Double Negative Is Positive: Anomalous Aortic Origin of the Left Coronary Artery Saved by a Bicuspid Aortic Valve.

Atypical aortic origin of coronary artery (AAOCA) is a rare heart condition that has been identified in only a few autopsy cases and in some patients who have undergone angiographic evaluation. AAOCA is known to be closely linked with aortic valve malformations, with the most common type being the bicuspid aortic valve (BAV). A 77-year-old male with a medical history of hypertension and diabetes presented with dyspnea and orthopnea for three days. During the eventual cardiac catheterization, it was noted that the left coronary artery had an anomalous origin from the right side, coursing between the aorta and pulmonary artery. Contrast-enhanced computerized tomography (CECT) also showed a type 2 BAV and a left main coronary artery arising lower at the level of the pulmonary trunk. The dyspnea in this patient was attributed to diastolic dysfunction, and surgical correction of the coronaries was not done. The patient was managed on an outpatient basis for heart failure. In this case, the patient had an anomalous origin of the left coronary artery and type 2 BAV, which posed significant cardiovascular complications. It is unclear if the presence of the concomitant type 2 BAV led to the origin of the anomalous left coronary artery being at a lower level through its effect on the developmental mechanics. This lower origin may have resulted in lower compressive forces on the coronary artery as the inter-arterial pressures would be lower closer to the heart and farther from the lungs. Our case report aims to highlight this complex presentation where the BAV likely provides a benefit in AAOCA cases.

First report from the European registry for anomalous aortic origin of coronary artery (EURO-AAOCA).

OBJECTIVES: Anomalous aortic origin of a coronary artery (AAOCA) is a group of rare congenital heart defects with various clinical presentations. The lifetime-risk of an individual living with AAOCA is unknown, and data from multicentre registries are urgently needed to adapt current recommendations and guide optimal patient management. The European AAOCA Registry (EURO-AAOCA) aims to assess differences with regard to AAOCA management between centres. METHODS: EURO-AAOCA is a prospective, multicentre registry including 13 European centres. Herein, we evaluated differences in clinical presentations and management, treatment decisions and surgical outcomes across centres from January 2019 to June 2023. RESULTS: A total of 262 AAOCA patients were included, with a median age of 33 years (12-53) with a bimodal distribution. One hundred thirty-nine (53.1%) were symptomatic, whereas chest pain (n = 74, 53.2%) was the most common complaint, followed by syncope (n = 21, 15.1%). Seven (5%) patients presented with a myocardial infarction, 2 (1.4%) with aborted sudden cardiac death. Right-AAOCA was most frequent (150, 57.5%), followed by left-AAOCA in 51 (19.5%), and circumflex AAOCA in 20 (7.7%). There were significant differences regarding diagnostics between age groups and across centres. Seventy-four (28.2%) patients underwent surgery with no operative deaths; minor postoperative complications occurred in 10 (3.8%) cases. CONCLUSIONS: Currently, no uniform agreement exists among European centres with regard to diagnostic protocols and clinical management for AAOCA variants. Although surgery is a safe procedure in AAOCA, future longitudinal outcome data will hopefully shed light on how to best decide towards optimal selection of patients undergoing revascularization versus conservative treatment.

Automatic 3D Segmentation and Identification of Anomalous Aortic Origin of the Coronary Arteries Combining Multi-view 2D Convolutional Neural Networks.

This work aimed to automatically segment and classify the coronary arteries with either normal or anomalous origin from the aorta (AAOCA) using convolutional neural networks (CNNs), seeking to enhance and fasten clinician diagnosis. We implemented three single-view 2D Attention U-Nets with 3D view integration and trained them to automatically segment the aortic root and coronary arteries of 124 computed tomography angiographies (CTAs), with normal coronaries or AAOCA. Furthermore, we automatically classified the segmented geometries as normal or AAOCA using a decision tree model. For CTAs in the test set (n = 13), we obtained median Dice score coefficients of 0.95 and 0.84 for the aortic root and the coronary arteries, respectively. Moreover, the classification between normal and AAOCA showed excellent performance with accuracy, precision, and recall all equal to 1 in the test set. We developed a deep learning-based method to automatically segment and classify normal coronary and AAOCA. Our results represent a step towards an automatic screening and risk profiling of patients with AAOCA, based on CTA.

Noninvasive Multimodality Imaging for the Assessment of Anomalous Coronary Artery.

