Anomalous Aortic Origin Of The Coronary Artery Research Articles

Lumped-parameter model as a non-invasive tool to assess coronary blood flow in AAOCA patients

Anomalous aortic origin of the coronary artery (AAOCA) is a rare disease associated with sudden cardiac death, usually related to physical effort in young people. Clinical routine tests fail to assess the ischemic risk, calling for novel diagnostic approaches. To this aim, some recent studies propose to assess the coronary blood flow (CBF) in AAOCA by computational simulations but they are limited by the use of data from literature retrieved from normal subjects. To overcome this limitation and obtain a reliable assessment of CBF, we developed a fully patient-specific lumped parameter model based on clinical imaging and in-vivo data retrieved during invasive coronary functional assessment of subjects with AAOCA. In such a way, we can estimate the CBF replicating the two hemodynamic conditions in-vivo analyzed. The model can mimic the effective coronary behavior with high accuracy and could be a valuable tool to quantify CBF in AAOCA. It represents the first step required to move toward a future clinical application with the aim of improving patient care. The study was registered at Clinicaltrial.gov with (ID: NCT05159791, date 2021-12-16).

Trends in surgical management of anomalous aortic origin of the coronary artery over 2 decades

ObjectiveTo evaluate outcomes of patients undergoing surgery for anomalous aortic origin of the coronary artery (AAOCA) at a tertiary care center and determine the influences of a coronary artery program on management strategies and outcomes. MethodsThis retrospective review of consecutive surgical patients who had isolated AAOCA at a tertiary care center between August 1, 1999, and October 31, 2022, compared patient characteristics, interventional timing, and surgical strategies before and after program inception in 2018. Comparisons between time periods and anatomical subgroups were performed using Fisher exact and Wilcoxon rank-sum tests. ResultsOf 149 surgical AAOCA patients, 102 (69%) had AAO of the right coronary artery. Compared with AAO of the left coronary artery (AAOLCA), AAO of the right coronary artery (AAORCA) was associated with greater athletic participation (intramural, varsity, and college-level) (74% vs 43%; P < .001) and preoperative functional imaging (72% vs 49%; P = .01), but were less likely to have ischemic changes on functional imaging (5% vs 23%; P = .03) or any postoperative complications (7% vs 19%; P = .04). Moderate or greater aortic insufficiency occurred postoperatively in 1 (1%) of AAORCA and 1 (3%) of AAOLCA patients. After the coronary artery program inception, there was an increase among patients with AAOCA undergoing preoperative computed tomography angiography (pre-2018: 39 out of 98 [40%] vs post-2018: 48 out of 51 [94%]; P < .001) and a decrease in isolated AAOCA unroofing procedures performed (30 [31%] vs 5 [10%]; P = .004). ConclusionsSurgical management of AAOCA evolved over time, and can be achieved with low instance of postoperative aortic insufficiency. Establishment of a coronary artery program has streamlined care.

Clinical analysis of sixty-nine children with anomalous aortic origin of the coronary artery

We aimed to analyse the clinical characteristics of children with different types of anomalous aortic origin of the coronary artery (AAOCA) at different ages, and to discuss the factors related to myocardial ischaemia. In this retrospective study, we included 69 children diagnosed with AAOCA using CT coronary angiography; we classified the participants based on the type of AAOCA, age, and high-risk anatomy. The clinical characteristics of the different AAOCA types and age groups were compared, and the correlation between manifestations and high-risk anatomy was analysed. Anomalous origin of the left coronary artery from the right coronary artery sinus, anomalous origin of the right coronary artery from the left coronary artery sinus, and a coronary artery origin without coronary sinuses was found in 10 (14.5%), 57 (82.6%), 2 (2.9%) patients, respectively. There were no significant differences in sex, clinical manifestations, percentage of positive myocardial injury markers, electrocardiogram, transthoracic echocardiography, or proportion of high-risk anatomy among the groups with different AAOCA types. According to age group, the proportion of asymptomatic infants and pre-schoolers was the highest (p < 0.001). Forty-three patients (62.3%) had high-risk anatomy and were more likely to present with severe symptoms and cardiac syncope (p < 0.05). Conclusion: There were no significant differences in the proportions of high-risk anatomy and clinical characteristics among children with different AAOCA types. We found a relation between the severity of AAOCA clinical symptoms and anatomical risk.What is Known:• Clinical symptoms in children with AAOCA are varied and the results of routine cardiological examinations lack specificity.• High-risk anatomical features, exercise, cardiac symptoms, and ALCA are risk factors for the occurrence of SCD in patients with AAOCA.What is New:• Compared the clinical characteristics of different types of AAOCA and ages.• Analysed the correlation between symptoms and high-risk anatomical features.

