Anomalous origin of the right coronary artery from the pulmonary artery. Two case reports

Anomalous origin of the right coronary artery originating from the pulmonary trunk (ARCAPA) is a rare but potentially fatal anomaly. We are presenting two cases of ARCAPA and reviewing the main previous published data on this lesion. The first patient presented at the age of 5 months with respiratory distress and severe chest infection. He was found to have heart murmur and cardiomegaly on chest X ray. Echocardiographic and angiographic data confirmed an ARCAPA associated to a large malalignment ventricular septal defect and distal pulmonary artery aneurysms. He underwent surgical closure of the ventricular septal defect and reimplantation of the ARCAPA on the aorta with good result.The second case is an 11 year old male patient, complaining of dizziness and chest pain on exertion. Echocardiographic and angiographic data confirmed ARCAPA associated to a valvular pulmonary stenosis. He was operated on successfully. He got also direct reimplantation of the anomalous coronary artery on the aorta and a pulmonary valve commissurotomy. An anomalous origin of the right coronary artery is a rare condition but may lead to myocardial ischemia and sudden death. Diagnosis is mainly made by echocardiography and confirmed by conventional coronary arteriography. Operative correction is the appropriate treatment for an anomalous coronary artery arising from the pulmonary trunk.     

Imaging of Right Coronary Artery Ostial Stents by Transesophageal Echocardiography

A 55-year-old female with a history of rheumatic mitral stenosis and coronary artery disease underwent a transesophageal echocardiographic examination to assess the feasibility of performing balloon mitral valvuloplasty. During the examination, an unusual echodensity was incidentally discovered in the right sinus of Valsalva which represented a coronary artery stent. Transesophageal imaging of ostial stents have rarely been described in the literature. Their characteristic echocardiographic findings are discussed.     

Bilateral congenital coronary ostial obstruction: Exertional syncope in an otherwise healthy 8‐year‐old

Congenital stenosis/atresia of a coronary artery is an exquisitely rare anomaly (Congenit Heart Dis, 2, 2007, 347) with increased risk of sudden death. Bilateral coronary obstruction is even more unusual but has been reported in conjunction with aortic valve disease, syphilis, and Takayasu's arteritis. To the best of our knowledge, obstruction of both coronaries in a pediatric patient has only been reported once (Ann Thorac Surg, 55, 1993, 1564). We present a patient with an intramural, anomalous aortic origin of the right coronary artery from the contralateral sinus (AAORCA) with near atresia of the left main coronary ostium. The diagnosis was made by echocardiogram and confirmed by catheterization and magnetic resonance imaging.     

An Unusual Presentation of an Anomalous Left Coronary Artery Arising from the Pulmonary Artery (ALCAPA) in an Adult: Anterior Papillary Muscle Rupture Causing Severe Mitral Regurgitation

We describe a 29-year-old male, previously in good health, with no history of angina pectoris and no risk factors for ischemic heart disease presenting with biventricular failure and severe mitral valve regurgitation. There were no signs or serological test results to suggest infective endocarditis. Transthoracic echocardiography (TTE) revealed severe anterior mitral valve prolapse secondary to papillary muscle rupture, severe mitral valve regurgitation, as well as an anterior myocardial wall hypokinesis. Parasternal short-axis view showed an anomalous left coronary artery arising from the pulmonary artery (ALCAPA), which was confirmed on coronary angiography. This is an unusual presentation of ALCAPA in an adult.     

Unique Echocardiographic Markers of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) in the Adult

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect in adults. We report a 38‐year‐old male presenting with exertional syncope. He was referred for the evaluation of multiple muscular ventricular septal defects diagnosed on an outpatient echocardiogram. Echocardiography revealed mild left ventricular enlargement, abnormal flow‐pattern in the ventricular septum and dilatation of the right coronary artery. Pulsed‐wave Doppler with sample volume placed in the coronary ostium showed systolic coronary flow predominancy. This unique finding is characteristic for ALCAPA and can differentiate it from other coronary anomalies. Coronary angiography confirmed ALCAPA syndrome. Surgical correction was planned.     

Anomalous right coronary artery origin from the pulmonary artery (ARCAPA) coexisting with obstructive atherosclerotic disease of the left coronary artery

Coronary artery anomalies arising from the pulmonary artery are rare, result in reversed flow in the affected coronary artery, and are commonly associated with myocardial ischemia in infancy or childhood. Uncorrected survival to adult age is rare. We present a 77-year-old individual with anomalous right coronary artery from the pulmonary artery (ARCAPA), diagnosed by coronary angiography, whose clinical presentation was governed by coexisting obstructive atherosclerotic coronary disease.     

Echocardiographic Diagnosis of a Partially Thrombosed Right Coronary Artery Aneurysm in a Patient with Polycystic Disease

This report describes a patient with polycystic disease of the kidneys and the liver and an aneurysm of the proximal right coronary artery. Transthoracic and transesophageal echocardiographic imaging showed a 5 times 5 cm oval-shaped, spherical tumor in the atrioventricular corner. The tumor was partially filled with homogenous echostructures. Coronary angiography showed aneurysmatic configuration of the right coronary artery, but no region demonstrating a mass of similar size and shape as seen by echocardiography. The finding of a partially thrombosed coronary artery aneurysm was subsequently confirmed by surgery.     

