Normal and anomalous coronary arteries: Definitions and classification

CURFKULUM

IN CAROlOLOGY

Normal and anomalous coronary Definitions and classification Paolo Angelini,

MD. Houston,

Texas

Coronary anomalies have intrigued clinicians, anatomists, and physiologists for years. Since the introduction of selective coronary angiography in the early 196Os, this segment of the systemic circulation has become a common subject of exploration. Currently, the United States alone, some 900,000 patients per year are being subjected to selective coronary angiography, and more than 300,000 per year are undergoing coronary angioplasty or bypass surgery. Between 1966 and 1987, coronary anomalies were the’ subject of more than 500 English-language articles cited in index Me&us. Whereas several different systems for classifying coronary anomalies have been proposed in the literature,1-23 no clear definition of normal coronary arteries has yet been proposed, and no uniform taxonomic criteria for identifying coronary anomalies have been established. This article surveys current knowledge concerning the embryogenesis of coronary arteries, proposes to define “normality” for such arteries, and suggests an organic method of classifying coronary anomalies, based on an extensive review of the pertinent literature. EMllBRYOLOGY

The genesis of the coronary circulation has received scant attention in the embryology literature.24-31 The available reports originate from general, descriptive embryologic studies. Experimental embryology has not yet devised a suitable animal model, mainly because of the lateness of the appearance of coronary arterial primordia in the chick embryo. (De la Cruz MV. Personal communication, Aug 1987). Most descriptions of the development of the coronary arterial bed mention three separate components (Fig. 1): (1) The sinusoids, which represent a prolongation of the trabeculae characteristic

From the Department of Cardiology, Texas Heart Institute. Received for publication July 18, 1988; accepted Sept. 1, 1988. Reprint requests: Paolo Angelini, MD, P.O. Box 20269, Texas Institute, Houston, TX 77225.

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of the spongiosa into the developing myocardium. These channels are the primitive sites of metabolic exchanges between the blood contained in the cardiac cavities and the cardiac mesenchyma, which at this time is composed mainly of cardiac jelly, In humans the heart starts beating as early as the twenty-second day after ovulation (horizon X of Streeter), and ebb-and-flow circulation of the blood begins a few days later (horizon XI). (2) The “in situ” vascular endothelial network, which appears separately in the subepicardium 31 days after ovulation (horizon XV). (3) The coronary buds (sprouts, or anlagen), which arise from the wall of the aortopulmonary trunk as it completes its division into the aorta and the pulmonary artery (horizons XV to XVI).2* Although most investigators have noted the early presence of coronary buds on the walls of both the aortic and the pulmonary trunks, the number of buds reported by different authors has varied 24-26*28-30 According to recent investigations by Conte’et al.,24 the in situ vascular network may actually induce the development of the coronary buds when it approaches the wall of the truncus arteriosus. During horizon XIX (after the completion of aortopulmonary septation and the formation of the semilunar valves), the second and third components of the coronary arterial bed fuse, and the coronary circulation begins to flow normally2s from the aortic trunk into the myocardial capillary network (and then into the coronary veins). Whereas the coronary ostia are probably formed quite early, soon after truncal septation, the distal coronary pattern remains characterized by a loose, intermingling network until the myocardial masses develop. As the coronary arteries become the predominant source of myocardial metabolic exchanges, after horizon XIX, the distribution and size of the major (epicardial) coronary arteries become strictly related to the extent of their dependent myocardium. A lack of coronary circulation during embryologic development would induce hypoplasia of the dependent myocardium; conversely, a relative reduction in the

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dependent myocardial mass would cause relative “hypoplasia” of its coronary branch. An extreme example of this phenomenon can be studied by observing the coronary circulation in cases of common ventricle, a congenital disorder in which the ventricular septum does not develop and the left anterior descending artery is absent. It is quite improbable, on embryologic grounds, that a true mismatch between the dependent myocardium and its related coronary arteries could occur. Therefore hypoplasia or atresia of a coronary artery or branch, or “missing” coronary arte~y,~~-~ is frequently a misnomer.37p38 Either the dependent myocardium is also hypoplastic (to designate such cases, one could suggest the term “true or secondary hypoplasia”) or, more commonly, the opposite coronary artery is relatively oversized (in which case it represents an alternative coronary pattern39*40). In both events the coronary circulation is effectively normal in global physiologic terms, and the observer is merely encountering a comparatively infrequent coronary arterial pattern. The fact that the coronary arteries develop after septation of the aortopulmonary truncus makes anomalous coursing of a coronary artery in the aortopulmonary septum embryologically “impossible.” Nevertheless, the literature includes cases in which a right coronary artery originates from the left coronary cusp and appears to cross the space between the aorta and the pulmonary artery (or vice versa in cases involving an anomalous left coronary artery or branch). In these unusual instances the anomalously coursing artery may actually cross the myocardium underlying the semilunar valves (the conal septum) rather than the aortopulmonary septum. This hypothesis is strengthened by the fact that systolic narrowing is occasionally detected during angiography.41,42 Only precise anatomic observations, however, not angiographic data, can confirm or disprove this hypothesis. The possibility still exists that coronary anomalies of this kind develop after the reabsorption of the aortopulmonary septum; at that stage a coronary bud could conceivably cross the area in question. CORONARY DISEASE

PATTERNS

IN CONGENITAL

HEART

By observing the coronary circulation in patients with congenital heart disorders, the investigator can study the results of nature’s own “experiments.“22,23*43 Although this is a subject that has been inadequately studied and poorly understood, the following comments may be in order: To a great extent the ventricular morphology

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1. Schematic representation of basic components involved in the embryogenesis of coronary arteries. Aorta (AO) and pulmonary (PA) trunks are shown at completion of septation; coronary buds (3a, 3b, 3c, 3d) emerge from semilunar sinuses. Rudiments of right (Ca), circumflex (Cb), and left anterior descending (Cc), coronary arteries are shown as isolated in situ vascular networks. At this stage sinusoids (Sn) are site of metabolic exchanges between intracavitary blood and cardiac jelly. Fig.

seems to determine the coronary pattern. For example, in ventricular inversion the coronary arteries are inverted and correspond to the respective ventricular morphology.” The coronary ostial morphology is quite variable in the presence of major congenital heart defects22~23~46~47 (much more so than in the absence of such defects); nevertheless, the coronary arteries tend to arise from the two aortic cusps, which are situated next to the aortopulmonary septum.“p48,4g Obviously this landmark is lost in common truncus arteriosus but not in pulmonary or aortic atresia. It is quite remarkable that in aortic atresia the coronary arteries still arise from the extremely hypoplastic aorta (the flow of which is inverted) and not from the pulmonary artery. The origin of the left anterior descending coronary artery is influenced by the development of the pulmonary conus. In tetrology of Fallot, which is characterized by uneven septation of the conus and the truncus arteriosus (at the expense of the pulmonary side), hypoplasia of the pulmonary infundibulum seems to be associated with a high probability of abnormal origination of the anterior descending branch from the right coronary artery (this anomaly is known as right anterior descending artery).60-55 A posterior pulmonary infundibulum, as in transposition of the great vessels, is most frequently crossed anteriorly by the left coronary artery. This

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arrangement is especially important in cases of pulmonary infundibular stenosis, when infundibular resection and patch repair, as are commonly done for tetralogy of Fallot, would compromise the anomalous coronary artery. During maturation of the fetus the primitive sinusoidal network gradually regresses simultaneously with the development of the compact outer layer of the myocardium.27,31 Trabeculae are still present in the mature heart, but their connections with the coronary arteries are small and physiologically insignificant, and their flow is directed toward the cardiac cavities (see “coronary-camera1 communications”). In two congenital anomalies-pulmonary atresia56-5s and hypoplastic left ventricle5gwell-developed, direct communications (sinusoidal channels) are frequently observed between the ventricular cavities and the epicardial coronary arteries. These “ventriculocoronary connections” are characterized by systolic flow from the suprasystemic pressure ventricular chamber into the aorta and thereby function as “venting” channels. Occasionally coronary arteries that may have been essentially normal during early development of the embryo will become obstructed or even totally occluded in later (fetal or neonatal) life. Such obstruction is typical of coronary ostial hypoplasia or atresia in the presence of a definite distal coronary bed that is supplied by collateral circulation from the opposite side. Congenital atresia of the left coronary ostium should be considered totally different from single (right) coronary artery, even though the right coronary artery supplies the entire coronary circulation in both conditions. Physiologically, coronary ostial atresia is usually associated with ischemic manifestations, whereas single coronary artery is not. Ontologically, the defects that lead to coronary ostial atresia must be acquired during later stages of fetal life (after horizon XIX), whereas single right coronary artery atresia is determined at an early stage (horizons XVI through XIX). Similarly, longer segments of a coronary artery or branch are occasionally found to be diffusely hypoplastic. These usually involve hypoplasia of the lumen (associated with a normal outer diameter) resulting from an endoproliferative process, with or without an inflammatory component, that occurs during fetal or neonatal life.38p60 This entity is different from primary coronary “hypoplasia” (in which the outer diameter is also diminished). NORMAL

CORONARY

ARTERIES

In biologic events the concept of normality can only be statistical. Whereas with some variables it is

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relatively easy to define normality (“a human being normally has two eyes”), with other variables it is more difficult (“What is the color of the normal eye, and what colors should be considered abnormal?“). With respect to quantifiable variables, normality can easily be based on a statistical criterion (the interval that comprises two or three standard deviations from the mean in a gaussian curve distribution); with respect to nonquantifiable variables, however, the establishment of normality can be more difficult. Moreover, on clinical grounds, a further complication arises from the observer’s implicit or openly expressed interest in the clinical relevance of a given variable. A clear example concerns one of the simplest aspects of the human coronary system: the number of coronary arteries. Most experts claim that there are normally two coronary arteries-the right and the left-thereby implying that the presence of three such arteries is abnormal. At the same time most authors accept that in close to 50% of the cases the conal artery arises independently from the aortic wall and has an ostium separate from that of the right coronary artery.“1*61*62 Because the clinician and the pathologist have little interest in this small artery, the typical description of normal coronary arteries omits it entirely. If the interest of the observer were the prevailing criterion, clinicians would be satisfied with a description of normality that included every variation that does not cause a clinical manifestation.K63 Such an attitude might have been justified in the early stages of our knowledge but is currently quite inadequate. Besides being illogical and scientifically inappropriate, the use of nonmorphologic criteria for defining and classifying anatomic events can also prove treacherous, since some conditions (such as muscular bridges or anomalous coursing of a coronary artery between the aorta and the pulmonary artery) have not yet been fully understood on clinical grounds. Consequently, the concept of normality that results from classifying coronary artery anomalies as “major” and “minor” or “significant” and “insignificant” is vague and confusing. It is proposed herein that normality be defined as “that which is observed in at least 1% of unselected cases.“64 This definition would allow for variations within normality (normal variants) and for less frequent morphologic entities (anomalies) that lie outside the 99% range of the normal spectrum but do not necessarily denote disease. Designating 1% as the cutoff point between normal and abnormal may seem rigid, empirical, or irrelevant. Such a criterion is indeed rigid, since it artifically differentiates between variations that form a continuous

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2. Diagram of aortic root (AR) and the aortic valve (AV), with suggestedapproach to determining “normal” site of right (R) and left (L) coronary ostia, respectively, in horizontal plane (circle) and longitudinal axis (vertical bars). Observed incidence of each coronary ostial site is expressedaspercentage of incidence encountered in normal population (in sectorsof aortic circumference for lateral variations; in 1 cm segmentsabove or below upper edge of sinus of Valsalva for longitudinal variations). Fig.

spectrum. Unfortunately, all biologic taxonomic systems are subject to this objection. Although such systems tend to be empirical, historical, and relative, this does not mean that they cannot still be useful and relevant. Whereas the preceding proposed criterion is easily justifiable (1% corresponds to two or three standard deviations) and readily applicable, it is based on the presumption that the incidence of a definite event is a known entity; this may be untrue, may be disputed from time to time, or may vary with different human races (Fig. 2). AlI of these considerations underscore the relatively of any taxonomic endeavor. Nevertheless, the need to demystify the term “coronary anomaly” and free it from clinical or physiologic implications should be obvious: as noted previously, a coronary anomaly is neither necessarily a congenital heart defect nor does it always cause a pathophysiologic repercussion. Essentially the coronary arteries can be described61 in terms of their ostia (with respect to number and location), main trunk (with respect to course, pattern, and size), primary branches, secondary (extramural) branches, intramural arteries and arterioles, and termination.

bronchi, branch” used.65

DEFINITIONS

left anterior descending and circurnfIex branches to have a split origin from the aorta. This phenomenon

OF NORMALITY

This term indicates a vessel that supplies myocardial blood flow. A subclavian artery that gives off an anomalous branch that feeds the myocardium is said to have a coronary branch. If a coronary artery gives off a branch that feeds the Coronary.

