Acute Angle Takeoff Research Articles

Rare Presentation of Myocardial Ischemia in a Patient with an Anomalous Right Coronary Artery

BACKGROUND: Coronary anomalies are coronary patterns that occur rarely in the general population. The most common type of these anomalies is the emergence of the right coronary artery (RCA) from the left coronary sinus, which in most patients is either asymptomatic or leads to sudden cardiac death (SCD). THE CASE: This case report presents the case of a 35-year-old male patient who had an inferior ST elevation myocardial infarction (STEMI) for which percutaneous coronary intervention was attempted and failed due to the patient going into cardiac arrest. The patient was subsequently managed medically in the cardiac intensive care unit and followed up outpatient with a cardiologist. The patient experienced symptoms of exertional angina and had a CT angiography of the heart and coronary vessels that identified an anomalous RCA emerging from the left coronary sinus. He was diagnosed with an anomalous RCA with malignant take-off from the left coronary sinus, and subsequent management was surgical with a coronary artery bypass graft (CABG) x1 using the right internal mammary artery (RIMA to RCA). Intraoperatively, the patient was found to have sclerosis of the RCA proximally for about 5cm. The patient did well post-operatively and had no symptoms of exertional angina upon outpatient follow up with both his cardiologist and cardiothoracic surgeon. CONCLUSION: Most cases of coronary anomalies have been found to be either completely asymptomatic or lead to SCD, especially in young patients, however very few cases in literature identified patients with this anomaly presenting with STEMI such as in this case. Another significant finding in this patient is sclerosis of the RCA, where although some articles propose that anomalous coronary arteries may be more susceptible to atherosclerosis, others conclude that there may be no association; concluding that factors such as intramural course, slit-like origin, and acute angle take-off might be more significantly associated. Moreover, patients with coronary anomalies rarely experience cardiac events solely due to their anomaly, such as with this patient. Finally, this patient’s significantly young age at 35 years old is significant given the mean age of patients undergoing CABG being 60.8 years in one cross-sectional study. Current management of symptomatic patients with these anomalies is under debate, with many patients undergoing CABG such as in this case, although one study shows no significant difference in mortality or 10-year survival with surgical intervention despite having higher surgical intervention rates. Percutaneous coronary intervention (PCI) of patients with coronary anomalies has been shown to be difficult or unsuccessful in most studies, although a few cases in literature were successful with tools such as multidetector CT (MDCT). Awareness of these anomalies and their possible presentation, risk factors, and radiologic findings is important especially given their potential for SCD. More research is needed to further elucidate the best treatment options and perhaps even improve the potential for non-invasive interventional techniques such as PCI.

Neo-aortic dimensions and coronary take-off angle in adolescent and young adult patients with transposition of the great arteries after the arterial switch operation

Abstract Objective Aim of this study was (1) to assess the relation between neo-aortic dimensions and coronary angle take-off in adult patients with transposition of the great arteries (TGA) late after the arterial switch operation (ASO) and (2) to evaluate if temporal increase in the neo-aortic dimensions is associated with a decrease of the coronary angle take off. Methods In this retrospective two-center cohort study, all patients with TGA who underwent ASO and had ≥1 CT-angiography (CTA) of the coronary arteries at the age of ≥ 16 years old were included. Neo-aortic dimensions and coronary angle take-off were measured with CTA, Figure 1. Results Eighty-one patients, 69% male and median age 21.0 (18.5-22.8) years, were included. Maximum neo-aortic diameter was 39.2±5.3mm (corrected for BSA 20.9±2.7mm/m2). Sixty-one (76%) patients had a coronary take-off angle of <45° at baseline. No correlation between the maximum neo-aortic diameter and coronary take-off angle of the right coronary artery (RCA) (R=0.161, p=0.150) and left coronary artery (LCA) (R=0.089, p=0.428) was found, Figure 2A-B. During a mean follow up of 7.9±3.1 years in patients with 2 CTAs (n=26), the average increase of maximum neo-aortic diameter at the level of sinus of Valsalva was 0.16±0.20mm/year and a poor correlation between the change in maximum aortic diameter and change in coronary take-off angle of the RCA (R=0.126, p=0.541) and LCA (R=0.311, p=0.158) was observed, Figure 2C-D. Conclusions This study reports slightly increased neo-aortic dimensions and high prevalence of acute coronary angle take-off in young adults late after ASO. The average neo-aortic growth rate of 0.16 mm/year at the level of the sinus of Valsalva is twice as fast as reported for healthy adults. However, no significant correlation was found between the neo-aortic dimensions and coronary take-off angle at baseline or during follow-up of almost 8 years in this group.Figure 1Figure 2

