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imaging modality as well. Due to the complexity of CHD, imaging specialists should understand the importance of proper patient and protocol selection for CCTA and, ideally, collaborate with adult congenital specialists to help plan and successfully execute the CCTA examination.
Coronary Artery Anomalies
Incidence and Defi nitions
Congenital coronary anomalies affect between 0.2 and 5.6 % of the general population [ 108112 ]. The incidence varies con- siderably between studies due to a number of factors including referral bias, fewer autopsies being performed and absence of strict diagnostic criteria. In addition, coronary anomalies can vary widely in severity. The presenting symptom for some anomalies may be sudden cardiac death; others may be entirely benign and never diagnosed. Some authors have reported that coronary anomalies may cause anywhere between 12 and 19 % of deaths among young athletes [ 113115 ].
In the normal coronary circulation, the left main coronary artery arises from the left ostium (located centrally within the left sinus of Valsalva) and gives rise to the left anterior descending artery and circumfl ex artery. The left anterior descending artery courses along the anterior interventricular groove, gives off septal perforators and supplies most of the septum, anterior wall and apex while the circumfl ex artery wraps posteriorly around the left atrioventricular groove. The right coronary artery arises from the right ostium (cen­trally located within the right sinus of Valsalva), giving off the marginal branch to the right ventricle and then curving posteriorly in the atrioventricular groove. Angelini and colleagues [ 116 ] suggest, in the context of known normal coronary anatomy, the following defi nitions: normal (seen in >1 % of the general population), normal variant (uncommon variant but seen in >1 % of the population) and anomaly (observed in <1 % of the population).
Clinical Signifi cance
Congenital coronary anomalies have been reported as the cause of chest pain, arrhythmias, myocardial infarction and sudden cardiac death. Yamanaka and colleagues [ 108 ], in a survey of 126,595 angiograms, classifi ed 80 % of anomalies as “benign” and 20 % as being potentially responsible for serious sequelae. The literature has consistently reported an association between certain coronary anomalies (such as anomalous left coronary artery from the pulmonary artery or origin of the left coronary artery from the right sinus of Valsalva) and adverse clinical outcomes. Such coronary variants may account for up to 15 % of sudden cardiac deaths
in athletes [ 114 , 116 ]. Outcomes in milder forms of coronary anomalies (i.e. “normal variants”) are less well defi ned. Coronary artery anomalies may also occur in concert with congenital heart defects (discussed later in this chapter).
Selected Lesions
Abnormal Coronary Origins: ALCAPA and ARCAPA
Anomalous origin of the left coronary from the pulmonary artery (ALCAPA, also called the Bland-White-Garland syn­drome) is a serious anomaly in which the left coronary artery arises from the pulmonary artery [ 117 ] (See Fig. 23.16 ). During fetal life, due to elevated pulmonary vascular resis­tance, myocardial perfusion is thought to be normal. However, upon birth, the pulmonary vascular resistance begins to fall, leading to insuffi cient perfusion of the left ventricle. Typically there is vigorous collateral formation from the right coronary artery but despite this, the majority present in infancy with symptoms of congestive heart failure. Others may never have symptoms and there are reports of patients reaching adulthood [ 118121 ] though it appears to still confer a risk of sudden cardiac death.
Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a much rarer anomaly which also has been associated with sudden cardiac death [ 122 ].
Coronary Artery Origin from the Improper Sinus Including Single Coronary Arteries
Coronary arteries may be seen to arise anomalously from the incorrect sinus, with an incidence of 0.92 % for origin of the right coronary artery from the left sinus and 0.15 % for left coronary artery from the right sinus [ 123 ]. Single coronary arteries are even rarer, occurring in approximately 0.024–
0.066 % of the population [ 108 , 124 , 125 ]. Although single coronary arteries are an isolated fi nding in the majority of cases, they may be associated with other cardiac defects or cause sudden cardiac death [ 125 , 126 ].
In broad terms, coronary arteries arising from the incor­rect sinus (including single coronary arteries) may take a number of routes including retrocardiac, retroaortic, infra­septal (supracristal), pre-pulmonary (crossing the right ven­tricular outfl ow tract) or intra-aortic (between the aorta and pulmonary artery) [ 123 ] (See Figs. 23.17 , 23.18 , 23.19 and
23.20 ). Of these, the intra-aortic course has been most consistently implicated as a possible cause of sudden cardiac death. The ostium of the anomalously placed artery may be slit-like, possibly predisposing to compression between the great arteries [ 127129 ]. Sudden cardiac death, when it occurs, is often precipitated by effort [ 129 ], which likely explains the increased incidence in athletes [ 113 , 115 , 130133 ].
