Anomalous Aortic Origin Of A Coronary Artery Research Articles

Prevalence and location of coronary artery disease in anomalous aortic origin of coronary arteries.

The prevalence and location of coronary artery disease (CAD) in anomalous aortic origin of a coronary artery (AAOCA) remain poorly documented in adults. We sought to assess the presence of CAD in proximal (or ectopic) and distal (or nonectopic) segments of AAOCA. We hypothesized that the representation of CAD may differ among the different courses of AAOCA. The presence of CAD was analyzed on coronary angiography and/or coronary computed tomography angiography in 390 patients (median age 64 years; 73% male) with AAOCA included in the anomalous coronary arteries multicentric registry. AAOCA mainly involved circumflex artery (54.4%) and right coronary artery (RCA) (31.3%). All circumflex arteries had a retroaortic course; RCA mostly an interarterial course (98.4%). No CAD was found in the proximal segment of interarterial AAOCA, whereas 43.8% of retroaortic AAOCA, 28% of prepulmonic AAOCA and 20.8% subpulmonic AAOCA had CAD in their proximal segments (P < 0.001). CAD was more prevalent in proximal than in distal segments of retroaortic AAOCA (OR: 3.1, 95% CI: 1.8-5.4, P < 0.001). On multivariate analysis, a retroaortic course was associated with an increased prevalence of CAD in the proximal segment (adjusted OR 3.4, 95% CI: 1.3-10.7, P = 0.022). Increased prevalence of CAD was found in the proximal segment of retroaortic AAOCA compared to the proximal segments of other AAOCA, whereas no CAD was observed in the proximal segment of interarterial AAOCA. The mechanisms underlying these differences are not yet clearly identified.

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

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. 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. 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. 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.

Fatal Coronary Artery Anomaly Concealed in Young Athletes with Exertional Syncope

Background. Syncope is a common symptom in children, many of which are benign and do not require treatment. Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital malformation but can be a risk for serious cardiovascular events, including sudden death as well as cardiogenic syncope. Case Report. We describe the case of a 14-year-old boy who suffered an initial syncope and afebrile seizure during a soccer game. A detailed medical history and imaging studies led to the diagnosis of the anomalous aortic origin of the left main coronary artery with an intramural course (AAOLCA-IM). Conclusion. Symptomatic AAOLCA-IM has the highest risk of sudden death among AAOCA, and surgical repair may be performed. Onset during exercise or preceding chest symptoms are suspicious signs of cardiogenic syncope and should be considered for cardiovascular imaging evaluation.

Anomalous Origin of Left Circumflex Artery from the Right Sinus of Valsalva in Cardiac Computed Tomography in a Group of 16,680 Patients—Radiologic and Clinical Characteristics

Background: Anomalous aortic origin of a coronary artery (AAOCA) is the most prevalent form of coronary anomaly. One variant of AAOCA is the anomalous origin of the left circumflex artery from the right sinus of Valsalva, which can be detected using cardiac computed tomography (CT). However, limited data are available regarding the natural history of this anomaly, its impact on myocardial function, and associated symptoms. Methods: We conducted a retrospective analysis of 16,680 CT exams (cardiac and chest) performed between 2015 and 2022 at our Heart Imaging Department, utilising a dual-source 128-slice CT scanner (SOMATOM Definition Flash, Siemens Healthineers, Forchheim, Germany). A registry of patients with anomalous origin of the circumflex artery from the right sinus of Valsalva (RCx) was established. The study included 56 cases of RCx (0.33%). Clinical information was obtained from medical records. RCx was defined as a circumflex artery originating from the right sinus of Valsalva (type I or II) or the right coronary artery (type III). Two researchers independently reevaluated each CT exam in our study group to ensure accurate radiologic descriptions and provide additional precise radiologic information regarding the anomaly, including high-risk features. Results: Our study comprised 56 patients, with approximately equal distribution between males (n = 30, 54%) and females (n = 26, 46%), and with a median age of 59 years. Coronary heart disease (CAD) was known in 23% of patients (n = 13), while 11% (n = 6) were obese (defined as a BMI &gt; 30 kg/m2), and 13% (n = 7) were diagnosed with type 2 diabetes. Only 9% of patients (n = 5) were smokers. Dyslipidemia was the most prevalent atherosclerotic risk factor, affecting approximately one third of patients (n = 17, 30%). In 14% (n = 8) of patients, heart failure was observed, while 13% (n = 7) were diagnosed with atrial fibrillation. Type I RCx was the most common subtype, identified in 48% of patients (n = 27) with this anomaly. Type II and Type III were found in 25% (n = 14) and 27% (n = 15) of patients, respectively. Conclusions: Our findings suggest that RCx is frequently encountered as an incidental finding, and we did not identify a consistent clinical characteristic in all patients with this type of anomaly. Furthermore, no gender predominance was associated with RCx. The natural history of this anomaly and its clinical implications seem benign. Further research is warranted to better understand this anomaly’s natural course and clinical implications.

