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Ann Thorac Surg 2000;69:S270-S297
© 2000 The Society of Thoracic Surgeons

Congenital Heart Surgery Nomenclature and Database Project: anomalies of the coronary arteries

Ali Dodge-Khatami, MDa, Constantine Mavroudis, MDa, Carl L. Backer, MDa

a Division of Cardiovascular-Thoracic Surgery, Northwestern University School of Medicine, Department of Surgery, Children’s Memorial Hospital, Chicago, Illinois, USA

Address reprint requests to Dr Mavroudis, Division of Cardiovascular-Thoracic Surgery, Children’s Memorial Hospital, 2300 Children’s Plaza, m/c 22, Chicago, IL 60614
e-mail: c-mavroudis{at}nwu.edu

Presented at the International Nomenclature and Database Conferences for Pediatric Cardiac Surgery, 1998–1999.

Abstract

The extant nomenclature for coronary artery anomalies is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. The seven major categories of coronary artery anomalies are: anomalous pulmonary artery origins of the coronaries, anomalous aortic origins of the coronaries, congenital atresia of the left main coronary artery, coronary artery fistulas, coronary artery bridging, coronary aneurysms, and coronary stenosis. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail, which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented, which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

I. Background

The incidence of coronary anomalies is 0.2% to 1.2% of the population. Various anomalies have been described since the 18th century, both clinically and on pathology studies, but the first landmark article, attempting a comprehensive classification, was by Ogden in 1969 [1], to which most authors refer, and from which the many modifications of nomenclature derive. Congenital variations of the coronary artery (Ogden, 1969) include:

Minor coronary variations
High take-off
Multiple ostia
Anomalous circumflex artery origin
Anomalous anterior descending artery origin
Absent proximal ostium/single ostium in other aortic sinus
Hypoplastic proximal coronary artery
Congenital proximal coronary artery
Congenital distal stenosis
Coronary artery from the posterior aortic sinus
Ventricular origin of an accessory coronary artery

Major coronary anomalies
Coronary "arteriovenous" fistula
Anomalous origin from the pulmonary artery
Left coronary artery
Right coronary artery
Both coronary arteries


Secondary coronary anomalies
Secondary coronary "arteriovenous" fistula
Variations in transposition of the great vessels
Variations in truncus arteriosus
Variations in tetralogy of Fallot
Ectasia of coronary arteries in supravalvular aortic stenosis
Mural coronary artery

This classification includes major, minor, and secondary anomalies on the basis of anatomical but not clinical considerations (ie, minor, arising directly from the aorta and distal distribution normal; major, abnormal origin from the pulmonary artery (PA) or abnormal communications with intracardiac structures). It is debatable whether minor and major are appropriate terms given the potential clinical and/or surgical significance.

The term anomalies of the coronary arteries will serve to define the first hierarchy level. The second hierarchy level is formed by the following diagnoses:

  1. Anomalous pulmonary origins of the coronaries (APOC)
  2. Anomalous aortic origins of the coronaries (AAOC)
  3. Congenital atresia of the left main coronary artery (CALM)
  4. Coronary arteriovenous fistulas (CAVF)
  5. Coronary artery bridging (CB)
  6. Coronary artery aneurysms (CAn)
  7. Coronary stenosis

In most instances, these anomalies are supposed in normal hearts, as the various coronary variations in complex congenital heart diseases are not encompassed in this review.

Hierarchy level 1

Coronary anomalies (CA)

Hierarchy level 2

Coronary anomalies (CA), NOS

Coronary anomalies (CA), Anomalous pulmonary origins of the coronaries (APOC)
Coronary anomalies (CA), Anomalous aortic origins of the coronaries (AAOC)
Coronary anomalies (CA), Congenital atresia of the left main (CALM)
Coronary anomalies (CA), Coronary arteriovenous fistulas (CAVF)
Coronary anomalies (CA), Coronary bridging
Coronary anomalies (CA), Coronary aneurysms (CAn)
Coronary anomalies (CA), Coronary stenosis

II. Analysis

A. Anomalous pulmonary origins of the coronaries (APOC)
Anomalous left coronary artery originating from the pulmonary artery (ALCAPA) is a rare congenital anomaly first described by Brooks [2] in 1885, and is present in 1 out of 300,000 live births. This anomaly may cause myocardial ischemia or infarction, mitral insufficiency, congestive heart failure, and death in early infancy, if not treated. The full clinical spectrum was reported by Bland, White, and Garland [3] in 1933, and the syndrome of angina and ischemic myocardial insult from abnormal coronary flow bears their name today. It is the most common of the APOC, which also includes the very rare anomalous right coronary artery from the PA (ARCAPA), anomalous circumflex from the PA (ACxPA) and anomalous right and left coronaries (both).

