Anomalies Coronaires Congénitales

Sep 26 2024
Anomalous aortic origin of coronary artery

Stephens EH, Jegatheeswaran A, Brothers JA, Ghobrial J, Karamlou T, Francois CJ, Krishnamurthy R, Dearani JA, Binsalamah Z, Molossi S, Mery CM.
Ann Thorac Surg. 2024;117:1074-86.

ABSTRACT: BACKGROUND Although anomalous aortic origin of a coronary artery (AAOCA) is associated with risk of sudden cardiac arrest (SCA), there is a spectrum of disease, with the appropriate management for many remaining unclear. Increasing data warrant review for an updated perspective on management. METHODS A panel of congenital cardiac surgeons, cardiologists, and imaging practitioners reviewed the current literature related to AAOCA and its management. Survey of relevant publications from 2010 to the present in PubMed was performed. RESULTS The prevalence of AAOCA is 0.4% to 0.8%. Anomalous left coronary artery is 3 to 8 times less common than anomalous right coronary, but carries a much higher risk of SCA. Nevertheless, anomalous right coronary is not completely benign; 10% demonstrate ischemia, and it remains an important cause of SCA. Decision-making regarding which patients should be recommended for surgical intervention includes determining anatomic features associated with ischemia, evidence of ischemia on provocative testing, and concerning cardiovascular symptoms. Ischemia testing continues to prove challenging with low sensitivity and specificity, but the utility of new modalities is an active area of research. Surgical interventions focus on creating an unobstructed path for blood flow and choosing the appropriate surgical technique given the anatomy to accomplish this. Nontrivial morbidity has been reported with surgery, including new-onset ischemia. CONCLUSIONS A proportion of patients with AAOCA demonstrate features and ischemia that warrant surgical intervention. Continued work remains to improve the ability to detect inducible ischemia, to risk stratify these patients, and to provide guidance in terms of which patients warrant surgical intervention. 

Lire la suite dans The Annals of Thoracic Surgery

Anocor Viewer 0 Comments
Sep 26 2024
Coronary artery anomalies – State of the art review

Lau W, Lee PT, Koh CH.
Curr Probl Cardiol. 2023;48. 101935. doi: 10.1016/j.cpcardiol.2023.101935.

ABSTRACT: Coronary artery anomalies (CAAs) comprise a wide spectrum of anatomic entities, with diverse clinical phenotypes. We present a case of an anomalous right coronary artery arising from the left aortic sinus with an interarterial course, a potentially fatal condition that can precipitate ischemia and sudden cardiac death. CAAs are increasingly detected in adults, mostly as incidental findings in the course of cardiac evaluation. This is due to the expanding use of invasive and noninvasive cardiac imaging, usually in the work-up for possible CAD. The prognostic implications of CAAs in this group of patients remain unclear. In AAOCA patients, appropriate work-up with anatomical and functional imaging should be performed for risk stratification. An individualized approach to management should be adopted, considering symptoms, age, sporting activities and the presence of highrisk anatomical features and physiologic consequences (such as ischemia, myocardial fibrosis, or cardiac arrhythmias) detected on multimodality imaging or other functional cardiac investigations. This comprehensive and up to date review seeks to crystallize current data in the recent literature, and proposes a clinical management algorithm for the clinician faced with the conundrum of managing such conditions.

Lire la suite dans Current Problems in Cardiology

Anocor Viewer 0 Comments
Jan 26 2024
Management of adults with anomalous aortic origin of the coronary arteries: State-of-the-art review

Gaudino M, Di Franco A, Arbustini E, Bacha E, Bates ER, Cameron DE, Cao D, David TE, De Paulis R, El-Hamamsy I, Farooqi KM, Girardi LN, Gräni C, Kochav JD, Molossi S, Puskas JD, Rao SV, Sandner S, Tatoulis J, Truong QA, Weinsaft JW, Zimpfer D, Mery CM.
J Am Coll Cardiol. 2023;82:2034-53.

ABSTRACT: As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used. In this paper, a multidisciplinary group of clinical and interventional cardiologists and cardiac surgeons performed a systematic review and critical evaluation of the available evidence on the interventional treatment of AAOCA in adult patients. Using a structured Delphi process, the group agreed on expert recommendations that are intended to complement existing clinical practice guidelines.

Lire la suite dans Journal of the American College of Cardiology

Anocor Viewer 0 Comments
Juil 17 2023
Anomalous coronary arteries – A state-of-the-art approach

Molossi S, Doan T, Sachdeva S.
Cardio Clin. 2023;41:51-69.

