A Case of Anomalous Right Coronary Artery With Catastrophic Presentation.

Anomalous coronary artery is a rare but potentially life-threatening alteration in the coronary vascular system that is related to an increased risk of myocardial ischemia, ventricular arrhythmias, heart failure, and sudden cardiac death (SCD). Here, we present the case of a young male who presented to the hospital after a witnessed sudden cardiac arrest. Bystander cardiopulmonary resuscitation was started immediately, and normal sinus rhythm was achieved after electrical cardioversion three times. He was admitted to the ICU for further care upon admission. A CT of the chest showed a potential vascular structure in between the aorta and the pulmonary trunk. He underwent cardiac catheterization, which identified minimal coronary artery disease with the anomalous takeoff of the right coronary artery from the left coronary cusp. A cardiac CT scan obtained also showed an anomalous right coronary artery (ARCA) with an inter-arterial course. After explaining available treatment options and obtaining informed consent, a surgical correction by cardiothoracic surgery was performed using the coronary artery bypass graft technique. The patient recovered well after the surgery and was discharged home. After two years of follow-up, he continued to live life normally without any symptoms. Early and accurate diagnosis of an anomalous coronary artery is imperative for timely intervention, as malignant coronary artery diseases can often have a catastrophic presentation with acute coronary syndromes, myocardial infarction, or SCD. We present here a case of successful diagnosis of ARCA and its prompt surgical correction using coronary artery bypass grafting technique in a young adult. Despite the availability of various other treatment options, our case underscores coronary artery bypass grafting as a viable choice for individuals with anomalous coronary arteries, particularly in urgent situations.

Coronary artery anomalies: what are they? when to suspect? how to treat?-a narrative review.

Background and Objective: In the literature have been widely discussed different classification criteria for coronary artery anomalies (CAAs), some authors have tried to categorize them only as "major" or "hemodynamically significant" anomalies versus "minor" or "not hemodynamically significant" ones. However, the most recent literature has concluded that all possible coronary anatomy should be taken into consideration in a comprehensive classification of CAAs. The aim of the article is to review the most recent literature regarding CAAs to provide a comprehensive overview of this challenging topic. Methods: We propose a narrative overview of the most impactful and recent literature, synthetizing and re-elaborating the most important articles concerning CAAs. Key Content and Findings: The important gap of knowledge on the specific characteristics of CAAs has led to a progressively increased interest of the current research in this field. Albeit their nature is still unclear, an increased awareness of their fatality is spreading among clinicians and the general population, mostly associated with their clinical relevance among young patients and athletes. On the other side, we do believe that clinical and hemodynamic repercussions are of crucial importance and should always be integrated to understand the true nature of this important pathology. Conclusions: In the field of pediatric cardiology, CAAs are one of the most fascinating and studied subject. We propose a state-of-the art review to provide a comprehensive and systematic description and subsequently an approach to the epidemiological, pathophysiological, and clinical aspects of the most important CAAs in the pediatric population.

Anatomical Variants of the Origin of the Coronary Arteries: A Systematic Review and Meta-Analysis of Prevalence.

PURPOSE: The most common anomaly is an anomalous left coronary artery originating from the pulmonary artery. These variants can be different and depend on the location as well as how they present themselves in their anatomical distribution and their symptomatological relationship. For these reasons, this review aims to identify the variants of the coronary artery and how they are associated with different clinical conditions. METHODS: The databases Medline, Scopus, Web of Science, Google Scholar, CINAHL, and LILACS were researched until January 2024. Two authors independently performed the search, study selection, and data extraction. Methodological quality was evaluated using an assurance tool for anatomical studies (AQUA). Pooled prevalence was estimated using a random effects model. RESULTS: A total of 39 studies met the established selection criteria. In this study, 21 articles with a total of 578,868 subjects were included in the meta-analysis. The coronary artery origin variant was 1% (CI = 0.8-1.2%). For this third sample, the funnel plot graph showed an important asymmetry, with a p-value of 0.162, which is directly associated with this asymmetry. CONCLUSIONS: It is recommended that patients whose diagnosis was made incidentally and in the absence of symptoms undergo periodic controls to prevent future complications, including death. Finally, we believe that further studies could improve the anatomical, embryological, and physiological understanding of this variant in the heart.

