Is the Prevalence of Coronary Artery Anomalies Different in Countries with Small Populations? - A Retrospective Study in Northern Cyprus.

BACKGROUND: Although the prevalence of coronary artery anomalies (CAA) is due to accidental and rare discoveries, it varies between different countries or geographies. CAA are rare congenital disorders having various clinical definitions. Its prevalence varies in angiographic and autopsy series in adult populations and is approximately 1% in average. While the incidence ranges from 0.2% to 5.64% in coronary angiographic (CAG) studies, it is around 0.3% in autopsy series. We aimed to estimate the frequency of CAA in our patient population. METHODS: The coronary angiographic data of 4099 consecutive adult patients, who underwent CAG between January 2019 and December 2020, were analyzed and retrospectively studied. RESULTS: The mean age of the total patients who underwent CAG was 61.59 ± 13.67 years (range, 18-98 years). CAA were found in 76 patients (1.85% incidence), origin and course anomaly in 62 patients (81.6%), and coronary artery termination anomaly in 14 patients (18.4%). Separate exits of the left anterior descending (LAD) and left circumflex (LCX) coronary artery from the left sinus of Valsalva (LSV) were the most common anomalies (36.84%). Coronary artery fistulas were seen in 14 (18.42%) patients. Abnormal origin of left circumflex artery (LCX) from the right coronary artery (RCA) or right sinus valsalva (RSV) was seen in 13 (17.11%) patients. Outflow anomalies from the contralateral coronary sinus were detected in 10 (13.16%) patients. CONCLUSION: The incidence and pattern of CAA in our patient population showed similarity with previous studies. Physicians should be aware of CAA that may be associated with potentially serious cardiac incidents, because recognition of these CAA is important for the decision of treatment procedures.

Anomalous origins of the coronary arteries: current knowledge and future perspectives.

PURPOSE OF REVIEW: The purpose of this review is to survey the contemporary literature surrounding congenital anomalies of origin of the coronary arteries and to identify remaining gaps in knowledge. RECENT FINDINGS: In recent years, lineage tracing analyses and mechanistic studies in model organisms have enhanced our understanding of the normal embryologic development of the coronary arteries, and how disruption of this intricate process can lead to congenital coronary anomalies. The true incidence of these anomalies remains unknown. Although a majority of cases are believed to be clinically silent, clinical presentation varies widely, from asymptomatic to sudden cardiac death. Cardiac computed tomography angiography and/or magnetic resonance angiography are the mainstay diagnostic modalities. Management of anomalous coronary arteries depends on the morphology and clinical presentation. Surgery is the gold-standard treatment for anomalous left coronary artery arising from the pulmonary artery and anomalous aortic origin of a coronary artery with intramural or interarterial course. SUMMARY: Several large multicenter initiatives are currently underway and should help address some of the numerous knowledge gaps surrounding the evaluation and management of anomalous coronary arteries.

Anomalías congénitas de arterias coronarias, estudio de aquellas con Importancia hemodinámica / Evaluation of the hemodynamically significant congenital anomalies of coronary arteries

Resumen: Las anomalías de las arterias coronarias son poco frecuentes, con una prevalencia de 0,21- 5,79%. Su presentación clínica es amplia, pudiendo ser asintomáticas o presentarse como isquemia miocárdica y muerte súbita, la que puede estar ligada o no al ejercicio. Existen varias clasificaciones, siendo las más usadas las que las agrupan desde un punto de vista anatómico en relación con el segmento afectado (origen, curso o terminación) y desde el punto de vista funcional (hemodinámicamente significativa y no significativa). Actualmente la Tomografía Computada Cardiaca se considera el estándar de referencia, siendo de suma importancia su adecuada caracterización ya que, de requerir tratamiento, este generalmente es quirúrgico. El objetivo de este trabajo es identificar, caracterizar y clasificar las anomalías congénitas de las arterias coronarias por su importancia hemodinámica mediante la presentación de casos clínicos y revisión de la literatura.

Abstract: Coronary artery anomalies are rare, with a prevalence ranging from 0.21 to 5.79%. Their clinical presentation is variable; being either asymptomatic or presenting as myocardial ischemia and sudden death, which may or may not be linked to exercise. There are several classifications, the most commonly used being those that sort them from an anatomical point of view in relation to the affected segment (origin, course or termination) and from a functional point of view (hemodynamically significant and not significant). Currently, Cardiac Computed Tomography is considered the reference standard allowing an adequate characterization of the anomaly, which is highly relevant since, if treatment is needed, it usually involves surgery. The purpose of this review is to identify, characterize and classify congenital anomalies of the coronary arteries according to their hemodynamic significance through the presentation of clinical cases and review of the literature.