ABSTRACT: Congenital coronary anomalies are not an infrequent occurrence and their clinical presentation typically occurs during early years, though may be manifested only in adulthood. In the setting of anomalous aortic origin of a coronary artery, this is particularly concerning as it inflicts sudden loss of healthy young lives. Risk stratification remains a challenge and so does the best management decision-making in these patients, particularly if asymptomatic. Standardized approach to evaluation and management, with careful data collection and collaboration among centers, will likely impact future outcomes in this patient population, thus allowing for exercise participation and healthier lives.

Lire la suite dans Cardiology Clinics

Anocor Viewer 0 Comments
Nov 3 2022
Therapeutic management of anomalous coronary arteries originating from the opposite sinus of Vasalva: current evidence, proposed approach, and the unknowing

Bigler MR, Kadner A, Räber L, Ashraf A, Windecker S, Siepe M, Padalino MA, Gräni C.
J Am Heart Asso. 2022. doi: 10.1161/JAHA.122.027098.

ABSTRACT: 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 assess- ment 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 manage- ment algorithm based on current evidence on multimodality invasive and noninvasive imaging findings and highlight remaining gaps of knowledge. 

Lire la suite dans Journal of the American Heart Association

Anocor Viewer 0 Comments
Nov 2 2022
Critical update and discussion of the prevalence, nature, mechanisms of action, and treatment options in potentially serious coronary anomalies

Angelini P, Uribe C.
Trends Cardiovasc Med. 2022. doi: 10.1016/j.tcm.2022.05.007.

ABSTRACT: As widely discussed in recent literature, coronary artery anomalies only occasionally lead to potentially serious myocardial ischemic events. The most important group of coronary anomalies has been called anomalous coronary artery origin from an abnormal sinus or a site in the ascending aorta (ACAOS). Only some cases of right- or left-sided intramural-course ACAOS (R-ACAOS-IM or L-ACAOS-IM) can potentially cause significant symptoms or sudden cardiac death, typically during exertion in athletes. After an ACAOS-IM case is qualitatively identified, it is necessary to establish the severity of associated stenosis (which is always present to some degree in ACAOS-IM). The 3 stages of a comprehensive diagnostic process are: 1. initial screening of high-risk populations (young elite athletes, optimally by use of magnetic resonance imaging [MRI]) to identify the prevalence of similar cases in large populations (the denominator of any risk calculation); 2. evaluating symptoms (chest pain, syncope, or sudden death) and performing stress testing; 3. in patients found to carry ACAOS-IM, evaluating the severity of coronary obstruction by intravascular ultrasonography, which is an objective, definitive, and quantifying imaging modality for this condition, essential in selected carriers of such anomalies. The possible treatment alternatives are discussed and updated.

Lire la suite dans Trends in Cardiovascular Medicine

Anocor Viewer 0 Comments
Nov 1 2022
Coronary artery anomalies

Dolgner S, Hickey E, Molossi S.
Heart. 2022;108:1063-70.

Introduction: While coronary artery anomalies (CAAs) are relatively rare overall, they are one of the most common types of congenital heart disease. Multiple classification schemes have been proposed, typically taking into account abnormalities of vessel origin, course, number and ventricular myocardium supply. These anomalies can occur in otherwise structurally normal hearts or associated with other types of congenital heart disease. While many CAAs are not associated with untoward outcomes, some may have a more concerning clinical course resulting in presentation with sudden cardiac death (SCD). Unfortunately, this dramatic presentation may be the first presenting symptom, though patients may have had prior symptoms that were not linked to the underlying potentially fatal diagnosis.5 This review will focus on the evaluation and management of CAA among adolescents and young adults with otherwise structurally normal hearts, primarily focusing on anomalous aortic origin of a coronary artery (AAOCA) at or above the opposite sinus of Valsalva, the second leading cause of SCD in the young. AAOCA may involve the right coronary artery (AAORCA) originating from the left (opposite anterior) sinus of Valsalva, the left coronary originating from the right (opposite anterior) sinus of Valsalva (AAOLCA), or, more rarely, the origin of a coronary artery from or near the ‘non-coronary’ (posterior) sinus. They are typically also categorised into the following groups based on their course: interarterial, subpulmonic (also called intraseptal or intraconal), pre-pulmonic, retroaortic and retrocardiac (figure 1). Based on this anatomical definition, lesions have historically been classified as normal variants without increased risk of mortality (typically including those variants without an …

Lire la suite dans Heart BMJ

Anocor Viewer 0 Comments
Fév 27 2022
Myocardial bridging: Diagnosis, functional assessment, and management

Sternheim D, Power DA, Samtani R, Kini A, Fuster V, Sharma S.
J Am Coll Cardiol. 2021;78:2196-212.

