Opposite Sinus Of Valsalva Research Articles

Anomalous origin of Left Main coronary artery from right sinus presenting as non-ST elevation ACS: A case Report

Congenital anomalies of the coronary arteries are a cause of sudden cardiac death. Of the known anatomic variants, anomalous origination of a coronary artery from an opposite sinus of Valsalva (ACAOS) remains a major clinical issue and a challenging condition to treat. Congenital coronary anomalies are likely to be under-recognized, as completing an anatomic assessment in a very large portion of the population would seem unfeasible. However, we present a case report with image of a 54-year-old female presented with acute non-ST elevation ACS for which she underwent diagnostic angiography of the coronary system which revealed a common origin of both right and left main coronary artery from right sinus of Valsa.

The role of coronary artery reimplantation for anomalous right coronary artery originating from the opposite sinus of Valsalva: preliminary outcomes and insights from a Latin American country.

Despite promising results, reimplantation appears to have fallen into oblivion among the multiple possible approaches for repairing anomalous coronary arteries. We describe the outcomes of 12 patients with an anomalous right coronary artery originating from the opposite sinus of Valsalva with an interarterial course who were surgically treated with this technique between 2018 and 2023 in 2 institutions in Bogota, Colombia. We provide preliminary evidence of the value reimplantation as a more than suitable technique, particularly in resource-constrained settings. It offers high rates of control of symptoms and functional class recovery while assessing all potential high-risk features, with a low risk of complications, even in middle-aged patients. We also advocate using noninvasive anatomical descriptions and patient symptoms over inducible ischaemia tests in decision making.

Surgery for Anomalous Origin of The Coronary Artery from The Opposite Sinus of Valsalva

Surgery for Anomalous Origin of The Coronary Artery from The Opposite Sinus of Valsalva

Management of anomalous origin of right coronary artery from left coronary sinus

Aberrant origin of coronary artery is a rare congenital anomaly associated with increased risk of myocardial ischemia and sudden death, with the highest risk lesions being those arising from the opposite sinus of Valsalva. We report a case with an aberrant right coronary artery arising superior to the left coronary cusp, with a slit-like ostium, having an inter-arterial and intramural course through the aortic root, that underwent repositioning of the right coronary artery. We believe such cases warrant surgical correction and reimplantation is a safe, effective and reproducible technique.

Clinical Outcomes in Adult Patients With an Anomalous Right Coronary Artery from the Left Sinus of Valsalva

An anomalous origin of the right coronary artery from the opposite sinus of Valsalva with an intramural course (R-ACAOS-IM) may cause sudden cardiac death in children and adolescents. However, the natural history and management of patients in whom this anomaly is detected later during adulthood remains uncertain. The goals of this study were to assess the impact of an R-ACAOS-IM on the clinical outcomes in an adult population and to determine if adult patients with this anomaly who do not have significant coronary artery disease (CAD) can be managed safely without surgical intervention. A database review identified patients aged >35 years with anomalous coronary arteries diagnosed by cardiac catheterization or coronary computed tomography angiography. The outcomes of patients with R-ACAOS-IM were compared with patients with anomalous left circumflex coronary arteries with retroaortic course (LCx-RA) (an anomaly not associated with ischemic events). The primary outcome was all-cause mortality. The study population consisted of 185 patients aged 59 ± 12 years. Clinical characteristics were similar in the R-ACAOS-IM (n=88) and LCx-RA (n=97) groups. At a follow-up of 6.6 ± 4.5years, there was no difference in mortality (hazard ratio 0.64, 95% confidence interval 0.32 to 1.28, p=0.20) when adjusted for gender, age, and CAD. A subgroup analysis of 88 patients with no obstructive CAD managed nonoperatively found no difference between the LCx and R-ACAOS-IM groups in mortality (hazard ratio 2.45, 95% confidence interval 0.45 to 13.40, p=0.30). There was no significant difference between the 2 groups in the composite outcome of death, nonfatal myocardial infarction, or survived cardiac arrest. The outcome of adult patients who have anomalous R-ACAOS-IM are similar to patients who have anomalous LCx-RA with a known benign course. In conclusion, these results suggest that most patients who survive this anomaly into adulthood may be managed conservatively without intervention.

