Interarterial Course Research Articles

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.

Inter-arterial course of the left coronary artery with a commissural origin in a 40-year-old patient with tricuspid atresia post Fontan shunt.

We report a case of an adult male with Tricuspid atresia post Fontan shunt in whom a rarecoronary anomaly was demonstrated on Computed Tomography angiography usingendoluminal views and volume rendering techniques.

Anomalous origin of the left coronary artery from the right sinus of Valsalva with separate ostia and inter-arterial course: MDCT angiography depiction.

We describe a case of anomalous left coronary artery origin from the right coronary sinus with separate ostia of right and left coronary arteries detected by 128-slice computed tomography (CT) coronary angiography. Apart from separate ostia, the left coronary artery had inter-arterial course between the aorta and the pulmonary trunk, also called as a malignant course.

131: ANOMALOUS RIGHT CORONARY ARTERY WITH AN INTERARTERIAL COURSE ORIGINATING FROM THE LEFT CORONARY CUSP

Introduction: Anomalies of coronary arteries arising from the opposite sinus are extremely rare, with an estimated prevalence of anomalous right coronary arteries arising from the left coronary cusp varying between 0.026 and 0.25%. While largely incidental, symptomatic patients vary in presentation. This case describes a young patient with no cardiac history who presented with acute coronary syndrome and was found to have an anomalous origin of the right coronary artery (RCA). Methods: Data was collected during the admission and follow-up a year later. Results: A 27-year-old male with a history of recent cocaine use presented to the hospital with several hours of chest pain described as constant, substernal, pressure-like, exertional, and with radiation to both arms. Laboratory analysis on admission showed normal cardiac biomarkers but the ECG showed ST-elevations in multiple inferior leads. Emergent cardiac catheterization with coronary angiography showed no epicardial or occlusive coronary artery disease; however, it did show an abnormal origin of the RCA. Subsequent CT angiogram of the coronary arteries confirmed an anomalous origin of the RCA from the left coronary cusp with an interarterial course between the aorta and the pulmonary artery, with concerns for impingement. Troponin level trended up to 21 ng/mL; however, we were unsure whether this was secondary to cocaine-induced vasospasms, RCA impingement, or a combination of both. Echocardiogram (ECHO) showed a normal ejection fraction with no valvular or wall motion abnormalities. Patient tolerated the stress test well, tolerating 11+ METS, which revealed no significant ECG changes and minimal areas of ischemia. Following discussion with all the cardiac specialties involved, the patient decided to hold off surgery at this time and continue with conservative medical management. Cocaine abstinence was advised. The patient has had no recurrent episodes in over a year. Conclusions: Coronary anomalies should be suspected in symptomatic young patients with acute coronary syndrome. Given the rarity of this condition, many physicians may be unaware of this disease and should consider this as a differential diagnosis. While surgical intervention is considered first line treatment in many cases, medical management remains a suitable option in select patients.

230 ANOMALOUS ORIGIN OF LEFT CIRCUMFLEX ARTERY FROM RIGHT SINUS OF VALSALVA: A RARE CASE BUT WITH GREAT CLINICAL RELEVANCE