Unroofing of an anomalous right coronary artery originating from the left coronary sinus

Coronary abnormalities, including the anomalous aortic origin of a coronary artery, coronary fistula and myocardial bridge, are among the most common congenital cardiovascular anomalies. A left coronary artery arising from the right cusp is less common than the right coronary artery arising from the left cusp but is more often found in autopsy series of sudden cardiac deaths. A slit-like/fish-mouth-shaped orifice, acute angle take-off, intramural course, interarterial course and hypoplasia of the proximal coronary artery have all been proposed as reasons for symptoms, ischaemia and sudden cardiac death. Surgical intervention is recommended for those patients with signs or symptoms of myocardial ischaemia. Intervention in asymptomatic patients with an interarterial/intramural left coronary artery from the right sinus of Valsalva is recommended due to the higher calculated risk of sudden cardiac death. In asymptomatic patients with an intramural right coronary artery from the left sinus of Valsalva, provocative testing is recommended. The slit-like orifice is more commonly seen in an anomalous right coronary artery arising from the left sinus, and we believe it is the major factor responsible for myocardial ischaemia. Our unroofing technique focuses on: (i) transecting the endothelial tissue flap to create a neo-ostium; (ii) limiting the flap resection to the intramural portion to avoid extra-aortic incision; and (iii) marsupialization of a neo-ostium with interrupted sutures to reapproximate the endothelium and prevent aortic dissection.

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.

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.

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.

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.

Abstract 17085: An Anomalous Outcome Of Renal Transplant

Case Presentation: A 65 year old man with diabetic renal disease developed acute dyspnea at rest on the first post-operative day after an uncomplicated deceased donor renal transplant. He was hypertensive, tachycardic, and hypoxemic. His electrocardiogram showed anterior ST elevations with reciprocal inferior ST depressions and his chest x-ray revealed pulmonary edema. He was given anti-platelet therapy, nitroglycerin, beta blocker and taken emergently for angiography. He had a thrombotic occlusion of a co-dominant anomalous left circumflex artery arising from the ostium of a moderate caliber right coronary artery. He underwent successful percutaneous coronary intervention. His post-revascularization echocardiogram showed a hypokinetic to akinetic inferior wall with preserved global left ventricular systolic function. Coronary CT for further assessment of the course of the anomalous artery was not pursued given minimal benefit and the risk of worsening graft function. He was discharged on dual anti-platelet therapy, beta blocker and a statin. Discussion: There is no consensus about risk stratification before renal transplant. Our patient’s pre-operative exercise treadmill test (ETT) showed reduced functional capacity at 5.6 metabolic equivalents, failure to reach target heart rate, with 1.0 mm down sloping ST depression in infero-lateral leads with no ischemic symptoms. While our patient had an equivocal ETT, his lack of angina argued against further work-up. Post-operatively, a pro-inflammatory state, increased shear stress, acquired thrombophilia and immunosuppressive medications can all contribute to plaque rupture with thrombus formation. Atherosclerosis due to diabetes and renal disease coupled with mechanical factors such as passive compression at the intraarterial course, acute angle take-off or valve-like closure of the slit orifice may have subjected our patient with an anomalous left circumflex artery to an acute ST-elevation MI.