Stent Angioplasty in Coronary Artery Anomalies With Intramural Course: When, Why, How, With What Results?

Although coronary artery anomalies include multiple disorders, few are likely to require intervention, given that the risk for critical sequelae (ie, sudden cardiac arrest and sudden cardiac death) is generally low. This article addresses which coronary artery anomaly carriers may need intervention and which interventions may be required. The recent introduction of stent angioplasty is discussed in particular, along with general reviews of nomenclature, various anatomical and functional presentations, quantitative diagnosis methods, and indications for surgical versus percutaneous intervention. Novel criteria for defining severe stenosis also are proposed. Optimal risk quantification depends on precise imaging that only intravascular ultrasonography or optical coherence tomography can reliably obtain. Accordingly, the technique of intravascular ultrasonography-monitored stent angioplasty is described in detail. Initial results from our group's study of 100 patients with right or left anomalous origin of a coronary artery from an opposite sinus of Valsalva with intramural course are reported. Future efforts should prospectively evaluate stent angioplasty in multicenter studies based on precise, consistent techniques and follow-up protocols, such as those initiated by our group. Comparisons with surgical results should be part of the program, with the understanding that detailed and complete results from those techniques will require long-term (5- to 10-year) studies.

Repeated Syncope During Exercise as a Result of Anomalous Origin of Left Coronary Artery With Intramural Aortic Course in a Teenage Boy

Anomalous origin of the left coronary artery from the opposite sinus of Valsalva with an intramural aortic course (L-ACAOS-IM) can cause syncope, sometimes as a prodrome of lethal events, including sudden cardiac death, in young athletes. The detailed mechanism of syncope in patients with L-ACAOS-IM is still unclear. This case report describes a 17-year-old boy who presented to the hospital because of syncope following chest pain with increasing frequency during exercise, such as playing soccer and running. In a treadmill exercise test, a decrease in blood pressure was seen (from 99/56 mm Hg to 68/38 mm Hg); chest pain and faintness accompanied by ST-segment elevation in lead aVR and ST-segment depression at other leads on electrocardiography were noted. These findings and symptoms disappeared spontaneously within a few minutes while clinicians prepared for emergency medications. Coronary computed tomography angiography (CCTA) showed that the origin of the left coronary artery (LCA) was the opposite sinus of Valsalva, and the course of the LCA was through the aortic wall toward the left coronary sinus. He was diagnosed with L-ACAOS-IM. After surgical treatment by unroofing the intramural part of the LCA and reconstructing a neo-ostium, he no longer experienced syncope during exercise. This case suggests that low cardiac output caused by myocardial ischemia, not life-threatening arrythmia, is a main mechanism of syncope in patients with L-ACAOS-IM. Consideration should be given to performing CCTA before an exercise stress test for young patients with syncope and chest pain to avoid the risk of severe myocardial ischemia.

Therapeutic Management of Anomalous Coronary Arteries Originating From the Opposite Sinus of Valsalva: Current Evidence, Proposed Approach, and the Unknowing.

Anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS) are a challenge because of their various anatomic and clinical presentation. Although the prevalence is low, the absolute numbers of detected ACAOS are increasing because of the growing use of noninvasive anatomical imaging for ruling out coronary artery disease. As evidence-based guidelines are lacking, treating physicians are left in uncertainty for the optimal management of such patients. The sole presence of ACAOS does not justify surgical correction, and therefore a thorough anatomic and hemodynamic assessment is warranted. Invasive and noninvasive multimodality imaging provides information to the clinical question whether the presence of ACAOS is an innocent coincidental finding, is responsible for the patient's symptoms, or even might be a risk for sudden cardiac death. Based on recent clinical data, focusing on the pathophysiology of patients with ACAOS, myocardial ischemia is dependent on both the extent of fixed and dynamic components, represented by anatomic high-risk features. These varying combinations should be considered individually in the decision making for the different therapeutic options. This state-of-the-art review focuses on the advantages and limitations of the common contemporary surgical, interventional, and medical therapy with regard to the anatomy and pathophysiology of ACAOS. Further, we propose a therapeutic management algorithm based on current evidence on multimodality invasive and noninvasive imaging findings and highlight remaining gaps of knowledge.