An unusual cause of cardiac arrest in a young infant.

BACKGROUND: Anomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva (AAOCA) is a rare congenital heart lesion. It is uncommon for patients with AAOCA to present with severe symptoms at a very young age. CASE PRESENTATION: We describe a very rare but critical presentation in a young infant with AAOCA that requires surgical repair and pacemaker placement. A three-month-old infant was referred because of syncope. Cardiac arrest occurred shortly after admission. The electrocardiogram indicated a complete atrioventricular block and a transvenous temporary pacemaker was implanted. A further coronary computed tomographic angiography (CTA) showed the anomalous origin of the right coronary artery from the left sinus of Valsalva. Coronary artery unroofing was performed due to an interarterial course with the intramural component, and a permanent epicardial pacemaker was implanted. The postoperative recovery was uneventful, and this patient was thriving and asymptomatic at the nine-month follow-up. However, the electrocardiogram still indicated a complete pacing rhythm. CONCLUSIONS: By timely diagnosis and treatment, this patient is successfully rescued. Although rare, AAOCA may be fatal even in infants.

Successful Management of Anomalous Lipton R-III Right Coronary Artery Chronic Total Occlusion.

Chronic total occlusion (CTO) of the coronary artery is a subset where cardiologists confront technical challenges most of the time during percutaneous coronary intervention (PCI). A congenital coronary anomaly is considered a critical challenge, especially when accompanied by CTO lesions. We report a case of a 64-year-old hypertensive and chronic smoker male who presented to our tertiary care center with chief complaints of Canadian Cardiovascular Society II angina. Coronary angiography revealed proximal right coronary artery CTO in a patient with an anomalous origin of coronary arteries arising from the right single sinus "Lipton R-III" which was managed successfully through PCI.

Anomalous Right Coronary Artery With Interarterial Course: Risk Stratification and Surgical Decision-Making Using Coronary Computed Tomography Angiography-Derived Fractional Flow Reserve.

An anomalous right coronary artery (RCA) takeoff, a rare congenital condition often characterized by an interarterial RCA course between the pulmonary artery and the ascending aorta, can lead to symptoms of angina pectoris (chest pain) or even sudden cardiac death (SCD) due to compression of the RCA, although most patients remain asymptomatic. In this case report, we highlight the utility of computed tomography angiography (CTA)-derived fractional flow reserve (FFR), a minimally invasive technique used to assess the hemodynamic significance of coronary lesions, in the risk stratification and surgical decision-making process for a 46-year-old female patient presenting with exertional dyspnea and an anomalous RCA takeoff with an interarterial course. The information obtained from this imaging modality was instrumental in determining that surgical repair did not need to be performed urgently and could be scheduled as an elective case in the future.

Anomalous origin of the circumflex artery from the right pulmonary artery.

Anomalous origin of the circumflex artery from the pulmonary artery (ACxAPA) is a rare but clinically significant condition in which the circumflex artery arises from either the main pulmonary artery or one of its main branches. Untreated patients with ACxAPA may develop severe heart failure or sudden cardiac death. Diagnosis is established with either catheter or CT angiography. We present a case of an adult male with no prior known cardiac history who was found to have ACxAPA after presenting to our institution in acute decompensated heart failure.

Anomalous Right Coronary Artery Clinical Presentations and Considerations.

Anomalous coronary artery presenting as syncope or acute decompensated heart failure complicated by cardiogenic shock is a relatively rare finding. Here, two unusual presentations are described in which an anomalous right coronary artery (RCA) with interarterial course was found following an initially negative workup. The first case describes a 71-year-old male with known non-ischemic cardiomyopathy presenting with acute decompensated heart failure and cardiogenic shock. The second case highlights a 44-year-old female presenting with intermittent angina and recurrent syncope of unknown etiology. These two cases suggest that the anatomy of coronary arteries and their anatomical variants may play a crucial role in the development of adverse cardiovascular outcomes. Utilizing cardiac computed tomography angiography with a lower threshold in patients presenting with cardiac signs, symptoms, and risk factors would lead to earlier detection of these anatomic anomalies and intervention either medically or surgically for potentially improved long-term outcomes.