Myocardial bridging (MB) is a congenital coronary anomaly in which a segment of the epicardial coronary artery traverses through the myocardium for a portion of its length. The muscle overlying the artery is termed a myocardial bridge, and the intramyocardial segment is referred to as a tunneled artery. MB can occur in any coronary artery, although is most commonly seen in the left anterior descending artery. Although traditionally considered benign in nature, increasing attention is being given to specific subsets of MB associated with ischemic symptomatology. The advent of contemporary functional and anatomic imaging modalities, both invasive and noninvasive, have dramatically improved our understanding of dynamic pathophysiology associated with MBs. This review provides a contemporary overview of epidemiology, pathobiology, diagnosis, functional assessment, and management of MBs.

Lire la suite dans Journal of the American College of Cardiology

Anocor Viewer 0 Comments
Fév 27 2022
Coronary artery anomalies

Gentile F, Castiglione V, De Caterina R.
Circulation. 2021;144:983-96.

ABSTRACT: Coronary artery anomalies (CAAs) are a group of congenital conditions characterized by abnormal origin or course of any of the 3 main epicardial coronary arteries. Although CAAs have been identified as a common underlying condition in young athletes with sudden cardiac death, the widespread use of invasive and noninvasive coronary imaging has led to increased recognition of CAAs among adults. CAAS are often discovered as an incidental finding during the diagnostic workup for ischemic heart disease. The clinical correlates and prognostic implication of CAAs remain poorly understood in this context, and guideline-recommended therapeutic choices are supported by a low level of scientific evidence. Several studies have examined whether assessment of CAA-related myocardial ischemia can improve risk stratification in these patients, suggesting that multimodality imaging and functional tests may be key in the management of CAAs. The aim of this review is to outline definitions, classification, and epidemiology of the most relevant CAAs, highlighting recent advances and the potential impact of multimodality evaluation, and to discuss current therapeutic opportunities. 

Lire la suite dans Circulation

Anocor Viewer 0 Comments
Fév 26 2022
Coronary artery fistulas: Indications, techniques, outcomes, and complications of transcatheter fistula closure

Al-Hijji M, El Sabbagh A, El Hajj S, Alkhouli M, El Sabawi B, Cabalka A, Miranda WR, Holmes DR, Rihal CS.
JACC Cardiovasc Interv. 2021;14:1393-1406.

Coronary artery fistulas (CAFs) are rare coronary anomalies that are usually diagnosed incidentally with cardiac imaging. Small CAFs are generally asymptomatic and can close over time, while some untreated medium or large CAFs can enlarge, leading to clinical sequelae such as cardiac chamber enlargement or myocardial ischemia. With the advancement of transcatheter equipment and techniques, CAFs have been increasingly closed using a percutaneous approach. However, the procedure is not free of limitations given the risk for myocardial infarction, device embolization, and fistula recanalization. In this review, the authors illustrate the contemporary procedural considerations, techniques, and outcomes of transcatheter CAF closure.

Lire la suite dans JACC: Cardiovascular Interventions

Anocor Viewer 0 Comments
Oct 15 2021
Hemodynamic relevance of anomalous coronary arteries originating from the opposite sinus of Valsalva-In search of the evidence

Bigler MR, Ashraf A, Seiler C, Praz F, Ueki Y, Windecker S, Kadner A, Räber L, Gräni C.
Front Cardiovasc Med. 2021. doi:10.3389/fcvm.2020.591326.

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.

Lire la suite dans Frontiers in Cardiovascular Medicine

Anocor Viewer 0 Comments
Oct 15 2021
The first multicentre study on coronary anomalies in the Netherlands: MuSCAT

Koppel CJ, Driesen BW, de Winter RJ, van den Bosch AE, van Kimmenade R, Wagenaar LJ, Jukema JW, Hazekamp MG, van der Kley F, Jongbloed MRM, Kiès P, Egorova AD, Verheijen DBH, Damman P, Schoof PH, Wilschut J, Stoel M, Speekenbrink RGH, Voskuil M, Vliegen HW.
Neth Heart J. 2021;29:311-17.

ABSTRACT: Current 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. Aim: To provide evidence-substantiated recommendations for diagnostic work-up, treatment and follow-up of patients with anomalous coronary arteries. Methods: A 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. Results: Primary 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. Conclusions: Structural 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. Keywords: Anomalous coronary artery from the opposite sinus of Valsalva; Anomalous coronary artery from the pulmonary artery; Coronary anomalies; Coronary arteriovenous fistula; Multicentre study.