Coronary

the term “anomalous origin of a bronchial (not “of a coronary branch”) should be ostia

Location. It is normal for coronary ostia to be located at the right and left aortic sinuses (the “coronary” sinuses). The ostia should be located in the center of each sinus, close to the free edge of the aortic cusp (Fig. 2). Adequate data are not yet available to identify exactly which portion of the aortic root is normally expected to have coronary ostia, but ostia located near the aortic valve commissures, in the posterior (noncoronary) sinus, or high in the aortic root (more than 1.0 cm above the cusp?) are definitely abnormal. Number. Two coronary ostia (one in each sinus) is the minimal requirement for normality, but three and four are considered normal variants. The third coronary ostium is usually the result of the conal branch originating independently from the aorta (rather than from the right coronary artery). The conal artery is present as a small accessory artery in 30 % to 50 % of normal human hearts.6l 62The second most frequent type of accessory ostium results from the absence of a left main trunk, which causes the

is said to occur in 0.5% to 8.0% of otherwise normal hearts?* The variance in the reported incidence of absence of the left main trunk is mostly due to the lack of a universally accepted terminology.66 If the

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Fig. 3. Three types of aortic origination of coronary arteries, according to orientation of proximal coronary segment. A, In most common pattern coronary artery is nearly orthogonal to aortic wall (both in vertical and horizontal axes). B, Less frequent variant shows nearly tangential origin. C, In an unusual anomaly, intussusception of coronary artery, proximal arterial segment is embeddedin aortic wall.

criterion

for this anomaly

is the presence of two

well-separated ostia, the incidence is probably less

than 1% ; if the criterion is the absence of a proper left main trunk, however, the incidence is more than 1%. More commonly, the aortic wall contains a single niche within which the ostia of the two left coronary branches (the left anterior descending and circumflex) are juxtaposed. Size. Rather than being described in arJsolute terms, the normal size of a coronary ostium should be defined according to the size of the related artery. Normally the aortic opening is equal to or larger than the diameter of the artery that originates from it. Orientation of coronary stems. The angle between the aortic wall and the proximal right and left coronary arteries is a relevant variable. Typically the proximal coronary arteries are oriented perpendicular to the aortic wall, but some variation in angula-

February 1989 Heart Journal

tion is commonly observed. Further studies are required to establish the limits of normality and thus clarify the definition of anomalies such as slitlike (tangential) origin of the coronary arteries, which involve extreme deviation from the normal orientation (Fig. 3). Coronary arteries. Although three or four separate arteries can originate from the aorta without being considered anomalous (see the foregoing section), an individual normally has only two main coronary arteries (or coronary systems&-the left and the right. For describing split origin of the conal branch or of the left anterior descending and circumflex branches, the term “normal variant” is appropriate. “Single coronary artery” (see below) refers to origination of both the right and left coronary arteries (as defined herein) from a single ostium; in such instances the presence of a separate conal-branch ostium is irrelevant. Left coronary artery. The left coronary system can best be defined according to its main stem and its two primary subdivisions: the left anterior descending and circumflex branches. Valid arguments have been advanced for designating the right, left, left anterior descending, and circumflex vessels as “arteries” and for referring to the more distal secondary vessels as “branches.“61 In 92.0% to 95.5 % of autopsy cases,‘jl, 67-69 the left coronary artery has a single initial stem or trunk of variable length (2.0 to 40.0 mm; mean 13.5 mm) and size (2.0 to 5.5 mm; mean luminal diameter 4.0 mm). It is essential, for normality, that both the left anterior descending and the left circumflex artery originate from the left coronary cusp, either directly or indirectly through a left main stem. The left anterior descending artery, a segment of the left coronary sytem, is characterized by its course along the anterior interventricular groove (sulcus). It is not essential for this artery to reach the cardiac apex or to have well-defined septal or diagonal branches, although it usually does both. Occasionally the left anterior descending branch proper reaches only the proximal portion of the anterior interventricular sulcus. In these instances the distal territory may be supplied by unusually long diagonal or right coronary branches (normal variants?).7n The circumflex branch, which is also a main segment of the left coronary system, is characterized by its course along the left-sided atrioventricular groove. It is not essential for the circumflex artery to have a branch that reaches the obtuse

margin of the heart, although it commonly does have such an extension (the obtuse marginal branch). The ramus intermedius, or medianus-a secondary nonessential branch of the left coronary

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defined by its distribution, intermediate between the diagonal and obtuse marginal branches; the medianus may arise from the left main trunk itself (trifurcation) or from the proximal left anterior descending or circumflex branch. Right coronary artery. This artery, which courses along the right atrioventricular groove, normally originates from the right coronary cusp and reaches (at least) the acute margin of the heart. The posterior descending artery is not an essential branch of the right coronary artery, since the former vessel may arise from the terminal portion of the circumflex artery (although this happens rather infrequently). The dominance pattern in a given case (i.e., the presence of right or left coronary predominance or balanced circulation) depends on the number of posterior descending arteries and their type of origin.71 All three above-named variants are normal, inasmuch as each can be found in more than 1% of human hearts.61,68,71.Is it normal for the acute marginal branch or anterior right ventricular branches to arise from the circumflex artery? Probably not. “Extreme” dominance of the left circumflex artery should therefore be classified as single left coronary artery (the dimunitive coronary branch that arises from the right cusp should then be called the conal branch). The exact dividing line between a normal variant and an anomaly can be established only by means of ad hoc statistical studies. Until such studies become available, the acute marginal branch appears to be a logical dividing line. At the other end of the spectrum, the obtuse marginal branch is probably the last vessel on the posterolateral left ventricular wall that can be thought of as originating from a normal right coronary artery. Beyond this point it is probably appropriate to refer to “extreme dominance of the right coronary artery” (an anomalous variant that could include both the origin of the circumflex branch from the right coronary artery and the single right coronary artery). With respect to the most distal secondary branches, only a few patterns are consistently observed and can therefore be considered as defining normality: 1. Anterior septal branches originate from the left anterior descending artery. It is abnormal for these branches to arise from other extramural vessels (the diagonal, ramus medianus, circumflex, and right coronary branches or the left main trunk). 2. The left anterior descending artery does not give rise to large epicardial right ventricular branches. 3. Extramural arteries do not cross each other. Therefore it is abnormal for one branch to cross an

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adjacent branch.72 Angiographically such crossing is sometimes mimicked by subendocardial vessels, which are occasionally quite large (especially in the presence of total occlusion of another branch, when they form part of a collateral circle). 4. Extramural arteries are expected to plunge into the myocardium only once, at their distal end. “Muscular bridge” or “intramural coronary artery” is the term used to denote an exception to this ru1e.73-75 5. Although still being investigated, it is probable that an ideal ratio exists with respect to the number of arterioles (and capillaries) per gram of related myocardium. This ratio could be altered congenitally (by an anomaly) or by an acquired condition. Patients who have angina in the presence of “normal” coronary arteries may indeed have a problem of this kind, either because of a congenitally inadequate number of small coronary ramifications or because of acquired myocardial hypertrophy. Theoretically it is conceivable that an individual could be born with “hypoplastic coronary arteries,“76’77 that is, vessels inadequate to meet the maximal metabol,ic needs of the dependent myocardium (diminished coronary reserve). As previously noted, however, a newborn coronary artery “too small” to meet basal metabolic needs could not support the growth of an adequate myocardial mass.38 6. Coronary arteries subdivide into smaller branches until they reach the arteriolar level where they terminate in the capillary network. Occasionally a small coronary artery will communicate with a cardiac cavity, producing a diminutive coronarycamera1 or coronary-sinusoidal connection. A limited number of these connections are commonly present in human hearts, especially in the left and right ventricles.61 How many of these are required for “normality” is not yet clear. A few hearts have been observed angiographically to have extremely numerous coronary-cameral communications of this kind (an anomaly that will be discussed later). 7. The small ramifications of the extramural coronary arteries tend to be of the terminal type, but diminutive (20 to 250 pm in diameter) anastomoses between adjacent territories (or collateral vessels) do exist in normal human hearts6’ The quantification of collaterals with respect to number and size has not yet been accomplished, but is likely that a wide spectrum exists. Anastamoses are sometimes present, also normally, between the coronary arteries and the systemic arteries of adjacent organs,61 including the vasa vasorum of the aorta, of the superior and inferior venae cavae, and of the pulmonary artery, as well as the pericardial arteries (along

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the reflection of the pericardium). Only rarely do contiguous coronary arteries have large (more than 250 h in diameter) connecting vessels in the absence of occlusion of an involved branch.78 Termination. Coronary arteries normally end in the myocardial capillary bed, but some communicate directly with the cardiac cavities. The difference between arteriocameral communications and coronary venous passageways to the cardiac cavities (Thebesian veins) is still’ open to discussion.61 Apparently, small arteriocameral communications (50 to 250 P in diameter) are frequently seen in human hearuP and should therefore be considered normal. CORONARY

ANOMALIES

Once the criteria for coronary artery normality have been established, the definition of coronary abnormalities has also been implicitly achieved; any anatomic feature that does not fulfill the criteria for normality is automatically an anomaly.5 Because anomalies are deviations from normal anatomy, their interpretation could be based on their mechanism of origin.7g Unfortunately, because advances in both descriptive and experimental embryology are lagging behind the insights of pathologic anatomy, or current understanding of normal and abnormal coronary embryologic development is conjectural and incomplete. The following summary of coronary anomalies is based on the observations concerning normal embryology presented earlier in this article. Because inference is a potentially dangerous logical process,8o its use will be proposed sparingly for the purpose of stimulating further study. A detailed description of the anatomic and clinical features of each coronary anomaly is beyond the scope of this presentation; interested readers are referred to the pertinent literature cited within the text. CLASSIFICATION

OF CORONARY

ANOMALIES

Coronary atresia. In a very few instances the extramural coronary arteries are totally absent (usually in the context of pulmonary atresia).81-85. Intrinsic coronary ostial anomalies. The coronary ostium in itself can be abnormal with respect to its intrinsic anatomy (within the proper aortic sinus) or size (ostial hypoplasia,@ fibrous endoproliferation 60,87,8a or atresia8g-g5). Usually associated with ectobic ostia, tangential origin of a coronary artery from the aortic wall (Fig. 3) is characterized by the fact that the proximal segment of the afFected artery is obliquely oriented with respect to the aortic wa11.96-101 Sometimes this course is also intramural,