Anomalous origin of the left coronary arterial tree through three stems--one from the pulmonary trunk

In an 18-year-old asymptomatic male athlete, the left anterior descending coronary artery was found to arise from the pulmonary trunk. The remainder of the left coronary arterial tree arose through two stems from the aorta. Collateral retrograde filling of the left anterior descending coronary artery from the right coronary artery and the left circumflex coronary artery was demonstrated, but we found no evidence of left-to-right shunting into the pulmonary trunk. The patient has chosen conservative treatment, thus offering an unusual opportunity to follow the natural course of this lesion, which may increase understanding of its natural history.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Pre- and Postoperative Echocardiography Findings in Three Unusual Cases

Diagnosis of anomalous left coronary artery from the pulmonary artery was prospectively established by color Doppler echocardiography in three patients. In two asymptomatic girls, aged 10 years and 5 years respectively, referred for evaluation of a murmur with normal ECG and chest X ray, two-dimensional and pulsed-Doppler examination showed no intracardiac abnormalities. Color flow mapping detected flow in a dilated right coronary artery and left coronary artery to pulmonary artery shunting. Color guided pulsed-Doppler examination permitted further evaluation of coronary flow. Another child had a dilated cardiomyopathy with an echo-dense anterolateral papillary muscle and mitral insufficiency. Postoperative echoes after subclavian to left coronary artery anastomosis and ligation of the left coronary artery at its origin showed residual high-velocity shunting resulting in reoperation in one case and a moderate supravalvular pulmonic stenosis in another. These findings further emphasize the benefit of color Doppler echocardiography in the pre- and postoperative evaluation of anomalous left coronary artery. (ECHOCARDIOGRAPHY, Volume 8, September 1991)     

Anomalous origin of the right coronary artery from the left anterior descending coronary artery

A 77-year-old male presented with a recent posterior myocardial infarction for coronary angiography. This angiogram revealed a rare, previously unreported anomalous origin of the right coronary artery from the proximal left anterior descending coronary artery distal to the first major diagonal branch. Cathet. Cardiovasc. Diagn. 42:308–309, 1997. © 1997 Wiley-Liss, Inc.     

Anomalous origin of the right coronary artery from the ascending aorta above the left coronary sinus

Ectopic origin of the right coronary artery is an infrequent anomaly. We report a case in which the right coronary artery arose from the ascending aorta above the left sinus. This anomaly was associated with a bicuspid aortic valve. Techniques for delineation of the ectopic origin of the right coronary artery are discussed.     

The role of echocardiography in anomalous origin of coronary artery from pulmonary artery (ALCAPA): Simple tool for a complex diagnosis

Anomalous origin of coronary artery from pulmonary artery (ALCAPA) is a rare coronary anomaly usually presenting in the first year of life. Adult presentation suggests a wide range of differential diagnosis such as myocarditis, dilated cardiomyopathy, and coronary artery disease. We have presented here the major distinctive echocardiographic features of ALCAPA through 4 cases. Since echocardiography is often used as the first imaging modality in these clinical scenarios, recognizing the characteristics of ALCAPA in a routine echocardiographic examination can transform a readily available, low‐cost tool into a valuable discriminative modality.     

Anomalous Origin of the Right Coronary Artery from the Pulmonary Trunk:

Anomalous origin of the right coronary artery from the pulmonary trunk is a rare congenital anomaly, which may be detected noninvasively by echocardiography. We report a case of definitive diagnosis of this anomaly using transesophageal echocardiography and Doppler color flow mapping in a 36-year-old woman. Of special interest is the fact that the most prominent echocardiographic feature was turbulent flow in the region of the interventricular septum streaming into the dilated RCA.     

An Unusual Combination of an Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) and a Right Coronary Artery System with Two Separate Ostia from the Aorta in an Adult

We describe a patient with an infrequent combination of variants in both the right and the left coronary arterial ostia, namely a combination of two separate right coronary artery (RCA) ostia from the aorta, and an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). To our knowledge, such a combination has not been previously reported. Based on published statistics for individual variants, such a combination is expected to occur approximately once for every 500,000 to one million live births. ALCAPA and dual RCA anatomy was characterized in our patient by echocardiography, conventional angiography, and multidetector computed tomography before and after Takeuchi repair. (Echocardiography 2010;27:E13-E17)     

Anomalous coronary circulation: left anterior descending and left circumflex coronary arteries arising from the right sinus of valsalva

Approximately 1% of adults who undergo cardiac catheterization have coronary anomalies. Patients may present with chest pain, arrhythmias, presyncope, and sometimes sudden cardiac death. Multidetector computed tomography (MDCT) is an excellent tool for identifying coronary artery anomalies and defining their course and relationship to the great vessels and surrounding structures; its value is incremental to conventional angiography. We present a rare case of a coronary anomaly involving three separate ostia at the right sinus of Valsalva for the left and right coronary vessels.     

