Aortic Origin Of 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.

Diagnosis and surgical treatment of high-risk anomalous aortic origin of coronary artery

Objective: To analyze the diagnosis and surgical treatment of high-risk anomalous aortic origin of coronary artery (AAOCA). Methods: This is a retrospective case series study. From January 2016 to July 2023, 24 cases of high-risk AAOCA underwent surgical treatment in Department of Cardiac Surgery, Guangdong Provincial People's Hospital. There were 18 males and 6 females, operatively aged (M (IQR)) 13 (26) years (range: 0.3 to 57.0 years). They were confirmed by cardiac ultrasound and cardiac CT, all of which had anomalous coronary running between the aorta and the pulmonary artery. There were 15 cases of the right coronary artery from the left aortic sinus of Valsalva, 6 cases of left coronary artery from the right aortic sinus of Valsalva, 3 cases of the sigle coronary artery. Only 3 patients had no obvious related symptoms (2 cases were complicated with a positive exercise stress test and 1 case with other intracardiac malformations), 21 cases had a history of chest tightness, chest pain, or syncope after exercise. Three patients suffered syncope after exercise and underwent cardiopulmonary resuscitation (2 cases were treated with an extracorporeal membrane oxygenerator (ECMO)). The gap from the first symptom to the diagnosis was 4.0 (11.5) months (range: 0.2 to 84.0 months). The detection rate of coronary artery abnormalities suggested by the first cardiac ultrasound was only 37.5% (9/24). Seven patients were complicated with other cardiac diseases (4 cases with congenital heart defects, 2 cases with coronary atherosclerotic heart disease, 1 case with mitral valve disease). Results: All 24 patients underwent surgical treatment (23 cases underwent abnormal coronary artery unroofing, 1 case underwent coronary artery bypass grafting), and 5 patients underwent other intracardiac malformation correction at the same time. There were no death or surgery related complications in the hospital for 30 days after the operation. A patient with preoperative extracorporeal cardiopulmonary resuscitation was continuously assisted by ECMO after emergency AAOCA correction and had complications such as limb ischemia necrosis and renal dysfunction after the operation. During the follow-up of 2.2 (3.3) years (range: 1 month to 7.2 years), one patient who previously underwent percutaneous transluminal coronary angioplasty with a stent implant experienced significant postoperative symptomatic relief, and the other discharged patients had no related symptoms. Conclusions: The accurate rate of initial diagnosis for high-risk AAOCA is still low, but the risk of cardiovascular accidents is high. For sports-related chest pain and other symptoms, more attention should be paid to the detection of AAOCA, especially for adolescents. Exercise stress testing can be helpful in evaluating the cardiovascular risk of asymptomatic AAOCA. Instant surgical treatment can achieve satisfactory curative effects.

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.

Abnormal aortic origin of coronary arteries

Abnormal aortic origin of coronary arteries

Abnormal aortic origin of coronary arteries

The anomalous aortic origin of the coronary arteries has an estimated prevalence of 0.02-5.7%. It can be associated with sudden death when it has an interarterial or intramural pathway or be damaged during interventions on the mitral, pulmonary and/or aortic annulus or percutaneous closure of an interatrial septal defect. To identify these patients by imaging techniques such as transthoracic color Doppler echocardiography (TTE), computed tomography (CT) multislice angiography or coronary angiography. The imaging techniques used for the detection of coronary anomalies were TTE, multislice coronary angiography or coronary angiography according to what is generally accepted. Fifteen patients were identified; in 12 of them the suspicion was due to TTE and in the remaining 3, CT multislice angiography was diagnostic. The circumflex artery was the coronary artery most involved, associated or not with another coronary anomaly (12/15 patients) and in the other three cases, the anomalous coronary artery had an interarterial course, with the right coronary arteries and the anterior descending coronary arteries being involved. The under diagnosis by TTE of coronary artery abnormalities may be due to the difficulty of visualization that is accentuated with age. Their detection is crucial because they can both, lead to sudden death associated with an intramural and/or interarterial pathway and complicate an interventional procedure on the interatrial septum or within the mitral, pulmonary and/or aortic rings.

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 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.

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.

Morphological Changes of Anomalous Coronary Arteries From the Aorta During the Cardiac Cycle Assessed by IVUS in Resting Conditions.