February 1989 Heart Journal

and the artery becomes embedded in the aortic wa11,*02a condition called “intussusception.” Ectopic coronary origination. This pattern is the result of the persistence of ectopic, additional, or atypical coronary buds, which connect with the right or left coronary artery or one of their branches,lo3 or are associated with anomalous connection of a coronary artery with the opposite coronary sinus or some other arterial structure. The various forms of this anomaly include: 1. Anomalous origin of the coronary arteries (involving one artery, one branch,104-1’0 or both111-“7) from the pulmonary trunk.161 63*gl, 118-173 In this disorder the anomalous orifice is located within either of the two semilunar sinuses, next to the aortopulmonary septum. 2. Anomalous origin of the coronary artery from an atypical aortic wall site174 (the noncoronary CUSP,‘~~ the aortic wall above the sinus,g8, ‘~-l~* or the coronary sinus opposite the one expected [see next section]). As mentioned previously, separate orgination of a conal branch from the right coronary CUSP’~$ or separate origination of the circumflex and left anterior descending branch from the left coronary cusp lies within the normal spectrum. However, origination of a septal branchlm directly from the aorta is considered an anomaly. 3. Anomalous origin of the right coronary artery,*sl-‘ss the left coronary artery,186v187 or the anterior descending,la circumflex, or septal branchlsg from the opposite coronary artery or sinus After arising abnormalwith respect to normal. 1go-1s4 ly, a coronary branch can arrive at the opposite side of the aorta by coursing1g5’ lg6: (1) posterior to the atrioventricular valves (as in most cases of single coronary artery), (2) posterior to the aorta,1g7-199(3) between the aorta and the pulmonary artery,198~200-207 (4) within the crista supraventricularis208 and the ventricular septum,20g-212 or (5) anterior to the pulmonary infundibulum213,214 (Fig. 4). When a coronary artery in its entirety arises from the opposite ostium, the term “single (right or left) coronary The determination of “right” artery” applies. 208,215-2zo or left” depends entirely on the aortic sinus that gives origin to the single coronary artery. 4. Ectopic origin of a coronary artery from an extracardiac vessel such as an innominate,221v222 subclavian, mammary,223 or carotid artery, a bronchial artery, the descending aorta,224 or a pulmonary branch.225-22g 5. Ectopic origin of a coronary artery from a ventricular cavity. In the absence of normal connections to the proper coronary ostium, a coronary artery may arise from the right or left ventricular

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cavity. This anomaly probably results from the development of exclusive connections between the in situ coronary network and the primitive myocardial sinusoids. The coronary-sinusoidal connections seen in cases of pulmonary valve atresiaBO have a different significance, since they coexist with otherwise normal coronary orifices and distribution patterns. Intramural coursing of a coronary artery (muscular bridge). In human hearts the main coronary arteries

and their branches are expected to course extramurally (epicardially). Sometimes, however, one of these vessels will have an abnormal subepicardial course (i.e., will become intussuscepted into the myocardium).231,232 Occasionally the intramural coronary artery will become intracavitary or subendocardial.233-236 The midsegment of the left anterior descending artery may be considered an exception, since it is intramural 5% to 80% of the time74); hence, a muscular bridge at this level should be considered a normal variant. Abnormal distal connections or termination. The development of abnormal connections between the coronary arterial network and its neighboring structures results in abnormal communications that may resemble fistulas. The fistulous character of a communication depends on the presence of a marked hemodynamic gradient and limited resistance (a large channei). As a consequence of torrential flow, there is a tendency for such communications to enlarge with time, although spontaneous closure is The following types have occasionally reported. 237-23s been described.240-2” 1. Connections with a cardiac cavity (the right or left atrium or ventricle), or coronary-cameral communications (fistulae). These may result in a wide spectrum of entities: small, normal, isolated arteriocame& fistulas255,256that differ from the Thebesian veins (which connect coronary veins with cardiac cavities), large arteriocameral flstulas (“functional” coronary fistulas),257-264 multiple small arteriocameral fistulas to the left ventricle,26s and arteriosinusoida1 connections (which are essentially identical to arteriocameral fistulas). Finally, “aorto-left ventricular tunne1s”266* 267probably result from direct communication between an abnormal coronary bud and a left ventricular sinusoid. 2. Coronary arteriovenous fistulas. These anomalies, which are the consequence of abnormal direct communication between in situ arterial and venous coronary plexi, may take the form of a single, large arteriovenous coronary fistula26s-271or multiple small fistulas272 or racemose angiomas.273 Because the presence of arterial flow in veins induces intimal and

4. Schematic view of coronal plane of heart showing possible variations in origin and course of coronary arteries as related to semilunar valves, atrioventricular valves, interventricular sulci, and cardiac margin. Anomalous arteries can take five possible routes: posterior (I), retroaortic (II), intertruncal (between aorta and pulmonary artery, III), intramuscular (within crista supraventricularis and ventricular septum; IV), and anterior (within pulmonary infundibulum; V). a, anterior interventricular sulcus; b, obtuse cardiac margin; c, acute cardiac margin; d, posterior interventricular sulcus; Ao, aorta; MV, mitral valve; Pa, pulmonary artery; TV, tricuspid valve. Fig.

medial hypertrophy, it may be difficult, even from a histologic standpoint, to demonstrate the coronary venous segment of a large fistulous tract. These fistulas usually terminate in the coronary sinus, as expected, because this structure is the normal drainage site for the coronary veins.27o 3. Coronary-to-extracardiac arterial or. venous connections. These structures result from the persistence of a vascular communication within the mediastinal mesoderm that may be normal in early embryogenesis; Examples include coronary-pulmonary fistulas (which usually involve small branches of the proximal coronary arteries)265,274-283and coronary-bronchial,66 coronary-pericardial, or coronaryphrenic communications. Diminutive, inconsequential connections between coronary and mediastinal systemic arteries are so commorP that they should probably be considered normal in humans, even though these connections are rarely recognized angiographically. Coronary-pulmonary fistulas may also be the result of the anomalous origin of supernumerary coronary arteries from the pulmonary artery. Indeed, in such instances, the normal anastomotic

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circles would elicit fistulous behavior as soon as the pulmonary pressure decreased below the systemic level shortly after birth. Fistulas between a coronary artery and an extracardiac vein (most frequently the superior vena cava%) are quite rare. Anomalies of coronary size. The diameter of a coronary artery can be congenitally too small (hypoplastic) or too large (e&sic). As discussed previously, the “ideal” normal diameter of a coronary artery or branch is a difficult concept to define. Theoretically it is easy to define as hypoplastic an artery that is inadequate to provide normal blood flow to the dependent myocardium (including the maximal blood-flow requirement, or “coronary reserve”). A practical definition of hypoplasia awaits the developments of scientific methods for determining the normal coronary reserve and cannot be established on purely anatomic grounds (the ideal “coronary size/dependent myocardial mass” ratio is difficult to determine on the basis of purely anatomic findings). Similarly the definition of coronary ectasia should be based on the expected “normal” luminal diameter, an as-yet vague variable that is best measured in terms of blood-flow velocity. Segmental ectasia (or hypoplasia) of a coronary artery, in which the disproportion to the distal coronary bed is obvious, is the only form of ectasia whose definition is currently agreed on by purist observers. Most cases of coronary ectasia or aneurysm285-28s are secondary or acquired (as a result of arteritis, trauma, or atherosclerosis) rather than primary congenital anomalies. Conclusions. The foregoing considerations were born out of current frustrations that the author has experienced in dealing with isolated reports of “new coronary anomalies.” An explicit description of normal coronary arteries is, de facto, the critical element missing from the current literature.21 The classification scheme proposed herein is partly tentative and is therefore open to discussion, common agreement, or both. If current technical difficulties related to the viability of an experimental model (the postloop chick embryo) are overcome, experimental embryology will come closer to providing a fundamental knowledge of basic morphogenetic mechanisms. Currently such mechanisms can only be inferred on the basis of human congenital abnormalities, which represent naturally occurring experiments. In itself the spectrum of coronary anomalies not only suggests that the coronary ostia and arterial trunks are subject to independent variability but also confirms the embryologic concept of separate primordia (mainly the coronary buds and the in situ vascular network). The recent interest in certain

coronary anomalies as possible causes of myocardial ischemia201* 232*2*g-314further stresses the importance of adopting a clear, universally accepted scheme for defining and classifying these heterogeneous entities. It is hoped that the foregoing discussion will stimulate-and help organize-further studies of normal and pathologic coronary anatomy and pathophysiology. GLOSSARY

Deviation (or variation) = a nonspecific term referring to an entity that is “different from” normal. Normal variant = a relatively infrequent deviation that is nevertheless within the spectrum of normality. Anomaly (anomalous variant or abnormality) = any congenital deviation that .is seen in less than 1% of otherwise normal individuals. Congenital malformation, defect, or disease = a congenital anomaly that has a negative functional or prognostic consequence. Only certain coronary anomalies qualify as a form of congenital heart disease. SUMMARY

Results of a comprehensive survey of the literature concerning coronary artery anatomy, embryology, and pathophysiology show the lack of an adequate definition of normal coronary arteries. To fill this gap, the present review considers the available data concerning, the embryogenesis of the coronary arteries and proposes a new definition of normality that refers to essential anatomic features. The concepts of normal variant versus anomaly are introduced, based on a statistical definition of the normal range (99% of the presentations observed in a normal, unselected population). Coronary anomalies are defined as those patterns found in less than 1% of the cases. The wide spectrum of coronary abnormalities is then organized according to a comprehensive classification scheme. For clinic&l purposes the conceptual difference between anatomic and pathophysiologic anomalies is stressed. The current paucity of experimental studies concerning normal and abnormal embryogenesis of the coronary arteries is found to be the major limitation to an understanding of this subject. ADDENDUM

Since the preparation of this article, a new report by Hutchins et al. has been published (Development of the coronary arteries in the embryonic human heart. Circulation 198&77:1250-7) regarding the

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development of the coronary arteries in 351 embryonic human hearts. Their findings essentially confirm those of previous reports. The hypothesis is proposed that the site of normal origin of the coronary arteries is determined by the configuration of the roots of the great arteries. REFERENCES

1. Hallman GL. Unusual coronary artery abnormalities [Editorial]. Chest 1976;70:4-5. 2. Baltaxe HA, Wixson D. The incidence of congenital anomalies of the coronary arteries in the adult population. Radiology 1977;122:47-52. 3. Levin DC, Fellows KE, Abrams HL. Hemodynamically significant primary anomalies of the coronary arteries. Angiographic aspects. Circulation 1978;58:25-34. 4. Roberta WC. Major anomalies of coronary arterial origin seen in adulthood. AM HEART J 1986;111:941-63. 5. Ogden JA. Congenital anomalies of the coronary arteries. Am J Cardiol 1970;25:474-9. 6. Alexander RW. Griffith GC. Anomalies of the coronarv arteries and their clinical significance. Circulation 1956; 14:800-5. 7. Blake HA, Manion WC, Mattingly TW, Baroldi G. Coronary artery anomalies. Circulation 1964;30:927-40. 8. Donaldson RM, Raphael MJ, Yacoub MH, Ross DN. Hemodynamically significant anomalies of the coronary arteries. Surgical aspects. Thorac Cardiovasc Surg 1982; 30:7-13. 9. Kucera J. Epidemiology and phenotype of congenital coronary artery anomalies. Analysis of 80 cases found in necropsies of stillborn fetuses and infants Cor Vasa 1978;20:369-78. 10. Paulin S. Major variation sin anatomical origin of the coronary arteries [Letter]. Cathet Cardiovasc Diagn 1976;2:105-7. 11. Engel HJ, Torres C, Page HL Jr. Major variations in anatomical origin of the coronary arteries: angiographic observations in 4,250 patients without associated congenital heart disease..Cathet Cardiovasc Diagn 1975;1:15?-69. 12. El-Said GM, Dawson Jr JT, Sandiform FM, Mullins CE, Hallman GL, Cooley DA, McNamara DG. Coronary artery anomalies. Diagnosis, indications and results of surgical management. Eur J Cardiol 1973;1:63-70. 13. Sharbaugh AH, White RS. Single coronary artery. Analysis of the anatomic variation, clinical importance, and report of five cases. JAMA 1974;230:243-6. 14. Ogden JA, Stansel Jr HC. Roentgenographic manifestations of congenital coronary artery disease. Am J Roentgenol Radium Ther Nucl Med 1971;113:538-53. 15. McNair M, Strickland B. Congenital anomalies of the coronary arteries. Proc R Sot Med 1971;64:839-44. 16. Somerville J, Ross DN, Left coronary artery from the pulmonary artery. Physiological considerations of surgical correction. Thorax 1970;25:207-12. 17. Gonzalez-Angulo A, Reyes HA, Wallace SA. Anomalies of the origin of coronary arteries. (special reference to single coronary artery). Angiology 1966;17:96-103. 18. Trivellato M, Angelini P, Leachman RD. Variations and coronary artery anatomy-normal vs abnormal. Cardiovasc Dis, Bull Tex Heart Inst 1980;7:357-60. 19. Martinez-Rios MA, Cisneros F, Gil M. El diagnostic0 angiografico de las anomalies congenitas de 10s vasos coronaries. Arch Inst Cardiol Mex 1976;46:750-7. 20. Roberta WC. Congenital coronary arterial anomalies unassociated with major anomalies of the heart or great vessels. In: Roberts WC, ed. Adult congenital heart diseases. Philadelphia: FA Davi Company, 1987:583-630.