PURPOSE OF REVIEW: Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital coronary anomaly with the potential to cause myocardial ischemia and adverse cardiac events. The presence of AAOCA anatomy itself does not necessarily implicate a need for revascularization. Therefore, the purpose of this review is to assess how noninvasive comprehensive anatomic- and physiologic evaluation may guide patient management. RECENT FINDINGS: The assessment of AAOCA includes an accurate description of the anomalous origin/vessel course including anatomical high-risk features such as a slit-like ostium, proximal narrowing, elliptic vessel shape, acute take-off angle, intramural course, and possible concomitant coronary atherosclerosis and hemodynamics. Various cardiac imaging modalities offer unique advantages and capabilities in visualizing these anatomical and functional aspects of AAOCA. This review explored the role of noninvasive multimodality imaging in the characterization of AAOCA by highlighting the strengths, limitations, and potential applications of the current different cardiac imaging methods, with a focus on the pathophysiology of myocardial ischemia and stress testing protocols.

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.

As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used. In this paper, a multidisciplinary group of clinical and interventional cardiologists and cardiac surgeons performed a systematic review and critical evaluation of the available evidence on the interventional treatment of AAOCA in adult patients. Using a structured Delphi process, the group agreed on expert recommendations that are intended to complement existing clinical practice guidelines.

Application of Patient-Specific Computational Fluid Dynamics in Anomalous Aortic Origin of Coronary Artery: A Systematic Review.

Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart condition with fixed and dynamic stenotic elements, potentially causing ischemia. Invasive coronary angiography under stress is the established method for assessing hemodynamics in AAOCA, yet it is costly, technically intricate, and uncomfortable. Computational fluid dynamics (CFD) simulations offer a noninvasive alternative for patient-specific hemodynamic analysis in AAOCA. This systematic review examines the role of CFD simulations in AAOCA, encompassing patient-specific modeling, noninvasive imaging-based boundary conditions, and flow characteristics. Screening articles using AAOCA and CFD-related terms prior to February 2023 yielded 19 publications, covering 370 patients. Over the past four years, 12 (63%) publications (259 patients) employed dedicated CFD models, whereas 7 (37%) publications (111 patients) used general-purpose CFD models. Dedicated CFD models were validated for fixed stenosis but lacked dynamic component representation. General-purpose CFD models exhibited variability and limitations, with fluid-solid interaction models showing promise. Interest in CFD modeling of AAOCA has surged recently, mainly utilizing dedicated models. However, these models inadequately replicate hemodynamics, necessitating novel CFD approaches to accurately simulate pathophysiological changes in AAOCA under stress conditions.

Treatment of Anomalous Coronary Arteries-Surgical Revascularisation Using the Pure Internal Thoracic Artery Technique.

OBJECTIVES: To evaluate the use of CABG utilising an isolated pedicled Right Internal Thoracic Artery (RITA) or Left Internal Thoracic Artery (LITA) or the Pure Internal Thoracic Artery (PITA) technique to treat anomalous aortic origin of coronary artery (AAOCA). METHODS: A retrospective review of all patients at our institution over an 8-year period (2013-2021) who underwent surgery for AAOCA was performed. Data assessed included patient demographics, initial presentation, morphology of coronary anomaly, surgical procedure, cross-clamp time, cardiopulmonary bypass time, and long-term outcome. RESULTS: A total of 14 patients underwent surgery, including 11 males (78.5%) with a median logistic EuroSCORE of 1.605 (IQR 1.34). The median age was 62.5 years (IQR 48.75). Presentation was angina (7 patients), acute coronary syndrome (5 patients), incidental findings in aortic valve pathology (2 patients). AAOCA morphology varied: RCA from left coronary sinus (6), RCA from left main stem (3), left coronary artery from the right coronary sinus (1), left main stem arising from right coronary sinus (2) and circumflex artery arising from the right coronary sinus (2). Overall, 7 patients had co-existing flow-limiting coronary artery disease. CABG was performed using either a pedicled skeletonized RITA, LITA or PITA technique. There was no perioperative mortality. Overall median follow-up time was 43 months. One patient presented with recurrent angina secondary to graft failure at 2 years and there were two non-cardiac-related deaths at 4 and 35 months. CONCLUSION: The use of internal thoracic artery grafts can provide a durable treatment option in patients with anomalous coronary arteries. The potential risk of graft failure in patients with no flow-limiting disease should be very carefully considered. However, a proposed benefit of this technique is the use of a pedicle flow to increase the long-term patency. More consistent results are obtained when ischaemia can be demonstrated preoperatively.