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Normal Variants and Minor Coronary Anomalies
Other coronary variants with minor or no signifi cant clinical sequelae include intramural course (also known as “myocardial bridging”), “absent” left main (better described
as dual origin of the left anterior descending and left circum­fl ex arteries) and geographical variations of the coronary ostia within the appropriate sinus (See Fig. 23.21 ).
Coronary Anomalies in Congenital Heart Disease
Multiple coronary anomalies have been reported in patients with d-transposition of the great arteries (d-TGA) [ 134 ]. Most frequently, the left coronary artery arises from the “anterior facing” sinus and the right coronary artery arises from the “posterior facing” sinus. As in normal hearts, there may be single coronary arteries, multiple coronary ostia or coronary arteries arising from an improper cusp. In patients who have undergone the arterial switch procedure (in which the coronary arteries are re-implanted into the neo-aorta), coronary events are not infrequent with a preva­lence of 2–11 % [ 135140 ]. Thus, it is critically important to evaluate on CCTA the coronary arterial origins and course to exclude kinking, compression and intimal thick­ening [ 135 , 141 , 142 ].
Patients with tetralogy of Fallot also commonly have cor­onary anomalies including single coronary arteries in 5 %, coronary arteries with anomalous origins in 5 % and a large conus artery in anywhere from 20 to 40 % [ 73 , 74 , 143 ]. Of particular concern is the variant in which the left anterior descending artery arises from the right coronary artery, crossing the right ventricular outfl ow tract in the process. This can pose diffi culties during surgery as the left anterior artery will be at risk when the chest is opened. Careful assessment for coronary anomalies in this population can
ab
Fig. 23.16 A 2 month old infant presented with failure to thrive. Axial views ( a ) and ( b ) demonstrating the LAD arising from the pulmonary artery (anomalous left coronary artery arising from the pulmonary artery or ALCAPA) (Note that the LCX arises normally from the aorta
(though its origin is posteriorly displaced) and the right coronary artery is dilated. AO aorta, PA pulmonary artery, LAD left anterior descending artery, RCA right coronary artery, LCX left circumfl ex artery
Fig. 23.17 This three-dimensional reconstruction demonstrates a sin­gle coronary artery, arising from the right coronary cusp. RCC right coronary cusp, LAD left anterior descending artery, RCA right coronary artery
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help guide surgical repair [ 144 ] and should therefore be excluded prior to any intervention to the right ventricular outfl ow tract.
Congenitally corrected transposition (l-TGA) typically
follows a similar pattern to d-TGA, in which the two main
coronary ostia arise from the “facing” sinus. As the coro­nary arteries not infrequently arise from the improper sinus, it may be helpful to describe both the origin of the anoma­lous artery in addition to the territory supplied. The left coronary artery will, as in normal individuals, supply the
ab
Fig. 23.18 ( a , b ) ( a ) Shown in this three-dimensional reconstruction is an anomalous right coronary artery originating from the left coronary cusp. ( b ) Curved multi-planar reconstruction demonstrates the slit-like
origin ( arrow ) of the right coronary artery. LCC left coronary cusp, LAD left coronary artery, RCA right coronary artery
ab
Fig. 23.19 ( a , b ) Three-dimensional reconstructions demonstrates a single coronary artery variant in which the left anterior descending artery courses anterior to the pulmonary artery and the circumfl ex
artery travels behind the aorta. AO aorta, PA pulmonary artery, LCX left circumfl ex artery, LAD left coronary artery, RCA right coronary artery
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morphologic left ventricle but arises from the “right facing” sinus. The right coronary artery will supply the morpho­logic (systemic) right ventricle but arises from the “left fac­ing” sinus [ 145 ] .
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Fig. 23.20 ( a , b ) ( a ) Three-dimensional reconstruction shows an anomalous right coronary artery arising from the left coronary cusp, following an intra-arterial course. ( b ) Curved multiplanar reconstruc-
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