Coronary anomalies 427 21. Roberta WC. Major anomalies of coronary arterial origin seen in adulthood. AM HEART J 1986;111:941-63. 22. Vlodaver Z, Neufeld HN, Edwards JE. Coronary arterial variations in the normal heart and in congenital heart disease. New York: Academic Press, Inc, 1975. 23. Neufeld HN, Schneeweiss A. Coronary artery disease in infants and children. Philadelnhia: Lea & Febieer. Publishers, 1983. 24. Conte G, Pellegrini A. On the development of the coronary arteries in human embryos, stages 14-19. Anat Embryo1 1984;169:209-15. 25. Bank1 H. Congenital malformations of the heart and great vessels. Synopsis of pathology, embryology and natural history. Baltimore: Urban and Swartzenberg, 1977:213-20. 26. Hackensellner HA. Akzessorische Kranzgefassanlangen der Arteria pulmonalis unter 63 menschlichen Embryonenserien mit einer grossten Lange von 12 bis 36 mm. Z Mikroskop Anat Forsch 1956;62:153. 27. Manasek FJ. Hi&genesis of the embryonic myocardium. Am J Cardiol 1970;25:149-68. 28. Sissman N. Developmental landmarks in cardiac morphogenesis: comparative chronology. Am J Cardiol 1970; 25:141-8. 29. Hirakow R. Development of the cardiac blood vessels in staged human embryos. Acta Anat 1983;115:220-30. 30. Licata RH. Coronary circulation: embryology. In: Abramson D, ed. Blood vessels and lymphatics. New York: Academic Press, Inc, 1962:258-61. 31. Pattern BM. Human embryology. New York: McGraw-Hill Book Company, Inc 1968;537-83. 32. Barresi V, Susmano A, Colandrea MA, Bogdonoff ML, Muenster JJ. Congenital absence of the circumflex coronary artery. Clinical and cinearteriographic observations. AM HEART J 1973;86:811-16. 33. Leitch AG, Caves PK. A case of Marfan’s syndrome with absent right coronary artery complicated by aortic dissection and right ventricular infarction. Thorax 1975;30: 352-4. 34. Bestetti RB, Costa RB, Oliveira JS, Rossi MA, Correa de Araujo R. Congenital absence of the circumflex coronary artery associated with dilated cardiomyopathy. Int J Cardiol 1985;8:331-5. 35. Donaldson RM, Raphael MJ. Mising coronary artery. Review of technical problems in coronary arteriography resulting from anatomical variants. Br Heart J 1982;47:6272. 36. Gentzler II RD, Gault JH, Liedtke AJ, McCann WD, Mann RH, Hunter AS. Congenital absence of the left circumflex coronary artery in the systolic click syndrome. Circulation 1975;52:490-6. 37. Sharbaugh AH, White RS. Single coronary artery. Analysis of the anatomic variation, clinical importance, and report of five cases. JAMA 1974;230:243-6. 38. Saji T, Yamamoto K, Hishiguchi R, Matauo N, Yabe Y. Hypoplastic left coronary artery. In association with occlusive intimal thickening of a coronary artery with ectopic ostium and with atresia of the left coronary ostium. Jpn Heart J 1985;26:603-12. 39. Menke DM, Waller DF, Pless JE. Hypoplastic coronary arteries and high takeoff position of the right coronary ostium. A fatal combination of congenital coronary artery anomalies in an amateur athlete. Chest 1985;88:299-301. 40. Ferguson DW, Henkle JQ, Haws CW. Absence of left anterior descending coronary artery associated with anomalous origin of left circumflex coronary artery from the right coronary artery: a case report and review. Cathet Cardiovasc Diagn 1985;11:55-61. 41. Keren A, Tzivoni D, G¬ C, Yalon S, Stern S. Origin of both coronary arteries from the same sinus of Valsalva: report of two cases. Cathet Cardiovasc Diagn 1982;8: 191-9. -

428

Angelini

42. Benge W, Martins JB, Funk DC. Morbidity associated with anomalous origin of the right coronary artery from the left sinus of Valsalva. AM H&T J 1980;99:96-160. 43. Ehren H. Wells TR. Landing BH. Association of common origin of the carotid’arteries kith anomalous origin the left coronary artery from the pulmonary artery. Pediatr Path01 1985;4:59-66. 44. Schneeweiss A, Deutsch V, Blieden LC, Shem-Tov A, Neufeld HN. “ComDlete-transDosition-like” coronarv arterial pattern in single ventricle-with inverted infundibulum and transposition of the great arteries. Cardiovasc Intervent Radio1 1981;4:148-50. 45. Reference deleted in proofs. 46. Anderson KR, McGoon DC, Lie JT. Vulnerability of coronary arteries in surgery for transposition of the great arteries. J Thorac Cardiovasc Surg 197&76:135-g. 47. Shrivastava S, Edwards JE. Coronary arterial origin in persistent truncas arteriosus. Circulation 1977;55:551-4. 48. Rossi MB, Ho SY, Anderson RH, Rossi-Filho RI, Lincoln C. Coronary arteries in complete transposition: the significance of the sinus node artery. Ann Thorac Surg 1986; 42~573-7. 49. Smith A, Arnold R, Wilkinson JL, Hamilton DI, McKay R, Anderson RH. An anatomical study of the patterns of the coronary arteries and sinus nodal artery in complete transposition. Int J Cardiol 1986;12:295-307. 50. Mever J. Chiariello L. Hallman GL. Coolev DA. Coronarv artery anomalies in patients with’tetralogy of Fallot. J Thorac Cardiovasc Surg 1975;69:373-6. 51. Meyer J, Reul Jr GJ, Chiariello L, Hallman GL, Cooley DA. Anomalous origin and distribution of coronary arteries. Review of 38 patients who underwent operation. J Cardiovast Surg (Torino) 1975;16:590-5. 52. Brenner JI, Burwell LR, Hubbell MM, Newton Jr MC, Krovetz LJ, Nolan SP. Single coronary artery with a fistula to the main pulmonary artery in a patient with tetralogy of Fallot. J Thorac Cardiovasc Surg 1977;73:738-41. 53. Hurwitz RA, Smith W, King H, Girod DA, Caldwell RL. Tetralogy of Fallot with abnormal coronary artery: 1967 to 1977. J Thorac Cardiovasc Surg 1980;80:129-34. 54. Landolt CC, Anderson JE, Zorn-Chelton S, Guyton RA, Hatcher Jr CR, Williams WH. Importance of coronary artery anomalies in operations for congenital heart disease. Ann Thorac Surg 1986;41:351-5. 55. Dabizzi RP, Caprioli G, Aiazzi L, Castelli C, Baldrighi G, Parenzan L, Baldrighi V. Distribution and anomalies of coronary arteries in tetralogy of Fallot. Circulation 1980; 61:95-102. 56. O’Connor WN, Stahr BJ, Cottrill CM, Todd EP, Noonan JA. Ventriculocoronary connections in hypoplastic right heart syndrome: autopsy serial section study of six cases. J Am Co11 Cardiol 1988;11:1061-72. 57. Lower RM, Fink HP, Petzy EL, Dunn MI, Diehl AM. Angiographic demonstration of intramyocardial sinusoids in pulmonary valve atresia with intact ventricular septum and hypoplastic right ventricle. N Engl J Med 1964;271:6872. 58. Cornell JH. Mvocardial sinusoids in pulmonary valvular atresia. Radiology 1966,86:421-4. 59. O’Connor WN. Cash JB. Cotrill CM. Johnson GL. Noonan JA. Ventriculocoronary connections in hypoplastic left hearts: an autopsy microscopic study. Circulation 1982; 661078-86. 60. Murai T, Kawaguchi Y, Inui M. Anomalous origin of the left coronary artery. Acta Path01 Jpn 1982;32:1075-83. 61. Baroldi G, -Scomazzoni G. Coronary circulation in the normal and natholoaic heart. Washington, DC: Department of the Army, United States Government Printing Office, 1967:5-90. 62. Schlesinger MJ, Zoll PM, Wessler S. The conus artery: a third coronary artery. AM HEART J 1949;38:823-36. 63. Donaldson RM, Raphael M, Radley-Smith R, Yacoub M.

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February 1989 Heart Journal

Angiographic diagnosis of anomalous origin of the right coronary artery from the pulmonary artery. Br J Radio1 1983;56:17-19. 64. Abrams HL. Coronary arteriography. A practical approach. Boston: Little, Brown & Co, ?983;283-99. -65. St. John-Sutton MG. Miller GA. Kerr IH. Trail1 TA. Coronary artery steal via large coronary artery to bronchial artery anastomosis successfully treated by operation. Br Heart J 1980;44:460-3. 66. Dicicco BS, McManus BM, Waller BF, Roberts WC. Separate aortic ostium of the left anterior descending and left circumflex coronary arteries from the left aortic sinus of Valsalva (absent left main coronary artery). AM HEART J 1982;104-153-4. 67. Dicicco BS, McManus BM, Waller BF, Roberts WC. Separate aortic ostium of the left anterior descending and left circumflex coronary arteries from the left aortic sinus of Valsalva (absent left main coronary artery). AM HEART J 1982;104:53. 68. James TN. Anatomy of the coronary arteries. New York: Hoeber Medical Division, Harper & Row Publishers, Inc, 1961. 69. Gould SE, Pathology of the heart. Springfield, IL: Charles C Thomas Publishers, 1960. 70. Spindola FH, Grose R, Solomon N. Dual left anterior descending coronary artery: angiographic description of important variants and surgical implications. AM HEART J 1983;105:445-55. 71. Schlesinger MJ. Relation of anatomic pattern to pathologic conditions of the coronary arteries. Arch Path01 1940; 30:403-15. 72. Muyldermans LL, Van den Heuvel PA, Ernst SM. Epicardial crossing of coronary arteries: a variation of coronary arterial anatomy. Int J Cardiol 1985;7:416-19. 73. Geiringer E. The mural coronary. AM HEART J 1951:41:35964. 74. Angelini P, Trivellato M, Donis J, Leachman RL. Myocardial bridges: a review. Prog Cardiovasc Dis 1983;26: 75-88. 75. Polacek P. Relation of myocardial bridges and loops on the coronary arteries to coronary occlusion. AM HEART J 1961;61:44-52. 76. De Feyter PJ, Wardeh R, Majid PA. Exercise-induced and variant form of angina pectoris in a patient with hypoplasia of the left coronary artery: clinical, metabolic and angiographic observations. Eur J Cardiol 1981;12:147-51. 77. Bates HR. Cardiomyopathy and hypoplastic coronary arteries [Letter]. JAMA 1974;230:1388. 78. Phillius DA, Berman J. A variation in the origin of the posterior descending coronary artery. Cardiovasc‘intervent Radio1 1984;7:75-7. 79. Schulze WB, Rodin AE. Anomalous origin of both coronary arteries. Report of a case with discussion of teratogenic theories. Arch Path01 1961;72:36-46. 80. Becker AE, Anderson RH. Cardiac embryology: a help or a hindrance in understanding congenital heart disease? In: Nora JJ. Takao A, eds. Coneenital heart disease: Causes and processes. Mount Kisco:NY: Futura Publishing Co, 1984;339-58. 81. Lenox CC, Briner J. Absent proximal coronary arteries associated with pulmonic atresia. Am J Cardiol 1972; 30:666-9. 82. Ueda K, Saito A, Nakano H, Hamazaki Y. Absence of oroximal coronary arteries associated with pulmonarv atre&a. AM HEART J i983;106:596-8. 83. Bvrum CJ. Blackman MS. Schneider B. Sondheimer HM. Kavey RE: Congenital atresia of the left coronary ostium and hypoplaaia of the left main coronary artery. AM HEART J 1980;99:354-8. 84. Hamazaki M. Congenital coronary arterio-ventricular fistulae, associated with absence of proximal coronary artery from aorta. Jpn Heart J 1982;23:271-7.