Fractional flow reserve in anomalous aortic origin of coronary arteries to evaluate hemodynamic consequences of high risk anatomical features

Abstract Introduction In patients with anomalous aortic origin of a coronary artery (AAOCA) fractional flow reserve (FFR) can be performed for further risk stratification of myocardial ischemia and sudden cardiac death. The aim of this study was to evaluate the hemodynamic consequences (accessed with FFR) of the high-risk anatomical features in AAOCA. Methods In this prospective cohort study, all consecutive patients with AAOCA in whom diagnostic work-up according to the MuSCAT trial, including FFR, was performed between July 2020 and January 2023 in our tertiary AAOCA referral center, were included. Presence and length of the intramural segment was assessed using the interluminal space (ILS) on the CTA and orifice geometry using IVUS. Results Thirty patients, 57% female and mean age at AAOCA diagnosis of 48.7±15.3 years, were included. Twenty-seven (90%) patients presented with an anomalous aortic origin of the right coronary artery. FFR adenosine was measured in all patients (Figure 1), and showed a significant relation with: 1. hypoplasia of the proximal segment on CTA (R=0.400, p=0.029); 2. the ILS at 2mm from the ostium on CTA (R=0.517, p=0.003); 3. the length of the intramural course on CTA (R=0.500, p=0.005); 4. the orifice shape on IVUS (R=0.460, p=0.011) and 5. the minimal lumen area on IVUS (R=0.371, p=0.044). Conclusions FFR was significantly reduced in AAOCA with the following high-risk anatomical features: increased hypoplasia of the proximal segment on CTA, lower ILS at 2mm from the ostium on CTA, longer intramural course on CTA, increased slitlike orifice shape on IVUS and lower minimal lumen area on IVUS. These anatomical high-risk features appear to be hemodynamically relevant in the pathophysiology of AAOCA.Figure 1

Abstract 13937: Socioeconomic Status in Pediatric Patients With Anomalous Aortic Origins of Coronary Arteries: An Individual- and Zip Code-Level Analysis

Introduction: Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden cardiac death in the young. Limited data is available on potential disparities on access to care and whether socioeconomic status is associated with AAOCA. Hypothesis: AAOCA occurrence, severity, and treatment relate to socioeconomic status and may unfold inequities to care. Methods: Single-center retrospective study of 553 pediatric patients with AAOCA residing in Texas. Zip code socioeconomic characteristics were extracted from American Community Survey 2021 5-year estimates. All Texas zip codes were ordered into quartiles by characteristic. Age at diagnosis, signs of ischemia, and surgery status were compared by race and zip code parameters. Mann-Whitney U test was for non-parametric variables and χ 2 test, Fisher’s test, or one-way ANOVA for categorical variables. Results: Table 1 depicts patients’ demographics. AAOCA patients tended to reside in zip codes at the 4 th quartile of income (39%, p=&lt;0.001), education (29%, p=0.003), and employment (42%, p=&lt;0.001) (Table 2). There was no difference in age at diagnosis, signs of ischemia, or occurrence of coronary surgery by individual race or zip code socioeconomic parameters. Conclusions: AAOCA pediatric patients tend to live in zip codes with higher socioeconomic status (SES). These differences may reflect disparities in access to diagnosis and care. Further studies are needed to determine the burden of AAOCA in low SES populations.