Coding of coronary arterial origin and branching in congenital heart disease: The modified Leiden Convention.

OBJECTIVES: Variations in coronary anatomy are common and may relate to the position of the coronary ostium relative to the aortic sinus, the angle of coronary take-off, or the course of the coronary arterial branches. Several classification systems have been proposed. However, they all lack a simple rationale that is applicable irrespective of the relative position of the great arteries, as well as in bicuspid aortic valves. We present a modification of a relatively simple system introduced in the early 1980s, designated the "Leiden Convention." METHODS: The first step of the Leiden Convention is that the clinician takes position in the nonfacing sinus of the aorta looking toward the pulmonary orifice. The right-hand facing sinus is sinus 1, and the left-hand facing sinus is sinus 2. The coronary branches arising from sinus 1 are annotated proceeding in a counterclockwise fashion toward sinus 2. "Usual" (normal) coronary anatomy would be 1R-2LCx. Given their clinical relevance, single sinus coronary arteries are discussed separately. RESULTS: This system was originally designed and highly applicable in hearts with an altered great artery relationship, such as in the variable and complicated patterns seen in transposition of the great arteries and double outlet right ventricle. The modified system also can be used in cases with normally related great arteries, cases with single sinus coronary arteries, and cases with bicuspid aortic valves. CONCLUSIONS: The modified Leiden Convention is not a strict classification but a simple coronary coding system that is broadly applicable.

Prevalence and Characteristics of Coronary Artery Anomalies Using Invasive Coronary Angiography in 6237 Consecutive Patients in a Single Center in Turkey.

BACKGROUND: Coronary artery anomalies (CAAs) include clinically and anatomically diverse types of congenital heart defects occurring in a complicated spectrum. The aim of the present study is to describe congenital anomalies of coronary arteries (CAs) and their variations, and also identify the prevalence of these anomalies in our center. METHODS: The study included a total of 6237 consecutive patients. Cine-angiographies and digital data of all patients undergoing coronary angiography (CAG) were reviewed by at least 2 independent and experienced observers. The Basic Anatomic Classification method was used to classify patients with CAAs. RESULTS: Of a total of 6237 patients, 2,313 were females (37.1%) and 3924 (62.9 %) were males. CAAs were detected in 247 out of 6237 patients (3.9%, 95% CI: 3.4-4.4). Of these patients, 48 (19.4%, 95% CI: 14.4-24.2) had absent left main coronary artery (LMCA), 21 (8.5%, 95% CI: 5-11.9) had anomalous origin from the appropriate sinus, 23 (9.3%, 95% CI: 5.7-12.9) had anomalous origin from structures other than appropriate sinus, 31 (12.6%, 95% CI: 8.5-16.6 ) had anomalous origin from the opposite sinus, 101 (40.9%, 95% CI: 34.8-46.9) had myocardial bridge, and 23 (9.3%, 95% CI: 5.7-12.9) had a coronary artery fistula. CONCLUSION: Our study results suggested that the total rate of coronary anomaly was found much higher than those reported in various invasive angiographic studies.

A New Variant of Dual Left Anterior Descending Artery Anomaly: Type XI.

A dual left anterior descending (LAD) artery is a rare congenital anomaly which is classified into different types based on the origin, course and termination of the short and long LAD arteries. To date, 10 variants of dual LAD artery anomalies have been described. We report a 44-year-old woman who was referred to the Department of Radiology, Royal Hospital, Muscat, Oman, in 2017. Coronary computed tomography angiography revealed a dual LAD artery anomaly in which the short and long LAD arteries shared a common ostium with the right coronary artery from the right coronary sinus. To the best of the authors' knowledge, this type of variant has not been previously reported in the literature.

Anatomic types of anomalous aortic origin of a coronary artery: A pictorial summary.

Anomalous aortic origin of a coronary artery is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. As commonly seen in congenital heart disease, a wide spectrum of anatomic variability is seen and hence, it is important for clinicians to document the precise anatomy and course of the anomalous vessel. This article describes coronary artery nomenclature using computerized tomography angiography and virtual angioscopy. These details are important for decision making, useful for surgical planning, and may have prognostic implications.

Interobserver variability in the classification of congenital coronary abnormalities: A substudy of the anomalous connections of the coronary arteries registry.