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85. Ng WL, Wai KH. Coronary arterial fistula. Arch Path01 Lab Med 1976;100:573-5. 86. Harada K, Fujiseki Y, Usami H, Ryujin Y, Seki I, Sakurai I. Myocardial infarction and left coronary ostial stenosis in infancy simulating anomalous origin of the left coronary artery. A case report. Jpn Heart J 1980;21:435-42. 87. Josa M, Danielson GK, Weidman WH, Edwards WD. Congenital ostial membrane of left main coronary artery. J Thorac Cardiovasc Sure 1981:81:338-46. 88. MacMahon HE, Dickinson PC. Occlusive fibroelastosis of coronary arteries in the newborn. Circulation 1967;35:3-9. 89. Blackman MS, Schneider B, Sondheimer HM. Absent proximal left main coronary artery in association with pulmonary atresia. Br Heart J 1981;46:449-51. 90. Kurosawa H, Wagenaar SS, Becker AE. Sudden death in a youth. A case of quadricuspid aortic valve with isolation of origin of left coronary artery. Br Heart J 1981;46:211-15. 91. Arciniegas E, Farooki ZQ, Hakimi M, Green EW. Management of anomalous left coronary artery from the pulmonary artery. Circulation 1980;62:1180-9. 92. Vidne BA, Nili M, Aygen M, Levy MJ. Congenital atresia of the left main coronary artery ostium. Scan J Thorac Cardiovasc Surg 1979;13:37-40. 93. Allen HD, Moller JH, Formanek A, Nicoloff D. Atresia of the proximal left coronary artery associated with supravalvular aortic stenosis. Suraical treatment. J Thorac Cardiovast Surg 1974;67:266-71: 94. Mullins CE, El-Said G, McNamara DG, Cooley DA, Treistman B, Garcia E. Atresia of the left coronary artery ostium. Repair by saphenous vein graft. Circulation 1972;46:98994. 95. Fortuin NJ, Roberts WC. Congenital atresia of the left main coronary artery. Am J Med 1971;50:385-9. 96. Menke DM, Jordan MD, Aust CH, Waller BF. Isolated and severe left main coronary atherosclerosis and thrombosis: a complication of acute angle takeoff of the left main coronary artery. AM HEART 5~1986;112:1319-20. 97. Virmani R. Chun PK. Goldstein RE. Robinowitz M. McAllister HA. Acute takeoffs of the coronary arteries along the aortic wall and congenital coronary ostial valve-like ridges: association with sudden death. J Am Co11 Cardiol 1984; 3:766-71. 98. Partridge JB. High leftward origin of the right coronary artery. Int J Cardiol 1986;13:83-8. 99. Lea IV JW, Page DL, Hammon Jr JW. Congenital ostial stenosis of the right coronary artery repaired by vein patch angioplasty. J Thorac Cardiovasc Surg 1986;92:796-8. 100. Mahowald JM, Blieden LC, Coe JI Edwards JE. Ectopic origin of a coronary artery from the aorta. Sudden death in 3 of 23 patients. Chest 1986;89:668-72. 101. Sacks JH, Londe SP, Rosenbluth A, Zalis EG. Left main coronary bypass for aberrant (aortic) intramural left coronary artery. J Thorac Cardiovasc Surg 1977;73:733-7. 102. Gittenberger de Groot AC, Sauer U, Quaegebeur J. Aortic intramural coronary artery in three hearts with transposition of the great arteries. J Thorac Cardiovasc Surg 1986;91:566-71. 103. Waller BF. Five coronary ostia: duplicate left anterior descending and right conus coronary arteries. Am J Cardiol 1983;51:1562. 104. Donaldson RM, Thornton A, Raphael MJ, Sturridge MF, Emanuel RW. Anomalous origin of the left anterior descending coronary artery from the pulmonary trunk. Eur J Cardiol 1979,10:295-300. 105. Roberts WC, Robinowitz M. Anomalous origin of the left anterior descending coronary artery from the pulmonary trunk with origin of the right and left circumflex coronary arteries from the aorta. Am J Cardiol 1984,54:1381-3. 106. Evans JJ. Phillins JF. Origin of the left anterior descending coronary artery from the pulmonary artery. 3-year angiographic follow-up after saphenous vein bypass graft and proximal ligation. J Am Co11 Cardiol 1984;3:219-24.

Coronary

anomalies

429

107. Tamer DF, Mallon SM, Garcia OL, Wolff GS. Anomalous origin of the left anterior descending coronary artery from the pulmonary artery. AM HEART J 1984;108:341-5. 108. Sinnh RN. Tavlor PC. Anomalous origin of the left anterior descending coronary artery from the pulmonary artery: surgical correction in an adult. Cathet Cardiovasc Diagn 1983;9:411-16. 109. Cooley DA, McNamara DG, Duncan JM, Ott DA. Internal mammary-anomalous left anterior descending coronary artery graft in 16-month-old infant with tetralogy of Fallot: 30-month follow-up. Ann Thorac Surg 1980;30:588-91. 110. Rivett JR, Berry CL. Aberrant coronary artery in association with a quadricuspid pulmonary valve. Virchows Arch [Pathol Anat] 1979;383:351-7. 111. Sennari E, Sato Y, Matsuoka Y, Yamamoto K, Okishima T, Hayakawa K. A case report of anomalous origin of a single coronary artery from the pulmonary artery associated with multiple cardiac malformations. Jpn Circ J 1982;46:32933. 112. Hoganson G, McPherson E, Piper P, Gilbert EF. Single coronary artery arising anomalously from the pulmonary trunk. Arch Path01 Lab Med 1983;107:199-201. 113. Keeton BR, Keenan DJ, Monro JL. Anomalous origin of both coronary arteries from the pulmonary trunk. Br Heart J 1983;49:397-9. 114. Heifetz SA, Robinowitz M, Mueller KH, Virmani R. Total anomalous origin of the coronary arteries from the pulmonary artery. Pediatr Cardiol 1986;7:11-18. 115. Bharati S, Szarnicki RJ, Popper R, Fryer A, Lev M. Origin of both coronary arteries from the pulmonary trunk associated with hypoplasia of the aortic tract complex: a new entity. J Am Co11 Cardiol 1984;3:437-41. 116. Goldblatt E, Adams AP, Ross IK, Savage JP, Morris LL. Single-trunk anomalous origin of both coronary arteries from the pulmonary artery. Diagnosis and surgical management. J Thorac Cardiovasc Surg 1984;87:59-65. 117. Monselise MB, Vlodaver Z, Neufeld HN. Single coronary artery: Origin from the pulmonary trunk in association with ventricular septal defect. Chest 1970;58:613-16. 118. Roberts WC. Anomalous origin of both coronary arteries from the pulmonary artery. Am J Cardiol 1962;10:595600. 119. Abbot ME. Anomalous origin from the pulmonary arteries. In: Osler W, ed. Osler’s modern medicine. Its theories and practice. Philadelphia: Lea & Febiger, Publishers, 1908: 420. 120. Bland EF, White PD, Garland J. Congenital anomalies of the coronary arteries: report of an unusual case associated with cardiac hypertrophy. AM HEART J 1933;8:787-806. 121. Edwards JE. Anomalous coronary arteries with special reference to arteriovenous-like communications. Circulation 1958;17:1001-6. 122. Edwards JE. The direction of blood flow in coronary arteries arising from the pulmonary trunk. Circulation 1964;29:163-6. 123. Moodie DS, Fyfe D, Gill CC, Cook SA, Lytle BW, Taylor PC, Fitzgerald R, Sheldon WC. Anomalous origin of the left coronary artery from the pulmonary artery (BlandWhite-Garland syndrome) in adult patients: long-term follow-up after surgery. AM HEART J 1983;106:381-8. 124. Jurishica AJ. Anomalous left coronary artery. Adult type. AM HEART J 1957;54:429-36. 125. Talner NS, Halloran KH, Mahdavy M, Gardner TH, Hipona F. Anomalous origin of the left coronary artery from the pulmonary artery. A clinical spectrum. Am J Cardiol 1965;15:689-95. 126. Likar I, Criley JM, Lewis KB. Anomalous left coronary artery arising from the pulmonary artery in an adult. A review of the therapeutic problem. Circulation 1966;33:727-32. 127. Roche AHG. Anomalous origin of the left coronarv arterv from the pulmonary artery in the adult. Report of &eventful ligation in two cases. Am J Cardiol 1967;20:561-5.

February

430

Angelini

128. Baue AE, Baum S, Blakemore WS, Zinsser HF. A later stage of anomalous coronary circulation with origin of the left coronary artery from the pulmonary artery. Coronary artery steal. Circulation 1967;36:878-85. 129. Jordan RA, Dry TJ, Edwards JE. Anomalous origin of the right coronary artery from the pulmonary trunk. Mayo Clin P;oc 1950;25;673-6: 130. Tingelstad JB, Lower RR, Eldredge WJ. Anomalous origin of the right coronary artery from the main pulmonary artery. Am J Cardiol 1972;30:670-3. 131. Grayzel DM, Tennant R. Congenital atresia of the tricuspid orifice and anomalous origin of the coronary arteries from the pulmonary artery. Am J Path01 1934-38;10:791. 132. Williams JW, Johnson WS, Boulware Jr JR. Case of tetralogy of Fallot with both coronary arteries arising from pulmonary artery. J Flat Med Assoc 1951;37:561-3. 133. Swan WC, Werthammer S, Aberrant coronary arteries: experiences in diagnosis with report of three cases. Ann Intern Med 1955;42:873-84. 134. Househam KC, Human DG, Fraser CB, Joffe HS. Anomalous left coronary artery from the pulmonary artery-a therapeutic dilemma. S Afr Med J 1983;63:325-7. 135. Depelchin P, Brion JP, Sobolski J. Anomalous origin of an accessory coronary artery from the main pulmonary artery: report of a case with arrhythmia, precordial pain and left heart failure. Eur Heart J 1985;6:548-51. 136. Cottrill CM, Davis D, McMillen M, O’Connor WN, Noonan JA, Todd EP. Anomalous left coronary artery from the pulmonary artery: significance of associated intracardiac defects. J Am Co11 Cardiol 1985;6:237-42. 137. Kory WP, Buck BE, Pickoff AS, Holzman B, Garcia OL. Single coronary artery originating from the right pulmonary artery. Pediatr Cardiol 1984;5:301-6. 138. Worsham C, Sanders SP, Burger BM. Origin of the right coronary artery from the pulmonary trunk: diagnosis by two-dimensional echocardiography. Am J Cardiol 1985;