Lire la suite dans Netherlands Heart Journal

Anocor Viewer 0 Comments
Oct 15 2021
Catheter selection and angiographic views for anomalous coronary arteries. A practical guide

Ben-Dor I, Weissman G, Rogers T, Slack M, Pichard A, Ben-Dor N, Hashim H, Bernardo N, Satler LF, Waksman R.
JACC Cardiovasc Interv. 2021;14:995-1008.

Although congenital coronary artery anomalies are relatively rare, they are the second most common cause of sudden cardiac death among young athletes. When encountered in the cardiac catherization laboratory, they are often challenging to selectively engage, requiring multiple catheters, plus increased contrast volume and radiation exposure. In the setting of acute coronary syndromes, it is not infrequent that percutaneous intervention is delayed because of the inability to engage an anomalous coronary artery. The aim of this review is to provide a comprehensive and concise overview of coronary artery anomalies, with particular attention to diagnostic and guide catheter selection for each type of anomaly and recommendations on how to recognize the vessel course angiographically.

Lire la suite dans JACC: Cardiovascular Interventions

Anocor Viewer 0 Comments
Oct 15 2021
Anomalous coronary arteries: what is known and what still remains to be learned ?

Molossi S, Sachdeva S.
Curr Opin Cardiol. 2020;35:42-51.

Purpose of review: To report what is known and unknown regarding coronary anomalies in children, particularly anomalous aortic origin of a coronary artery, efforts undertaken to answer several questions regarding evaluation and management of this challenging young population, and where the future is heading. Recent findings: Patients with anomalous aortic origin of a coronary artery (AAOCA) present as an incidental finding at least half of the time, advanced imaging is essential to define anatomic characteristics of this lesion, assessment of myocardial perfusion with stress cardiac magnetic resonance imaging is feasible and contributes to risk stratification, certain patient populations require invasive assessment of coronary flow with measurement of fractional flow reserve, and surgical intervention can be safely performed through long-term data on impact to prevent sudden events is lacking. Summary: Optimal risk stratification in AAOCA is yet to be defined, though substantial strides are being made with a standardized approach to the evaluation and management of these patients. Continued collaboration among centers and the scientific community will positively impact patients and families living with AAOCA.

Lire la suite dans Current Opinion in Cardiology

Anocor Viewer 0 Comments
Oct 15 2021
Embryology of coronary arteries and anatomy/pathophysiology of coronary anomalies. A comprehensive update

Tomanek R, Angelini P.
Int J Cardiol. 2019;281:28-34. 

Objectives: This paper reviews new findings in both embryology of coronary arteries and in clinical observations of coronary artery anomalies. Focus: Our presentation emphasizes studies based on: 1) newer methods of coronary development in animals and humans, and 2) intravascular ultrasonography to interpret pathophysiology and guide treatment of coronary anomalies. Conclusions: New data reveal the roles of many cellular interactions and signaling pathways involved in the normal and abnormal formation of the coronary arterial system and the consequences of their defective formation. Pathogenetic developmental mechanisms include dysfunction of the Notch and Hypo signaling pathways, angiogenic and arteriogenic molecules, and neural crest cells.We addressed numerous clinically significant coronary anomalies and their prevalence in a general population (especially those characterized by an ectopic origin with aortic intramural course), and point out the critical relevance of understanding the variable mechanisms of coronary dysfunction, especially, fixed versus phasic stenoses or intermittent spasm, and individual severity of clinical presentations.

Lire la suite dans International Journal of Cardiology

Anocor Viewer 0 Comments
Oct 15 2021
Features associated with myocardial ischemia in anomalous aortic origin of a coronary artery: A Congenital Heart Surgeons’ Society study

Jegatheeswaran A, Devlin PJ, McCrindle BW, Williams WG,  Jacobs ML, Blackstone EH, DeCampli WM, Caldarone CA, Gaynor JW, Kirklin JK, Lorber RO, Mery CM, St. Louis SD, Molossi S, Brothers JA.
J Thorac Cardiovasc Surg. 2019;158:822-34.