In 1989, Smith and associates [4] described various patterns pertaining to ALCAPA and their possible surgical implications.

The sinus of the pulmonary valves are designated right hand (sinus number 1), and left hand (sinus number 2), as viewed from the nonfacing sinus of the pulmonary trunk toward the aorta. Sinus 1 of the aorta faces sinus 2 of pulmonary trunk and vice versa; both nonfacing sinuses are at the two extremities (Fig 1).

  1. Anomalous origin of the left main coronary artery from the pulmonary artery (ALCAPA)
    1. From right handed sinus (sinus 1)
    2. From nonfacing pulmonary sinus
    3. From left-handed sinus (sinus 2)
    4. From commissure between sinus 1 and nonfacing sinus
    5. From commissure between sinus 2 and nonfacing sinus
    6. From commissure between sinus 1 and sinus 2
    7. High takeoff from left or right pulmonary arteries

  2. Anomalous origin of the right coronary artery (ARCAPA)
  3. Anomalous origin of the circumflex coronary artery from the PA (ACxPA)
  4. Anomalous right and left coronaries from the PA (both)



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  		Fig 1. Diagrammatic representation of aortic and pulmonary artery origins of the left main coronary artery in normal and anomalous conditions. Cephalic views depict a person in the nonfacing sinus with the right hand always signifying sinus 1 and the left hand always signifying sinus 2.

 
An origin impinging on a commissure between sinuses would complicate transfer or tunnel operations from possible interference with pulmonary valve leaflet movement, and potential resultant pulmonary insufficiency. A high takeoff above the sinopulmonary bar could be fatal if a PA banding is performed.

The proposed classification by hierarchy levels for APOC is as follows.

APOC hierarchy level 3

CA, APOC, NOS

CA, APOC, Anomalous left coronary from the pulmonary artery (ALCAPA)
CA, APOC, Anomalous right coronary from the pulmonary artery (ARCAPA)
CA, APOC, Anomalous circumflex from the pulmonary artery (ACxPA)
CA, APOC, Anomalous left and right coronaries from the pulmonary artery (Both ALCAPA and ARCAPA)

APOC hierarchy level 4

CA, APOC, NOS
CA, APOC, ALCAPA, NOS

CA, APOC, ALCAPA, Origin sinus 1 (right-handed pulmonary sinus)
CA, APOC, ALCAPA, Origin sinus 2 (left-handed pulmonary sinus)
CA, APOC, ALCAPA, Origin nonfacing pulmonary sinus
CA, APOC, ALCAPA, Origin from commissure between sinus 1 and nonfacing sinus
CA, APOC, ALCAPA, Origin from commissure between sinus 2 and nonfacing sinus
CA, APOC, ALCAPA, Origin from commissure between sinus 1 and sinus 2
CA, APOC, ALCAPA, Origin from left PA
CA, APOC, ALCAPA, Origin from right PA

CA, APOC, ARCAPA, NOS

CA, APOC, ARCAPA, Origin sinus 1 (right-handed pulmonary sinus)
CA, APOC, ARCAPA, Origin sinus 2 (left-handed pulmonary sinus)
CA, APOC, ARCAPA, Origin nonfacing pulmonary sinus
CA, APOC, ARCAPA, Origin from commissure between sinus 1 and nonfacing sinus
CA, APOC, ARCAPA, Origin from commissure between sinus 2 and nonfacing sinus
CA, APOC, ARCAPA, Origin from commissure between sinus 1 and sinus 2
CA, APOC, ARCAPA, Origin from left PA
CA, APOC, ARCAPA, Origin from right PA