55:232-3. 139. Steussy HF, Caldwell RL, Wills ER, Waller BF. High takeoff of the left main coronary artery from the pulmonary trunk: potentially fatal combination with pulmonary trunk banding. AM HEART J 1984;108:619-21. 140. Fisher J, McDonald G, Brinker J, Neil1 CA, Donahoo JS, Baughman KL. Transpulmonary artery correction of anomalous origin of the left coronary artery by saphenous vein graft. Cathet Cardiovasc Diagn 1983;9:373-80. 141. AufderHeide JF, Gutierrez FR, Hartmann AF, Weldon CS, Biello DR. Improvement of left ventricular function after ligation of the anomalous left coronary artery during infancy. Pediatr Cardiol 1982;3:229-35. 142. Achtel RA, Zaret BL, Iben AB, Hurley EJ. Surgical correction of congenital left coronary artery-main pulmonary artery fistula in association with anomalous right coronary artery. J Thorac Cardiovasc Surg 1975;70:46-51. 143. Bregman D, Brennan FJ, Singer A, Vinci J, Parodi EN, Cassarella WJ, Edie RN. Anomalous origin of the right coronary artery from the pulmonary artery. J Thorac Cardiovasc Surg 1976;72:626-30. 144. Thompson SI, Vieweg WV, Alpert JS, Hagan AD. Anomalous origin of the right coronary artery from the left sinus of Valsalva with associated chest pain: report of two cases. Cathet Cardiovasc Diagn 1976;2:397-402. 145. Gutgesell HP, Pinsky WW, DePuey EG. Thallium-201 myocardial perfusion imaging in infants and children. Value in distinguishing anomalous left coronary artery from congestive cardiomyopathy. Circulation 1980;61: 596-9. 146. Bermudez GA, Abdelnur R, Midell AI, Replogle R. Anomalous origin of the right coronary artery from the pulmonary artery with large left-to-right shunt (anomalous right coronary artery). Cathet Cardiovasc Diagn 1979;5:371-84. 147. Wilson CL, Dlabal PW, McGuire SA. Surgical treatment of anomalous left coronary artery from pulmonary artery:

American

follow-up

Heart

1989 Journal

in teenagers and adults. AM HEART .I 1579;98:

440-6. 148. Lerberg DB, Ogden JA, Zuberbuhler JR, Bahnson HT. Anomalous origin of the right coronary artery from the pulmonary artery. Ann Thorac Surg 1979;27:87-94. 149. Takeuchi S, Imamura H, Katsumoto K, Hayashi I, Katohgi T, Yozu R, Ohkura M, Inoue T. New surgical method for repair of anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg 1979,78:7-11. 150. Hamilton DI, Ghosh PK. Donnelly RJ. An operation for anomalous origin of left, coronary artery. Br Heart .J 1979;41:121-4. 151. Shrivastava S, Casteneda AR, Molier dH. Anomalous left coronary artery from pulmonary trunk. Long-term followup after ligation. J Thorac Cardiovasc Surg 1978;76:

130-4. 152. Alstrup P, Madsen T, Jagt T. Left coronary artery originating from the pulmonary artery correction and total myocardial blood flow measurements. J Cardiovasc Surg (Torino) 1978;19:169-76. 153. Almeida De Oliveira S, Diament J, Bezerra De Carvalho V, Arie S, Macruz R, Zerbini EJ. Anomalous origin of the left coronary artery from the pulmonary artery. Surgical repair of an unusual form. J Cardiovasc Surg (Torino) 1977; l&599-605. 154. Wilson CL, Dlabal PW, Holeyfield RW, Akins CW, Knauf DG. Anomalous origin of left coronary artery from pulmonary artery. Case report and review of literature concerning teen-agers and adults. J Thorac Cardiovasc Surg 19’77; 73:887-93. 155. Grace RR, Angelini P, Cooley DA. Aortic implantation of anomalous left coronary artery arising from pulmonary artery. Am J Cardiol 1977;39:609-13. 156. Anzai N, Okada T, Takanashi Y, Yamada M, Sano A. Anomalous origin of left coronary artery from pulmonary artery. Chest 1976;70:775-7. 157. Chaitman BR, Bourassa MG, Lesperance J, Dominguez JL, Saltiel J. Aberrant course of the left anterior descending coronary artery associated with anomalous left circumflex origin from the pulmonary artery. Circulation 1975;52: 955-8. 158. Choh JH, Levinsky L, Srinivasan V, Idbeis B, Subramanian S. Anomalous origin of the left coronary artery from the pulmonary trunk: its clinical spectrum and current surgical management. Thorac Cardiovasc Surg 1980;28:239-42. 159. Driscoll DJ, Nihill MR, Mullins CE, Cooley DA, McNamara DG. Management of symptomatic infants with anomalous origin of the left coronary artery from the pulmonary artery. Am J Cardiol 1981;47:642-8. 160. Akasaka T, Itoh K, Ohkawa Y, Nakayama S, Miyamoto H, Nishi T, Satoh H. Takarada M. Surgical treatment of anomalous origin of the left corona& artery from the pulmonary artery associated with tetralogy of Fallot. Ann Thorac Surg 1981;31:369-74. 161. Bourlon F, Kreitmann P, Jourdan J, Grinneiser D, Schmitt R, Dor V. anomalous origin of left coronary artery with aortopulmonary window-a case report with surgical correction and delayed control. Thorac Cardiovasc Surg 1981; 29:91-2. 162. Salomon J, Baltazar R, Mower MM, Goldman S. Anomalous origin of the right coronary artery from the pulmonary artery. Am J Med Sci 1981;281:152-6. 163. Driscoll DJ, Garson Jr A, McNamara DG. Anomalous origin of the left coronary artery from the right pulmonary artery associated with complex congenital heart disease. Cathet Cardiovasc Diagn 1982;8:55-61. 164. Coe JY, Radley-Smith R, Yacoub M. Clinical and hemodynamic significance of anomalous origin of the right coronary artery from the pulmonary artery. Thorac Cardiovasc Surg 1982;30:84-7. 165. Laborde F, Marchand M, Leca F, Jarreau MM, Dequirot A, Hazen E. Surgical treatment of anomalous origin of the left

Volume

117

Number

2

166.

167.

168.

169. 170.

171.

172.

173. 174. 175.

176.

177.

178. 179. 180.

181. 182. 183. 184.

coronary artery in infancy and childhood. Early and late results in 20 consecutive cases. J Thorac Cardiovasc Surg 1981;82:423-8. Stephenson LW, Edmunds Jr LH, Friedman S, Meijboom E, Gewitz M, Weinberg P. Subclavian-left coronary artery anastomosis (Meyer operation) for anomalous origin of the left coronary artery from the pulmonary artery. Circulation 1981;64:1130-3. Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases. Circulation 1968;38:403-25. Reis RL, Cohen LS, Mason DT. Direct measurement of instantaneous coronary blood flow after total correction of anomalous left coronary artery. Circulation 1969;39 (suppl):229-34. Askenazi J, Nadas AS. Anomalous left coronary artery originating from the pulmonary artery. Report on 15 cases. Circulation 1975;51:976-87. Chiariello L, Meyer J, Reul Jr GJ, Halhnan GL, Cooley DA. Surgical treatment for anomalous origin of left coronary artery from pulmonary artery. Ann Thorac Surg 1975; 19443-50. Neches WH, Mathews RA, Park SC, Lenox CC, Zuberbuhler JR, Siewers RD, Bahnson HT. Anomalous origin of the left coronary artery from the pulmonary artery. A new method of surgical repair. Circulation 1974;50:582-7. El-Said GM, Ruzyllo W, Williams RL, Mullins CE, Hallman GL, Cooley DA, McNamara DG. Early and late result of saphenous vein graft for anomalous origin of left coronary artery from pulmonary artery. Circulation 1973; 48(suppl 3):2-6. Perry LW, Scott LP, Anomalous left coronary artery from pulmonary artery: report of 11 cases: review of indications for and results of surgery. Circulation 1970;41:1043-52. Kimbiris D, Iskandrian AS, Segal BL, Bemis CE. Anomalous aortic origin of coronary arteries. Circulation 1978; 58:606-15. Palomo AR, Schrager BR, Chahine RA. Anomalous origin of the right coronary artery from the ascending aorta high above the left posterior sinus of Valsalva of a bicuspid aortic valve. AM HEART J 1985:109:902-4. Young Hyman PJ, Tommaso’CL, Singleton RT. A new double coronary artery anomaly: the right coronary artery originating above the coronary sinus giving off the circumflex artery. J Am Co11 Cardiol 1984;4:1329-31. King BD, Ambrose JA, Stein JH, Ro JH, Herman MV. Anomalous origin of the right coronary artery from the ascending aorta above the left coronary sinus. Cathet Cardiovasc Diagn 1982;8:277-80. Yans J, Kumar SP, Kwatra M. Anomalous origin of the right coronary artery above the left sinus of Valsalva. Cathet Cardiovasc Diagn 1978;4:407-12. Vacek JL, Stock PD, Davis WR. Aberrant origin of the right ventricular coronary artery: a report of two cases. Cathet Cardiovasc Diagn 1984;10:369-76. Palomo AR, Schrager BR, Chahine RA. Anomalous separate origin of the septal perforator coronary artery from the left sinus of valsalva. Cathet Cardiovasc Diagn 1984;lO: 385-8. Husaini SN, Beaver WL, Wilson JJ, Lath RD. Anomalous right coronary artery arising from the left mainstem. Cathet Cardiovasc Diagn 1983;9:407-9. Berdoff R, Haimowitz A, Kupersmith J. Anomalous origin of the right coronary artery from the left sinus of Valsalva. Am J Cardiol 198658656.7. Barbour DJ, Roberts WC. Origin of the right from the left main coronary artery (single coronary ostium in aorta). Am J Cardiol 1985;55:608-9. Muus CJ, McManus BM. Common origin of right and left coronary arteries from the region of left sinus of Valsalva:

Coronary anomalies

185. 186.

187. 188. 189.

190.

191.

192. 193.

194.

195. 196.

197.

43 1

association with unexpected intrauterine fetal death. AM HEART J 1984;107:1285-6. Brandt III B, Martins JB, Marcus ML. Anomalous origin of the right coronary artery from the left sinus of Valsalva. N Engl J Med 1983;309:596-8. Moodie DS, Gill C, Loop FD, Sheldon WC. Anomalous left main coronary artery originating from the right sinus of Valsalva: pathophysiology, angiographic definition, and surgical approaches. J Thorac Cardiovasc Surg 1980; 80:198-205. Davia JE, Green DC, Cheitlin MD, DeCastro C, Brott WH. Anomalous left coronary artery origin from the right coronary sinus. AM HEART J 1984;108:165-6. Dalal JJ, West RO, Parker JO. Isolated anomaly of the left anterior descending coronary artery. Cathet Cardiovasc Diagn 1984;10:189-93. Rath S, Har-Zahav Y, Battler A, Agranat 0, Schneeweiss A, Rabinowitz B, Neufeld HN. Frequency and clinical significance of anomalous origin of septal perforator coronary artery. Am J Cardiol 1986;58:657-8. Roberts WC, Waller BF, Roberta CS. Fatal atherosclerotic narrowing of the right main coronary artery: origin of the left anterior descending or left circumflex coronary artery from the right (the true “left-main equivalent”). AM HEART J 1982;104:638-41. Chaitman BR, Lesperance J, Saltiel J, Bourassa MG. Clinical, angiographic, and hemodynamic findings in patients with anomalous origin of the coronary arteries. Circulation 1976;53:122-31. Saenz CB, Taylor JL, Soto B, Nanda NC, Kirklin JK. Acute myocardial infarction in a patient with anomalous right coronary artery. AM HEART J 1986;112:1092-4. Chaitman BR, Lesperance J, Saltiel J, Bourassa MG. Clinical, angiographic, and hemodynamic findings in patients with anomalous origin of the coronary arteries. Circulation 1975;53:122-31. Liberthson RR, Dinsmore RE, Bharati S, Rubenstein JJ, Caulfeld J, Wheeler EO, Harthorne JW, Lev M. Aberrant coronary origin from the aorta. Diagnosis and clinical sienificance. Circulation 1974:50:774-g. Klmbiris D. Anomalous origin of the left main coronary artery from the right sinus of Valsalva. Am J Cardiol 1985;55:765-9. Ishikawa T, Brandt PW. Anomalous origin of the left main coronary artery from the right anterior aortic sinus: angiographic definition of anomalous course. Am J Cardiol 1985;55:770-6. Silverman KJ, Bulkley BH, Hutchins GM. Anomalous left circumflex coronary artery: “normal” variant of uncertain clinical and pathologic significance. Am J Cardiol 1978; 41:1311-14.