Anomalous aortic origin of coronary artery (AAOCA) with intramural segment is associated with risk of sudden cardiac death, probably related to a compressive mechanism exerted by the aorta. However, the intramural compression occurrence and magnitude during the cardiac cycle remain unknown. We hypothesized that (1) in end diastole, the intramural segment is narrower, more elliptic, and has greater resistance than extramural segment; (2) the intramural segment experiences a further compression in systole; and (3) morphometry and its systolic changes vary within different lumen cross-sections of the intramural segment. Phasic changes of lumen cross-sectional coronary area, roundness (minimum/maximum lumen diameter), and hemodynamic resistance (Poiseuille law for noncircular sections) were derived from intravascular ultrasound pullbacks at rest for the ostial, distal intramural, and extramural segments. Data were obtained for 35 AAOCA (n=23 with intramural tract) after retrospective image-based gating and manual lumen segmentation. Differences between systolic and end-diastolic phases in each section, between sections of the same coronary, and between AAOCA with and without intramural tract were assessed by nonparametric statistical tests. In end diastole, both the ostial and distal intramural sections were more elliptical (P<0.001) than the reference extramural section and the correspondent sections in AAOCA without intramural segment. In systole, AAOCA with intramural segment showed a flattening at the ostium (-6.76% [10.82%]; P=0.024) and a flattening (-5.36% [16.56%]; P=0.011), a narrowing (-4.62% [11.38%]; P=0.020), and a resistance increase (15.61% [30.07%]; P=0.012) at the distal intramural section. No-intramural sections did not show morphological changes during the entire cardiac cycle. AAOCA with intramural segment has pathological segment-specific dynamic compression mainly in the systole under resting conditions. Studying AAOCA behavior with intravascular ultrasound during the cardiac cycle may help to evaluate and quantify the severity of the narrowing.

Surgical repair of the anomalous aortic origin of the coronary arteries: a single-center experience.

Anomalous aortic origin of the coronary artery is a rare congenital cardiac anomaly. The aim of this study was to present our experience with patients who underwent surgery for the anomalous aortic origin of the coronary artery. This was a retrospective review of our experience with patients who had anomalous aortic origin of the coronary artery from 2019 to 2022. Seven patients were managed for anomalous aortic origin of the coronary artery, including five males and two females. The median age of the patients were 16 years (IQR, 14.5-26 years). Five patients had anomalous aortic origin of the right coronary artery and two patients had anomalous aortic origin of the left coronary artery. Five patients were treated surgically, one patient refused surgical treatment despite myocardial ischaemia symptoms, and the other one was not operated because she had no symptoms. Two patients underwent pulmonary root anterior translocation and left main coronary artery unroofing procedure, one patient underwent right coronary artery unroofing procedure, one patient underwent pulmonary artery lateral translocation procedure, and the last patient underwent right coronary artery osteal translocation procedure. The post-operative mortality or myocardial infarction was not observed in any patient. Patients were followed for a median of 10 months (IQR, 6.75-20.5 months) after repair. The data suggest that surgical repair of anomalous aortic origin of the coronary artery can be performed confidently and can be very effective for relieving myocardial ischaemia symptoms. Different surgical techniques can be used in anomalous aortic origin of the coronary artery according to the course and origin of the coronary arteries. To the our knowledge, pulmonary root anterior translocation and coronary artery unroofing procedure were performed for the first time in the literature.

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

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). 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. 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. 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.

Safety and Efficacy of Surgical Correction of Anomalous Aortic Origin of Coronary Arteries: Experiences from two Tertiary Cardiac Centers.

This study aims to describe the outcomes of surgical correction for anomalous aortic origin of coronary artery (AAOCA) with regard to symptom relief. We performed a retrospective multicentre study including surgical patients who underwent correction for AAOCA between 2009 and 2022. Patients who underwent concomitant cardiac procedures were also included. However, to analyze symptom relief we only assessed the subgroup of symptomatic patients who underwent isolated correction for AAOCA. A total of 71 consecutive patients (median age 55, range 12-83) who underwent surgical correction for AAOCA were included in the study. Right-AAOCA was present in 56 patients (79%), left-AAOCA in 11 patients (15%), single coronary ostium AAOCA in 4 patients (6%). Coronary unroofing was performed in 72% of the patients, coronary reimplantation in 28% and an additional neo-ostium patchplasty in 13% of the patients. In 39% of the patients a concomitant cardiac procedure was performed. During follow-up, no cardiovascular-related death was observed. Three patients (4.2%) had a myocardial infarction and underwent postoperative coronary artery bypass grafting. Six patients (8.5%) needed postoperative temporary mechanical circulatory support. Among the 34 symptomatic patients who underwent isolated AAOCA correction, 70% were completely asymptomatic after surgery, 12% showed symptom improvement and no symptom improvement was observed in 18% of the patients (median follow-up 3.5 years, range 0.3-11.1). Correction for AAOCA can be safely performed with or without concomitant cardiac procedures. Performing AAOCA correction leads to a significant improvement in symptoms for most patients.