High take-off of coronary arteries contributing to lethal myocardial ischemia: report of two cases

Abstract Introduction/Objective High take-off coronary arteries (HTO) are defined by coronary ostia arising above the sinotubular junction (STJ). Although asymptomatic in most individuals, there is increasing evidence that HTO is a risk factor for sudden cardiac death. Here we present two patients where HTO contributed to death. Methods/Case Report Patient A was a 52 year old morbidly obese male with atypical chest pain, new inferior ST elevations and troponinemia. Multiple coronary angiographies did not reveal any stenosis, but 3 stents were placed in the RCA due to concern for vasospasm. 3 days later the patient died. Autopsy revealed cardiac tamponade and non- atherosclerotic ischemic heart disease with remote apical LV scar and diffuse patchy interstitial fibrosis in the myocardium, which could be attributed to HTO of the RCA 0.5 cm above the STJ and early intramuscular courses of both coronaries. Multiple angiographies likely caused iatrogenic coronary injury and subsequent tamponade. Patient B was a healthy 33 year old female at 34 weeks gestation, who developed anaphylaxis during IV iron infusion for severe iron deficiency anemia. She was transferred to the OR for emergent C-section. Minutes after delivery she died. Autopsy revealed HTO, with coronary ostia being 0.4 cm and 0.7 cm above the STJ, respectively and acute angle take-off of LCA. No atherosclerosis was noted. The inability to increase myocardial perfusion through the coronary arteries during a high stress situation due to pregnancy, iron deficiency anemia and anaphylaxis likely contributed to lethal myocardial ischemia. Results (if a Case Study enter NA) NA Conclusion HTO and other coronary artery anomalies (CAAs) should be considered in cases of cryptogenic acute and chronic myocardial ischemia. Hemodynamic characterization of HTO, including those &amp;lt; 1 cm above the STJ in presence and absence of other CAAs may help better understand their pathophysiologic significance. Antemortem diagnosis requires high clinical suspicion and appropriate surgical intervention could be life-saving.

Assessment of the intramural segment of interarterial anomalous coronary arteries originating from the opposite sinus of Valsalva on CT angiography

Abstract Background Of the coronary anomaly variants, an anomalous coronary artery originating from the opposite sinus (AAOCA) with an interarterial course poses the highest the risk of sudden cardiac death. Assessment of high risk anatomical characteristics can be done with Computed Tomography Angiography (CTA). High risk features are a slit-like ostium, acute angle take-off, proximal narrowing and an intramural course. For an intramural course no clear-cut CT parameters exist. Purpose To deduct new CTA criteria to identify an intramural course as well as the length of the intramural segment based on peroperative findings. Material and methods Twenty patients were included that received unroofing surgery of the right or left AAOCA between 2010 and 2019. All patients had a pre-operative CTA (0.5–1mm slice-thickness) performed. The presence of the intramural segment was measured peroperatively by the surgeon and used as indicator for CTA evaluation. Using multiplanar reconstructions, CTA images were rotated perpendicular to the horizontal plane of the aortic valve annulus and AAOCA to assess the distance between the aortic and AAOCA lumen and the shape of the AAOCA. This was done at every 2mm for the length of the intramural course as described by the surgeon (Figure 1). Results Analysis of 20 patients (40% male, AAORCA n=17, age at diagnosis AAOCA 45.6±10.5 years), showed a mean intramural length of 11.5±2.4mm at surgery. The median distance between the aortic and AAOCA lumen was 0.76mm (IQR 0.72–0.97mm) for the intramural segment. At the distal end of the intramural part (indicated by no. 5 in Figure 1), the mean distance was 1.20mm±0.27mm. The median ratio between the antero-posterior and transverse diameter of the AAOCA lumen at the distal end of the intramural part was 0.94 (IQR 0.88–0.99). Along the intramural part (Figure 1, no. 1–4) this ratio was 0.56mm±0.11mm, indicating a more flattened ostial shape along the course of the vessel. Conclusions Results indicate that an aortic to AAOCA lumen distance of ≤0.76mm on CTA is suggestive of an intramural course. A distance between the aorta and AAOCA of ≥1.2mm combined with an antero-posterior to transverse diameter ratio of 0.94 of the AAOCA indicates that the intramural trajectory has ended. Funding Acknowledgement Type of funding sources: None.