198. Cheitlin MD, DeCastro CM, McAllister HA. Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva. A not so minor congenital anomaly. Circulation 1974;50:780-7. 199. Joswig BC, Warren SE, Vieweg WV, Hagan AD. Transmural myocardial infarction in the absence of coronary arterial luminal narrowing in a young man with single coronary arterial anomaly. Cathet Cardiovasc Diagn 1978;4:297304. 200. Liberthson RR, Dinsmore RE, Fallon JT. Aberrant coronary artery origin from the aorta. Report of 18 patients, review of literature and delineation of natural history and management. Circulation 1979;59:748-54. 201. Barth III CW, Roberts WC. Left main coronary artery originating from the right sinus of Valsalva and coursing between the aorta and pulmonary trunk. J Am Co11Cardio‘i 1986;7:366-73. 202. Rou PR, Saunders A, Sowton GE. Review of variations in origin of left circumflex artery. Br Heart J 1975;37:287-92. 203. White NK, Edwards JE. Anomalies of the coronary arteries. report of four cases. Arch Path01 1948;45:766-71.

432

Angelini

American

204. Antopol W, Kugel MA. Anomalous origin of the left circumflex coronary artery. AM HEART J 1933;8:802-6. 205. Murnhv DA. Rov DL. Sohal M. Chandler BM. Anomalous origin of left main coronary artery from anterior sinus of Valsalva with myocardial infarction. J Thorac Cardiovasc Surg 1978;75:282-5. 206. Barth III CW, Roberts WC. Left main coronary artery originating from the right sinus of Valsalva and coursing between aorta and pulmonary trunk. J Am Co11 Cardiol 1986;7:366-71. 201. Topaz 0, Edwards JE. Pathologic features of sudden death in children, adolescents, and young adults. Chest 1985; 871476-82. 208. Kelley MJ, Wolfson S, Marshall R. Single coronary artery from the right sinus of Valsalva: angiography, anatomy, and clinical significance. Am J Roentgen01 1977;128:25762. 209. Schulte MA, Waller BF, Hull MT, Pless JE. Origin of the left anterior descending coronary artery from the right aortic sinus with intramyocardial tunneling to the left side of the heart via the ventricular septum: a case against clinical and morphologic significance of myocardial bridgine. AM HEART J 1985:110:499-501. 210. Miyers DG, McManus BM, McCall D, Walsh RA, Quaife MA. Single coronary artery with the right coronary artery arising from the first septal perforator. Cathet Cardiovasc Diagn 1984;10:479-84. 211. Roberts WC, Dicicco BS, Waller BF, Kishel JC, McManus BM, Dawson SL, Hunsaker III JC, Luke JL. Origin of the left main from the right coronary artery or from the right aortic sinus with intramyocardial tunneling to the left side of the heart via the ventricular septum: the case against clinical significance of myocardial bridge or coronary tunnel. AM HEART J 1982;104:303-5. 212. Saner HE, Saner BD, Dykoski RK, Edwards JE. Origin of anterior descending coronary artery from right aortic sinus. Intramyocardial tunneling to the left side of the heart. Arch Path01 Lab Med 1984;108:642-3. 213. Reference deleted in proofs. 214. Simkoff WL, Murphy ES, DeMots H, Khonsari S, Abbruzzese P. Anomalous origin of the right coronary artery from the left anterior descending: angiographic diagnosis in a patient with coronary artery disease. Cathet Cardiovasc Diagn 1982;8:49-53. 215. Banchi A. Morfologia della arteriae coronariae cordis. Arch Ital Anat Embriol-1903;3:89-97. 216. Ogden JA, Goodyear AVN. Patterns of distribution of the single coronary artery. Yale J Biol Med 1970;43:11. 217. Smith JC. Review of single coronary artery with report of 2 cases. Circulation 195O;i:1168-75. 218. Linton MJ. Barrv WH. Obrez I. Silverman J. Wexler L. Isolated single coronary artery: diagnosis, angiographic classification, and clinical significance. Radiology 1979; 130:39-47. 219. Vuthoori S, Waisser E, Angelini P. Case report. Triple origin of left coronary arteries from right coronary artery: unusual case of single coronary artery. Clin Cardiol 1980; 3:67-g. 220. Murphy ME. Single coronary artery. AM HEART J 1967; _”

,

74557-61.

221. Robicsek F. Origin of the left anterior descending coronary artery from the left mammary artery. AM HEART J 1984; 108:1377-8.

222. Davis JS, Lie JT. Anomalous origin of a single coronary artery from the innominate artery. Angiology 1977;28: 775-8. 223. Robicsek F, Sanger PW, Daugherty HK, Gallucci V. Origin of the anterior interventricular (descending) coronary artery and vein from the left mammary vessels. A prevlously unknown anomaly of the coronary system., J Thorac Cardiovasc Surg 1967;53:602-4. 224. Cheatham JP, Ruyle NA, McManus BM, Bammel GE.

225.

226.

227.

228.

229.

230.

231.

232. 233. 234. 235. 236. 231.

238. 239. 240.

February l!XW Heart Journal

Origin of the right coronary artery from the descending thoracic aorta: angiographic diagnosis and unique coronary artery anatomy at autopsy. Cathet Cardiovasc Diagn 1987;13:321-4. Ott DA, Cooley DA, Pinsky WW, Mullins CE. Anomalous origin of circumflex coronary artery from right pulmonary artery. Report of a rare anomaly. J Thorac Cardiovasc Surg 1978;76:190-4. Bharati S, Chandra N, Stephenson LW, Wagner HR, Weinberg PM, Lev M. Origin of the left coronary artery from the right pulmonary artery. J Am Co11 Cardiol 1984; 3:1565-g. Ott DA, Cooley DA, Pinsky WW, Mullins CE. Anomalous origin of circumflex coronary artery from right pulmonary artery: report of a rare anomaly. J Thorac Cardiovasc Surg 1978;76:190-4. Doty DB, Chandramouli B, Schieken RE, Lauer RM, Ehrenhaft JL. Anomalous origin of the left coronary artery from the right pulmonary artery. J Thorac Cardiovasc Surg 1976;71:787-91. Honey M, Lincoln JC, Osborne MP, de Bono DP. Coarcta tion of aorta with right aortic arch. Report of surgical correction in 2 cases: one with associated anomalous origin of left circumflex coronary artery from the right pulmonary artery. Br Heart J 1975;37:937-45. Waldman JD. Lamberti JJ. Mathewson JW. George L. Surgical closure of the tricuspid valve for’ pulmonary atresia, intact ventricular septum, and right ventricle to coronary artery communications. Pediatr Cardiol 1984; 5:221-3. Noble J, Bourassa MG, Petitclerc R, Dyrda I. Myocardial bridging and milking effect of the left anterior descending coronary artery: normal variant or obstruction? Am J Cardiol 1976;37:993-9. Roberts WC, Silver MA, Sapala JC. Intussusception of a coronary artery associated with sudden death in a college football player. Am J Cardiol 1986;57:179-80. Parsonnet V. Intracavitary coronary arteries. [Letter]. Ann Thorac Surg 1985;40:206. Ochsner JL, Mills NL. Surgical management of diseased intracavitary coronary arteries. Ann Thorac Surg 1984; 38356-62. de Zwaan C, Wellens HJ. Left ventricular aneurysm subsequent to cleavage of myocardial bridging of a coronary artery. J Am Co11 Cardiol 1984;3:1345-8. Huhta JC, Edwards WD, Danielson GK. Supravalvular mitral ridge containing the dominant left circumflex coronary artery. J ThoracCardiovasc Surg 1981;81:577-9. Griffiths SP, Ellis K, Hordof AJ, Martin E, Levine OR, Gersony WM. Spontaneous complete closure of a congenital coronary artery fistula. J Am Call Cardiol 1983;2:116973. Mahoney LT, Schieken RM, Lauer RM. Sponataneous closure of a coronary artery fistula in childhood. Pediatr Cardiol 1982;2:311-12. Shubrooks Jr SJ, Naggar CZ. Spontaneous near closure of coronary artery fistula. Circulation 1978;57:197-9. Wilde P, Watt I. Congenital coronary artery fistulae: six new cases with a collective review. Clin Radio1 1980;31:30111.

241. Vogelbach KH, Edmiston WA, Stenson RE. Coronary artery-left ventricular communications: a report of two cases and review of the literature. Cathet Cardiovasc Diagn 1979;5:159-67. 242. Schneeweiss A, Rath S, Neufeld HN. Bilateral congenital coronary artery fistula. Thorax 1981;36:697-8. 243. Meyer J, Reul GJ, Mullins CE, McCoy J, Hallman GL, Cooley DA. Congenital fistulae of the coronary arteries. Clinical considerations and surgical management in 23 patients. J Cardiovasc Surg (Torino) 1975;16:506-11. 244. John S, Perianayagam WJ, Muralidiaran S, Nandakumar V, Mansfield R, Krishnaswamy S, Sukumar IP, Cherian G.

Volume Number

245.

246. 247.

248. 249. 250. 251. 252. 253. 254. 255.

256. 257. 258. 259. 260. 261. 262.

263. 264. 265.

