Anomalous Circumflex Coronary Artery Research Articles

Angiographic recognition and percutaneous intervention of an occluded anomalous circumflex coronary artery causing acute myocardial infarction: a case report

Coronary arteries with anomalous origin are unusual but can be responsible for myocardial infarction. Acute occlusion of an anomalous coronary artery is not only easily missed on angiography but also technically challenging for percutaneous intervention. In this report, we present our experience in a patient with an anomalous circumflex being the culprit vessel in acute myocardial infarction. We report a case of a 74-year- old male patient presented with sudden chest pain. The diagnosis of occluded anomalous circumflex coronary artery was made by invasive coronary angiography shortly after admission. The patient was stented with a 2.5 x 18mm bare metal stent and was discharged with intensive medical treatment and regular follow-up. Interventional cardiologists should keep in mind there are several anomalous origin, the most frequent anomaly being a circumflex artery with origin from the right coronary artery or the right sinus of Valsalva.

To Intervene or Not Intervene? That Is the Question. Do New Wall Motion Changes After Aortic Valve Replacement in the Presence of an Anomalous Circumflex Coronary Artery Dictate Action?

To Intervene or Not Intervene? That Is the Question. Do New Wall Motion Changes After Aortic Valve Replacement in the Presence of an Anomalous Circumflex Coronary Artery Dictate Action?

Surgical Aortic Valve Replacement in the Setting of Anomalous Circumflex Coronary Artery

BackgroundThe anomalous circumflex coronary artery (ACCA) from the right coronary artery or sinus of Valsalva lies in proximity to the aortic valve annulus. This study sought to determine the prevalence of injury to the ACCA during surgical aortic valve replacement (SAVR). MethodsWe queried the databases of the Departments of Cardiovascular Surgery and Cardiovascular Diseases of Mayo Clinic, Rochester, Minnesota for all patients who underwent SAVR in the setting of an ACCA. The study investigators identified 31 patients operated on from September 2002 through December 2018. The end point was myocardial ischemia in the distribution of the ACCA. ResultsThe patients’ mean age was 69 ± 11 years, sex was female in 8 patients (26%), and ejection fraction was 62% (interquartile range, 59% to 68%). No patient underwent exploration of the ACCA, but 5 (16%) had a coronary artery bypass graft to the ACCA. No patient demonstrated myocardial infarction or underwent perioperative intervention on the ACCA; however, discharge echocardiography showed new lateral wall motion abnormality in 5 (16%) patients that was associated with a reduction in ejection fraction of −11% from baseline (P = .007). Coronary artery bypass graft to the ACCA was not protective of new lateral wall motion abnormality (P = .968). Mortality was 34% ± 10% at 10 years and was not associated with new lateral wall motion abnormality (log-rank test P = .183). ConclusionsClinically apparent myocardial infarction was not identified after SAVR, but echocardiographic evidence of myocardial ischemia in the distribution of the ACCA was identified in 16% of patients. Protective adjuvant intervention on the ACCA may be indicated. Further study is warranted.

ACCAPA: anomalous circumflex coronary artery origin from pulmonary artery.

Anomalous circumflex coronary artery origin from pulmonary artery is a very rare congenital heart disease. Misdiagnosis of this condition may lead to inadequate myocardial protection during cardiopulmonary bypass surgery.

Outcomes in Anomalous Aortic Origin of a Coronary Artery Following a Prospective Standardized Approach