Abstract Introduction The anomalous connection of the left circumflex artery (LCx) to the right coronary artery (RCA) or sinus is the most frequent coronary artery (CA) anomaly. Among them, only those with an interarterial course are regarded as hidden conditions at risk of myocardial ischemia (MI) and sudden cardiac death (SCD). We report an uncommon of anomalous origin of LCx from the right sinus of Valsalva and a retroaortic path causing MI. Case presentation A 61-year-old man presented to the emergency department complaining palpitations and chest discomfort for an hour. He only had history of hypertension. Physical examination was unremarkable. The ECG demonstrated atrial flutter with a 2:1 conduction ratio and a ventricular rate of 157 bpm and ST segment depression in leads V4-6. Transthoracic echocardiography did not reveal segmental kinetic anomalies but a five-chamber apical view showed a “RAC sign”, typical of anomalous retroaortic course of the left coronary artery. The patient was treated with intravenous infusion of amiodarone. He restored sinus rhythm and symptoms regressed completely, but the ECG taken after conversion showed flattened T waves in leads V5-6 and negative T waves in I and aVL. Cardiac enzymes had transient increase. After the acute episode ended the patient underwent cardiac computed tomography angiography (CTA) with evidence of anomalous origin of LCx from the right sinus with a retroaortic course. A coronary angiography excluded obstructive atherosclerotic coronary lesions. Nuclear myocardial perfusion imaging revealed reversible small subsegmental perfusion defects in mid inferolateral wall and apical lateral wall. We established a medical treatment with beta-blocker. Discussion Our patient had anomalous connection of the LCx branch to the right sinus of Valsalva with a retroaortic course. Although this anomaly is usually considered benign, cases of association with SCD and MI have been reported. The factor responsible for this pathogenicity could be high orifice, ostial stenosis, slit-like/fish-mouth-shaped orifice and acute-angle take-off. As cardiac CTA did not reveal any of these characteristics, we hypothesized that the increased cardiac output and expansion of the great vessels during tachycardia could cause compression of the retroaortic segment or angling at its origin and generate ischemia. Repolarization abnormalities at ECG are well documented during supraventricular tachycardia as a response to pacing-induced stress. These changes are usually diffused and disappear after conversion to sinus rhythm. In this case they appeared hours later, accompanied by cardiac enzyme buildup. As the epicardial coronary arteries did not show any pathology, we suggest that the patient had transient ischemia due to LCx anomaly. We confirmed it by myocardial perfusion imaging. As for the management of this anomaly in adults, surgery is recommended as class IC in patients with typical angina symptoms who present with evidence of stress-induced myocardial ischemia in a matching territory or high-risk anatomy. Our patient has never had clear manifestations of angina. All these elements together with the age of our patient motivated us to use a conservative approach. Conclusions We report a case of anomalous origin of LCx from right sinus of Valsalva causing transient myocardial ischemia in a patient that has always been asymptomatic. This anomaly has been and continues to be considered benign, nevertheless we suggest to judge the clinical significance of this kind of CA anomaly on a case-by-case integrated approach.

253 EXCERCISE-INDUCED PULMONARY HYPERTENSION AND CORONARY ARTERY ANOMALY: AN UNUSUAL ASSOCIATION

Abstract A healthy 55-year-old competitive sportsman, with familial history of early sudden cardiac death, came to our attention following the finding of asymptomatic exercise-induced myocardial ischemia (ST depression in V3- V6). Computed tomography (CT) coronary angiogram excluded atherosclerotic disease but showed a dominant right coronary artery (RCA) originating from left Valsalva sinus and with an interarterial course between the ascending aorta and the pulmonary artery. Both the trunk of the pulmonary artery and the right ventricle were reported as mildly dilated Transthoracic echocardiography showed a normal left ventricle (LV), while confirmed right ventricle (RV) dilatation, moderate tricuspid regurgitation (TR) and interatrial septal aneurysm. Stress echocardiography excluded inducible ischemia, but highlighted exercise-induced pulmonary hypertension (PH) (PAPs 25 → 70 mmHg), while a transesophageal echo confirmed a left-to-right shunt due to a 8-mm Patent Foramen Ovale (PFO). Rheumatological, thromboembolic and parenchymal lung diseases were excluded as possible causes of exercise-induced PH; a rest right heart catheterization (RHC) showed a Qp/Qs ratio at the upper limits of normal range without PH. Nuclear stress testing identified trivial asymptomatic septal ischemia. The patients started medium dose of beta-blockers and early follow-up was planned. This clinical case raises some questions: why is RV dilated? What role does PFO play? What is the cause of the inducible ischemia? What is the pathogenesis of exercise-induced PH? Pulmonary Arterial Hypertension, in early stages, often manifests only under exertion, but it is usually characterized by exercise dyspnea and low oxygen saturation. Moreover, intracardiac shunts may be involved in developing PH. In this specific case, pulmonary trunk may be expanded by pressure overload, causing the compression of aberrant RCA and consequent ischemic abnormalities. RV dilatation might be explained by exercise-induced PH itself, but it was not severe and has to be demonstrated by exercise right heart catheterization. The main questions are: which is the primary etiology of right chamber enlargement and myocardial ischemia? Which is the most appropriate therapeutic approach (i.e. conservative vs interventional)?