CA, APOC, ACxPA, NOS

CA, APOC, ACxPA, Origin sinus 1 (right-handed pulmonary sinus)
CA, APOC, ACxPA, Origin sinus 2 (left-handed pulmonary sinus)
CA, APOC, ACxPA, Origin nonfacing pulmonary sinus
CA, APOC, ACxPA, Origin from commissure between sinus 1 and nonfacing sinus
CA, APOC, ACxPA, Origin from commissure between sinus 2 and nonfacing sinus
CA, APOC, ACxPA, Origin from commissure between sinus 1 and sinus 2
CA, APOC, ACxPA, Origin from left PA
CA, APOC, ACxPA, Origin from right PA

CA, APOC, Both ALCAPA and ARCAPA

GUEST EDITORS’ NOTE: If "CA, APOC, Both ALCAPA and APCAPA" is chosen, one must then code both the ALCAPA and ARCAPA separately as subsequent diagnoses in order to fully describe the origin of the ALCAPA and ARCAPA.

B. Anomalous aortic origins of coronary arteries (AAOC)
Anomalous aortic origins of the coronaries are considered minor anomalies by Ogden’s classification [1] and represent one-third of all coronary anomalies [2]. All three coronaries may be involved. Most anomalous aortic origins are considered benign except aberrant origin of the left main coronary artery (LMCA) from the right aortic sinus of Valsalva (RASV) and aberrant origin of the right coronary artery (RCA) from the left aortic sinus of Valsalva (LASV), which are associated with cardiac symptoms and sudden death [5–15]. The fatal potential of a minor coronary anomaly was first described by Jokl in 1962 [16].

AAOC hierarchy level 3

CA, AAOC, NOS

CA, AAOC, Origin of left main from the right aortic sinus of Valsalva (LMCA from RASV)
CA, AAOC, Origin of right coronary artery from the left aortic sinus of Valsalva (RCA from LASV)
CA, AAOC, Origin of left anterior descending coronary artery from the right aortic sinus of Valsalva (LAD from RASV)
CA, AAOC, Origin of left anterior descending coronary artery from the right coronary artery (LAD from RCA)
CA, AAOC, Origin of circumflex coronary artery from the right aortic sinus of Valsalva (Cx from RASV)
CA, AAOC, Origin of circumflex coronary artery from the right coronary artery (Cx from RCA)
CA, AAOC, Single coronary artery
CA, AAOC, Inverted coronary arteries
CA, AAOC, Other anomalous aortic origin of coronary artery, specify

LMCA from RASV
This represents the most serious coronary anomaly of origin, associated with the highest incidence of symptoms and sudden death [5–9]. This risk is further increased if the course is between the great vessels [6, 7, 9], as high as 82% [6]. Four courses of the LMCA from the RASV are possible in relation to the great vessels: (1) anterior to PA; (2) Posterior to the aorta (Ao); (3) between the great vessels; and (4) septal course through conal septum (beneath right ventricular infundibulum) [7].

In almost all cases of aberrant LMCA from RASV, the RCA provides the dominant circulation [10, 11]. If the course of the LMCA is between the great vessels, the LMCA provides 1 or 2 branches to the proximal ventricular septum; if the aberrant LMCA is posterior to the aorta, there are no septal branches from the left but rather from the RCA [10].

Single coronary artery
Single coronary artery is a minor anomaly by Ogden’s classification with a high association of complex congenital heart disease [17]. It has a rare incidence of 0.04%, and was first described by Morgagni in 1761 who noted that 2 coronaries are normal but a single one is the exception. The first classification was proposed by Smith [18] in 1950: type 1, one artery supplying the entire heart, the other truly absent, left or right in equal distribution; type 2, a single artery subdivides into 2 branches with distribution patterns corresponding to normal right and left coronary artery (most common pattern) [17]; and type 3, other.

An additional subdivision of type 2 was proposed by Sharbaugh [17] in 1974: type 2a, the branch that is the missing artery of origin passes anterior to the great vessels; type 2b, the branch that is the missing artery passes between the great vessels; and type 2c, the branch that is the missing artery passes posterior to the great vessels. The artery of origin from the aorta, which then gives the missing branch, is designated as left or right coronary artery.

This classification (left or right and types 1, 2a, 2b, 2c, and 3) is thorough, and is the proposed nomenclature. As mentioned previously, there exists a high incidence of associated coronary heart disease (41%). These include transposition of the great vessels (TGV), tetralogy of Fallot (TOF), truncus arteriosus, coronary arteriovenous fistula, endocardial fibroelastosis, and bicuspid aortic valve. Type 2 is the most common form, and type 2a is associated with TGV and TOF.