Outcomes of a protocolized approach for surgical unroofing of intramural anomalous aortic origin of coronary artery in children and adults

ObjectiveManagement of anomalous aortic origin of coronary arteries has been variable, and limited data are available on early and late outcomes. MethodsWe report a single institution's experience with a protocolized approach to 148 consecutive patients who underwent surgical unroofing of intramural anomalous aortic origin of coronary arteries (June 2003 to December 2020). The management algorithm included preoperative and postoperative cross-sectional and echocardiographic imaging, exercise testing, and a standardized operative technique. ResultsThe median age of the cohort was 44.4 years (range, 4 months to 83 years); 130 patients had an anomalous right coronary artery, and 19 patients had an anomalous left coronary artery. Surgical unroofing was an isolated procedure in 118 patients (80%) and a concomitant procedure in 30 patients (20.3%). There were 2 (1%) early deaths; both were reoperations for aortic root or valve replacement. Over a median follow-up of 9.5 (interquartile range, 5-12.3) years, there were 5 late deaths, 3 due to noncardiac causes and 2 due to unknown cause. Late survival after anomalous aortic origin of coronary arteries repair at 10 and 15 years was 94.5% and 94.5%, respectively. There were no early or late deaths in the pediatric cohort with a median follow-up of 10.9 years (interquartile range, 6.9-12.1). At a median clinical follow-up of 3.9 years (interquartile range, 1.1-9.5), 36 patients had chest pain but none with evidence of ischemia related to the unroofing. ConclusionsSurgical unroofing of anomalous aortic origin of coronary arteries can be performed safely with low early mortality, even in the setting of concomitant procedures. Late survival is excellent, with the vast majority being symptom free.

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.

Abstract 11835: Different Dynamic Luminal Compression of Intramural Segment During Cardiac Cycle in Anomalous Aortic Origin of Coronary Arteries Revealed by Ivus Gated Sequences

Introduction: Anomalous Aortic Origin of Coronary Artery (AAOCA) with intramural (IM) segment is associated with risk of sudden cardiac death (SCD). The pathogenic mechanism is believed to be the IM compression caused by the aorta expansion during effort. However, the IM compression occurrence and magnitude during the cardiac cycle remain unknown. Hypothesis: We hypothesized that the IM segment is subjected to dynamic compression during different phases of cardiac cycle with a luminal area and roundness smaller in systole than in end-diastole. Methods: Lumen dynamics was derived from IVUS pullbacks of N=35 AAOCA (N=23 with IM tract) after retrospective image-based gating to identify cardiac cycles. The cyclic change of area (A) and roundness (R=minimum/maximum lumen axis) of ostial, distal IM, and extramural (EM) tracts were described after manual lumen segmentation. The systolic phase was identified, in each patient, by estimating normal PQ and QT intervals from image-based gating. Results are presented with median and interquartile range. One-tailed Wilcoxon signed-rank test (p&lt;0.025) assessed differences between systolic and diastolic phases in each tract. Results: A systolic reduction of roundness was observed in ostium [R systole = 0.5 (0.2), R diastole = 0.6 (0.3), flattening: -7% (10), p=0.01] and distal IM segment [R systole = 0.7 (0.2), R diastole = 0.8 (0.2), flattening: -6% (16), p=0.004]. No-IM segments showed normal behaviour and remained circular during the entire cardiac cycle. The distal IM had an area reduction during systole [A systole = 8.3 (2.3) mm 2 , A diastole = 8.1 (2.9) mm 2 , narrowing: -4% (11), p=0.009] that was not observed in all other segments (Figure 1). Conclusion: AAOCA with IM segment has pathological dynamic compression mainly in systole. Studying AAOCA behaviour with IVUS during the cardiac cycle may help to evaluate and quantify the severity of the narrowing.

Coronary unroofing does not fits all anomalous aortic origin of coronary arteries.