Decision making in anomalous aortic origin of a coronary artery.

INTRODUCTION: There are many uncertainties surrounding anomalous aortic origin of a coronary artery (AAOCA) including the pathophysiology of sudden cardiac death, how to best risk stratify patients, how to best evaluate patients, who would benefit from exercise restriction, who should undergo surgical intervention, and which operation to perform. AREAS COVERED: The goal of this review is to provide a comprehensive but succinct overview of AAOCA to help clinicians with the difficult task of navigating optimal evaluation and treatment of an individual patient with AAOCA. EXPERT OPINION: Beginning in year 2012, some of our authors proposed an integrated, multi-disciplinary working group which has become the standard management strategy for patients diagnosed with AAOCA. A multi-disciplinary team with a focus on shared decision-making with the patients/families is likely necessary to optimize outcomes. Long-term follow-up and research are needed to improve our understanding of AAOCA.

Detection of anomalous aortic origin of a coronary artery (AAOCA) by echocardiogram: When does computed tomographic angiography add value?

BACKGROUND AND OBJECTIVE: There is limited literature comparing TTE and CCTA in children with suspected AAOCA. To determine the distribution of various coronary anomalies comparing TTE and CCTA data, and define the added value advanced imaging brings in clinical decision-making. MATERIALS AND METHODS: Retrospective review of data was obtained in patients aged 0-18 years who underwent TTE and CCTA for suspected AAOCA. Patient demographics, CCTA and TTE findings, and interventions performed were recorded. RESULTS: 100 consecutive patients were included (60% male), mean age 11 years (7 days-18 years old). In 93 patients, CCTA detected 94 anomalous coronaries. Definitive coronary abnormality was reported on TTE in 77 patients; 76 of which were confirmed by CCTA, 1 patient was found to have a normal variant. Suspected anomalous origin was reported in 16 patients on TTE, 13 of which were abnormal on CCTA. The coronary origin was not seen on TTE in 6 patients; of these, 3 had AAOCA on CCTA and 3 had hypoplastic RCA with left dominant system. Only 1 patient who had a normal TTE was found to have AAOCA on CCTA. CCTA was better than TTE in defining ostial characteristics and the course of the anomalous coronary artery, and detecting myocardial bridge. CONCLUSIONS: CCTA adds value in diagnosing AAOCA when the coronary origins are not well assessed or suspected anomalous origin is suggested on TTE. In addition, when a confident definitive diagnosis of AAOCA is reported on TTE, CCTA demonstrates better performance in determining additional features of AAOCA.

Symptomatic Interarterial Course Of Coronary Arteries In Middle Aged Patients.

Anomalous aortic origin of a coronary artery (AAOCA) is the most common congenital abnormality and is sometimes associated with various life-threatening conditions. We present the cases of a 35-year-old male and a 50-year-old female with complaints of chest pain. Patients had anomalous aortic origin of coronary arteries with the interarterial course and were treated surgically. By literature review, we came to know that the approach to treat patients with anomalous aortic origin of coronary arteries should be largely individualized and there is no ample scientific data to support any specific diagnostic modality and treatment option.

Coronary artery disease in adults with anomalous aortic origin of a coronary artery.

Objectives: This study sought to characterize coronary artery disease (CAD) among adults diagnosed with an anomalous aortic origin of a coronary artery (AAOCA). We hypothesized that coronaries with anomalous origins have more severe CAD stenosis than coronaries with normal origins. Methods: This single-center study of 763 adults with AAOCA consisted of 620 patients from our cardiac catheterization database (1958-2009) and 273 patients from electronic medical records query (2010-2021). Within left main, anterior descending, circumflex, and right coronary arteries, the CAD stenosis severity, assessed by invasive or computer tomography angiography, was modeled with coronary-level variables (presence of an anomalous origin) and patient-level variables (age, sex, comorbidities, and which of the four coronaries was anomalous). Results: Of the 763 patients, 472 (60%) had obstructive CAD, of whom, 142/472 (30%) had obstructive CAD only in the anomalous coronary. Multivariable modeling showed similar CAD stenosis severity between coronaries with anomalous versus normal origins (P = .8). Compared with AAOCA of other coronaries, the anomalous circumflex was diagnosed at older ages (59.7 ± 11.1 vs 54.3 ± 15.8 years, P < .0001) and was associated with increased stenosis in all coronaries (odds ratio, 2.7; 95% confidence interval, 2.2-3.4, P < .0001). Conclusions: Among adults diagnosed with AAOCA, the anomalous origin did not appear to increase the severity of CAD within the anomalous coronary. In contrast to the circumflex, AAOCA of the other vessels may contribute a greater ischemic burden when they present symptomatically at younger ages with less CAD. Future research should investigate the interaction between AAOCA, CAD, and ischemic risk to guide interventions.