Accuracy of computed tomography angiography and structured reporting of high-risk morphology in anomalous aortic origin of coronary artery: comparison with surgery.

Morphological features including interarterial course, intramural course, high ostial location and slit-like ostium are presumed risk factors for sudden cardiac death in children with anomalous aortic origin of the coronary artery (AAOCA). To facilitate clinical risk stratification, the diagnostic accuracy of CT angiography for individual risk factors in the setting of AAOCA must be established. We assessed diagnostic accuracy of standardized CT angiography interpretation for morphological characteristics that might determine risk in children with AAOCA by comparing them to surgical findings. We created a standardized protocol for CT angiography of AAOCA and retrospectively evaluated diagnostic performance in 25 consecutive surgical patients. Relevant morphological variables in AAOCA were assessed by three independent blinded readers, with surgery as the reference standard. We used Cohen kappa coefficients and accuracies to assess agreement between readers and surgical findings, and we calculated intraclass correlation coefficients to compare length of the intramural course. CT angiography correctly identified AAOCA in all patients. For the three readers, accuracies for detecting ostial stenosis were 84%, 94% and 96%; for high ostial origin, accuracies were 76%, 78% 82%; for intramurality using the peri-coronary fat sign, accuracies were 98%, 96% and 92%; and for intramurality using oval shape of coronary artery, accuracies were 98%, 94% and 92%. The intraclass correlation coefficients (ICCs) for predicting intramural length among the three readers were 0.67, 0.75 and 0.81 using peri-coronary fat, and 0.69, 0.50 and 0.81 using oval shape, respectively. CT angiography reliably identified AAOCA in all children and detected the presence of intramurality with high accuracy.

A case of anomalous aortic origin of coronary artery associated with a coved‐type electrocardiogram

Brugada syndrome (BrS) is a sudden cardiac death syndrome characterized by a coved-type electrocardiogram (ECG). Different disorders, such as ischemia, can emulate a Brugada-pattern ECG (Brugada phenocopy). We report herein, the first case of surgical epicardial electrophysiological mapping in a successfully resuscitated patient with an anomalous aortic origin of the coronary artery (AAOCA) associated with a coved-type ECG. It was debatable whether the coved-type ECG and the abnormal arrhythmogenic substrate in the epicardial right ventricular outflow tract were derived from BrS or from repetitive ischemia due to AAOCA; however, the epicardial electrophysiological mapping helped in deciding the treatment strategy.

Anomalous aortic origin of a coronary arteries: Is unroofing procedure always appropriate?

Anomalous aortic origin of the coronary artery (AAOCA) with an inter-arterial ± intra-mural course is rare anomaly that carries a high risk of ischemic event and even sudden death. Unroofing of the intramural course had been adopted as the gold standard. However, there are anatomical forms where the alternative techniques require to be considered. We reviewed our cohort between 1999 and 2019. Results 38 patients underwent surgical intervention. Median age was (Mean age was 42.5 ± 2.7 years). Twenty-eight patients (74%) had right AAOCA, 9(23%) left AAOCA and one (3.2%) both left and right AAOCA. One patient had a left coronary artery coursing within the subpulmonary muscular conus. Twenty-nine (76%) patients presented with symptoms, 9 (23%) patients were asymptomatic. Symptoms were chest pain for 24 patients, syncope or lipothymia for 15 patients, 1 patient had a cardiac arrest during exercise and for 8 patients the diagnosis was made by chance. Four patients had a positive ischemic test. Repair techniques included 27 unroofing procedures (67.7%) with an associated translocation of the pulmonary artery for 10 patients in early experience. For 6 patients the unroofing was not-feasible because of the absence of an intra-mural distinct segment or was intraoperatively judged not inappropriate and had a reimplantation of the AAOCA. Four patients had a coronary artery bypass grafting (CABG) and three translocation of the pulmonary artery. There was no early or late mortality. CT scan was performed in 31 cases showed a patent coronary artery ostium. Seventeen patients underwent postoperative ischemia testing with no evidence of ischemia. Surgical correction in AAOCA is mandatory for symptomatic and asymptomatic patients with evidence of myocardial ischemia under stress or restricted coronary artery segment. Surgical unroofing remains the gold standard but is not appropriated in all particular forms: alternative techniques should be considered in the latter. Surgical results are promising.