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

The objective of this study was to analyze echocardiographic characteristics of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and to explore the diagnostic value of transthoracic echocardiography. The echocardiographic characteristics of 8 patients hospitalized with ALCAPA from 2000 to 2005 were analyzed retrospectively, including the results of real time three-dimensional echocardiography in one case, and compared with angiographic results. Eight cases included 6 older type patients and 2 infant type patients. Echocardiography showed abnormal vessel inserting into pulmonary artery (PA), continuous shunt into PA and intercoronary collateral signals within the ventricular septum in all cases and bifurcate structure of the abnormal vessel with retrograde filling in 4 cases. The morphological and functional changes and valvular regurgitation induced by insufficient myocardial perfusion were also evaluated. In former 4 patients, 2 cases were misdiagnosed as right coronary artery-PA fistula and the other 2 cases were given an uncertain diagnosis of anomalous origin of the coronary artery because of the visualization of the echo-free linear structure which apparently arose from the aorta resembling a normal left coronary artery. The latter 4 patients were correctly diagnosed by excluding the aforementioned interference. The diagnosis of ALCAPA was confirmed by angiocardiography in all patients and by intraoperative findings in 4 patients. Based on the apprehension of ultrasonic features and the enhancement of diagnostic alertness, the echocardiography can evaluate ALCAPA accurately and give more information than angiography. It may be the first diagnostic choice.     

Primary percutaneous coronary intervention of an anomalous right coronary artery originating from the left sinus of valsalva

The presence of an anomalous origin of a coronary artery as the infarct related vessel during primary percutaneous coronary intervention for acute ST elevation myocardial infarction is rare and may present a technical challenge. We reported on a primary coronary percutaneous intervention performed in a right coronary artery originating from the left coronary sinus. The technical tips and tricks of treating congenital coronary anomalies are reviewed.     

Two-Dimensional Echocardiographic Identification of Peripheral Coronary Artery Ectasia in an Adult

Coronary artery aneurysms (ectasia) have been reported to be present in up to 4.9% of patients undergoing coronary angiography. In children with Kawasaki's disease or coronary arteriovenous fistula, the transthoracic two-dimensional echocardiographic findings associated with peripheral vessel aneurysms have been previously described. Echocardiographic imaging of peripheral coronary artery segments in the adult is difficult and to date only imaging of large, proximal coronary artery aneurysms has been reported. We report a case of an adult patient with angiographically documented extensive right coronary artery ectasia in which peripheral vessel aneurysms were identified with transthoracic echocardiography.     

Isolated Single Coronary Artery Presenting as Acute Coronary Syndrome: Case Report and Review

Congenital single coronary artery is commonly associated with complex congenital heart diseases and manifests in infancy or childhood. But isolated single coronary artery is a rare congenital anomaly which can present as acute coronary syndrome in adults. The aim of the work is to discuss on isolated single coronary artery in two adults presenting as acute coronary syndrome. The first case underwent coronary angiography (CAG) through right radial route, but switched over to femoral for confirmation of diagnosis and due to radial spasm. An aortic root angiogram was done to rule out presence of any other coronary ostia. It revealed a single coronary artery originating from right sinus of valsalva. After giving rise to posterior descending artery branch at crux, it continued in the atrioventricular groove to the anterior basal surface of the heart and traversed as anterior descending artery. There was no atheromatous occlusive stenosis. This is R-I type single coronary artery as per Lipton classification. In the second case, angiography was completed through right radial route. It revealed a single coronary artery arising from right aortic sinus. Anterior descending and circumflex branch were originating from proximal common trunk of the single coronary artery and supplying the left side of the heart. The right coronary artery has diffuse atheromatous disease without significant stenosis in any major branch. This is R-III C type as per Lipton classification. A coronary anomaly of both origin and course is very rare. It may be encountered in adults evaluated for atherosclerotic coronary heart disease. Knowledge and understanding of anatomical types of this congenital anomaly will reduce time, anxiety, complications during CAG and cardiac surgery.     

Right coronary artery cirsoid with fistulous connection to the coronary sinus

A 61-yr-old woman was referred to our hospital for evaluation of a suspected right atrial myxoma. The transesophageal echocardiogram suggested the presence of an anomalous right coronary artery with fistulous connection to the coronary sinus. At cardiac catheterization, an oxygen stepup in the right atrium indicated a 1.3:1.0 left-to-right shunt. Aortic root angiography showed a large and calcified right coronary artery cirsoid draining to the coronary sinus, which appeared remarkably dilated. In this rare anomaly, cardiac catheterization is necessary, not only to quantify the magnitude of the left-to-right shunt, which is an important requirement for the indication to surgical treatment, but also to confirm the echocardiographic diagnosis.© 1993 Wlley-Liss, Inc