Anomalous aortic origin of a coronary artery (CA) is the second leading cause of sudden cardiac death in young athletes. Management is controversial and longitudinal follow-up data are sparse. We aim to evaluate outcomes in a prospective study of anomalous aortic origin of CA patients following a standardized algorithm. Patients with anomalous aortic origin of a CA were followed prospectively from December 2012 to April 2017. All patients were evaluated following a standardized algorithm, and data were reviewed by a dedicated multidisciplinary team. Assessment of myocardial perfusion was performed using stress imaging. High-risk patients (high-risk anatomy-anomalous left CA from the opposite sinus, presence of intramurality, abnormal ostium-and symptoms or evidence of myocardial ischemia) were offered surgery or exercise restriction (if deemed high risk for surgical intervention). Univariate and multivariable analyses were used to determine predictors of high risk. Of 201 patients evaluated, 163 met inclusion criteria: 116 anomalous right CA (71%), 25 anomalous left CA (15%), 17 single CA (10%), and 5 anomalous circumflex CA (3%). Patients presented as an incidental finding (n=80, 49%), with exertional (n=31, 21%) and nonexertional (n=32, 20%) symptoms and following sudden cardiac arrest/shock (n=5, 3%). Eighty-two patients (50.3%) were considered high risk. Predictors of high risk were older age at diagnosis, black race, intramural course, and exertional syncope. Most patients (82%) are allowed unrestrictive sports activities. Forty-seven patients had surgery (11 anomalous left CA and 36 anomalous right CA), 3 (6.4%) remained restricted from sports activities. All patients are alive at a median follow-up of 1.6 (interquartile range, 0.7-2.8) years. In this prospective cohort of patients with anomalous aortic origin of a CA, most have remained free of exercise restrictions. Development of a multidisciplinary team has allowed a consistent approach and may have implications in risk stratification and long-term prognosis.

VD05 BENTALL OPERATION IN BICUSPID VALVE AND ANOMALOUS LEFT CIRCUMFLEX CORONARY ARTERY: ANATOMICAL AND TECHNICAL IMPLICATIONS

Abstract Bicuspid aortic valve (BAV) is often associated with coronary abnormalities. The finding of BAV associated with an anomalous circumflex coronary artery (ACCA) originating from the right Valsalva sinus (VS) and surrounding the non-coronary VS is however a rather rare occurrence. We report a case of successful Bentall operation with a composite button technique in a 56-year-old man with aortic root dilatation, BAV insufficiency and coexisting ACCA. In this case a 25 SJM composite graft was implanted in supraannular fashion rather than intraannular to avoid: 1) ACCA injury or obstruction of the proximal course near the annulus by suture ligation, 2) compression by prosthetic ring, 3) distortion of ACCA during ostia reimplantation. We demonstrated that attention to the anatomic relationship of ACCA to the aorta is crucial and prosthesis supraannular implantation technique allows a safe aortic root replacement.

Familial clustering of cardiac conditions in patients with anomalous aortic origin of a coronary artery and myocardial bridges.

Anomalous aortic origin of a coronary artery is the second leading cause of sudden cardiac arrest/death in young athletes in the United States of America. Limited data are available regarding family history in this patient population. Patients were evaluated prospectively from 12/2012 to 02/2017 in the Coronary Anomalies Program at Texas Children's Hospital. Relevant family history included the presence of CHD, sudden cardiac arrest/death, arrhythmia/pacemaker use, cardiomyopathy, and atherosclerotic coronary artery disease before the age of 50 years. The presence of one or more of these in 1st- or 2nd-degree relatives was considered significant. Of 168 unrelated probands (171 patients total) included, 36 (21%) had significant family history involving 19 (53%) 1st-degree and 17 (47%) 2nd-degree relatives. Positive family history led to cardiology referral in nine (5%) patients and the presence of abnormal tests/symptoms in the remaining patients. Coronary anomalies in probands with positive family history were anomalous right (27), anomalous left (five), single right coronary artery (two), myocardial bridge (one), and anomalous circumflex coronary artery (one). Conditions present in their family members included sudden cardiac arrest/death (15, 42%), atherosclerotic coronary artery disease (14, 39%), cardiomyopathy (12, 33%), CHD (11, 31%), coronary anomalies (3, 8%), myocardial bridge (1, 3%), long-QT syndrome (2, 6%), and Wolff-Parkinson-White (1, 3%). In patients with anomalous aortic origin of a coronary artery and/or myocardial bridges, there appears to be familial clustering of cardiac diseases in approximately 20% of patients, half of these with early occurrence of sudden cardiac arrest/death in the family.