Sudden Cardiac Death in a Young Athlete: Imaging Techniques to Evaluate the Etiology

Anomalous aortic origin of the coronary artery (AAOCA) is a rare form of congenital heart disease with varying implications. Although most of the population with this congenital heart defect may remain completely asymptomatic and never know of the diagnosis, the risk of sudden cardiac death among healthy children and young adults during or just after exercise is significant. A previously healthy adolescent female athlete presented to the pediatric intensive care unit after sudden cardiac arrest on the playing field. She was successfully resuscitated, and further imaging revealed a diagnosis of AAOCA. She underwent surgical correction of her defect without complications. AAOCA remains a rare and often undetected form of congenital heart disease based on the asymptomatic nature of the defect; however, with proper imaging, a diagnosis can be made to allow for correct treatment.

Anomalous Aortic Origin of Left Coronary Artery Leading to Sudden Cardiac Arrest in Young Female

Purpose: Anomalous aortic origin of the coronary artery(AAOCA) is an uncommon but important cause of sudden death in young patients. Vigilance is required to ascertain a prompt diagnosis and expedite surgery to prevent further fatal events. We describe a case of a 20-year-old female with an anomalous left coronary artery origin, whose presentation and path to diagnosis and treatment is outlined. Methodology: A 20-year-old female presented following an out of hospital cardiac arrest (OOHCA) whilst swimming. ROSC was achieved with CPR and a single defibrillation from an AED. History revealed recurrent episodes of chest pain and syncope over the previous 4 years. She was evaluated 3 years prior by cardiology; TTE and EST were found to be normal and EP review for prolonged QTS stratified her as a low risk for QTS. She was discharged without further investigations. CTCA performed following her arrest revealed an anomalous left coronary artery, arising from the right coronary sinus, passing intramurally between the aortic root and pulmonary trunk. This was repaired by deroofing the left coronary artery across the top of the left and right commisure of the aortic valve. The aortic valve commisure was re-suspended with good outcome. Results: Surgery provided excellent immediate results and exercise stress test at 6 months was negative. She remained asymptomatic with no further episodes of chest pain or syncope and is now able to perform strenuous activities. Conclusion: The AAOCA arising from the opposite sinus of Valsalva is a rare but an important condition which targets children and adolescents with an often fatal outcome if diagnosis is missed.

Anomalous Aortic Origin of a Coronary Artery Is Not Always a Surgical Disease.

Anomalous aortic origin of the coronary artery (AAOCA) from the opposite sinus of Valsalva with an interarterial course has become a high-profile lesion as a result of its association with sudden cardiac death in otherwise young and healthy individuals. Despite our incomplete knowledge of its pathophysiology and natural history, surgical intervention is often recommended. Evidence now shows AAOCA to be relatively common, with lower than previously suspected rates of sudden cardiac death. Analysis of this information reveals that AAOCA is not always a surgical disease. Future multi-institutional studies will continue to define those subgroups best served by observation or surgery.