Anomalous aortic origin of coronary artery (AAOCA) is the second leading cause of sudden cardiac death in children and young adults. Intramural-interarterial course is the most frequent anatomic variation and coronary unroofing is widest adopted for surgical management. Symptoms recurrence is described regardless of the technique used. This study aims to describe how an anatomic patient-centered approach aimed to restore a normal coronary artery take-off is associated with symptoms resolution. From 2008 to 2021, 25 patients were operated on for an AAOCA at a median age of 20 years. Nineteen patients had a right AAOCA and six had left AAOCA. Intramural course was present in 18 patients. Seventy-six percent were symptomatic. No episodes of aborted sudden cardiac death before surgery was described in the population. Surgical technique used were coronary unroofing in 18 patients, coronary neo-ostioplasty in 3, coronary Reimplantation in 3, and main pulmonary artery re-location in 1. No hospital mortality or reoperation was observed in our experience as well as major complications related to surgery. Mean hospital length of stay was 8.5 days. None of patients reported symptoms recurrence at follow-up. Young athletes returned to play competitive sport. Postoperative computed tomography scan evaluation showed a general improvement of the take-off angle. AAOCA requires a patient anatomic-based surgical management. There is not a single surgical technique that can fits all anatomic subtype of AAOCA. Surgical techniques may be selected on the base of the preoperative images and intraoperative findings. In our experience, this policy is associated with no symptoms recurrence.

Anomalous Aortic Origin of Coronary Artery: A Cases Series

Congenital coronary artery anomalies (CCAA) are not that common, they are usually found in 0.3 to 5.6% of people undergoing coronary angiography [1], these numbers are still to be widely confirmed since they can be biased by the fact that angiography or autopsy are usually preformed in patients with suspected cardiac disease. However, they are particularly important as they occupy the second place of most common cause of sudden death in young athlete according to a series reported by Basso et al., [2]. Definition, clinical presentation, physiopathology of symptoms, diagnostic workup and prognosis, can be challenging and varies according to different subgroups established by classifications, this last one can falls into two paths, anatomical based on the description of origin, course, termination and functional based on the degree of ischemia that can be caused by it. The management and treatment of these anomalies is still not firmly entrenched, it depends mostly on the patient, circumstances of diagnosis and proof of hemodynamic significance. In this case series of 3 patients, we will focus mainly of anomalous aortic origin of a coronary artery, encountered in our cathlab.

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.

An Atypical Anomalous Aortic Origin of the Left Coronary Artery With Intra-Arterial Wall Course Pretending a Normal Migration on Imaging Screening: A Case Report

BackgroundAn anomalous aortic origin of a coronary artery (AAOCA) has been considered as a dominant cause of sudden cardiac death (SCD) among young age children. Therefore, it is critical to identify AAOCA timely to avoid lethal events. Recently, accumulating cases of right or left coronary arteries originating from inappropriate locations at the sinus of Valsalva have been identified. Here, we report a rare case of AAOCA with an intra-arterial wall course pretending normal migration on imaging screening in a patient who suffered from syncope.Case summaryA 7-year-old male without a previous history of cardiovascular and cerebrovascular diseases suddenly suffered from sharp chest pain and syncope after intensive exercise. The electrocardiogram showed that the ST segment of multiple leads was depressed by more than 0.05 mV, and biomarkers indicated severe myocardial injuries. The left ventricular ejection fraction (LVEF) decreased dramatically to 23%. Fulminant myocarditis and cardiomyopathy were therefore excluded. However, a relatively normal coronary artery origin, which arose from the left coronary sinus, presented on echocardiography and cardiac CT angiography (CTA). It is difficult to draw an association between severe clinical manifestations and slight malformations on echocardiography and CTA. Furthermore, selective coronary angiography revealed that an anomalous left coronary artery arose from the superior margin of the inappropriate sinus, developed an intramural wall course and finally exits the left sinus of Valsalva and migrated between the aorta and the pulmonary artery, which induced severe myocardial infarction during exercise. Then, the patient received surgical correction with a modified unroofing procedure. After 2 months of intensive treatment, the patient was discharged and remained asymptomatic through 18 months of follow-up.ConclusionAAOCA, especially anomalous left coronary artery (ALCA), represents a major potential risk of SCD. We reported an atypical manifestation of ALCA arising from the inappropriate sinus of Valsalva and merging into the intra-arterial wall to develop a strange course and then sprout between the aorta and the pulmonary artery. The diversity of AAOCA might present as a relatively normal course under non-invasive radiological imaging scanning.