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<0.001) (Figure 2). Interobserver agreement evaluation showed good reproducibility of ILS (intraclass correlation coefficient 0.77, p<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.

Percutaneous Intervention With GuideLiner Catheter in 4 Patients With Anomalous Coronary Artery Originating From Opposite Sinus of Valsalva.

Percutaneous intervention in anomalous coronary arteries originating from the opposite sinus of Valsalva is complicated by their unusual location and course, which makes selective cannulation difficult. The GuideLiner (Vascular Solutions, Inc.) is a monorail guide extension catheter designed to advance beyond the tip of a mother guide catheter to enable deep intubation of a coronary artery, provide extra support, and improve coaxial alignment. We describe the cases of 4 patients with an anomalous coronary artery originating from the opposite sinus of Valsalva-including 2 with acute myocardial infarction-who underwent successful percutaneous coronary intervention with use of a GuideLiner catheter.

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.

Angioplasty of Anomalous Coronaries Arising from the Opposite Sinus with an Interarterial Course, is it Safe?

BackgroundThe coronary artery with an interarterial course CAIAC is the most threatening coronary anomaly, especially if it concerns the left coronary. Percutaneous coronary intervention PCI is scarcely described given its low prevalence and lack of long-term outcome data. Therefore, we assessed through this case series the feasibility and safety of PCI in this population.MethodsThis is an observational multicentric study including patients with CAIAC arising from the opposite sinus of Valsalva. The primary endpoints were immediate angiographic success and target lesion revascularization.ResultsDuring the period of the study, we performed 27235 PCI in six Cath labs, 26 procedures concerning abnormal coronaries including 12 with CAIAC. The median age was 57 years extremes: 43–78 with male predominance 1:11. Anomalous coronary artery was Right coronary artery RCA in eight patients, Left main LM in three patients, and left anterior descending LAD in one patient. The stenosis was located in all cases in proximal segments beyond the inter-arterial course proximal LAD, the superior genius of the RCA, or the proximal segment of mid-RCA. Five patients showed slit-like ostium and all have an angle take-off <45° on CT scan. After a median follow-up of 24 months, four subjects presented target lesion revascularization TLR, all were initially treated with either a bare-metal stent or with balloons.ConclusionsPCI of patients with CAIAC is feasible and appears safe. The operator should carefully analyze the angiogram before PCI to choose the appropriate guiding catheter and should be acquainted with the different techniques for improving backup.

Bicuspid aortic valve and anomalous right coronary artery from the opposite coronary sinus

We report on a patient with bicuspid aortic valve and anomalous right coronary artery from the opposite sinus without evidence of intramural course. Different authors support the universal presence of intramural course in patients with origin of the right coronary artery from the opposite sinus of Valsalva in normal heart. The occurrence of both bicuspid aortic valve and the absence of intramural course may not be accidental. This might suggest a developmental interaction between bicuspid aortic valve and anomalous coronary artery. Large observational study including characterisation by intravascular ultrasonography in patients with bicuspid aortic valve and anomalous coronary is needed.