NASCI case of the month: a case of anomalous right coronary artery arising from the pulmonary artery.

Coronary artery anomalies are rare but potentially fatal abnormalities with occasional striking imaging findings radiologists should recognize.

Percutaneous Coronary Intervention in a Patient Presenting With Inferior Myocardial Infarction and an Anomalous Left Main Artery Originating From the Right Coronary Sinus.

We present a case of a patient with inferior myocardial infarction (MI) and anomalous left main artery originating from the right coronary sinus. The left main artery and right coronary artery originated from the right coronary sinus but with separate ostia. The patient underwent revascularization of the right coronary artery with balloon angioplasty and a drug-eluting stent. Despite being rare, these anomalies can be life-threatening depending on the course of the artery, and when atherosclerotic disease is present, a revascularization strategy can be challenging. Knowing the existence of the left main artery anomaly is important to choose the right guide catheter to achieve successful cannulation and decrease the risk of complications, radiation exposure, and contrast usage.

Intramural aortic stenting of an anomalous right coronary artery arising from the left valsalva sinus: A leap into the dark?

A 69-year-old man with unstable angina underwent coronary angiography showing no lesion in the left coronary artery and critical stenosis in the proximal right coronary artery (RCA) arising from the left sinus of Valsalva.

Hidden Coronary Artery Ostium and Sudden Death.

A 1-week-old girl died suddenly and unexpectedly. At autopsy the major finding was of a right dominant coronary artery circulation with an inapparent left coronary artery ostium. After careful examination, an anomalous origin of the left coronary artery was found with the ostium located in the non-coronary cusp immediately adjacent to the commissure of the non- and left coronary cusps. The ostium was of small caliber with an obliquely oriented artery (<45°) with no ostial ridges. The artery coursed anteriorly past the left coronary cusp between the aorta and the left atrial appendage to then follow its usual course inferiorly along the anterior aspect of the left ventricle. The reminder of the autopsy was unremarkable. Death was, therefore, attributed to an anomalous and hypoplastic left coronary artery (and ostium) with an acute angle of take-off. Tracing coronary arteries in the very young may be technically difficult due to their small size, thus identifying the location of ostia is important. This may be difficult when the ostium was located close to a commissure.

An Unusual Presentation of Syncope in an Elderly Male Leading to a Diagnosis of an Anomalous Right Coronary Artery.

Various coronary artery anomalies have been identified in modern literature with most being benign in nature. Generally, these anomalous vessels are clinically silent due to their non-obstructive or benign course. It is vital to identify patients with malignant courses of these vessels as their initial presenting symptom might be sudden cardiac death. A 74-year-old male presented to the hospital following an episode of syncope and incontinence. Denying any symptoms of chest pain or shortness of breath, the patient did admit to having a six-month history of intermittent lightheadedness and one prior episode of syncope that was attributed to physical activity. Cardiac nuclear stress testing revealed a large reversible inferior wall defect indicating a defect with the right coronary artery. Cardiac catheterization demonstrated a history of coronary artery disease and revealed an anomalous origin of the right coronary artery. A coronary CT angiogram identified the right coronary artery as having an abnormal origin from the left sinus of Valsalva with a malignant interarterial route. The patient underwent a coronary artery bypass graft to correct the issue. There were no major postoperative complications. Treatment guidelines for patients suffering from malignant coronary artery anomalies are limited. Despite multiple surgical interventions available, data regarding conservative medical management is limited and should be of consideration in future studies.

A Cadaveric Case Report of a Double Right Coronary Artery and Its Clinical Implications.

A double right coronary artery is one of the rarest variations of the coronary arterial system. This case report presents a unique variation: a right coronary and an accessory coronary artery arise from a single ostium within the right aortic sinus. The two vessels appeared externally to have a common trunk but were partitioned internally by a carina-like septum. This report underscores the importance of understanding the embryological development of coronary arteries and recognizing potential variations. It discusses the specific variation observed in this case and its clinical implications. The aim is to contribute to the limited literature on this condition and highlight the importance of recognizing and managing such anomalies in clinical procedures.