Abstract 12529: Exercise Patterns and Body Mass Index in a Large Cohort of Patients With Anomalous Aortic Origins of Coronary Arteries

Introduction: Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden cardiac death in the young. Exercise patterns in relation to BMI and surgery status are lacking. Hypothesis: Exercise patterns are variable in AAOCA and relate to BMI and surgery. Aim: To identify differences in exercise patterns and BMI in patients with AAOCA and their relation to surgery status. Methods: Single-center retrospective study of 573 patients with AAOCA (median age = 11.9 years, IQR 6-15), 63% males. Exercise patterns were classified as recreational or organized/competitive. BMI percentiles were stratified as normal (5 th to ≤ 85 th %ile), overweight (85 th to ≤ 95 th %) and obese (≥ 95 th %ile). Exercise patterns and BMI were compared across demographic parameters, presence of ischemia, and surgery status. Mann-Whitney U test was for non-parametric and χ 2 test for categorical variables. Results: Two-thirds of patients exercise recreationally, 1/3 are overweight (BMI &gt; 85 th %ile) and 20% are obese (BMI &gt;95 th %ile) (Table 1). Those who had surgery were more likely to exercise competitively (24% vs 15%, p=0.026). Myocardial ischemia was more likely in those who were exercise restricted (34% vs 14%, p=&lt;0.001). There was no difference in BMI percentile in those with ischemia or who exercised competitively (Table 2). Conclusion: Most patients with AAOCA exercise recreationally and 1 in 5 is obese. Surgery positively relates to competitive exercise. BMI or exercise patterns are unrelated to ischemia but associated with exercise restriction. Future studies are needed to determine optimal exercise recommendations to promote active lifestyle while minimizing the risk of sudden death.

Invited Commentary: Notwithstanding the Significance of Anatomy in Anomalous Aortic Origin of a Coronary Artery, Physiology Is Important, Too.

The management strategies for anomalous aortic origin of a coronary artery (AAOCA) are based on anatomy, symptoms, and stress tests for evidence of ischemia. These strategies remain associated with low levels of evidence. Stress tests for ischemia or ventricular dysfunction, the only widely used physiological tests, are not adequately reliable. Additional physiological metrics are needed to build reliable strategies. Fractional flow reserve (FFR) and instantaneous wave-free ratio (iFR) are physiological measurements that are used in assessing acquired coronary artery disease (CAD). In this commentary, we describe FFR and iFR and review studies supporting their utility in assessing CAD. We describe a few small studies of their use in assessing AAOCA. Finally, we comment on the unique features of AAOCA that mandate further investigation on how to conduct and interpret FFR/iFR measurements. Even at a point where we understand how to do this, determining the effectiveness of these measurements in improving outcomes and guiding management will require lengthy and challenging trials. A collective effort of institutions that manage AAOCA will be essential.

Assessment of Intramural Segment Compression in Anomalous Coronary Arteries through Patient-Specific Finite Element Modeling