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 14549: Malignant Anomalous LMCA With an Interarterial Course: Atypical Presentation

A 42-year-old healthy female presented to the emergency department with coughing and shortness of breath, complicated by syncope. Before loss of consciousness, the patient reported feeling lightheaded, but no chest pain or palpitations were appreciated. Following a negative work up for cardiogenic syncope, she was discharged home with a presumed diagnosis of vasovagal syncope. Over the next 12 months, the patient had recurrent episodes of shortness of breath followed by either a presyncopal prodrome or full syncopal episodes. Each episode came on with coughing and dyspnea without significant exertion. 18 months after the original event, she was re-evaluated for cardiac etiologies of her symptoms, and CT coronary angiogram showed an anomalous left main coronary artery arising from the right coronary cusp (AAOLCA) with an interarterial course, which was subsequently confirmed on coronary angiography (figure 1). Anomalous Left Coronary Arteries of Aortic Origin (AAOLCA) present a diagnostic dilemma for cardiologist, given their vague presenting symptoms, unknown prevalence in the general population, and high risk for Sudden Cardiac Death (SCD). This patient’s syncopal episodes and presyncopal symptoms that were inconsistent with a cardiac etiology represent an atypical presentation, although there is no classic presentation for which AAOLCA is the most likely diagnosis. AAOLCA’s with an interarterial course, as seen in this patient, account for the majority of SCD caused by anomalous coronary arteries, despite making up just 1.3% of all identified cases. Referral for surgical correction is recommended for all patients AAOLCA with an interarterial course, to reduce risk of SCD. Further research is warranted regarding the true prevalence of AAOLCA in the general population, the risk of SCD associated with various AAOLCA’s, and validated screening tools for utilization of coronary CTA or MRA to identify AAOLCA’s prior to major cardiac events.

Abstract 15162: Anomalous Right Coronary Artery From the Left Sinus of Valsalva With an Interarterial Course: Impact on Clinical Outcomes in the Adult Population

Introduction: Anomalous right coronary arteries (RCA) arising from the left sinus of Valsalva with an interarterial course (lAC) may cause sudden cardiac death in children and adolescents. However, the natural history and management of patients in whom this anomaly is first detected during adulthood remains uncertain. Methods: Database review identified patients ≥35 years old with anomalous coronary arteries diagnosed by cardiac catheterization or coronary computed tomographic angiography. The outcomes of patients with anomalous RCA with IAC treated conservatively were compared to patients with anomalous left circumflex (LCX) coronary arteries with retroaortic course (an anomaly not associated with ischemic events). The primary outcome was all-cause mortality. Secondary outcomes included survived cardiac arrest and myocardial infarction. Results: The study population consisted of 185 patients (115 men, 70 women) aged 59 ± 12. Clinical characteristics were similar in the RCA (n=88) and LCX (n=97) groups. At follow-up of 6.6 ± 4.4 years there were no statistically significant differences between the 2 groups in mortality (p=0.11) or in secondary outcomes (P=0.89). Conclusions: The outcome of adult patients who had an anomalous RCA with lAC were similar to patients who had anomalous LCX with a known benign course. These results suggest that most patients who survive this anomaly into adulthood may be managed conservatively without intervention.

Abstract 12499: Anomalous Coronary Arteries Are Associated With Ischemia in the Ischemia Trial

Introduction: Coronary anomalies are detected in 0.3-2.3% of patients undergoing coronary computed tomography angiography (CCTA). Data are scarce on atherosclerosis severity in anomalous arteries and relationship to ischemia on stress testing. Hypothesis: High risk features of anomalous coronary arteries are associated with ischemia, even in the absence of coronary stenosis on CCTA. Methods: 5,903 participants enrolled in the ISCHEMIA trial program with site-determined moderate or severe ischemia underwent CCTA. A core laboratory assessed atherosclerosis severity and made note of anomalous coronary origin. A single reader at the CCTA core laboratory re-evaluated all identified anomalies to assess high risk features and origin, blinded to stress test results. Patients were excluded if stress test results were unavailable. We assessed the relationship between type of anomaly and vessel with ischemia in the subtended territory on stress imaging, and stenosis severity. Results: There was anomalous origin of a coronary artery in 56 of 5,903 CCTA cases (0.9%). Age, sex and CAD risk factors were similar between patients with vs. without anomalies. Anomalous origin affected the left circumflex (LCX) in 28, right coronary artery (RCA) in 21, left main (LM) in 4, and left anterior descending (LAD) in 3. Ischemia was severe on stress testing in 58.2%, moderate in 34.5%, mild in 3.6% and absent in 3.6%. High risk anomalies were present in 17/56 (30.3%, 16/21 RCA and 1/4 LM), including inter-arterial course in 28.6%, intra-mural course in 1.8%, and slit-like orifice in 12.5%. Most anomalous coronaries (82.9%) were associated with ischemia in the subtended territory. Among anomalies associated with ischemia, 37.9% had &lt;50% stenosis and 62.1% had &lt;70% stenosis (Figure). Of those with &lt;70% stenosis, 27.6% had high risk features. Conclusions: Anomalous coronary arteries in stable patients are associated with ischemia, even in the absence of severe atherosclerotic disease.