OBJECTIVE: The diagnosis of anomalous connections of the coronary arteries (ANOCOR) requires an appropriate identification for the management of the patients involved. We studied the observer variability in the description and classification of ANOCOR between a nonexpert group of physicians and a group of expert physicians, using the ANOCOR cohort. PATIENTS AND DESIGN: Consecutive patients identified by 71 referring cardiologists were included in the ANOCOR cohort. Anomalous connection was diagnosed by invasive and/or computed tomography coronary angiography. Angiographic images were reviewed by an angiographic committee with experience in this field. Both investigators and angiographic committee filled out a questionnaire to classify each anomaly with the type of coronary artery involved, the site of anomalous connection, and the initial course. Observer variability between investigators and angiographic committee was assessed by κ statistics. Anomalous connection with a preaortic course was defined as at-risk. RESULTS: Among 472 patients of the ANOCOR cohort, 496 abnormalities were identified with a preaortic course present in 31%. The agreement for the type of artery was excellent (κ = 0.92, 95% CI = 0.86-0.98, P < .05), while the agreement for the site of anomalous connection was moderate (κ = 0.50, 95% CI = 0.42-0.58, P < .05), and the agreement for the initial course was only fair (κ = 0.32, 95% CI = 0.28-0.37, P < .05). Observer agreement for the identification of at-risk forms was moderate (κ = 0.497, 95% CI = 0.40-0.59, P < .05). CONCLUSIONS: Observer variability in the assessment of anomalous connection of the coronary arteries between nonexperienced and experienced physicians can be significant. We found that expert physicians provide a more robust classification in comparison with nonexpert physicians. Therefore, referral to physicians with a relevant experience should be considered, especially if an anomaly at-risk is suspected.

Anatomical Classifications of the Coronary Arteries in Complete Transposition of the Great Arteries and Double Outlet Right Ventricle with Subpulmonary Ventricular Septal Defect.

Objective To discuss the anatomical morphologies of the coronary arteries and frequencies of unusual coronary arteries in complete transposition of the great arteries and double outlet right ventricle (DORV) associated with a subpulmonic ventricular septal defect (VSD). Methods Between March 1999 and August 2012, 1,078 patients with complete transposition of the great arteries or DORV with subpulmonary VSD underwent arterial switch operations (ASOs) and were visually evaluated to classify their coronary artery morphology during open heart surgery. Results The coronary arteries could be classified into five patterns with several subtypes. Unusual coronary arteries were observed in 248 of the 1,078 cases, providing a frequency of 23.01%. The frequencies of the patients with transposition of the great arteries with intact ventricular septum (TGA/IVS), TGA/VSD, and DORV with subpulmonary VSD were 17.65, 23.28, and 31.84%, respectively. The most common morphologies were the right coronary artery (RCA) originating from sinus 1 and circumflex (CX) originating from sinus 2 (1R, AD; 2CX; 26.50%); the CX originating from sinus 2 (1AD; 2R, CX; 21.36%); the RCA, left anterior descending artery, and CX originating from single sinus 2 (2R, AD, CX; 13.24%). The in-hospital mortalities of the patients with or without unusual coronary arteries after ASO were 14.1 and 6.02%, respectively. Conclusion Patients with complete transposition of the great arteries or DORV with subpulmonary VSD have a high frequency of unusual coronary arteries, which might greatly impact on the mortality for ASO. Improving the preoperative diagnostic criteria for coronary artery morphology may significantly increase the success rate for ASOs.

The Coincidence of 3 Different Rare Coronary Artery Anomalies in an Adult Patient With Untreated Kawasaki Disease: Case Report.

The coincidence of 3 different rare coronary artery anomalies is extremely rare, and has not been reported so far.We report multiple imaging findings of a giant coronary artery aneurysm, which has a fistulous connection to the right ventricle associated with anomalous origin of the anterior descending coronary artery from the right coronary artery in a 67-year-old woman who suffered with a 20-year history of progressively chest distress on exertion and a history of untreated Kawasaki disease in her childhood.The patient received surgical treatment. The aneurysm was resected and openings at both ends being oversewn. And the fistula was also closed directly. She recovered and discharged uneventfully.The coincidence of 3 different rare coronary artery anomalies in adult patient with untreated Kawasaki disease is a rare and complicated condition, in which surgical treatment is recommended.

Reporting a novel variant of type VI dual left anterior descending artery: a rare coronary anomaly.

A dual left anterior descending artery (LAD) is a rare congenital coronary anomaly and is classified into six types based on the origin and course of the long LAD. In type VI dual LAD, the short LAD arises from the left main coronary artery, and the long anomalous LAD, from the proximal right coronary artery (RCA), coursing between the right ventricular outflow tract and aortic root to reach its normal course in the mid interventricular groove. We present a novel variant of type VI dual LAD where the course of the long LAD is the same but the origin is not from the RCA but, instead, separately, from the right coronary sinus.