Computed Tomography Derived Coronary Triangulated Orifice Area-Deduction of a New Parameter for Follow-up After Surgical Correction of Anomalous Aortic Origin of Coronary Arteries and Call for Validation.

Introduction: Anomalous aortic origin of a coronary artery (AAOCA) from the opposite sinus of Valsalva is a rare congenital abnormality. Computed tomography angiography (CTA) is primarily used as a diagnostic tool to evaluate the anatomy and identify potentially malignant AAOCA variants. Limited data is available on the role of CTA during postoperative follow-up. We aimed to develop an objective CTA derived parameter for diagnostic evaluation and follow-up after surgical correction of AAOCA and correlate the anatomical features to the postoperative outcome.Methods: All consecutive patients who underwent surgical repair of AAOCA from 2001 to 2018 and had pre and postoperative CTA imaging available were included. A retrospective analysis of the pre- and postoperative CTA and the outcomes was performed. The origin and course of the anomalous coronary artery and the ostial dimensions were evaluated and correlated with restenosis of operated coronary artery. To allow an accurate evaluation of the effective orifice area at diagnosis and after surgical repair we deduce and propose a new parameter—the coronary triangulated orifice area (CTOA).Results: Out of the 54 patients who underwent surgical treatment for AAOCA, 11 fulfilled the inclusion criteria. The median follow-up was 19 months [IQR 3;42]. The mean age at surgery was 41 ± 16 years, with six patients (55%) being male. Postoperatively, the angle between the proximal coronary artery and the aortic wall increased from 20 ± 5° to 28 ± 9° (p < 0.01) and ostial diameter in the transversal plane increased from 4.1 ± 2.5 mm to 6.2 ± 2.7 mm (p < 0.01). The median CTOA increased significantly from 1.6 mm2 [IQR 0.9;4.9] to 5.5 mm2 [IQR 3;11.8] (p < 0.005). During follow-up, in three patients a restenosis of the operated coronary artery was suspected. In these patients, the CTOA only showed a limited postoperative increase of ≤ 1.4 mm2.Conclusions: CTA can play an important role in the evaluation of the pre- and postoperative anatomy in AAOCA patients. CTOA may be of use in conjunction with the acute angle take-off and ostial diameter order to comprehensively evaluate the operated ostium after unroofing or patch angioplasty.

Symptomatic Anomalous Right Coronary Artery from the Opposite Sinus of Valsalva with a Malignant Course in a Filipino Adult Patient: A Case Report

Anomalous coronary artery from the opposite sinus of Valsalva (ACAOS) is a rare congenital condition with reported incidence of 1.07%. It is classically associated with risk for sudden death (SCD) based on autopsy reports. Several anatomic features of this anomaly have been correlated with its symptomatology and risk of SCD, and surgical revascularization has been suggested by guidelines. However, based on recent reviews, SCD risk in ACAOS patients may be overestimated and that these patients may be managed conservatively. We present a case of a 53-year-old athletic Filipino male who consulted for sudden chest heaviness after a basketball game. He was initially managed as a case of acute coronary syndrome. However, subsequent coronary angiography revealed an anomalous right coronary artery arising from the left coronary sinus. Coronary computed tomography angiography showed an acute angle take-off of the right coronary artery with an interarterial course between the pulmonary artery and the aorta. The patient was managed conservatively with good outcomes. The patient’s management and a review of literature on ACAOS are reported in this paper. KEYWORDS: ACAOS, coronary artery anomaly, sudden cardiac death.

Sudden Death Caused by Anomalous Origin of the Coronary Artery During Exercise.

Anomalous origin of the coronary artery (AOCA) is a rare, but important cause of sudden cardiac death among young athletes. Nine autopsy cases (8 male, 1 female; mean age, 17.9 years; age range, 11-31 years) of sudden death during or just after exercise caused by AOCA were reviewed. The exercises performed at the time of death were running (4 cases), soccer (2 cases), and baseball, swimming and kendo (Japanese swordsmanship) (1 case each). In 6 cases, the left coronary artery arose from the right sinus of Valsalva, and in 3, the right coronary artery from the left sinus. The coronary arteries passed between the pulmonary artery and the aorta with an acute angle takeoff from the orifice. Three cases had cardiovascular manifestations prior to death. In cases with cardiovascular manifestations, novel imaging methods should be considered to prevent sudden death.