Abstract 13039: A Rare Case of Anomalous Aortic Origin of Right Coronary Artery From Left Coronary Cusp Presenting With Recurrent Atypical Chest Pain

Background: The incidence of anomalous aortic origin of the right coronary artery (AAORCA) is between 0.026% and 0.25%. There is limited data regarding medical versus surgical management. We present a case of AAORCA which did not “qualify” for surgical intervention but remained symptomatic on medical management. Presentation: A 58-year-old female with paroxysmal atrial fibrillation presented for the eighth time in the past two years with recurrent atypical chest pain and lightheadedness. Vital signs and cardiac biomarkers were normal. Work-up: EKG showed normal sinus rhythm. TTE revealed EF 68%. Myocardial perfusion imaging was normal but exercise stress test showed ventricular ectopy, ventricular bigeminy, short bursts of ventricular tachycardia (VT) at peak exercise, and a 7-beat run of VT. A 30-day event monitor revealed a 17-beat run of VT at a rate of 173 bpm (Figure A). Management: Cardiac catheterization showed the dominant right coronary artery (RCA) arising from the left coronary cusp with a shared ostium to the left coronary system (Figure B-D). A coronary CT scan confirmed dominant RCA arising from left coronary cusp (Figure E) with an interarterial course (Figure F) and slit-like appearance of the proximal RCA, with a likely intramural course (Figure G).She was managed conservatively with restriction of vigorous exercise, up-titration of beta-blockers, addition of anti-anginal agents and implantation of loop recorder. Conclusion: The most updated consensus statement for AAORCA recommends surgical intervention for those with signs or symptoms of myocardial ischemia (true angina, findings on provocative testing, aborted sudden cardiac arrest or non-vagally-mediated arrhythmia).Although our patient did not “qualify” for surgical intervention, she continued to experience refractory symptoms. Hence, it is of utmost importance to consider surgical intervention in patients who have failed medical management, for improved quality of life.

Coronary Stent Placement in an Interarterial Anomalous Right Coronary Artery: An Alternative Approach in a Multimorbid High-risk Surgical Patient.

Anomalous aortic origin of a coronary artery is a rare congenital anomaly and potential aetiology for sudden cardiac death. However, the mere presence of this anomaly does not portend clinical significance, and there are many factors that contribute to limiting coronary blood flow in these patients. The standard of care for symptomatic individuals is surgical management with coronary unroofing although not all cases are amenable to surgery. We report the case of an anomalous right coronary artery with interarterial course managed by percutaneous coronary intervention due to surgical contraindication secondary to comorbidities. The proposed mechanism of action culminating in aborted sudden cardiac death is unique and involves aggravated pulmonary hypertension in an individual with severe comorbid pulmonary disease.

A novel method to identify an intramural segment in interarterial anomalous coronary arteries on CT-angiography