Anomalous origin of coronary artery: taxonomy and clinical implication / Origem anômala da artéria coronária: taxonomia e implicação clínica

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

Objetivo: A origem anômala da artéria coronária é incomum. As taxonomias de origem anômala da artéria coronária são inconsistentes e complexas. Os debates conceituais e terapêuticos permanecem. O objetivo do presente estudo é reavaliar o conceito de origem anômala da artéria coronária e discutir os riscos potenciais e fundamentos para o tratamento desta anomalia, com base em revisão de literatura. Métodos: A revisão da literatura foi feita com termos das taxonomias, incluindo origem anômala da artéria coronária “simples”, “múltipla” e “complexa”. Resultados: A origem anômala da artéria coronária pode ser simplesmente classificada de acordo com a origem ectópica da artéria coronária. Há um par de variações anatômicas complexas: “múltipla”, envolvendo mais de uma artéria coronária ou ramo, que podem ser subdivididos em dois subtipos: A) mais de uma das artérias coronárias ou ramos decorrentes de um lugar; e B) duas artérias coronárias/ramos decorrentes de sítios ectópicos separados; e tipo “complexo”, associado à doença cardíaca adquirida ou defeitos cardíacos congênitos. Conclusão: A morte súbita cardíaca de origem anômala da artéria coronária está associada com as características anatômicas incluindo curso anormal, descolamento de ângulo agudo e anormalidades ostiais. A aterosclerose é propensa a ocorrer na artéria coronária ectópica e retroaórtica, do lado direito. O tratamento cirúrgico é uma terapia definitiva. Revascularização do miocárdio simples não é recomendada devido aos riscos potenciais do fenômeno de “roubo” do fluxo coronário e patência ruim dos enxertos com a artéria torácica interna. Manobras modificadas como reimplante ostial coronário, destelhamento do segmento coronário impingido e implantação de stent coronário são defendidas em seu lugar. .

Congenital coronary artery anomalies for the pathologist.

Congenital coronary artery anomalies are rare. Pathologists are exposed to those in mainly two settings; in association with sudden death and usually extreme exercise in young adults, and in association with complex congenital heart disease in the pediatric and perinatal population. Pediatric pathologists, other pathologists and pathologists' assistants performing pediatric or forensic autopsies therefore need to be familiar with coronary artery anomalies.

Prevalence and types of coronary artery fistulas detected with coronary CT angiography.

OBJECTIVE: The prevalence of coronary artery fistula (CAF) based on coronary angiographic findings has been reported. However, the number of incidentally found CAFs is increasing as coronary CT angiography (CTA) has become popular. The purpose of this study was to determine the prevalence and types of CAFs detected with coronary CTA. MATERIALS AND METHODS: Between March 2009 and November 2011, 6341 patients underwent coronary CTA at one institution. The prevalence of CAF was retrospectively evaluated, and the morphologic features were analyzed, including vessel of origin, drainage site, size, and presence of an aneurysmal sac. We also analyzed cardiac and pulmonary findings. RESULTS: Among 6341 patients, 56 (0.9%) patients had CAF. The types of CAF detected, in decreasing frequency, were coronary to pulmonary artery fistula (43 cases [76.8%]), coronary to bronchial artery fistula (five cases [8.9%]), coronary artery to cardiac chamber fistula (five cases [8.9%]), combined coronary to pulmonary and coronary to bronchial artery fistula (two cases [3.6%]), and coronary artery to superior vena cava fistula (one case [1.8%]). Lung parenchymal or vascular anomaly was more frequently noted in coronary to bronchial artery fistulas, combined coronary to pulmonary and coronary to bronchial artery fistulas, and coronary artery to superior vena cava fistulas than in coronary to pulmonary artery and coronary artery to cardiac chamber fistulas. CONCLUSION: The prevalence of CAF at coronary CTA was 0.9%, which is higher than the known prevalence based on conventional angiographic findings (0.05-0.25%). Furthermore, the most common type of CAF in this study was coronary to pulmonary artery, whereas coronary artery to ventricle fistula was previously considered the most common type in studies conducted with conventional angiography. Coronary CTA is a useful, noninvasive imaging modality for the detection of CAF.

Coronary anomalies encountered in the acute setting: an imaging review.

A broad spectrum of congenital coronary anomalies may be discovered on imaging and sometimes in the emergency setting on computed tomography (CT). Most coronary artery anomalies are of academic interest; however, a minority can cause morbidity and mortality and symptoms such as angina, myocardial infarction, or arrhythmias. These anomalies are usually discovered as an incidental finding on CT examinations as part of the diagnostic workup for other pathology or on dedicated coronary computed tomography angiography (CCTA) as part of the evaluation for a coronary cause of chest pain. The purpose of this pictorial review is to demonstrate the types of coronary anomalies and to enhance the clinicians' understanding of the imaging classifications and clinical implications.