Background: Anomalous Aortic Origin of a Coronary Artery (AAOCA) is a congenital condition that can lead to ischemia and sudden cardiac death. Current diagnostic tools are unable to fully quantify the pathological behavior that occurs mainly with physical effort. Methods: Patients’ computed tomography scans and centerline-based geometric quantities were used to develop three-dimensional computer-aided design models of the main anatomical variants of AAOCA. Blood pressure ranging from rest to extreme effort was simulated through structural finite element analyses, and the pressurized geometries were analyzed to evaluate coronary lumen cross-sectional areas and variations at the different loading conditions. Results: We simulated 39 subjects, demonstrating the ability to reproduce accurately the patient-specific anatomy of different AAOCA variants and capture pathological behaviors. AAOCAs with intramural courses showed compression along the proximal segment with a caliber reduction ranging from 0.14% to 18.87% at different pressure levels. The percentage of proximal narrowing relative to the distal segment was greater than any other type of anomalous course and exceeded 50% with simulated exertion. Conclusions: The present study proposes a computational pipeline to investigate conditions not reproducible in clinical practice, providing information to support decision-making in the management of AAOCA patients.

Noninvasive Multimodality Imaging for the Assessment of Anomalous Coronary Artery.

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. 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 possibleconcomitant 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.

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.

A Survey on the Management of Anomalous Aortic Origins of the Coronary Arteries.

The management of patients with an anomalous aortic origin of a coronary artery (AAOCA) remains controversial despite the publication of the 2017 American Association for Thoracic Surgery (AATS) expert guidelines. We surveyed the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and the Pediheart.net online community regarding their care of patients with anomalous origins of the right or left coronary from the opposite cusp with inter-arterial courses and compared them to the AATS guidelines. We received 111 complete responses. Four notable variations from the AATS recommendations were identified. Respondents were more likely to use ECG exercise testing than the stress imaging recommended in the AATS guidelines. For a 16-year-old with AAOCA, recommendations for surgery generally followed the AATS guidelines. However, for asymptomatic left AAOCA without signs of ischemia on stress imaging, only 69.4% felt surgery was appropriate or somewhat appropriate. In the setting of a 16-year-old with right AAOCA free from signs or symptoms of ischemia, respondents were more likely to recommend surgery if the patient was a competitive athlete, a topic not directly addressed in the AATS guidelines. After surgical treatment of AAOCA, only 24% of respondents recommended lifelong antiplatelet therapy despite recommendations for this in the AATS guidelines. Respondents recommendations were generally consistent with the 2017 AATS guidelines but with important variations in the use of stress imaging, indications for surgery in asymptomatic left AAOCA, the impact of identification as a competitive athlete and duration of postoperative antiplatelet therapy.

Clinical characteristics of anomalous aortic origin of a coronary artery in children

Objective: To analyze the clinical characteristics, diagnosis and treatment of anomalous aortic origin of a coronary artery (AAOCA) in children. Methods: There were 17 children diagnosed with AAOCA from January 2013 to January 2022 in Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine.Their clinical manifestations, laboratory and imaging data, treatment and prognosis were retrospectively analyzed. Results: These 17 children included 14 males and 3 females, with the age of (8.7±3.5) years. There were 4 anomalous left coronary artery (ALCA) and 13 anomalous right coronary artery (ARCA). Seven children presented with chest pain or chest pain after exercise, three patients presented with cardiac syncope, one complained chest tightness and weakness, and the other six patients had no specific symptoms. Cardiac syncope and chest tightness occurred in patients with ALCA. Fourteen children had the dangerous anatomical basis of myocardial ischemia caused by coronary artery compression or stenosis on imaging. Seven children had coronary artery repair, of whom two were ALCA and five were ARCA. One patient had received heart transplantation because of heart failure. The incidence of adverse cardiovascular events and poor prognosis in ALCA group was higher than that in ARCA group (4/4 vs. 0/13, P<0.05). They were followed up in the outpatient department regularly for 6 (6, 12) months; except for the one who lost visit, the rest of the patients had a good prognosis. Conclusions: Cardiogenic syncope or cardiac insufficiency usually occurs in ALCA, and adverse cardiovascular events and poor prognosis are more common in ALCA than in ARCA. Early surgical treatment should be considered for children with ALCA and ARCA accompanied by myocardial ischemia.

Computed tomography angiography (CTA) of anomalous aortic origin of a coronary artery (AAOCA): Which measurements are accurate and reliable?