Abstract Background/Introduction An anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) with an interarterial course can be assessed using Computed Tomography Angiography (CTA) for the presence of high-risk characteristics associated with sudden cardiac death. These features include a slit-like ostium, acute angle take-off, and degree of proximal luminal narrowing. However, no robust CTA criteria currently exist to determine the presence of an intramural segment. Purpose The aim of this study is to deduct a method to accurately identify an intramural course of interarterial ACAOS on CTA imaging. Methods All consecutive adult patients with an interarterial ACAOS that were evaluated at the two academic hospitals between January 2010 and July 2019 were screened for inclusion. Inclusion criteria were availability of a preoperative CTA-scan (0.5–1mm slice-thickness) and peroperative confirmation of the intramural segment. Using multiplanar reconstruction of the CTA, the distance between the lumen of the aorta and the lumen of the ACAOS (defined as “interluminal space” (ILS)) was assessed at 2mm intervals along the intramural segment (Figure 1). Results Twenty-five patients (64% female, mean age 46 years, 88% right ACAOS) were included. Analysis showed a mean ILS of 0.69mm±0.15mm at 2mm from the ostium. At the end of the intramural segment where the ACAOS becomes non-intramural, the mean ILS was significantly larger (1.27±0.29mm, p&amp;lt;0.001) (Figure 2). Interobserver agreement evaluation showed good reproducibility of ILS (intraclass correlation coefficient 0.77, p&amp;lt;0.001). ROC-analysis demonstrated that at a cut-off ILS of ≤0.95mm, an intramural segment can be diagnosed with 100% sensitivity and 84% specificity. Conclusion(s) The ILS is introduced as novel and robust CTA parameter to identify an intramural course of interarterial ACAOS. An ILS of ≤0.95mm is indicative of an intramural segment with 100% sensitivity and 84% specificity. Funding Acknowledgement Type of funding sources: None.

Detection of Coronary Artery and Aortic Arch Anomalies in Patients with Tetralogy of Fallot Using CT Angiography.

Background: Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD). Furthermore, the prevalence of anomalous origin of a coronary artery is higher in patients with TOF than in the general population (6% vs. ≤1%). Preoperative assessment of cardiovascular anatomy using computed tomography (CT) angiography enables the adaptation of the surgical approach to avoid potentially overlooked anomalies. Our purpose was to determine the prevalence of coronary artery and aortic arch anomalies in a cohort of TOF patients. Methods: In this retrospective analysis, data were collected from CT reports (2015–2021) of 105 TOF patients. All images were acquired using a 64-slice multi-detector CT (MDCT) scanner. Results: The median age of the patients was 38.7 months, with a male-to-female ratio of 1.39. The overall prevalence of coronary artery anomalies (CAAs) was 7.61% (8 of 105 cases). The anomalous origin and course of coronary arteries across the right ventricular outflow tract (RVOT; prepulmonic course) were defined in 5.71% of cases (six patients). In four of these, the left anterior descending artery (LAD) originated from the right coronary artery (RCA), while in two cases, the RCA arose from the LAD. In the remaining two patients, the coronary arteries followed an interarterial course. The most frequent anomalous aortic arch pattern in the overall TOF population was the right aortic arch (RAA) with mirror image branching, seen in 20% of patients (21 cases). The most frequent anomaly of the supra-aortic trunks was bovine configuration, found in 17.14% (18 cases). Conclusions: The prevalence of CAAs and aortic arch anomalies detected by CT angiography was in line with the data reported in anatomical specimens. Therefore, this technique represents a powerful tool for the evaluation of congenital cardiovascular anomalies.

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.

449 A Novel Method To Identify An Intramural Segment In Interarterial Anomalous Coronary Arteries On Ct-angiography

Introduction: An anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) with an interarterial course can be assessed using Computed Tomography Angiography (CTA) for the presence of high-risk characteristics associated with sudden cardiac death. These features include a slit-like ostium, acute angle take-off, and degree of proximal luminal narrowing. However, no robust CTA criteria currently exist to determine the presence of an intramural segment. The aim of this study is to deduct a method to accurately identify an intramural course of interarterial ACAOS on CTA imaging.

A comprehensive analysis of the intramural segment in interarterial anomalous coronary arteries using computed tomography angiography.

AimsAn anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) with an interarterial course can be assessed using computed tomography angiography (CTA) for the presence of high-risk characteristics associated with sudden cardiac death. These features include a slit-like ostium, acute angle take-off, proximal luminal narrowing, and an intramural segment. To date, no robust CTA criteria exist to determine the presence of an intramural segment. We aimed to deduct new CTA parameters to distinguish an intramural course of interarterial ACAOS.Methods and resultsTwenty-five patients with an interarterial ACAOS (64% female, mean age 46 years, 88% right ACAOS) from two academic hospitals were evaluated. Inclusion criteria were the availability of a preoperative CTA scan (0.51 mm slice thickness) and peroperative confirmation of the intramural segment. Using multiplanar reconstruction of the CTA, the distance between the lumen of the aorta and the lumen of the ACAOS [defined as ‘interluminal space’ (ILS)] was assessed at 2 mm intervals along the intramural segment. Analysis showed a mean ILS of 0.69 ± 0.15 mm at 2 mm from the ostium. At the end of the intramural segment where the ACAOS becomes non-intramural, the mean ILS was significantly larger (1.27 ± 0.29 mm, P < 0.001). Interobserver agreement evaluation showed good reproducibility (intraclass correlation coefficient 0.77, P < 0.001). Receiver operator characteristic analysis demonstrated that at a cut-off ILS of <0.95 mm, an intramural segment can be diagnosed with 100% sensitivity and 84% specificity.ConclusionThe ILS is introduced as a novel and robust CTA parameter to identify an intramural course of interarterial ACAOS. An ILS of <0.95 mm is indicative of an intramural segment.