Patient-specific fluid-structure simulations of anomalous aortic origin of right coronary arteries.

Objectives: Anomalous aortic origin of the right coronary artery (AAORCA) may cause ischemia and sudden death. However, the specific anatomic indications for surgery are unclear, so dobutamine-stress instantaneous wave-free ratio (iFR) is increasingly used. Meanwhile, advances in fluid-structure interaction (FSI) modeling can simulate the pulsatile hemodynamics and tissue deformation. We sought to evaluate the feasibility of simulating the resting and dobutamine-stress iFR in AAORCA using patient-specific FSI models and to visualize the mechanism of ischemia within the intramural geometry and associated lumen narrowing. Methods: We developed 6 patient-specific FSI models of AAORCA using SimVascular software. Three-dimensional geometries were segmented from coronary computed tomography angiography. Vascular outlets were coupled to lumped-parameter networks that included dynamic compression of the coronary microvasculature and were tuned to each patient's vitals and cardiac output. Results: All cases were interarterial, and 5 of 6 had an intramural course. Measured iFRs ranged from 0.95 to 0.98 at rest and 0.80 to 0.95 under dobutamine stress. After we tuned the distal coronary resistances to achieve a stress flow rate triple that at rest, the simulations adequately matched the measured iFRs (r = 0.85, root-mean-square error = 0.04). The intramural lumen remained narrowed with simulated stress and resulted in lower iFRs without needing external compression from the pulmonary root. Conclusions: Patient-specific FSI modeling of AAORCA is a promising, noninvasive method to assess the iFR reduction caused by intramural geometries and inform surgical intervention. However, the models' sensitivity to distal coronary resistance suggests that quantitative stress-perfusion imaging may augment virtual and invasive iFR studies.

Anomalous aortic origin of the left coronary artery: A rare case of an origin from the noncoronary sinus.

BACKGROUND: Anomalous aortic origin of the coronary artery (AAOCA) refers to a congenital abnormality of the origin and/or course of a coronary artery that arises from the aorta. It can be classified according to the sinus from which the coronary artery arises. The most common type is an anomalous origin of the right coronary artery. CASE PRESENTATION: Herein, we report a 7-year-old male that was diagnosed with AAOCA. Although the patient had not complained of obvious symptoms before the onset, echocardiography showed that the left coronary artery originated from the noncoronary sinus at an anatomical location. CONCLUSION: AAOCA is a rare congenital disease that often causes a sudden death in young athletes. Originating from the noncoronary sinus at the level of the sinotubular junction in the anomalous origin of the left coronary artery is even more rare which is presented in this case. Surgical intervention should be actively performed if the patient has symptoms or if the anatomy is complex.

Pattern, behavior, and clinical implications of electrocardiographic changes in patients undergoing repair of anomalous aortic origin of coronary arteries.

OBJECTIVES: Surgical repair in anomalous aortic origin of a coronary artery aims at mitigating the risk of sudden cardiac death in a subset of patients. The pattern and behavior of electrocardiogram changes in a large cohort of these patients are lacking. We aim to describe postoperative electrocardiogram changes in this population and its clinical implications on follow-up. METHODS: All patients aged less than 21 years who underwent surgical repair for anomalous aortic origin of a coronary artery between December 2012 and June 2020 at our institution were considered for inclusion. Electrocardiograms were reviewed at 5 defined time intervals, from preoperative to 90-day follow-up, with attention to significant findings of ST-segment changes, abnormal T waves, and pathologic Q waves. The electrocardiogram changes were analyzed for correlation with surgical reintervention and medium-term outcomes. RESULTS: Sixty-two patients met inclusion criteria (median age 13.7 years, 61% male). ST-segment changes in the initial postoperative period were seen in 52 patients (84%), all resolving over time. Abnormal T waves were seen in 19 patients (31%), occurred commonly at the predischarge period, and mostly resolved over time. Pathologic Q waves were observed in only 1 patient and associated with reintervention due to coronary artery stenosis. There was no association between postoperative electrocardiogram changes and inducible myocardial ischemia, ventricular dysfunction, or restriction from exercise at follow-up. CONCLUSIONS: ST-segment changes and T-wave abnormalities are commonly seen in the postoperative period after anomalous aortic origin of a coronary artery repair, tend to resolve over time, and are not associated with adverse medium-term outcomes. Pathologic Q waves were associated with the need for early coronary reintervention.