BackgroundThe variation and accuracy of computed tomography angiography (CTA) features of anomalous aortic origin of a coronary artery (AAOCA) have not been completely characterized. We evaluated anomalous right (AAORCA) and left (AAOLCA) coronary arteries by CTA, with an emphasis on reproducibility, comparison with surgical measurements, and effect of nitroglycerin. MethodsCTAs were interpreted for location of coronary origin & exit from the aorta, course, and dominance; minor & major diameters of the proximal & distal coronaries; shape of orifice; and intramural length. Relationships between vessel measurements, body surface area (BSA), and nitroglycerin use were evaluated. Comparisons between CTA and surgical measurements included intramural length, surgical probe size pre-intervention vs. CTA proximal AAOCA diameter, and surgical probe size post-intervention vs. CTA distal AAOCA diameter. ResultsOf 104 patients [81 (78%) AAORCA], all but 1 were intramural. Compared to AAOLCA, AAORCA patients were more likely to have a high origin (91% vs. 31%, p ​< ​0.01), and slit-like orifice (58% vs. 26%, p ​< ​0.01). When CTAs with nitroglycerin were compared to those without, no difference in proximal AAOCA dimensions was identified; however distal AAOCA and normal-origin coronary dimensions were larger in scans with nitroglycerin. Aside from slit-like orifice, reliability of coronary CTA measurements, including proximal AAOCA minor diameter and intramural length, was moderate to good between readers. In a subgroup of 54 patients, proximal AAOCA minor diameter and intramural length had good agreement and correlation with surgery. ConclusionProximal AAOCA dimensions and intramural length are reproducible variables. The lack of difference in proximal AAOCA dimensions with nitroglycerin may reflect abnormal vessel mechanics. Multicenter studies are an important next step in understanding the generalizability of our findings.

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

Background and objectiveThere 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 methodsRetrospective 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. Results100 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. ConclusionsCCTA 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.

Toward More Granular Guidelines in AAOCA: Associating Anatomical Details With Specific Surgical Strategies.

Patients with anomalous aortic origin of a coronary artery (AAOCA) require imaging to clarify the multiple potential anatomic sites of obstruction (fixed or dynamic). Once repaired, the pathway of blood to the myocardium must not encounter: (1) intrinsic ostial stenosis, (2) obstruction from compression or distortion near the commissure or the intercoronary pillar, (3) stenosis where the artery exits the aortic wall (due to an acutely angled "take-off"), (4) compression due to a pathway between the great vessels, (5) stenosis or compression along an intramural course, or (6) compression due to an intramuscular (intraseptal/intraconal) course. Detailed anatomic evaluation of each of these locations allows the surgeon to select an appropriate repair strategy, and each of these abnormal anatomic features should be "matched" with a particular surgical correction. We speculate that the most common surgical repair, unroofing with or without tacking, is often inadequate, as in isolation, it may not allow for correction with a large orifice from the appropriate sinus, without an interarterial course. While the evidence base is insufficient to call these recommendations formal guidelines, these recommendations should serve as a basis for further validity testing, and ultimate evolution to more granular guidelines on AAOCA management.

Anomalous Aortic Origin of Coronary Arteries in Children: Postoperative High-risk Anatomic Features