Anomalous Connection of the Coronary Artery Causing ST Segment Elevation Myocardial Infarction: A Case Report

Background: With an angiographic prevalence approaching 1%, Anomalous connections of the Coronary arteries (ANOCOR) are rare. The term connection is preferred over origin since the coronary arteries do not grow out of the aorta but grow into the aorta from the peri truncal ring of coronary arterial vasculature. Very often these anatomical shapes are incidental findings without clinical impact; however, in some specific cases, clinical symptoms may occur arriving to sudden cardiac death (SCD), this is particularly true when there is a an interarterial course between the ascending aorta and the pulmonary.&#x0D; Case Presentation: This case is about patient that we receive in emergency department presenting with an inferior ST segment elevation myocardial infarction (STEMI) which was found to be caused by compression of the proximal right coronary artery (RCA) between the aorta and the pulmonary trunk. Surgery was indicated by the heart team for the treatment of this condition.&#x0D; Conclusions: This case presents a severe manifestation of proximal anomalous connection of the right coronary artery with a “malignant” course between the aorta and the pulmonary artery. This variant has been called malignant because of its association with sudden death especially in young asymptomatic athletes. In current guidelines for symptomatic ANOCOR with interarterial course the treatment recommended is surgery.

Multimodality Imaging Assessment of Anomalous Aortic Origin of the Left Main Coronary Artery Presenting With Syncope and Non-ST Elevation Myocardial Infarction.

Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital abnormality associated with myocardial ischemia and sudden cardiac death. We present a case of a 20 year old previously healthy male presenting with exertional syncope and non-ST elevation myocardial infarction. Coronary computed tomography angiography showed an anomalous left main coronary artery arising from the right coronary cusp with a slit-like appearance, acute angle origin, intramural course, and a subsequent inter-arterial course between the main pulmonary artery and the proximal aorta. Cardiac magnetic resonance imaging demonstrated myocardial infarction in the distribution of the left main coronary artery. The patient underwent successful surgical correction with unroofing of the left main coronary artery. He has had no syncopal episodes or recurrence of chest pain and returned to full duty status in the United States Marine Corps. This case report demonstrates the evaluation and management of a patient with AAOCA.

Diagnosis and treatment of anomalous aortic origin of coronary artery: A twenty-year retrospective study of experience and decision-making in children and young adults

Anomalous aortic origin of a coronary artery, particularly in the presence of inter-arterial course between the great arteries, has been found to be associated with sudden death in young people. This study reports a single-center experience in the management of anomalous aortic origin of a coronary artery, with or without inter-arterial course, by focusing specifically on presentation, diagnosis, and patient outcome. From March 1993 to February 2018, 100 patients (70 males) were diagnosed with anomalous aortic origin of a coronary artery, including 27 left coronary artery from the right sinus, 60 right coronary artery from the left sinus, and 13 other anomalies. Patients with inter-arterial course between the great arteries presented more frequently with syncope and/or sudden death compared to patients without (23.4% vs. 0%, P = 0.026). Diagnosis was reached using first-line transthoracic echocardiography in 65% of cases. Surgical repair was performed in 61 patients (61%). All operated patients (60 direct implantations, 1 unroofing) had been diagnosed with inter-arterial course between the great arteries, and were asymptomatic at a mean (±SD) postoperative follow-up of 4.9 ± 5.3years. An inter-arterial course of the anomalous right or left coronary arteries arising from the opposite sinus is associated with life-threatening events. Direct reimplantation of coronary artery is reliable, and should be discussed even in asymptomatic patients.