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Surgical treatment of congenital coronary artery fistula. Thorax 1981;36:359-4. Macri R, Capulzini A, Fazzini L, Cornali M, Verunelli F, Reginato E. Congenital coronary artery fistula: report of five patients, diagnostic problems and principles of manaeement. Thorac Cardiovasc Sum 1982:30:167-‘71. Urrutia SCO, Falaschi G, Ott DA, Cooley DA. Surgical management of 56 patients with congenital coronary artery fist&s. Ann Thorac Surg 1983;35:396-57. Wilcox WD, Neal MJ, Alpert BS, Taylor AB, Dooley KJ. Localized occurrence of congenital coronary artery fistula in the southeast United States. Am J Cardiol 1986;57: 361-3. Hobbs RE, Millit HD, Raghavan PV, Moodie DS, Sheldon WC. Coronary artery fistulae: a lo-year review. Cleve Clin Q 1982;49:191-7. Lowe JE, Oldham Jr HN, Sabiston Jr DC. Surgical management of congenital coronary artery fistulas. Ann Surg 1981;194:373-86. Rittenhouse EA, Doty DB, Ehrenhaft JL. Congenital coronary artery-cardiac chamber fistula. Review of operative management. Ann Thorac Surg 1975;20:468-85. De Nef JJ, Varghese PJ, Losekoot G. Congenital coronary artery fistula: report of 17 cases with a note on natural history of lesion. Br Heart J 1971;33:150. Jaffe RB, Glancy DL, Epstein SE, Brown BG, Morrow AG. Coronary arterial-right heart fistulae. Long-term observations in seven patients. Circulation 1973;47:133-43. Floyd WL, Young WG, Johnsrude IS. Coronary arterialleft atria1 fistula. Case with obstruction of the inferior vena cava by a giant left atrium. Am J Cardiol 1970;25:716-22. De Nef JJ, Varghese PJ, Losekoot G. Congenital coronary artery fistula. Analysis of 17 cases. Br Heart J 1971;33:85762. Krajcer Z, Leachman RD, Lufschanowski R, Cooley DA, Angelini P. Anomalous left coronary artery from pulmonary artery. Unusual case complicated by coronary arterial disease and fistula from coronary artery to left ventricle. Chest 1978;74:102-4. Effler DB, Sheldon WC, Turner JJ, Groves LK. Coronary arteriovenous fistulae: diagnosis and surgical management. Report of fifteen cases. Surgery 1967;61:41-8. Kiso I, Itoh T, Morishita M, Kato K, Ishikura Y. Blood flow and pressure measurements of right coronary artery to left ventricle fistula. Thorax 1978;33:253-6. Aytac A, Bilgic A, Olga R, Olgunturk R, Saylam A. Congenital right coronary artery-right atrial fistula. Jpn Heart J 1980;21:409-15. Chen W, Woo KS, Kong SM, Mok CK. Coronary artery to left ventricle fistulas: echocardiographic features. Cardiovast Intervent Radio1 1982;5:241-5. Start TJ, Bowman FO, Hordof AJ. Congestive heart failure in a newborn secondary to coronary artery-left ventricular fistula. Am J Cardiol 1986;58:366-7. Adams P, Morris L, Ross I. Congenital left coronary artery-right ventricular fistula. Aust Paediatr J 1983; 19:47-50. Midell AI, Bermudez GA, Replogle R. Surgical closure of left coronary artery-left ventricular fistula: the second case reported in the literature and a review of the five previously reported cases of coronary artery fistula terminating in the left ventricle. J Thorac Cardiovasc Surg 1977;74:199203. Muraki H, Sakai M, Uozumi Z. Coronary artery fistula draining into left ventricle: case report and review, Jpn Heart J 1976;17:531-9. Abrams LD, Evans DW, Howarth FH. Coronary arteryright ventricular fistula treated surgically. Br Heart J 1967;29:132-4. Gillebert C, Van Hoof R, Van de Werf F, Piessens J, De Geest H. Coronary artery fist&s in an adult population. Eur Heart J 1986;7:437-43.

Coronary

anomalies

433

266. Sung C, Leachman RD, Zerpa F, Angelini P, Lufschanowski R. Aortico-left ventricular tunnel. AM HEART J 1979; 98:87-92. 267. Levy MJ, Lillehei CW, Anderson RH, Amplatz K, Edwards JE. Aortic-left ventricular tunnel. Circulation 1963;27:84154. 268. Croom III RD, Wilcox BR, Abney III RL. Right coronary artery-coronary sinus arteriovenous fistula. Ann Thorac Surg 1967;4:182-8. 269. Ogden JA, Stansel Jr HC. Coronary arterial fistulas terminating in the coronary venous system. J Thorac Cardiovasc Surg 1972;63:172-82. 270. MacMillan RM, Shahriari A, Sumithisena Fender BK, Maranhao V, Clark D. Contrast-enhanced tine computed tomography for diagnosis of right coronary artery to coronary sinus arteriovenous fistula. Am J Cardiol 1985;56: 997-8. 271. Kimbiris D, Kasparian H, Knibbe P, Brest AN. Coronary artery-coronary sinus fistula. Am J Cardiol 197($26:532-g. 272. Sheikhzadeh A, Stierle U, Langbehn AF, Thoran P, Diederich KW. Generalized coronary arterio-systemic (left ventricular) fistula. Case report and review of literature. Jpn Heart J 1986;27:533-44. 273. Arani DT, Greene DG, Klocke FJ. Coronary artery fistulas emptying into left heart chambers. AM HEART J 1978; 96438-43. 274. Macchi RJ, Fabregas RA, Chiarielli HO, Bourdet JC, Lhez 0, Stagnaro R. Anomalous communication of the left coronary artery with a peripheral branch of the right pulmonary artery. Chest 197&69:565-S. 275. Lukacs L, Arvay A. Bilateral congenital coronary arterypulmonary artery fistulas. Report of two cases and review of the literature. J Cardiovasc- Surg (Torino) 1986;27:99-3. 276. Oeasawara K. Aizawa T. Fuiii J. Watanabe H. Uchida E. Kitoh K. A case with fistul& from both coronary arterie; and the left bronchial artery to the pulmonary artery. Jpn Heart J 1985;26:597-601. 277. Dark JH. Pollock JC. Coronarv arterv-nulmonarv arterv fistula in’tetralogy of Fallot with pulmonary atreiia. Eur Heart J 1985;6:714-16. 278. Bairn DS, Kline H, Silverman JF. Bilateral coronary artery-pulmonary artery fistulas. Report of five cases and review of the literature. Circulation 1982:65:810-15. 279. Mori K, Onoe T, Ooka T. Three main coronary arteries to pulmonary artery fistula. Jpn Circ J 1981;45:209.12. 280. DeBakey ME, Lawrie GM. Right coronary artery-to-right pulmonary artery fistula: surgical correction. J Thorac Cardiovasc Surg 1980;80:225-7. 281. Francis CK, Sacheti CK, Cohen RB. Fistulous communication between the left coronary artery and main pulmonary artery: a thirteen-year follow-up. Cathet Cardiovasc Diagn 1979;5:357-66. 282. Feigenberg Z, Kaplinsky E, Kissin L, Levy MJ. Unusual congenital coronary arterio-venous fistulae with surgical correction. Vast Surg 197’7;11:14-18. 283. Bjork L. Angiographic demonstration of extracardial.anastomoses to the coronarv arteries. Radiolozv 1966:87: 274-7. 284. Phillips SJ, Goodman P, Goldfine R, Pall L, Rubenfire M, Jaron D, Kantrowitz A. Blood flow measurements during repair of a right coronary artery superior vena cava fistula. Ann Surg 1973;177:63-5. 285. Sheikhzadeh A, Ghabussi P. A survey of uncommon forms of coronary arteries. Jnn Heart J 1982:23:725-31. 286. Lim CH, Tan NC, Ti L, Seah CS, Tan D. Giant congenital aneurysm of the right coronary artery. Am J Cardiol 1977;39:751-3. 287. Demany MA, Zimmerman HA. Congenital anomalies of the coronary arteries. A report of three cases. Angiology 1967; 18370-7. 288. Bjork L. Ectasia of the coronary arteries. Radiology 1966; 87:33-4. -I

February

434

Angelini

American

289. Lynch P. Soldiers, sport and sudden death. Lancet 1980; 1:1235-7. 290. Iskandrian AS, Hakki AH, Bemis CE. Myocardial ischemia in a patient with anomalous origin of the left main coronary artery. Cathet Cardiovasc Diagn 1986;12:48-50. 291. Imamura T, Nakagawa S, Koiwaya Y, Tanaka K, Saisho K, Sumiyoshi A. Recurrent myocardisl infarction and unexpected sudden death in a case of d-loop d-transposition of the great arteries associated with single coronary artery. Clin Cardiol 1986;9:77-81. 292. Barth III CW, Bray M, Roberts WC. Sudden death in infancy associated with origin of both left main and right coronary arteries from a common ostium above the left sinus of Valsalva. Am J Cardiol 1986:57:365-6. 293. Edelstein J, Juhasz RS. Myocardial infarction in the distrubition of a patent anomalous left circumflex coronary artery. Cathet Cardiovasc Diagn 1984;10:171-6. 294. Isner JM, Shen EM, Martin ET, Fortin RV. Sudden unexpected death as a result of anomalous origin of the right coronary artery from the left sinus of Valsalva. Am J Med 1984;76:155-8. 295. Visscher DW, Miles BL, Waller BF. Tunneled (“bridged”) left anterior descending coronary artery in a newborn without clinical or morphologic evidence of myocardial ischemia. Cathet Cardiovasc Diagn 1983;9:493-6. 296. Hanzlick RL, Stivers RR. Sudden death due to anomalous right coronary artery in a 26-year-old marathon runner. Am J Forensic Med Path01 1983;4:265-8. 297. Hanzlick R, Stivers RR. Sudden death in a marathon runner with origin of right coronary artery from the left sinus of Valsalva [Letter]. Am J Cardiol 1983;51:1467. 298. Liberthson RR, Gang DL, Custer J. Sudden death in an infant with aberrant origin of the right coronary artery from the left sinus of Valsalva of the aorta: case report and review of the literature. Pediatr Cardiol 1983;4:45-8. 299. Bloomfield P, Ehrlich C, Folland ED, Bianco JA, Tow DE, Parisi AF. Anomalous right coronary artery: a surgically correctable cause of angina pectoris. Am J Cardiol 1983; 51:1235-7. 300. Mintz GS, Iskandrian AS, Bemis CE, Mundth ED, Owens JS. Myocardial ischemia in anomalous origin of the right coronary artery from the pulmonary trunk. Proof of a coronary steal. Am J Cardiol 1983;51:610-12. 301. Cheng TO. Left coronary artery-to-left ventricular fistula: demonstration of coronary steal phenomenon. AM HEART J 1982;104:870-2.

302. Ahmed SS, Haider

B, Regan TJ. Silent

left coronary

303.

304. 305.

306.

307. 308.

309.

310. 311. 312.

313. 314.

Heart

,989 Journal

artery-cameral fistula: probable cause of myocardial isch emia. AM HEART J 1982;104:869-70. Liberthson RR, Zaman L, Weyman A, Kiger R, Dinsmore RE, Leinbach RC, Strauss HW, Buckley MJ. Aberrant origin of the left coronary artery from the proximal right coronary artery: diagnostic features and pre- and postoperative course. Clin Cardiol 1982;5:377-81. Hobbs RE, Millit HD, Raghavan PV, Moodie DS, Sheldon WC. Congenital coronary artery anomalies: clinical and therapeutic implications. Cardiovasc Clin 1981;12:43-58. Rajfer SI, Oetgen WJ, Weeks Jr KD, Kaminski RJ, Rocchini AP. Thallium-201 scintigraphy after surgical repair of hemodynamically significant primary coronary artery anomalies. Chest 1982;81:687-92. Roberts WC, Siegel RJ, Zipes DP. Origin of the right coronary artery from the left sinus of valsalva and its functional consequences: analysis of 10 necropsy patients. Am J Cardiol 1982;49:863-8. McClellan JT, Jokl E. Congenital anomalies of coronary arteries as cause of sudden death associated with physical exertion. Am J Clin Path01 1968;50:229-33. Chee TP, Jensen DP, Padnick MB, Cornell WP, Desser KB. Myocardial bridging of the left anterior descending coronary artery resulting in subendocardial infarction. Arch Intern Med 1981;141:1703-4. Ahmad M, Merry SL, Haibach H. Evidence of impaired myocardial perfusion and abnormal left ventricular function during exercise in patients with isolated systolic narrowing of the left anterior descending coronary artery. Am J Cardiol 1981;48:832-6. Kafrouni G, Khan AH, Wolfsen JL. Single right coronary artery: clinical and angiographic findings with surgical management. Ann Thorac Surg 1981;32:80-4. Young MW, Hamby RI, Zaret B. Anomalous origin of the left coronary artery: angiographic and myocardial perfusion scintigraphic correlates. Chest 1981;79:473-5. Moodie DS, Cook SA, Gill CC, Napoli CA. Thallium-201 myocardial imaging in young adults with anomalous left coronary artery arising from the pulmonary artery. J Nucl Med 1980;21:1076-9. Warren SE, Alpert JS, Vieweg WV, Hagan AD. Normal single coronary artery and myocardial infarction. Chest 1977;72:540-3. Haravon A, Franklin M, Krauthamer MJ. Congenital coronary artery to left ventricle fistula with angina pectoris. Coronary steal syndrome? NY State J Med 1972; 72:2196-200.