Anomalous Aortic Origin of a Coronary Artery: Surgical Repair With Anatomic- and Function-Based Follow-Up

Anomalous aortic origin of the coronary artery (AAOCA) with an interarterial (IAC) course is an uncommon congenital anomaly. Surgical indications and repair techniques have evolved. We have managed 259 adult patients with AAOCA over 40 years. Our management strategy includes anatomic- and function-based surveillance to select surgical candidates. We reviewed our surgical cohort and analyzed anatomic and functional outcomes. We queried our heart center databases to obtain the names of all patients with AAOCA managed at our institution between 1974 and 2014. We performed a retrospective chart review. Two hundred fifty-nine patients were managed for AAOCA. Sixty-one underwent surgical intervention. Twenty-six with associated coronary atherosclerosis were excluded. Thirty-one who underwent surgical repair were analyzed. Mean age was 42.5 ± 2.7 years. Twenty-four patients (77.4%) had right AAOCA. Six (19.4%) had left AAOCA. One (3.2%) had bilateral coronary anomalies. Repair techniques included 21 unroofing procedures (67.7%), 6 translocations (19.4%), and 4 coronary artery bypass grafting (CABG) procedures (12.9%). Mean follow-up was 3.8 ± 0.8 years. Thirteen patients underwent follow-up anatomic testing with computed tomography. Twelve of these patients had widely patent coronary arteries, and 1 patient had mild coronary artery stenosis. Seventeen patients underwent functional testing. Fifteen of these patients had no evidence of ischemia. One patient had reversible ischemia after CABG, and 1 had subclinical ischemia after unroofing. There was 1 late mortality from endocarditis. Our multidisciplinary program uses a treatment algorithm to select patients with AAOCA for surgical intervention. Only a small subset requires an operation, and we favor unroofing and translocation techniques. With this paradigm, outcomes are excellent, as validated with anatomic- and function-based testing.

Direct Reimplantation as an Alternative Approach for Treatment of Anomalous Aortic Origin of the Right Coronary Artery

Anomalous aortic origin of the coronary artery (AAOCA) is a rare cardiac anomaly associated with myocardial ischemia, infarction, and even sudden death. We report 2 patients presenting with AAOCA of the right coronary artery originating from the left coronary sinus with an initial intramural course. In both patients, we performed uncomplicated direct reimplantation of the right coronary artery into the right coronary sinus. For this purpose, the right coronary artery was dissected from the transmural emersion point out of the aortic wall, subsequently as proximal as possible transected and directly reimplanted into the right coronary sinus. In both cases, the postoperative course was uneventful, and cardiac computed tomography confirmed excellent postoperative results after 1 year of follow-up. This technique is a convenient and easily performable approach, characterized by short cross-clamp times and avoidance of aortotomy, and thus is an excellent alternative strategy.

Direct reimplantation as an alternative approach for treatment of anomalous aortic origin of the right coronary artery - A case series

Objectives: Anomalous aortic origin of the coronary artery (AAOCA) is a rare cardiac anomaly, associated with myocardial ischemia, infarction and even sudden death.

Surgery for Anomalous Aortic Origin of the Coronary Artery

Anomalous aortic origin of the coronary artery (AAOCA) has been associated with coronary ischemia, myocardial infarction, and sudden death. Advances in echocardiography and computed tomography have identified at-risk patients. Treatment options include unroofing strategies in symptomatic and asymptomatic patients. We review our experience for efficacy and safety. Between 1998 and 2008, we performed coronary unroofing in 22 patients with AAOCA without aortic commissural detachment. Of 7 patients with "left from right" AAOCA, 4 had chest pain only, 1 had syncope, 1 had myocardial infarction, and 1 was asymptomatic. Of 15 patients with "right from left" AAOCA, 11 had chest pain only, 4 had syncope, and none were without symptoms. Median age was 15 years (range, 5 to 54). Eight patients had concomitant procedures, most commonly patent foramen ovale closure. There were no deaths or complications. Mean cross-clamp time was 53 minutes. Mean length of stay was 4 days. Postoperative evaluation included echocardiography, computed tomography angiogram, stress thallium, stress echocardiography, and exercise stress test. In all patients, the repaired coronary was patent, with demonstrated flow. Mean follow-up was 17 months (range, 1 to 63). Anomalous aortic origin of the coronary artery is emerging as an identifiable disease entity associated with symptoms or sudden death. We conclude that coronary unroofing is a safe and effective therapy for symptomatic patients. Surgery for asymptomatic patients has been more controversial, with growing advocacy for patients with "left from right" as opposed to "right from left" origins. Prospective studies will be required to answer these questions.