Sudden death of a post-partum female with anomalous origin of the coronary artery with bicuspid aortic valve.

Modern techniques have enabled routine diagnosis of congenital cardiac defects, notwithstanding, there will be some that remain undiagnosed and asymptomatic until adulthood. But this is rarely the case with the patent foramen ovale, with the anomalous aortic origin of the left main coronary artery and bicuspid aortic valve. This case describes the sudden death of a female in her post-partum period due to cardiac tamponade following a ruptured aneurysm of the coronary artery at its origin at the sinus of Valsalva. Autopsy shows patent foramen ovale findings and anomalous aortic origin of coronary artery (AAOCA) and bicuspid aortic valve. The association of AAOCA with bicuspid aortic valve and patent foramen ovale is rare.

Characterization of hemodynamics in anomalous aortic origin of coronary arteries using patient-specific modeling.

The anomalous aortic origin of coronary arteries (AAOCA) is a congenital disease that can lead to sudden cardiac death (SCD) during strenuous physical activity. Despite AAOCA being the second leading cause of SCD among young athletes, the mechanism behind sudden cardiac death remains mostly unknown. Computational fluid dynamics provides a powerful tool for studying how pathologic anatomy can affect different hemodynamic states. The present study investigates the effect of AAOCA on patient hemodynamics. We performed patient-specific hemodynamic simulations of interarterial AAOCA at baseline and in the exercise state using our massively parallel flow solver. Additionally, we investigate how surgical correction via coronary unroofing impacts patient blood flow. Results show that patient-specific AAOCA models exhibited higher interarterial time-averaged wall shear stress (TAWSS) values compared to the control patients. The oscillatory shear index had no impact on AAOCA. Finally, the coronary unroofing procedure normalized the elevated TAWSS by decreasing TAWSS in the postoperative patient. The present study provides a proof of concept for the potential hemodynamic factors underlying coronary ischemia in AAOCA during exercise state.

Flow Dynamics in Anomalous Aortic Origin of a Coronary Artery in Children: Importance of the Intramural Segment.

This study aims to assess the differences in pressure, fractional flow reserve (FFR) and coronary flow (with increasing pressure) of the proximal coronary artery in patients with anomalous aortic origin of a coronary artery with a confirmed ischemic event, without ischemic events, and before and after unroofing surgery, and compare to a patient with normal coronary arteries. Patient-specific flow models were 3D printed for 3 subjects with anomalous right coronary arteries with intramural course, 2 of them had documented ischemia, and compared with a patient with normal coronaries. The models were placed in the aortic position of a pulse duplicator and precise measurements to quantify FFR and coronary flow rate were performed from the aortic to the mediastinal segment of the anomalous right coronary artery. In an ischemic model, a gradual FFR drop (emulating that of pressure) was shown from the ostium location (∼1.0) to the distal intramural course (0.48). In nonischemic and normal patient models, FFR for all locations did not drop below 0.9. In a second ischemic model prior to repair, a drop to 0.44 was encountered at the intramural and mediastinal intersection, improving to 0.86 postrepair. There is a difference in instantaneous coronary flow rate with increasing aortic pressure in the ischemic models (slope 0.2846), compared to the postrepair and normal models (slope >0.53). These observations on patient models support a biomechanical basis for ischemia and potentially sudden cardiac death in aortic origin of a coronary artery, with a drop in pressure and FFR in the intramural segment, and a decrease in coronary flow rate with increasing aortic pressure, with both improving after corrective surgery.

Déjà Vu: Recurrent Sudden Cardiac Arrests in a Child With an Anomalous Left Coronary Artery.

Sudden cardiac arrest from anomalous coronary artery from the opposite sinus of Valsalva is rarely observed in children under 10 years of age. We report a 7-week-old infant with a brief resolved unexplained event from left anomalous aortic origin of a coronary artery who underwent unroofing and again developed syncope at 8 years of age. Ischemia was detected by stress echocardiography both times. (Level of Difficulty: Advanced.).