Aortic Root Sign

Appearance: The aortic root sign describes an anomalous circumflex coronary artery (ACCA) that emerges from the contralateral coronary sinus, and travels underneath the aorta to supply blood to its normal vascular territory. Explanation: The moniker, “aortic root sign,” was first coined by Page et al1 on conventional angiography to describe ACCA as arising from the proximal right coronary artery or right coronary sinus. The aberrant vessel courses inferiorly behind the aortic root within the groove between the left atrium and aorta,2 hence the term “aortic root sign” (Fig. 1).FIGURE 1: Fifty-six-year-old patient with atypical chest pain. A, Right anterior oblique ventriculogram from left heart catheterization demonstrates anomalous course of the left circumflex (LCX) en face below the aortic root (classic aortic root “dot” sign; thin arrow) (LV=left ventricle). B, Electrocardiogram-gated cardiac computed tomography (CT) matched in projection of the cardiac catheterization reveals the same signature appearance of the anomalous LCX (thin arrow). C, CT correlate of the conventional aortic root “dot” sign seen on an axial image from an electrocardiogram-gated CT reveals the anomalous LCX coursing inferior to the noncoronary sinus of the aortic root (medium arrow) (RA=right atrium, RV=right ventricle, LA=left atrium). D, Volume-rendered image of the aortic root demonstrates coronary origin from the right sinus of Valsalva (asterisk) with immediate takeoff of the anomalous LCX (arrowhead) from the normal right coronary artery (large arrow).Discussion: Traditionally coronary artery anomalies (CAAs) were thought to have little significance, and were often discovered as incidental findings on angiography. However, patients may present with clinically significant disease.2 In fact, CAAs are the second most common cause of sudden cardiac death in young athletes in the United States.2 ACCA was first described by Antopol and Kugel.3 The prevalence of ACCA reported in the coronary angiography literature ranges from 0.18% to 0.67%, making it the most common CAA. Some authors postulate that the oblique angulation of its origin predisposes ACCA to atherosclerosis. However, most clinicians agree that ACCA is a benign, asymptomatic anomaly. Indeed, the retroaortic course of ACCA, as described by the aortic root sign, rarely leads to ischemia or sudden cardiac death. On the contrary, CAAs that travel along an interarterial course between the aorta and pulmonary artery are more prone to ischemia during strenuous exercise.2 Despite the low risk of ischemia and sudden death with ACCA, recognition of this anomaly is still of great clinical importance because failure to identify this aberrant vessel can lead to adverse patient outcomes, especially during cardiac surgery. Therefore, it is crucial for the angiographer as well as the radiologist to be adept at recognizing ACCA and the other coronary anomalies, especially given the ease of diagnosis with noninvasive coronary imaging techniques, such as multidetector computed tomography4 and cardiac magnetic resonance imaging.5

An Unusual Case of Angina in the Young

Anomalous circumflex coronary artery from the pulmonary artery (ACCAPA) is a rare variant of anomalous left coronary artery from the pulmonary artery (ALCAPA), which has previously been described in 11 adult case reports since it was first discovered in 1992.

Twin Circumflex Artery: a rare coronary artery anomaly

A circumflex coronary artery anomalously originating from the right sinus of Valsalva is the most common coronary anomaly; however dual origin of the circumflex artery is a rare anomaly. We describe here the report of 'twin' circumflex arteries with the anomalous circumflex coronary artery arising from the right coronary sinus and a circumflex artery from left coronary artery.Keywords: Coronary artery anomalies, twin circumflex arteries, right sinus of valsalva

Percutaneous intervention on anomalous circumflex coronary arteries — a single centre experience

