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Coronary anomalies may be classified according Angelini et al. Another classification divides coronary anomalies into hemodynamically significant and non-hemodynamically significant. Anomalies classified as hemodynamically significant include: 1 anomalies of origination with interarterial course; 2 anomalous origin in the pulmonary artery; 3 atresias; 4 congenital fistulas 8. The origination and proximal course of the anomalous coronary arteries constitute the main prognostic factors. Figures 2 to 4 demonstrate the main anomalies of coronary arteries origination and course, and Figure 5 depicts a sagittal oblique chest computed tomography image showing the four proximal courses that a coronary with anomalous origination may take, as described below.
A pathway between the aorta and the pulmonary arterial trunk.
It is the most frequent type of hemodynamically significant anomaly 8. Several causes are suggested to explain the higher incidence of sudden death in individuals with this type of anomaly. Some authors argue that the vessel with this course would be prone to obstruction during exercise, due to compression by the aorta and the pulmonary artery; but this hypothesis is rejected by some 8.
Course between the posterior region of the aorta noncoronary sinus and the interatrial septum. No vascular structure is found in this region. In spite of not being associated with the hemodynamic repercussion, it plays a relevant role in cases of cardiac valve surgery. It is generally related to origination anomalies of the left coronary trunk and of the circumflex artery 8. Anterior course to the right ventricle output tract and to the pulmonary arterial trunk, most commonly associated with anomaly of the left coronary trunk. It may be associated with angina, but generally with no hemodynamic repercussion.
The most frequently found anomalous coronary arteries with transseptal pathway are the left coronary trunk and the anterior descending artery 8.
Article - Normal Coronary Anatomy and Anatomic Variations
The main types of origination and course anomalies are highlighted, according to the classification proposed by Angelini et al. Separated originations of the anterior descending and circumflex arteries are not frequently found 0. They may cause difficulties in catheterization during angiography, but they allow for the development of collateral circulation in the event of proximal obstruction in one of those vessels 2.
It is associated with a higher incidence of myocardial bridging and left dominance 4 Figure 6. Axial coronary computed tomography angiography and MIP reconstruction showing the origination of the anterior descending artery DA and circumflex CX artery directly from the left coronary sinus.
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Ao Asc, ascending aorta. It is defined as origin of a coronary artery or left coronary trunk more than 1 cm above the sinotubular junction 8. It usually does not present with clinical repercussion, however the preoperative identification of this anomaly is important in case of ascending aorta surgery and may cause difficulties in catheterization during angiography. Most frequently, it occurs in the right coronary artery, sometimes in association with a bicuspid aortic valve 8 Figure 7. Anomalous origination of the coronary artery from the pulmonary arterial trunk.
It is among the differential diagnoses for marked cardiomegaly in the neonatal period 8. The most common presentation is the left coronary trunk originating from the pulmonary artery and the right coronary artery originating from the aorta Bland-White-Garland syndrome 2. Such an anomaly leads to myocardial ischemia due to the coronary steal phenomenon, where the flow is redirected from the high-pressure system of the right coronary artery to the low-pressure pulmonary system by means of right coronary-left coronary collaterals 4 , 8.
In the literature, there are reports of late presentation of such syndrome in adults, probably caused by development of collaterals from the right coronary artery 4. Right coronary artery originating from the left coronary sinus or as a branch of a single coronary artery is found in 0. Fig 8A: Axial coronary computed tomography angiography with MIP showing anomalous origination of right coronary artery CD from the left coronary sinus SCE , with interarterial course between the pulmonary artery trunk TP and the ascending aorta Ao Asc malignant course.
Fig 8C: Axial coronary computed tomography angiography with MIP showing another patient with anomalous origination of right coronary artery CD from the left coronary sinus and interarterial course between the pulmonary artery trunk TP , anteriorly, and ascending aorta Ao ASC , posteriorly malignant course. CX, circumflex artery. Left coronary trunk originating from the right coronary sinus or as a branch of a single coronary artery occurs in 0. Fig 9A: Benign course. Axial coronary computed tomography angiography with MIP showing anomalous origination of left coronary trunk TCE from the right coronary sinus SCD , with retroaortic course between the ascending aorta Ao Asc , anteriorly, and the left atrium AE , posteriorly.
Fig 9B: Malignant course. Axial coronary computed tomography angiography with MIP showing anomalous origination of the left coronary trunk TCE in the right coronary sinus SCD , with interarterial course between the pulmonary artery trunk TP , anteriorly, and the ascending aorta Ao Asc , posteriorly.