Anomalous Aortic Origin of Coronary Arteries in the Young: Echocardiographic Evaluation With Surgical Correlation

ObjectivesThis study sought to compare findings from institutional echocardiographic reports with imaging core laboratory (ICL) review of corresponding echocardiographic images and operative reports in 159 patients with anomalous aortic origin of a coronary artery (AAOCA). The study also sought to develop a “best practice” protocol for imaging and interpreting images in establishing the diagnosis of AAOCA. BackgroundAAOCA is associated with sudden death in the young. Underlying anatomic risk factors that can cause ischemia-related events include coronary arterial ostial stenosis, intramural course of the proximal coronary within the aortic wall, interarterial course, and potential compression between the great arteries. Consistent protocols for diagnosing and evaluating these features are lacking, potentially precluding the ability to risk stratify patients based on evidence and plan surgical strategy. MethodsFor a prescribed set of anatomic AAOCA features, percentages of missing data in institutional echocardiographic reports were calculated. For each feature, agreement among institutional echocardiographic reports, ICL review of images, and surgical reports was evaluated using the weighted kappa statistic. An echocardiographic imaging protocol was developed heuristically to reduce differences between institutional reports and ICL review. ResultsA total of 13%, 33%, and 62% of echocardiograms were missing images enabling diagnosis of intra-arterial course, proximal intramural course, and high ostial takeoff, respectively. There was poor agreement between institutional reports and ICL review for diagnosis of origin of coronary artery, interarterial course, intramural course, and acute angle takeoff (kappa = 0.74, 0.11, –0.03, 0.13, respectively). Surgical findings were also significantly different from those of reports, and to a lesser extent ICL reviews. The resulting protocol contains technical recommendations for imaging each of these features. ConclusionsPoor agreement between institutional reports and ICL review for AAOCA suggests need for an imaging protocol to permit evidence-based risk stratification and surgical planning. Even then, delineation of echocardiographic details in AAOCA will remain imperfect.

Anomalous origin of coronary artery: taxonomy and clinical implication.

ObjectiveAnomalous origin of coronary artery is uncommon. The taxonomies of anomalous origin of coronary artery are inconsistent and complex. Conceptual and therapeutic debates remain. The aim of the present study is to reappraise the concept of anomalous origin of coronary artery and to discuss the potential hazards and treatment rationale of this anomaly on basis of literature review.MethodsA comprehensive literature review was made in terms of the taxonomies including “simple”, “multiple” and “complex” types of anomalous origin of coronary artery.ResultsAnomalous origin of coronary artery can be simply categorized according to the ectopically originated coronary artery. There are a couple of complex anatomical variants: “multiple” type, involving more than one coronary artery or branch, which can be subdivided into 2 subtypes, A) more than one coronary arteries or branches arising from one place; and B) two coronary arteries/branches arising from separate ectopic sites; and “complex” type, associated with acquired heart disease, or congenital heart defects.ConclusionSudden cardiac death in anomalous origin of coronary artery is associated with the anatomical features including abnormal coursing, acute angle take-off and ostial abnormalities. Atherosclerosis is prone to be in the right-sided ectopic and retroaortic coursing coronary artery. Surgical treatment is a definitive therapy. Simple coronary artery bypass grafting is not recommended due to the potential hazards of coronary steal phenomenon and poor patency of mammary arterial grafts, and modified maneuvers such as coronary ostial reimplantation, impinged coronary segment unroofing and coronary stent deployment are advocated instead.