Anomalies of the origin and course of the circumflex artery are amongst the most common seen at coronary angiography. There is limited information regarding patient and procedural characteristics, technical feasibility and outcomes associated with percutaneous intervention (PCI) to these vessels. The aim of this study is to examine our experience with PCI to anomalous circumflex vessels and compare this to some aspects of percutaneous intervention on non-anomalous circumflex vessels. Over a 41 month period, 20 PCI procedures on anomalous circumflex vessels were identified and 1550 PCI procedures on non-anomalous circumflex arteries. In 9 anomalous cases, the circumflex arose from the left coronary cusp, in 7 cases from the right coronary cusp, and in the remaining 4 cases from the proximal right coronary artery. There were no differences in demographics or pattern or severity of coronary disease between the 2 groups. A higher proportion of patients with anomalous vessels presented acutely. Screening times were longer in the anomalous group. All 20 procedures were associated with immediate procedural success. There was one peri-procedural myocardial infarction unrelated to anomalous circumflex intervention. After a median follow-up period of 7.3 months, the only major adverse cardiac event recorded in the anomalous group was an ischaemia-driven revascularisation to a non-target vessel branch. We describe techniques which can be used to improve support and facilitate successful PCI to anomalous circumflex vessels. PCI to anomalous circumflex vessels may be technically challenging, but is feasible and carries favourable short and long-term clinical outcomes. This single centre observational study demonstrates that percutaneous coronary intervention to anomalous circumflex coronary arteries although technically challenging can be performed with satisfactory procedural success rates and favourable short and longer-term clinical outcomes. It describes various techniques that can be employed to optimise successful intervention.

Letters to Editor Non-invasive detection of concomitant coronary artery anomaly and atherosclerotic coronary disease using transthoracic Doppler echocardiography

Letters to Editor Non-invasive detection of concomitant coronary artery anomaly and atherosclerotic coronary disease using transthoracic Doppler echocardiography

Left circumflex coronary artery originating from distal right coronary artery: An unusual case with 13-year follow-up

In this report we describe a patient with the left circumflex coronary artery originating from the distal right coronary artery as a terminal extension of it. Other origin anomalies related to the circumflex coronary artery may be associated with sudden death and adverse cardiac events, but no data are available about the clinical consequences of this rare anomaly. We also report the clinical follow-up of this patient over a 13-year period, with anomalous circumflex coronary artery arising as a terminal extension of the right coronary artery.

Anomalous Twin Circumflex Artery Identified By Invasive Coronary Angiography and Non-Invasive Multidetector CT Angiography in A 75 Year Old Caribbean Male

Coronary artery anomalies are clinically important as there have been reports of sudden death, fatal and non-fatal myocardial infarction associated with exercise in persons with certain types of unusual coronary anatomy. Anomalous origin of the circumflex artery is not an uncommon finding; however dual origin of the circumflex artery is a rare anomaly. An extensive search of literature indicates that there have been only two such prior reports, both with nondominant anomalous left circumflex arteries. We describe here the first report of 'twin' circumflex arteries with the anomalous dominant circumflex coronary artery arising from the right coronary trunk and a non-dominant circumflex artery from left coronary artery. This was diagnosed by conventional coronary angiography and then confirmed with 64-slice multidetector computed axial tomographic (MDCT) angiography. To the best of our knowledge, this is the first report of twin circumflex coronary artery clearly demonstrated by both invasive and non-invasive techniques. No such confirmation by MDCT angiography has previously been reported in literature.

Mouse Models of Congenital Heart Defects

One of the puzzling things about genetic disruptions in mice that affect heart development is the overwhelming number of conotruncal anomalies and the infrequency with which certain other malformations are reported. For instance, venous malformations such as total anomalous pulmonary venous return (TAPVR) or partial anomalous pulmonary venous return (PAPVR) have not been reported in a mutant mouse. Coronary artery anomalies are reported infrequently, and when they are reported it is because they are obvious. Conotruncal anomalies such as common trunk, double-outlet right ventricle, and pulmonary stenosis with overriding aorta are frequently associated with mouse germ line and conditional gene deletions. Although conotruncal malformations represent the highest percentage of congenital heart defects in the human population, TAPVR and PAPVR occur in a significant number of babies. Further, coronary anomalies affecting the stems of the coronary arteries frequently are seen in conotruncal malformations in babies but are rarely reported in mouse models. Article see p 314 Isolated TAPVR is a rare lesion and occurs in only about 1 in 17 000 live births.1 PAPVR is much more common and has been reported in 1 in 160 individuals (0.6%) and 0.4% to 0.7% of postmortem examinations, with 90% of these associated with an atrial septal defect.2 The majority of patients with TAPVR have symptoms of cyanosis and congestion within the first year of life. If left untreated, about 80% would die. In contrast, PAPVR frequently is not discovered until adulthood and is not usually a cause of death. Although the overall incidence of PAPVR in autopsy cases has been estimated to be 0.7%,3 the true prevalence may be higher,4 as several reports have appeared with PAPVR as an incidental finding that in most of these cases is asymptomatic. PAPVR can present as an isolated structural abnormality, but it …