BackgroundThis study aimed to assess postoperative presumed high-risk anatomic features (HRAFs) by using computed tomographic angiography (CTA) in patients with anomalous aortic origin of a coronary artery (AAOCA) after surgical unroofing vs transection and reimplantation (TAR) if unroofing was thought to provide unsatisfactory results. MethodsThe study included 62 children with postoperative CTA performed at a median of 3 months (interquartile range, 3-4 months) after unroofing (n = 45) and TAR (n = 17). HRAFs included slitlike ostium, intramural course, acute angle takeoff (<45o), interarterial course, proximal stenosis >50%, or course through a thickened intercoronary pillar. ResultsMedian age at surgery was 13.8 years (interquartile range, 10.5-15.8 years). None of the patients had a slitlike ostium or an intramural course on postoperative CTA. Acute takeoff was seen in 100% after unroofing and in 2 of 17 (12%) after TAR (P < .001). After unroofing, the interarterial course improved to 35 of 45 (78%) from 43 of 45 (96%) (P = .003), and a thickened intercoronary pillar improved to 10 of 45 (22%) from 22 of 45 (49%) (P = .0001), compared with none seen after TAR. Preoperative intramural length <5 mm was associated with a postoperative thickened intercoronary pillar in right AAOCA after unroofing (P = .0004). Severe coronary stenosis occurred in 2 of 17 (12%) after TAR, and both patients needed urgent revision procedures. All patients except 2 (97%) returned to exercise activities at a median follow-up of 4.9 years (range, 0.6-9.2 years). ConclusionsThe slitlike ostium and intramural course resolved in all patients. Residual acute angle takeoff, an interarterial course, and mild coronary narrowing related to a thickened intercoronary pillar were common after unroofing. TAR allows resolution of all HRAFs, although severe narrowing requiring surgical revision happened only in TAR. Long-term studies are needed to understand the clinical significance of these residual presumed HRAFs.

Percutaneous treatment of a CTO in an anomalous right coronary artery: A rupture paved the way for new insights.

An anomalous aortic origin of a coronary artery (AAOCA) from the opposite sinus, with an interarterial course, has been associated with an increased risk of myocardial ischemia and sudden death. As the exact pathophysiology of AAOCA is not well understood, the clinical management is also not well defined. With increased use of non-invasive imaging, the diagnosis of AAOCA is increasing and the association of anomalous origin and atherosclerotic disease is becoming a more important topic. We report a rare case of AAOCA chronic total occlusion (CTO). A 40-year-old Caucasian man was referred for invasive coronary angiography (ICA) due to typical chest pain and positive myocardial scintigraphy. ICA demonstrated CTO of an anomalous right coronary artery (ARCA) originating from the left side of the ascending aorta with an interarterial course. There was no lesion in the left coronary artery. During the procedure, unexpected rupture of the coronary artery occurred after dilatation with a small balloon at low pressure. The complication in this case was handled with good procedural final result but was an occasion for a food for thought. Coronary artery perforations are rare but life-threatening procedural complications that are usually caused by predisposing anatomical and procedural factors. We issue a warning on the risk of complications during complex percutaneous coronary intervention of these arteries, and we reconsidered the pathophysiology of the anomaly in a way that could change the approach to the disease. Based on this complication, we hypothesized that the wall of the artery could be fragile due to histopathological alterations, which could have a role in the pathophysiology of coronary malignancy. Future autopsy studies should be focused on the analysis of the arterial wall of the patient affected by sudden death with this anomaly.

459 CT Angiography Measurements Of Anomalous Aortic Origin Of A Coronary Artery: Inter-observer Agreement And Comparison To Intra-operative Inspection

Introduction: Morphologic features of anomalous aortic origin of a coronary artery (AAOCA) by CT angiography (CTA) are associated with risk of ischemia and are used to guide surgical referral. However, their inter-observer agreement and comparison to intra-operative inspection have not been well characterized.

Surgery for Anomalous Aortic Origin of a Coronary Artery (AAOCA) in Children and Adolescents: A Meta-Analysis.

We conducted a systematic review and meta-analysis of the literature to assess the outcomes of surgery for the anomalous aortic origin of a coronary artery in children and young adults (<30 years). Thirteen publications were selected, including a total of 384 patients. Unroofing of the intramural segment was adopted in 92% (95% CI: 81%-98%) of cases, with pooled early and late mortality of 0% (95% CI: 0%-0.3%) and 0.1% (95% CI: 0%-1%), respectively. Reoperation for aortic regurgitation was anecdotal. Surgical management of anomalous aortic origin of coronary arteries can be achieved with excellent results in pediatric patients, but concerns remain about the durability of surgery.