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.

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.

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. .

Angioplastía de circunfleja en paciente con doble arteria descendente anterior tipo IV: Propuesta para actualizar la clasificación de Spindola-Franco / Circumflex angioplasty in a patient with type IV dual left anterior descending coronary artery: Proposal for updating the classification of Spindola-Franco

La anatomía normal de las arterias coronarias incluye una serie de variantes respecto a su origen, distribución y recorrido, que hacen que no existan 2 pacientes con coronarias exactamente iguales. La arteria descendente anterior puede tener variantes anatómicas que no afectan el aporte normal de sangre al territorio miocárdico correspondiente. En nuestros 25 años de trabajo y más de 11,000 coronariografías realizadas, sólo habíamos encontrado un paciente con un doble sistema, tipo IV, de la arteria descendente anterior. En este artículo mostramos el caso de una paciente con la anomalía coronaria descrita que presentaba, además, una estenosis severa de la arteria circunfleja que fue tratada exitosamente por vía percutánea. También, se propone una actualización de la clasificación de Spindola-Franco, donde se mantienen los 4 tipos originales y se añaden 7 variantes anatómicas o subtipos.

The normal anatomy of coronary arteries includes a kind of variants with regard to its origin, distribution and route, which makes that 2 patients do not have coronary arteries exactly alike. Left anterior descending artery could have anatomical variations that do not affect the normal supply of blood to the corresponding myocardial territory. In our 25 years of experience, and more than 11,000 coronary angiographies, we have found only one patient with a type IV Classification; dual left anterior descending coronary artery. In this article we show the case of a patient with the described coronary anomaly, presenting a severe stenosis of the circumflex artery which was successfully treated percutaneously. Besides, a proposal for updating the classification of Spindola-Franco is made, where the 4 types previously described were kept invariable, and 7 anatomical variants or subtypes were added.

A simplified categorization for common arterial trunk.

OBJECTIVE: A common arterial trunk is a solitary trunk that exits the heart through a common ventriculo-arterial junction and supplies directly the systemic, pulmonary, and coronary arterial pathways. It remains to be determined, however, how best to subclassify those hearts fulfilling this definition. The time-honored classification is based on the morphology of the pulmonary arteries, but an alternative approach also places emphasis on the nature of the systemic pathways. We evaluated our experience to establish whether these different approaches can be reconciled. METHODS: We examined 28 autopsied hearts with common arterial trunks; the specimens were drawn from the archives of three institutions. Based on our analysis, we simplified classification into hearts with aortic or pulmonary dominance. We used this approach to categorize 42 patients who had undergone surgical correction at Children's Memorial Hospital in Chicago. RESULTS: All autopsied hearts could be assigned to groups with either aortic or pulmonary dominance of the common arterial trunk, with 20 and 8 specimens, respectively, fitting into these categories. Pulmonary dominance was found only when the aortic component of the trunk was hypoplastic and an arterial duct supplied the majority of flow to the descending aorta. Only in this setting did we observe pulmonary arteries arising from the sides of the major pathway, and only in this setting was the aortic component discrete from the pulmonary component within the pericardial cavity. CONCLUSIONS: This simple approach to classification reconciles the existing disparate categorizations of patients having common arterial trunks and it emphasizes the principal morphologic determinant of surgical outcome.

Anomalies of the coronary arteries: nomenclature and classification.

Abnormalities of the coronary arterial circulation represent a broad spectrum of anomalies. Some of these coronary arterial anomalies are of little clinical significance and are generally discovered either as incidental findings at autopsy or at the time of diagnostic coronary angiography in patients with atherosclerotic ischaemic cardiac disease. Some of these coronary arterial anomalies, however, are associated with a markedly abnormal natural history that may include significant cardiac morbidity very early in infancy, or increased risk of cardiac sudden death in childhood or adolescence. It is therefore important that we have a system for the naming and classification of these anomalies, to stratify them according to the associated risk and to develop rational approaches to evaluation and management. To apply any system of nomenclature reliably and responsibly, it is essential that each term be associated with a definition that is known and accepted by users of terminology. Such definitions are included in the specifications of both the Society of Thoracic Surgeons Congenital Heart Surgery Database and the European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Database. These databases use the Diagnostic Short List of the version of the International Paediatric and Congenital Cardiac Code derived from the nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons. This article will provide the nomenclature and definitions used by these databases for anomalies of the coronary arteries.

Surgical management of congenital coronary arterial anomalies in adults.

Congenital anomalies of the coronary arteries are an uncommon, but important, cause of pain in the chest, myocardial ischaemia and even sudden cardiac death, especially in young individuals. This paper focuses on the surgical treatment of congenital anomalies of the coronary arteries in adults; indications for surgery and the different surgical options will be reviewed.

Coronary artery anomalies in adult patients.