Anomalous origin of left main coronary artery from the right sinus of Valsalva.

BACKGROUND: Anomalous coronary arteries are rare congenital variations with cases ranging from asymptomatic to life-threatening. Given the wide variability of coronary anomalies, it is challenging to predict their clinical consequences. Here, we present the 'malignant' variant - interarterial course of the left coronary artery between the aorta and pulmonary trunk - given the highest risk of sudden cardiac death among the various coronary anomalies. CASE PRESENTATION: Our case presents a 22-year-old male presenting to the emergency department after a syncopal episode that occurred while the patient was driving a motor vehicle. Initial Computed Tomography (CT) of the chest performed as part of the trauma work-up revealed a rare case of an anomalous origin of the left main coronary artery (LMCA) originating from a common ostium with the right coronary artery (RCA). The LMCA was found to have a malignant course, as it was positioned between the aorta and pulmonary artery. Given the high risk of sudden cardiac arrest with this congenital variant, the patient underwent coronary artery bypass grafting. CONCLUSION: Anomalous coronary arteries remain the second leading cause of sudden cardiac death in young adult patients. The risk of sudden cardiac death depends on the congenital variant of the anomalous coronary artery as well as the course these vessels take. This case highlights a rare congenital variant featuring both the LMCA and RCA originating from a common ostium, with the LMCA having a malignant course, a variant with the highest risk of sudden cardiac death.

Percutaneous coronary intervention for ventricular fibrillation in the setting of an anomalous right coronary artery.

We present a case of a quadriplegic male who developed ventricular fibrillation associated with an anomalous aortic origin of the right coronary artery. Successful revascularization was achieved with percutaneous coronary intervention. This case highlights the application of an unconventional approach to resolve ischemia in a patient with prohibitive surgical risk.

Anomalous Left Coronary Artery from the Pulmonary Artery: How to Diagnose and Treat.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary anomaly that can present in childhood or adulthood with a multitude of symptoms depending on the age of presentation. It should be suspected in infants presenting with heart failure in the setting of left ventricular systolic dysfunction and associated mitral regurgitation from papillary muscle ischemia. Adults with ALCAPA may present with cardiac ischemic symptoms. Prompt diagnosis with echocardiography and cross-sectional chest imaging is important to guide surgical intervention and improve the patients' survival and prognosis. The goal of surgery is to establish a dual-coronary system with mid-term results revealing progressive recovery of left ventricular function and improvement in mitral regurgitation. Patients with ALCAPA should maintain life-long follow-up with a cardiologist with congenital heart disease expertise for surveillance of post-operative complications.

Application of Patient-Specific Computational Fluid Dynamics in Anomalous Aortic Origin of Coronary Artery: A Systematic Review.

Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart condition with fixed and dynamic stenotic elements, potentially causing ischemia. Invasive coronary angiography under stress is the established method for assessing hemodynamics in AAOCA, yet it is costly, technically intricate, and uncomfortable. Computational fluid dynamics (CFD) simulations offer a noninvasive alternative for patient-specific hemodynamic analysis in AAOCA. This systematic review examines the role of CFD simulations in AAOCA, encompassing patient-specific modeling, noninvasive imaging-based boundary conditions, and flow characteristics. Screening articles using AAOCA and CFD-related terms prior to February 2023 yielded 19 publications, covering 370 patients. Over the past four years, 12 (63%) publications (259 patients) employed dedicated CFD models, whereas 7 (37%) publications (111 patients) used general-purpose CFD models. Dedicated CFD models were validated for fixed stenosis but lacked dynamic component representation. General-purpose CFD models exhibited variability and limitations, with fluid-solid interaction models showing promise. Interest in CFD modeling of AAOCA has surged recently, mainly utilizing dedicated models. However, these models inadequately replicate hemodynamics, necessitating novel CFD approaches to accurately simulate pathophysiological changes in AAOCA under stress conditions.