ABSTRACT: Objectives: We sought to determine anatomic features associated with evidence of myocardial ischemia and sudden cardiac events (arrest or death) for patients with anomalous aortic origin of a coronary artery. Methods: We enrolled 560 patients, less than or equal to 30 years, at diagnosis from 40 institutions. Ischemia was defined as the presence of exertional syncope, a sudden cardiac event (arrest/death), or abnormal investigation results. Data on detailed anatomic features were abstracted from echocardiography, computed tomography, magnetic resonance imaging, operative, and/or surgeon-completed reports. Results: There were 236 patients with negative ischemia test results, and 49 with evidence of ischemia (including 18 who presented with a sudden cardiac event); 275 asymptomatic patients who had not undergone provocative ischemia testing were excluded from primary analyses. Patients with ischemia (vs without), were more likely to have left anomalous coronary arteries (28/49 vs 46/236; P < .0001). Of patients with ischemia (vs without), those with anomalous left coronary arteries were more likely to have an intramural coronary artery course, or a high or slit-like coronary artery orifice. Of patients with ischemia (vs without), those with anomalous right coronary arteries were more likely to have a longer intramural course. Among patients with ischemia, the occurrence of sudden cardiac events was not shown to have any associated anatomic features. Conclusions: Anatomic features including coronary artery involved, intramural course and length, and orifice anomalies were associated with evidence of myocardial ischemia for patients with anomalous aortic origin of a coronary artery. These features might importantly inform risk stratification and decisions regarding surgical management. Keywords: anomalous aortic origin of a coronary artery; congenital heart disease; database; epidemiology; ischemia.

Lire la suite dans The Journal of Thoracic and Cardiovascular Surgery

Anocor Viewer 0 Comments
Oct 15 2021
Anomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva

Cheezum MK, Liberthson RR, Shah NR, Villines TC, O’Gara PT, Landzberg MJ, Blankstein R.
J Am Coll Cardiol. 2017;69:1592-608.

Anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva is increasingly recognized by cardiac imaging. Although most AAOCA subtypes are benign, autopsy studies report an associated risk of sudden death with interarterial anomalous left coronary artery (ALCA) and anomalous right coronary artery (ARCA). Despite efforts to identify high-risk ALCA and ARCA patients who may benefit from surgical repair, debate remains regarding their classification, prevalence, risk stratification, and management. We comprehensively reviewed 77 studies reporting the prevalence of AAOCA among >1 million patients, and 20 studies examining outcomes of interarterial ALCA/ARCA patients. Observational data suggests that interarterial ALCA is rare (weighted prevalence ¼ 0.03%; 95% confidence interval [CI]: 0.01% to 0.04%) compared with interarterial ARCA (weighted prevalence ¼ 0.23%; 95% CI: 0.17% to 0.31%). Recognizing the challenges in managing these patients, we review cardiac tests used to examine AAOCA and knowledge gaps in management.

Lire la suite dans Journal of the American College of Cardiology

fillweb.anocor 0 Comments
Oct 15 2021
Multimodality imaging in individuals with anomalous coronary arteries

Gräni C, Buechel RR, Kaufmann PA, Kwong RY.
JACC Cardiovasc Imaging. 2017;10:471–81.

Anomalous coronary arteries (ACA) represent a congenital disorder with an anomalous location of the coronary ostium and/or vascular course. Although most individuals with ACA are asymptomatic and remain undiagnosed, some ACA variants are clinically significant leading to symptoms and even adverse cardiac events. Currently, disease prevalence, pathophysiological mechanisms, risks of sudden cardiac death, and the optimal assessment and treatment strategies among subtypes of ACA remain largely unknown. Consequently, there is a lack of guidelines regarding imaging, sport restriction, and treatment options in individuals with ACA at all ages. Cardiac imaging techniques may play a pivotal role in the assessment of individuals with ACA and may offer guidance toward an optimal treatment strategy. This state-of-the-art review highlights current challenges and future perspectives with a special focus on the role of noninvasive multimodality imaging in patients with ACA.

Lire la suite dans JACC: Cardiovascular Imaging

Anocor Viewer 0 Comments
Oct 15 2021
Decision analysis to define the optimal management of athletes with anomalous aortic origin of a coronary artery

Mery CM, Lopez KN, Molossi S, Sexson-Tejtel SK, Krishnamurthy R, McKenzie ED, Fraser CD, Cantor .
J Thorac Cardiovasc Surg. 2016;152:1366-75.

ABSTRACT: Objectives: The goal of this study was to use decision analysis to evaluate the impact of varying uncertainties on the outcomes of patients with anomalous aortic origin of a coronary artery. Methods: Two separate decision analysis models were created: one for anomalous left coronary artery (ALCA) and one for anomalous right coronary artery (ARCA). Three strategies were compared: observation, exercise restriction, and surgery. Probabilities and health utilities were estimated on the basis of existing literature. Deterministic and probabilistic sensitivity analyses were performed. Results: Surgery was the optimal management strategy for patients <30 years of age with ALCA. As age increased, observation became an equivalent strategy and eventually surpassed surgery as the treatment of choice. The advantage on life expectancy for surgery over observation ranged from 2.6 ± 1.7 years for a 10-year-old patient to -0.03 ± 0.1 for a 65-year old patient. In patients with ARCA, observation was the optimal strategy for most patients with a life expectancy advantage over surgery of 0.1 ± 0.1 years to 0.2 ± 0.4 years, depending on age. Surgery was the preferred strategy only for patients <25 years of age when the perceived risk of sudden cardiac death was high and the perioperative mortality was low. Exercise restriction was a suboptimal strategy for both ALCA and ARCA in all scenarios. Conclusions: The optimal management in anomalous aortic origin of a coronary artery depends on multiple factors, including individual patient characteristics. Decision analysis provides a tool to understand how these characteristics affect the outcomes with each management strategy and thus may aid in the decision making process for a particular patient.