Exercise-Induced Myocardial Ischemia in a Case of Anomalous Origin of the Left Main Coronary Artery from the Noncoronary Sinus of Valsalva

We report a case of anomalous origin of the left main coronary artery (LCA) from the noncoronary sinus of valsalva (LCANCS) in a young healthy patient who presented with syncope and cardiopulmonary arrest during exercise. The enhanced computed tomography showed acute angle take-off (AAT) of LCA, and the exercise stress thallium-201 myocardial scintigraphy demonstrated a large defect at the LCA perfusion region. We propose that the coexistence of AAT and resulting ischemia causes sudden cardiac death during exercise in the patients with LCANCS.

Ectopic Origin of Coronary Arteries from the Aorta in Syrian Hamsters (Mesocricetus auratus)

An ectopic origin of the coronary artery from the aorta beyond the sinotubular junction, a condition commonly referred to as 'coronary artery high take-off', has been described in man and C57BL/6 mice. The present paper reports this congenital coronary artery anomaly in the Syrian hamster (Mesocricetus auratus). Hearts from 14 individuals, aged 53-350 days, were examined by means of a corrosion-cast technique, scanning electron microscopy or histological and immunohistochemical techniques. In 11 hamsters, the right coronary artery was the ectopic vessel. In the other three animals there was a solitary coronary ostium in the aorta. In all cases, the ectopic coronary artery originated at an acute angle and a valve-like ridge was in front of the coronary artery ostium. The ectopic arteries examined microscopically showed an intramural trajectory within the aortic wall. In the hearts with a solitary ostium in the aorta, the left main coronary artery coursed between the aorta and the pulmonary artery. In man, all of these anomalous conditions place the individual at risk of myocardial ischaemia and sudden death. However, none of the affected hamsters had clinical signs of disease. Intimal thickenings of increasing size with age were present in the intramural coronary artery segment of eight hamsters aged 106 days or older, examined histologically. The present findings fit with the notion that coronary arteries with acute angle take-off and an intramural course are subjected to unusual wear and tear, leading to tissue changes in the vessel wall.

Anomalous origin of the right coronary artery: Depiction at whole-heart coronary magnetic resonance angiography

Anomalous origin of the right coronary artery (RCA) is a rare congenital anomaly, which may cause myocardial ischemia and sudden death. Whole-heart coronary magnetic resonance angiography (MRA) was performed in a 61-year-old woman who had exercise-induced myocardial ischemia. MRA revealed that the RCA originated from the left sinus of Valsalva, separately from the left main coronary artery and it coursed between the ascending aorta and pulmonary artery. There was acute angle take-off of the RCA from the aorta. This is the first report describing whole-heart coronary MRA findings of the anomalous origin of the RCA.

Multislice computed tomographic findings of the anomalous origins of the right coronary artery: evaluation of possible causes of myocardial ischemia

Anomalous right coronary arteries (RCA) arising from the left sinus of Valsalva may cause myocardial ischemia. We evaluated morphological features of anomalous RCA by using multislice computed tomography (MSCT) in relation to myocardial ischemia provoked by myocardial perfusion single-photon emission computed tomography. MSCT was performed in a total of 3, 212 patients by using an Aquillion 16 and a Light Speed Ultra. Retrospective ECG-gated image reconstruction was performed. Volume rendering, axial and curved multiplanar reformatted images were analyzed for the determination of the origin and course of the RCA, the take-off angle of the RCA from the aorta, and size of the RCA orifice. Furthermore, virtual angioscopic images were also used for the evaluation of the RCA orifice structure. Anomalous origins of the RCA were found in 15 patients. In 13 patients, the RCA arose from the left sinus of Valsalva, and in 2 patients it arose from the left main coronary artery as a single coronary artery. The RCA coursed anteriorly between the ascending aorta and pulmonary artery in 14 patients, whereas it had a retroaortic course in 1 patient. Acute angle take-off (<30 degrees ) of the RCA from the aorta and the left main coronary artery was observed in 8 patients, intramural course of the RCA within the aortic wall was observed in 6 patients and a small RCA orifice was observed in 4 patients. Exercise-induced myocardial ischemia was present in 5 patients. Coursing between the aorta and pulmonary artery, acute angle take-off and intramural course were thought to be major causes of exercise-induced ischemia in patients with anomalous origins of the RCA.