BACKGROUND: Coronary artery anomalies are discovered in less than 1% of angiography series. Since the number of angiographies and coronary bypass operations are increasing significantly every day, these anomalies are of clinical importance. MATERIAL/METHODS: 58,023 coronary angiographies were performed in the cardiology clinic of our hospital from 1978 to 2001. Coronary artery anomalies were discovered in 257 of these cases (0.44%). The mean age of these patients was 51.9+/-11.4 years (18-82). 80% were male (n=207). RESULTS: The circumflex artery (CXA) was the most frequently involved vessel (51.1%). Coronary arteries originating from the pulmonary artery were not encountered in our series due to the natural history of the disease. In 54 patients with coronary artery fistulae, 18 (33.3%) were closed by operation. Perioperative mortality was 5.5%. Acquired coronary artery fistulae or aneurysms due to trauma or inflammatory diseases are completely different entities and beyond the scope of this article. In 76 patients, open heart surgery was required for additional lesions, 57 of which were coronary artery bypass grafts. CONCLUSIONS: Being usually asymptomatic, coronary artery anomalies are usually discovered incidentally in the adult population. These pathologies are important for practical purposes, especially for interventional cardiologists, radiologists and cardiac surgeons, who should be aware of these anatomical entities. LMCA originating from the right coronary system has been reported to result in sudden death and myocardial ischemia, so these mostly asymptomatic patients must be followed closely.

Repair of an anomalous left coronary artery.

We describe a patient with an anomalous single coronary artery who presented with a syndrome of atypical chest pain. Coronary angiography revealed a single right coronary ostium, with a narrowed left coronary artery originating at the right coronary ostium. The proximal portion of the left coronary artery that was narrowed was noted to run in the aortic wall. We describe the operative management of this patient using ostial remodeling.

Surgical management of congenital obstruction of the left main coronary artery with supravalvular aortic stenosis.

OBJECTIVE: Stenosis of the left main coronary artery is a recognized complicating feature of supravalvular aortic stenosis. We have retrospectively identified three anatomic subtypes of left main coronary obstruction in patients with supravalvular aortic stenosis, each necessitating a distinct surgical approach. METHODS: From 1991 to 1998, 9 patients underwent surgical repair of supravalvular aortic stenosis and left main coronary stenosis. Five patients (group 1) had obstruction from near-circumferential thickening of the left main ostium, 2 patients (group 2) had restricted coronary flow due to fusion of an aortic valve leaflet to the supravalvular ridge, and 2 patients (group 3) had diffuse narrowing of the left main coronary artery. Group 1 patients were treated with patch aortoplasty encompassing the left main ostium and supravalvular aortic stenosis. Group 2 patients were treated with excision of the fused leaflet from the aortic wall and patch aortoplasty. Group 3 patients were treated with bypass grafting and aortoplasty. RESULTS: Surgical strategy was determined by coronary angiography and intraoperative assessment of coronary anatomy. There was 1 early death. All surviving patients underwent echocardiography with or without postoperative catheterization. The mean postoperative supravalvular gradient for 7 patients was 8 mm Hg (range 2-15 mm Hg). One patient required reoperation for a residual aortic gradient as a result of aortic arch involvement. No evidence of left main coronary artery stenosis was seen in groups 1 and 2; bypass grafts were patent in group 3 patients at a mean follow-up of 54.8 months. CONCLUSION: Three subtypes of left main coronary stenosis with supravalvular aortic stenosis are described. Each anatomic type mandates an individual surgical approach. Favorable surgical outcomes are achievable with each category.

Classification of origin and proximal epicardial course of coronary arteries in transposition of the great arteries.

The complexity of the variability in aortic origin and epicardial course of the coronary arteries relative to the pedicles of the great arteries in hearts with transposition of the great arteries is evident in that there is no simple classification to include all the possible variations of the coronary artery patterns. Therefore, a simplified and yet all-inclusive classification is proposed in which the origin of the coronary arteries is defined as seen by an observer looking from the pulmonary artery toward the aorta. This promote and classification should expedite and promote the international exchange of data regarding coronary artery anatomy in transposition of the great arteries and should assist in the surgical management of this condition.

Anatomy of the proximal coronary arteries as a risk factor in primary repair of common arterial trunk.

The objective of this study was to investigate whether the proximal coronary arterial anatomy is a risk factor in surgical treatment of common arterial trunk, with special focus on the value of preoperative angiocardiography. A retrospective analysis was performed of all 22 patients who underwent primary surgical repair of the common arterial trunk, with a mean follow-up of 5.1 years. In 18 patients preoperative angiocardiography was performed. Anatomical features (angiocardiographical, surgical as well as post-mortem) of the proximal coronary arteries were investigated. With standard biplane angiocardiography single and dual coronary arterial systems could adequately be distinguished. However, the position of the coronary orifices in relation to the sinus of Valsalva could not adequately be identified. Three patients had coronary abnormalities without surgical consequences. In 2 cases the surgical approach had to be modified due to the coronary anatomy. Early mortality was 23% (5/22) and was correlated with worse functional class (p < 0.05) and earlier date of operation (p < 0.05). Late mortality was 5% (1/22). Five patients were reoperated, without mortality. Fourteen surviving patients are in functional class I, and 2 in class II. A further improvement of the surgical therapy of common arterial trunk might be provided by adequate appreciation of the proximal coronary arterial anatomy at surgery.