Lire la suite dans The journal of Thoracic and Cardiovascular Surgery

Anocor Viewer 0 Comments
Oct 15 2021
Coronary artery anomalies overview: The normal and the abnormal

Villa ADM, Sammut E, Nair A, Rajani R, Bonamini R, Chiribiri A.
World J Radiol. 2016;8:537-55.

ABSTRACT: The aim of this review is to give a comprehensive and concise overview of coronary embryology and normal coronary anatomy, describe common variants of normal and summarize typical patterns of anomalous coronary artery anatomy. Extensive iconography supports the text, with particular attention to images obtained in vivo using non-invasive imaging. We have divided this article into three groups, according to their frequency in the general population: Normal, normal variant and anomaly. Although congenital coronary artery anomalies are relatively uncommon, they are the second most common cause of sudden cardiac death among young athletes and therefore warrant detailed review. Based on the functional relevance of each abnormality, coronary artery anomalies can be classified as anomalies with obligatory ischemia, without ischemia or with exceptional ischemia. The clinical symptoms may include chest pain, dyspnea, palpitations, syncope, cardiomyopathy, arrhythmia, myocardial infarction and sudden cardiac death. Moreover, it is important to also identify variants and anomalies without clinical relevance in their own right as complications during surgery or angioplasty can occur.

Lire la suite dans World journal of radiology

Anocor Viewer 0 Comments
Oct 15 2021
Congenital coronary artery anomalies: a bridge from embryology to anatomy and pathophysiology-a position statement of the development, anatomy, and pathology ESC Working Group

Perez-Pomares JM, De la Pompa JL, Franco D, Henderson D, Yen Ho S, Houyel L, Kelly RG, Sedmera D, Sheppard M, Sperling S, Thiene G, Van den Hoff M, Basso C.
Cardiovasc Res. 2016;109:204-16.

Congenital coronary artery anomalies are of major significance in clinical cardiology and cardiac surgery due to their association with myocardial ischaemia and sudden death. Such anomalies are detectable by imaging modalities and, according to various definitions, their prevalence ranges from 0.21 to 5.79%. This consensus document from the Development, Anatomy and PathologyWorking Group of the European Society of Cardiology aims to provide: a definition of normality that refers to essential anatomical and embryological features of coronary vessels, based on the integrated analysis of studies of normal and abnormal coronary embryogenesis and pathophysiology; an animal model-based systematic survey of the molecular and cellular mechanisms that regulate coronary blood vessel development; an organization of the wide spectrum of coronary artery anomalies, according to a comprehensive anatomical and embryological classification scheme; current knowledge of the pathophysiological mechanisms underlying symptoms and signs of coronary artery anomalies, with diagnostic and therapeutic implications. This document identifies the mosaic-like embryonic development of the coronary vascular system, as coronary cell types differentiate from multiple cell sources through an intricate network of molecular signals and haemodynamic cues, as the necessary framework for understanding the complex spectrum of coronary artery anomalies observed in human patients.

Lire la suite dans Cardiovascular Research

Anocor Viewer 0 Comments
Oct 14 2021
Anomalous aortic origin of a coronary artery: Toward a standardized approach

Mery CM, Lawrence SM, Krishnamurthy R, Sexson-Tejtel SK, Carberry KE, McKenzie ED, Fraser Jr CD.
Semin Thorac Cardiovasc Surg. 2014;26:110-22.

Anomalous aortic origin of a coronary artery (AAOCA) is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. It is the second most common cause of sudden cardiac death in young athletes. Its exact prevalence, the pathophysiological mechanisms that cause sudden cardiac death, the actual risk of death for the different types of AAOCA, the optimal way to evaluate these patients, and whether any treatment strategies decrease the risk of sudden cardiac death in patients diagnosed with AAOCA are unknown. This article analyzes what is currently known and unknown about this disease. It also describes the creation of a dedicated multidisciplinary coronary anomalies program and the development of a framework in an initial attempt to standardize the evaluation and management of these patients.

