and 3.2 mm for 10 years; and for right coronary/aorta ratios the limits were 0.17 for one month to one year, 0.18 for one to six years, 0.19 for six to 10 years, and 0.20 for more than 10 years. All patients with dilated cardiomyopathy had normal right coronary artery diameters and right coronary artery/aorta ratios (0.10-0.13). Those patients with anomalous origin of the left coronary artery from the pulmonary trunk had larger than normal right coronary artery diameter and a significant increase in the right coronary artery/aorta ratio (0.21-0.29). The presence of an anomalous left coronary artery was likely if the diameter of the right coronary artery or the right coronary artery/aorta ratio was larger than the normal 95% limits of prediction.     

Atypical Bland-White-Garland syndrome: a 58-year-old woman with stenosis of the pulmonary origin of the left coronary artery

The anomalous origin of the left coronary artery from the main pulmonary trunk (also known as Bland-White-Garland syndrome) is a rare congenital malformation that occurs in 0.4% of patients with cardiac anomalies. We present an adult case (a 58-year-old woman) of atypical Bland-White-Garland syndrome. The patient displayed a stenosis at the ostium of the anomalous origin of the left coronary artery and an aortopulmonary fistula. Using conventional angiography, it was not possible to differentiate between an anomalous origin of the pulmonary coronary artery and total stenosis of the left main coronary artery in combination with a pulmonary fistula. However, transesophageal echocardiography (TEE) succeeded in making this differential diagnosis. CONCLUSION: If there is subtotal or total occlusion, TEE can be used for detection of coronary vessel morphology, particularly in cases of coronary anomalies.     

Anomalous origin of the right coronary artery from the pulmonary artery associated with tetralogy of Fallot: description of the pre-surgical diagnosis and surgical repair

Anomalous origin of the right coronary artery from the pulmonary artery is a rare congenital defect. We describe the case of an infant with anomalous origin of the right coronary artery from the pulmonary artery in association with tetralogy of Fallot. This patient had a pre-operative echocardiographic diagnosis, which was confirmed by angiography, and later underwent a successful surgical repair.     

Isolated, anomalous origin of the left anterior descending coronary artery from the right coronary artery with angina pectoris

The case of a 51-year-old woman with exertional angina pectoris and isolated anomalous origin of the left anterior descending coronary artery from the right coronary artery is reported. This anomalous artery was not narrowed, coursed in front of the pulmonary artery and did not present either anatomical derangement at the take off or intramyocardial course. The mechanism of ischaemia could not be identified.     

Anomalous origin of the left main coronary artery from the right sinus of Valsalva

The clinical and angiographic findings in 10 patients with anomalous origin of the left main (LM) coronary artery from the right sinus of Valsalva are presented. The initial course of the anomalous artery was between the aorta and the pulmonary trunk in 6 patients (5 men and 1 woman), and posterior to the aorta in 4 patients (3 women and 1 man). The best coronary angiographic projection to determine the course of the LM coronary artery in relation to the aorta and pulmonary trunk was the right anterior oblique and lateral projections, with a catheter in the pulmonary trunk as a guide. In the right anterior oblique projection left ventriculogram, an increased density at the base of the aorta was seen in all patients with posterior-to-aorta course of the anomalous artery. The blood supply to the first portion of the ventricular septum is provided by 1 or 2 descending septal branches from the anomalous LM artery, when the anomalous vessel is coursing between the aorta and pulmonary trunk. When the anomalous LM courses posteriorly to the aorta, it does not provide any septal branches. In patients with this anomaly, the blood supply to the first portion of the septum is usually provided by descending septal branches originating from the right sinus of Valsalva or from the first portion of the right coronary artery. Six of the 10 patients had severe obstructive coronary artery disease. Four of the 6 patients had significant LM coronary artery disease.