Anterior descending or circumflex arteries originating from the right coronary sinus. The circumflex artery is the one that most commonly presents anomalous origin, occurring in 0. Retroaortic pathway is its most common course, and there is no association with sudden death 2 Figure Axial coronary computed tomography angiography with MIP showing anomalous origination of the circumflex artery CX from the right coronary sinus SCD , with retroaortic course between the ascending aorta Ao Asc , anteriorly, and the left atrium AE , posteriorly.
The right coronary artery CD and the anterior descending artery DA have normal origination from the right and left coronary sinuses, respectively. The anterior descending artery with anomalous origin rarely occurs in individuals with a normal cardiac anatomy. A single coronary artery originates from a single aortic root ostium. This is an extremely rare anomaly 0. Fig 11A: Oblique axial coronary computed tomography angiography with MIP showing single, short coronary trunk asterisk with calcified atheromatous plaque, originating from the right coronary sinus and giving origin to the right coronary artery CD and the left coronary trunk TCE , which bifurcates into the anterior descending artery DA and the circumflex artery CX , both travelling anteriorly to the pulmonary arterial trunk TP.
Fig 11B: Invasive coronary angiography catheterization identifying the same anatomical division demonstrated at coronary computed tomography angiography. Such patients present with high risk for sudden death as the main trunk courses interarterialy.
Left Coronary Artery (LCA)
In addition, a proximal obstruction in the main trunk might be devastating, due to the unfeasibility of collateral circulation development. The normal coronary artery evetually branchs into a capillary bed in the myocardium. In cases where the coronary artery ends in a cardiac chamber or in a low-pressure vascular structure such as a pulmonary vessel, the steal phenomenon may occur, leading to inappropriate myocardial perfusion 8 Figure Termination in a chamber or low-pressure vessel may cause increase in caliber and tortuosity of the artery.
Most patients are asymptomatic, and the most frequent symptoms and signs include atypical chest pain, dyspnea, exercise-related syncope or pre-syncope, arrhythmia and left ventricular dysfunction 1 , 6. Patients above the age of 30 diagnosed with coronary anomalies in adulthood present a lower risk for sudden death, a fact that is, many times, taken into consideration in the therapeutic decision making process As compared with normal arterial segments, coronary arteries with anomalous course are not more susceptible to obstructive atherosclerotic diseases 5.
The risk for sudden death of athletes presenting with anomalous coronary artery origination is 79 times higher than in non-athlete individuals Imaging methods are essential for the diagnosis of congenital coronary anomalies, since it is practically impossible to make a diagnosis by means of anamnesis, physical examination and electrocardiography, or even functional tests.
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Transthoracic echocardiography presents limitations for such a characterization, especially as performed on adults and in the absence of a purpose-orientes study 1 , Brothers et al. Transesophageal echocardiography may be useful in the characterization of coronary arteries origination and proximal course 9 , but few reports are found in the literature in addition to fact that this is a semi-invasive method, not capable of demonstrating the entire pathway of such vessels 13 , Currently, computed tomography angiography or magnetic resonance imaging are considered as being the goldstandard to demonstrate the coronary anatomy 6 , 11 , Computed tomography angiography detects not only the anomalous origination of such vessels, but also their course and relationship with other mediastinal structures, allowing for multiplanar and volumetric reformations, which play an essential role in the prognosis and evaluation for therapeutic approach 1 , 4.
It is important to highlight that for the characterization of coronary anomalies, a specific protocol for computed tomography angiography with electrocardiographic synchronization is required, since in the absence of synchronization with heartbeats, pulse artifacts may generate images simulating an anomalous origination of the right coronary from the left coronary sinus with interarterial course The disadvantage of computed tomography as compared with magnetic resonance imaging is the utilization of ionizing radiation.
New techniques have allowed for the reduction of radiation doses to even lower levels than those utilized in digital coronary angiography, while maintaining its excellent spatial resolution 4. Magnetic resonance imaging is also a good noninvasive method capable of demonstrating the coronary arteries origination and course, but its spatial resolution is significantly lower than that obtained by the new multidetector computed tomography apparatuses, in addition to its longer acquisition time The 3.
Surgical treatment is generally the approach of choice for coronary anomalies of origination and course. However, the impact of such an approach on the survival of adult patients is still uncertain A high number of authors indicate surgical treatment for anomalies of left coronary artery origination 1 , 7 , 10 - In cases of right coronary origination anomalies, the treatment is more controversial and is usually less agressive, depending on the clinical findings; and there are studies reporting a favorable evolution of some patients without surgical treatment Other described techniques include: 1 unroofing, or fenestration of the intramural coronary segment, considered to be a simple and safe technique with reproducible results 23 ; 2 ectopic artery reimplantation in the correct coronary sinus technically difficult 1 , 7 , Such techniques may yield better long-term outcomes, being utilized in children and in some adults