Lire la suite dans Seminars in Thoracic and Cardiovascular Surgery

Anocor Viewer 0 Comments
Oct 14 2021
Novel imaging of coronary artery anomalies to assess their prevalence, the causes of clinical symptoms, and the risk of sudden cardiac death

Angelini P.
Circ Cardiovasc Imaging. 2014;7:747-54.

In a fundamental 1974 article, Cheitlin et al of the Armed Forces Institute of Pathology emphasized the special role of anomalous aortic origin of the coronary arteries and differentiated this condition from the other coronary artery anomalies (CAAs) as being associated with an increased frequency of sudden cardiac death (SCD) in young persons, especially during strenuous exertion. More recently, CAAs have also been considered possible causes of clinically disabling symptoms, including dyspnea, angina pectoris, and syncope, especially in young adults.Clinicians and epidemiologists have identified the need to prevent not only SCD in young persons, especially athletes or military recruits, but also other CAA-related symptoms in persons of any age.Much of the available information concerning the incidence of SCD in carriers of CAAs is lacking a denominator (measure of the carriers at risk). The most notable study of SCD incidence is a classic 2004 article by Eckart et al, who reported the mortality rate (>25 years) that US military recruits experienced during a 2-month-long boot-camp training period. All the recruits were involved in strenuous exercise and had undergone routine screening based on a history and physical examination performed by general practitioners. Of the 23 million recruits, 64 died of SCD (0.28 per 100 000 per 2 months, or 1.68 per 100 000 per year). Of these deaths, 21 (33%) were attributed to CAAs, specifically anomalous origin of the left coronary artery from the right sinus of Valsalva with an interarterial course.

Lire la suite dans Circulation Cardiovascular Imaging

Anocor Viewer 0 Comments
Oct 14 2021
Abnormal origins of the coronary arteries from the aortic root

Hoffman JIE.
Cardiol Young. 2014;24:774-91.

Background: Isolated anomalous left main coronary artery (ALMCA) from the right aortic sinus of Valsalva (RASV) with an interarterial course between the pulmonary trunk and aorta is a rare congenital abnormality. We performed an evidence based systematic overview spanning 4 decades to assess the prevalence, clinical features and management of this anomaly. Methods: A computerized search spanning 40 years was conducted to identify articles describing cases of ALMCA arising from the RASV with an interarterial course. The bibliographies of all relevant articles were also searched. Results: The search identified 264 cases. Age ranged from 3.5 months to 87 years.Male/female ratio was 2.9/1. Forty-nine percent of the cases were diagnosed postmortem. Cardiac catheterization was the most common diagnostic tool (41.7%) followed by echocardiography, magnetic resonance imaging (MRI) and computerized assisted tomography. Fifty-seven (21.6%) cases underwent surgical procedureswith nomortality and low morbidity. Conclusions: ALMCA from the RASV is associated with increased risk of sudden death, notably in young patients. Unfortunately the majority are diagnosed postmortem. More than a third present with sudden cardiac death. Echocardiography, computerized assisted tomography and cardiac MRI are valuable non-invasive diagnostic tools. Cardiac catheterization provides a definitive diagnosis in the majority. Surgical correction is the mainstay of treatment with low risk and good anatomic and functional results.

Lire la suite dans Cardiology in the Young

Anocor Viewer 0 Comments
Oct 14 2021
Anomalous aortic origin of coronary arteries from the opposite sinus: A critical appraisal of risk

Penalver JM, Mosca RS, Weitz D, Phoon CKL.
BMC Cardiovasc Disord. 2012;12:83.

Background: Anomalous aortic origin of the coronary artery (AAOCA) from the opposite sinus of Valsalva with an interarterial course has received much attention due to its association with sudden death in otherwise healthy individuals. AAOCA is relatively common and may have significant public health implications. While our knowledge of its pathophysiology and natural history remains incomplete, an emphasis has been placed on surgical correction. Discussion: In 2005 we published a review examining the rates of sudden death with AAOCA, as well as complications of surgical management. Evidence now points even more strongly to lower rates of sudden death, while surgical outcomes data now better documents associated risks. Summary: Armed with this updated information, we agree with the need for a national registry to better track patients with AAOCA. We submit that the risks of surgical management outweigh any benefits in the asymptomatic patient with anomalous right coronary artery, and expectant management should also be strongly considered even in asymptomatic patients with anomalous left coronary artery.