Inverted coronary arteries
Inverted coronary arteries implies the exact reverse anatomy of what is considered the norm. In this anomaly, the left main coronary arises from the RASV (sinus 1) and the right coronary artery takes its origin from the LASV (sinus 2). The left main coronary then branches normally into the left anterior descending artery and the circumflex artery.

AAOC hierarchy level 4

CA, AAOC, NOS
CA, AAOC, LMCA from RASV, NOS

CA, AAOC, LMCA from RASV, Origin anterior to pulmonary artery
CA, AAOC, LMCA from RASV, Origin posterior to aorta
CA, AAOC, LMCA from RASV, Origin between great vessels
CA, AAOC, LMCA from RASV, Septal course through conal septum

CA, AAOC, RCA from LASV, NOS

CA, AAOC, RCA from LASV, Origin anterior to aorta
CA, AAOC, RCA from LASV, Origin between great vessels

CA, AAOC, LAD from RASV
CA, AAOC, LAD from RCA
CA, AAOC, Cx from RASV, NOS

CA, AAOC, Cx from RASV, Origin anterior great vessels
CA, AAOC, Cx from RASV, Origin posterior great vessels

CA, AAOC, Cx from RCA artery
CA, AAOC, Single coronary, NOS

CA, AAOC, Single coronary artery, Left
CA, AAOC, Single coronary artery, Right

CA, AAOC, Inverted coronary arteries, NOS
CA, AAOC, Other anomalous aortic origin of coronary artery, specify

AAOC hierarchy level 5

CA, AAOC, Single Coronary, Left, NOS

CA, AAOC, Single coronary artery, Left, Type 1: one artery supplies entire heart
CA, AAOC, Single coronary artery, Left, Type 2: divides in 2 coronaries, right and left
CA, AAOC, Single coronary artery, Left, Type 3: other

CA, AAOC, Single coronary artery, Right, NOS

CA, AAOC, Single coronary artery, Right, Type 1: one artery supplies entire heart
CA, AAOC, Single coronary artery, Right, Type 2: divides in 2 coronaries, right and left
CA, AAOC, Single coronary artery, Right, Type 3: other

AAOC hierarchy level 6

CA, AAOC, Single coronary artery, Left, Type 2: divides in 2 coronaries, right and left, NOS

CA, AAOC, Single coronary artery, Left, Type 2a: divides in 2 coronaries, Right branch anterior to great vessels
CA, AAOC, Single coronary artery, Left, Type 2b: divides in 2 coronaries, Right branch between great vessels
CA, AAOC, Single coronary artery, Left, Type 2c: divides in 2 coronaries, Right branch posterior to great vessels

CA, AAOC, Single coronary artery, Right, Type 2: divides in 2 coronaries, right and left, NOS

CA, AAOC, Single coronary artery, Right, Type 2a: divides in 2 coronaries, Left branch anterior to great vessels
CA, AAOC, Single coronary artery, Right, Type 2b: divides in 2 coronaries, Left branch between great vessels
CA, AAOC, Single coronary artery, Right, Type 2c: divides in 2 coronaries, Left branch posterior to great vessels

C. Congenital atresia of the left main coronary artery (CALM)
Congenital atresia of the left main coronary artery (CALM) is an extremely rare minor anomaly by Ogden’s classification with approximately 40 cases described in the literature. Until the mid 1970s it was considered as a single coronary artery, until Lurie and associates [19] defined it as a distinct entity with a flow pattern and physiology of its own. In single coronary artery, a single left or right coronary (RCA) gives flow to the entire heart in a centrifugal anterograde pattern, from the aorta to the periphery, with decreasing diameter of the vessels, as distal progression towards the capillaries is achieved. In CALM, a single RCA does feed the entire heart, but flow in the left anterior descending (LAD) as well as into the Cx is centripetal, and therefore retrograde, depending on collaterals from the RCA [20]. Collateral channels are through the circle of Vieussens which includes the conal artery, intraseptals, and apical-anterior and posterior ventricular anastomoses [20, 21]. There is also an anastomosis of the anterior interventricular branches of the RCA and the LAD, as well as final ramifications of the posterior descending branch of the RCA anastomosing with the LAD [20]. There is no ostium of the left coronary, as the proximal left main trunk ends blindly. Most importantly, the LAD and Cx arteries are located in their normal anatomic positions and connect in the usual fashion [21, 22]. An association is found with supravalvular aortic stenosis, especially in William’s syndrome [21, 23, 24]. No classification exists, and therefore no nomenclature confusion can arise.