Chronicle of a death foretold. It is time for echocardiographic screening in young athletes

BackgroundThe novel “Chronicle of a death foretold” by Gabriel Garcia Marquez is a story of a sudden death which could have been prevented. In 1976, within the University of Maryland basketball program and only 8 weeks apart, two athletes died suddenly during physical exertion. They were affected by hypertrophic cardiomyopathy and Marfan syndrome and in both cases an echocardiogram would have prevented the tragic epilogue. This coincidence drew everyone's attention and experts' interest on sudden death in sports. Methods and resultsEven in recent Italian history, unexpected deaths continue to affect athletes but surprisingly any real knowledge regarding the numbers and the impact of those tragedies must take medical literature and non-medical press into consideration. Herein we report the clinical case of a 13-year-old patient with a bicuspid aortic valve, whose mother was alarmed by the news of a young boy who died because of an anomalous origin of coronary artery (AOCA) which had not been diagnosed at transthoracic echocardiography (TTE). Her obstinacy induced the physicians to repeat TTE and led to the same diagnosis in her son: actually, his right coronary artery originated from the opposite sinus of Valsalva. The suspicion was confirmed by coronary CT scan and, thanks to appropriate therapy, the boy now fares well. ConclusionsAOCA is the second most common cause of sudden death in young athletes. Although AOCA is often undetectable at ECG, TTE increases sensitivity of preparticipation screening. It could therefore allow us to avoid such coincidences and prevent sudden juvenile death.

Clariant contributes to China's carbon neutrality goal with innovative MegaMax catalyst for CO2-based methanol synthesis

The novel “Chronicle of a death foretold” by Gabriel Garcia Marquez is a story of a sudden death which could have been prevented. In 1976, within the University of Maryland basketball program and only 8 weeks apart, two athletes died suddenly during physical exertion. They were affected by hypertrophic cardiomyopathy and Marfan syndrome and in both cases an echocardiogram would have prevented the tragic epilogue. This coincidence drew everyone's attention and experts' interest on sudden death in sports.Even in recent Italian history, unexpected deaths continue to affect athletes but surprisingly any real knowledge regarding the numbers and the impact of those tragedies must take medical literature and non-medical press into consideration. Herein we report the clinical case of a 13-year-old patient with a bicuspid aortic valve, whose mother was alarmed by the news of a young boy who died because of an anomalous origin of coronary artery (AOCA) which had not been diagnosed at transthoracic echocardiography (TTE). Her obstinacy induced the physicians to repeat TTE and led to the same diagnosis in her son: actually, his right coronary artery originated from the opposite sinus of Valsalva. The suspicion was confirmed by coronary CT scan and, thanks to appropriate therapy, the boy now fares well.AOCA is the second most common cause of sudden death in young athletes. Although AOCA is often undetectable at ECG, TTE increases sensitivity of preparticipation screening. It could therefore allow us to avoid such coincidences and prevent sudden juvenile death.

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.

Primary percutaneous coronary intervention in a patient with anomalous origin of the left coronary artery from the opposite sinus of Valsalva and left main coronary artery occlusion

Introduction. Congenital coronary anomalies are detected in about 5% of all performed coronarographies. Coronary artery (CA) anomalies (CAA), considered to be of great risk, are the ones where the CA arises from the opposite sinus (anomalous origination of CA from opposite sinus, ACA-OS) of Valsalva. These anomalies are detected in about 1% of cases. This report shows a unique case of a patient with anterior wall ST-elevation myocardial infarction (STEMI) caused by left main CA (LMCA) occlusion, which arose from the right coronary cusp and had an interarterial course, successfully treated with primary percutaneous coronary intervention (PCI). Case report. A 46-year-old male patient was admitted to the hospital due to STEMI of the anterior region. On admission, the patient was hypertensive (150/100 mmHg) in sinus rhythm (heart rate 70/min), Killip I. After the initial examination and admitting dual antiplatelet therapy, the patient underwent urgent coronarography. Coronarography was performed using the transradial approach. The right CA had no significant stenosis and was easily cannulated, whereas the left CA could not be cannulated at the usual position. Attempts to cannulate the left CA with multiple catheters of various curves were unsuccessful. The conclusion was that there was a CA anomaly, and the cannulation of the anomalous left CA, which arose from the opposite (right) coronary cusp (anomalous aortic origin of the left CA, AAOLCA), was successfully performed with a Multipurpose catheter. Moreover, the LMCA was occluded in the distal segment. Two drug-eluting stents (DES) were implanted, but the patient developed the no-reflow phenomenon and cardiogenic shock. After the patient was stabilized, computed tomography (CT) coronarography was performed, and AAOLCA with an interarterial course was registered. During the follow-up period, single photon emission computed tomography (SPECT) was per-formed, and in the staged procedure, a stent was implanted into the proximal circumflex artery using the T and protrusion (TAP) technique. Conclusion. Patients with STEMI and the anomalies of CAs are very rare. As such, these patients represent a great challenge for revascularization. Possessing the knowledge of anatomic varieties is paramount when it comes to these patients to treat them adequately with primary PCI.