Lire la suite dans BMC Cardiovascular Disorders

Anocor Viewer 0 Comments
Oct 13 2021
Anomalous origination of a coronary artery from the opposite sinus

Lim JCE, Beale A, Ramcharitar S. 
Nat Rev Cardiol. 2011; 8(12): 706-19

Anomalous origination of a coronary artery from the opposite sinus (ACAOS) is estimated to be present in 0.2-2.0% of the population. In the majority of individuals, ACAOS has no hemodynamic or prognostic implications, but in a minority of cases, typically where the anomalous coronary artery takes an interarterial course to reach its correct myocardial territory, it can precipitate ischemia and sudden cardiac death (SCD). With the growing use of CT coronary angiography (CTCA) in the investigation of ischemic heart disease, we can expect increasing rates of incidental detection of this anomaly. Although CTCA and magnetic resonance coronary angiography can effectively characterize these lesions anatomically, they fail to describe and quantitatively assess the basic defect that leads to coronary insufficiency, such as mural intussusception. The key challenge lies in the identification of which patients are at risk of SCD and, therefore, who should be offered corrective surgical or (potentially) percutaneous intervention. Conventional, noninvasive stress testing has limited sensitivity, but emerging, invasive stress tests, which utilize intravascular ultrasonography and measurements of fractional flow reserve, show the potential to provide more-accurate hemodynamic and prognostic assessment.

Lire la suite dans Nature Reviews Cardiology

Anocor Viewer 0 Comments
Oct 12 2021
Anomalous interarterial left coronary artery: An evidence based systematic overview

Moustafa SE, Zehr K, Moohadam M, Lorenz EC, Mookadam F.
Int J Cardiol. 2008;126:13-20

Background: Isolated anomalous left main coronary artery (ALMCA) from the right aortic sinus of Valsalva (RASV) with an interarterial course between the pulmonary trunk and aorta is a rare congenital abnormality. We performed an evidence based systematic overview spanning 4 decades to assess the prevalence, clinical features and management of this anomaly. Methods: A computerized search spanning 40 years was conducted to identify articles describing cases of ALMCA arising from the RASV with an interarterial course. The bibliographies of all relevant articles were also searched. Results: The search identified 264 cases. Age ranged from 3.5 months to 87 years.Male/female ratio was 2.9/1. Forty-nine percent of the cases were diagnosed postmortem. Cardiac catheterization was the most common diagnostic tool (41.7%) followed by echocardiography, magnetic resonance imaging (MRI) and computerized assisted tomography. Fifty-seven (21.6%) cases underwent surgical procedureswith nomortality and low morbidity. Conclusions: ALMCA from the RASV is associated with increased risk of sudden death, notably in young patients. Unfortunately the majority are diagnosed postmortem. More than a third present with sudden cardiac death. Echocardiography, computerized assisted tomography and cardiac MRI are valuable non-invasive diagnostic tools. Cardiac catheterization provides a definitive diagnosis in the majority. Surgical correction is the mainstay of treatment with low risk and good anatomic and functional results.

Lire la suite dans International Journal of Cardiology

Anocor Viewer 0 Comments
Sep 26 2021
Coronary Artery Anomalies An Entity in Search of an Identity

Angelini P.
Circulation. 2007;115:1296-305.

ABSTRACT: Coronary artery anomalies (CAAs) are a diverse group of congenital disorders whose manifestations and pathophysiological mechanisms are highly variable. The subject of CAAs is undergoing profound evolutionary changes related to the definition, morphogenesis, clinical presentation, diagnostic workup, prognosis, and treatment of these anomalies. To understand the clinical impact of CAAs, the fundamental challenge is the firm establishment, for a particular type of CAA, of a mechanism capable of interference with the coronary artery’s function, which is to provide adequate blood flow to the dependent myocardium. The present review focuses on anomalous origination of a coronary artery from the opposite sinus–the subgroup of CAAs that has the most potential for clinical repercussions, specifically sudden death in the young. For this subgroup, solid diagnostic screening protocols should be established, especially for athletes and other young individuals subjected to extreme exertion. Intravascular ultrasonography is the preferred means to evaluate the mechanisms responsible for ischemia in anomalous origination of a coronary artery from the opposite sinus and other potentially significant CAAs. Patients symptomatic of anomalous origination of a coronary artery from the opposite sinus may undergo medical treatment/observation, coronary angioplasty with stent deployment, or surgical repair. To be competent to advise CAA carriers, especially in the context of sporting or military activities, cardiologists should undergo specific training in these disorders. Only multicenter collaboration on protocols dedicated to CAAs can give rise to the large-scale studies needed to define the prognosis and optimal treatment of these disorders.

Lire la suite dans Circulation

Anocor Viewer 0 Comments