D. Coronary arteriovenous fistulas (CAVF)
Coronary arteriovenous fistulas (CAVF) are considered a major coronary anomaly by Ogden’s classification [1], and represent an anomaly of termination. CAVF were first described by Krause in 1865 [25] and are present in 1 of 50,000 live births [26–28] (0.002% of the general population). They are visualized in 1 of 500 patients undergoing catheterization (0.2% to 0.25%). They are the most common of hemodynamically significant coronary lesions [26–31], and comprise nearly half of all coronary artery anomalies [32]. They may be congenital or acquired (traumatic, infectious, or iatrogenic). CAVF may be isolated in 55% to 80% of cases [25, 28, 32, 33] or associated with other congenital heart disease in 20% to 45%. Associated anomalies include TOF, atrial septal defect, patent ductus arteriosus, ventricular septal defect (VSD), pulmonary atresia—intact ventricular septum, and superimposed coronary artery disease (35%) [26]. Single fistulas are most common, ranging from 74% to 90% [26, 28, 30, 32, 33]. Multiple fistulas are present in 10.7% to 16% [26, 27, 33] of cases, and fistulas originate from both coronaries in 4% to 18% [26–28, 33]. Nomenclature is based on a descriptive analysis of the vessel of origin and the chamber of termination (Table 1). There exists one angiographic classification by Sakakibara and associates (1966) [34]: type A (proximal type), proximal coronary segment dilated to the origin of fistula, distal end normal; and type B (distal type), coronary dilated over entire length, terminating as a fistula in the right side of the heart (end-artery type), proximal to fistula, regular branching of coronary.


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  		Table 1. Coronary Arteriovenous Fistulas

 
This classification has surgical implications. Type A is treated by epicardial ligation of the fistula, distal to the fistula origin, maintaining normal branch flow, and no cardiopulmonary bypass (CPB) is required. Type B requires ligation of the precapillary end by intracameral purse-string sutures at the site of termination, and requires CPB.

CAVF hierarchy level 3

CA, CAVF, NOS

CA, CAVF, Congenital
CA, CAVF, Acquired

CAVF hierarchy level 4

CA, CAVF, Congenital, NOS

CA, CAVF, Congenital, Single
CA, CAVF, Congenital, Multiple

CA, CAVF, Acquired, NOS

CA, CAVF, Acquired, Single
CA, CAVF, Acquired, Multiple

Each fistula will then be described using the following descriptive hierarchy:

CAVF description: hierarchy level 3

CA, CAVF Type A—Proximal
CA, CAVF Type B—Distal

CAVF description: hierarchy level 4

CA, CAVF, Type A—Proximal, NOS

CA, CAVF Type A—Proximal, Origin—Cx
CA, CAVF Type A—Proximal, Origin—Diagonal
CA, CAVF Type A—Proximal, Origin—LAD
CA, CAVF Type A—Proximal, Origin—Marginal
CA, CAVF Type A—Proximal, Origin—RCA
CA, CAVF Type A—Proximal, Origin—Single coronary

CA, CAVF Type B—Distal, NOS

CA, CAVF Type B—Distal, Origin—Cx
CA, CAVF Type B—Distal, Origin—Diagonal
CA, CAVF Type B—Distal, Origin—LAD
CA, CAVF Type B—Distal, Origin—Marginal
CA, CAVF Type B—Distal, Origin—RCA
CA, CAVF Type B—Distal, Origin—Single coronary

CAVF description: hierarchy level 5

CA, CAVF Type A—Proximal, NOS
CA, CAVF Type A—Proximal, Origin—Cx, NOS

CA, CAVF Type A—Proximal, Origin—Cx, Drainage—LA
CA, CAVF Type A—Proximal, Origin—Cx, Drainage—LV
CA, CAVF Type A—Proximal, Origin—Cx, Drainage—RA
CA, CAVF Type A—Proximal, Origin—Cx, Drainage—RV
CA, CAVF Type A—Proximal, Origin—Cx, Drainage—Pulmonary artery
CA, CAVF Type A—Proximal, Origin—Cx, Drainage—Superior vena cava
CA, CAVF Type A—Proximal, Origin—Cx, Drainage—Coronary sinus