Transesophageal Echocardiography of an Anomalous Circumflex Coronary Artery: Anatomy and Implications

Transesophageal Echocardiography of an Anomalous Circumflex Coronary Artery: Anatomy and Implications

Percutaneous coronary intervention with stent deployment in anomalously‐arising left circumflex coronary arteries

Anomalous origin of the left circumflex coronary artery from the right sinus of Valsalva or proximal right coronary artery is the most common congenital coronary anatomical abnormality. Whether such vessels are particularly predisposed to atherosclerotic disease in their proximal portion remains controversial. Successful balloon angioplasty has been described, but thus far only six isolated cases of stent deployment in anomalous circumflex vessels have been described in the interventional literature. Single-center retrospective case study of twenty-two patients with anomalous circumflex coronary arteries. Twelve patients (11/12 male, average age (63 +/- 3) years) underwent successful percutaneous coronary intervention (PCI) with stent deployment in the retroaortic segment of the anomalous vessel. Significant obstructive coronary disease in the retroaortic portion was found in the majority of anomalous left circumflex vessels (16/22, 73%), but did not predict the presence of significant two or three vessel coronary disease (P = 0.14, 0.63). PCI was successful in all cases attempted, with favourable short/medium-term event-free survival. The retroaortic portion of anomalously-arising circumflex coronary arteries is selectively predisposed to atherosclerotic disease. PCI to these vessels is feasible but may be technically challenging.

Images in Anesthesia: Transesophageal echocardiography images of anomalous circumflex coronary artery

Images in Anesthesia: Transesophageal echocardiography images of anomalous circumflex coronary artery

Compression of anomalous circumflex coronary artery by a prosthetic valve ring

Anomalous origin of the circumflex coronary artery from the right aortic sinus, with a retroaortic course, is usually without consequence. We report a patient who underwent aortic valve replacement for bicuspid aortic valve. The prosthesis sewing ring distorted the circumflex, producing myocardial infarcts and sudden death during exercise.

Diagnosis and Operation for Anomalous Circumflex Coronary Artery

Background. Origin of the left circumflex coronary artery from the right sinus of Valsalva is the most common anatomic variation of the coronary artery circulation. However, there are few reports about the operative approach to this anomalous vessel.Methods. Forty patients having this anomaly were identified from 10,216 adult cardiac catheterization procedures. Forty percent of the anomalous circumflex coronary arteries (ACCAs) had critical atherosclerotic lesions. Eighty cases needed bypass grafting.Results. For diagnosis of ACCA, the aortic root sign was positive in 94.9% of the diagnosed patients and the nonperfused myocardium sign was found in 92.5%. Eighty percent of ACCAs were larger than 2 mm in radiographic diameter before their passage into the atrioventricular groove. However, after emerging from the atrioventricular groove, 70% measured less than 1.5 mm. Consequently, a technique was developed to bypass the proximal ACCA and was used in 2 cases. Six other patients with more distal disease and larger vessels underwent conventional bypass grafting.Conclusions. The aortic root sign and nonperfused myocardium are useful in diagnosing ACCA. The ACCA is usually too small for use of the conventional graft technique. Therefore, a technique was developed to graft more proximally and was applied successfully in 2 cases.(Ann Thorac Surg 1997;63:377–81)