The first multicentre study on coronary anomalies in the Netherlands: MuSCAT

BackgroundCurrent guidelines on coronary anomalies are primarily based on expert consensus and a limited number of trials. A gold standard for diagnosis and a consensus on the treatment strategy in this patient group are lacking, especially for patients with an anomalous origin of a coronary artery from the opposite sinus of Valsalva (ACAOS) with an interarterial course.AimTo provide evidence-substantiated recommendations for diagnostic work-up, treatment and follow-up of patients with anomalous coronary arteries.MethodsA clinical care pathway for patients with ACAOS was established by six Dutch centres. Prospectively included patients undergo work-up according to protocol using computed tomography (CT) angiography, ischaemia detection, echocardiography and coronary angiography with intracoronary measurements to assess anatomical and physiological characteristics of the ACAOS. Surgical and functional follow-up results are evaluated by CT angiography, ischaemia detection and a quality-of-life questionnaire. Patient inclusion for the first multicentre study on coronary anomalies in the Netherlands started in 2020 and will continue for at least 3 years with a minimum of 2 years of follow-up. For patients with a right or left coronary artery originating from the pulmonary artery and coronary arteriovenous fistulas a registry is maintained.ResultsPrimary outcomes are: (cardiac) death, myocardial ischaemia attributable to the ACAOS, re-intervention after surgery and intervention after initially conservative treatment. The influence of work-up examinations on treatment choice is also evaluated.ConclusionsStructural evidence for the appropriate management of patients with coronary anomalies, especially (interarterial) ACAOS, is lacking. By means of a structured care pathway in a multicentre setting, we aim to provide an evidence-based strategy for the diagnostic evaluation and treatment of this patient group.Supplementary InformationThe online version of this article (10.1007/s12471-021-01556-9) contains supplementary material, which is available to authorized users.

Hemodynamic Relevance of Anomalous Coronary Arteries Originating From the Opposite Sinus of Valsalva-In Search of the Evidence.

Coronary artery anomalies (CAA) represent a heterogeneous group of congenital disorders of the arterial coronary circulation, defined by an anomalous origin of the coronary ostium and/or vessel course. Of particular interest are anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS). The interarterial variants (with the anomalous vessel situated between the great arteries) are historically called “malignant,” based on an anticipated higher risk for myocardial ischemia and sudden cardiac death (SCD), especially affecting young patients during strenuous physical activity. However, the interarterial course itself may not be the predominant cause of ischemia, but rather represents a surrogate for other ischemia-associated anatomical high-risk features. As the exact pathophysiology of ACAOS is not well-understood, there is a lack of evidence-based guidelines addressing optimal diagnostic work-up, downstream testing, sports counseling, and therapeutic options in patients with ACAOS. Therefore, treating physicians are often left with uncertainty regarding the clinical management of affected patients. This review focuses on the pathophysiologic consequences of ACAOS on myocardial ischemia and discusses the concept of the interplay between fixed and dynamic coronary stenosis. Further, we discuss the advantages and limitations of the different diagnostic modalities and give an outlook by highlighting the gaps of knowledge in the assessment of such anomalies.