CA, CAVF Type A—Proximal, Origin, Diagonal, NOS

CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—LA
CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—LV
CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—RA
CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—RV
CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—Pulmonary artery
CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—SVC
CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—Coronary sinus

CA, CAVF Type A—Proximal, Origin—LAD, NOS

CA, CAVF Type A—Proximal, Origin—LAD, Drainage—LA
CA, CAVF Type A—Proximal, Origin—LAD, Drainage—LV
CA, CAVF Type A—Proximal, Origin—LAD, Drainage—RA
CA, CAVF Type A—Proximal, Origin—LAD, Drainage—RV
CA, CAVF Type A—Proximal, Origin—LAD, Drainage—Pulmonary artery
CA, CAVF Type A—Proximal, Origin—LAD, Drainage—SVC
CA, CAVF Type A—Proximal, Origin—LAD, Drainage—Coronary sinus

CA, CAVF Type A—Proximatl, Origin—Marginal, NOS

CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—LA
CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—LV
CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—RA
CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—RV
CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—Pulmonary artery
CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—SVC
CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—Coronary sinus

CA, CAVF Type A—Proximal, Origin—RCA, NOS

CA, CAVF Type A—Proximal, Origin—RCA, Drainage—LA
CA, CAVF Type A—Proximal, Origin—RCA, Drainage—LV
CA, CAVF Type A—Proximal, Origin—RCA, Drainage—RA
CA, CAVF Type A—Proximal, Origin—RCA, Drainage—RV
CA, CAVF Type A—Proximal, Origin—RCA, Drainage—Pulmonary artery
CA, CAVF Type A—Proximal, Origin—RCA, Drainage—SVC
CA, CAVF Type A—Proximal, Origin—RCA, Drainage—Coronary sinus

CA, CAVF Type A—Proximal, Origin—Single coronary, NOS

CA, CAVF Type A—Proximal, Origin—Single coronary, Drainage—LA
CA, CAVF Type A—Proximal, Origin—Single coronary, Drainage—LV
CA, CAVF Type A—Proximal, Origin—Single coronary, Drainage—RA
CA, CAVF Type A—Proximal, Origin—Single coronary, Drainage—RV
CA, CAVF Type A—Proximal, Origin—Single coronary, Drainage—Pulmonary artery
CA, CAVF Type A—Proximal, Origin—Single coronary, Drainage—SVC
CA, CAVF Type A—Proximal, Origin—Single coronary, Drainage—Coronary sinus

CA, CAVF Type B—Distal, NOS
CA, CAVF Type B—Distal, Origin—Cx, NOS

CA, CAVF Type B—Distal, Origin—Cx, Drainage—LA
CA, CAVF Type B—Distal, Origin—Cx, Drainage—LV
CA, CAVF Type B—Distal, Origin—Cx, Drainage—RA
CA, CAVF Type B—Distal, Origin—Cx, Drainage—RV
CA, CAVF Type B—Distal, Origin—Cx, Drainage—Pulmonary artery
CA, CAVF Type B—Distal, Origin—Cx, Drainage—SVC
CA, CAVF Type B—Distal, Origin—Cx, Drainage—Coronary sinus

CA, CAVF Type B—Distal, Origin—Diagonal, NOS

CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—LA
CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—LV
CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—RA
CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—RV
CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—Pulmonary artery
CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—SVC
CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—Coronary sinus

CA, CAVF Type B—Distal, Origin—LAD, NOS

CA, CAVF Type B—Distal, Origin—LAD, Drainage—LA
CA, CAVF Type B—Distal, Origin—LAD, Drainage—LV
CA, CAVF Type B—Distal, Origin—LAD, Drainage—RA
CA, CAVF Type B—Distal, Origin—LAD, Drainage—RV
CA, CAVF Type B—Distal, Origin—LAD, Drainage—Pulmonary artery
CA, CAVF Type B—Distal, Origin—LAD, Drainage—SVC
CA, CAVF Type B—Distal, Origin—LAD, Drainage—Coronary sinus

CA, CAVF Type B—Distal, Origin—Marginal, NOS

CA, CAVF Type B—Distal, Origin—Marginal, Drainage—LA
CA, CAVF Type B—Distal, Origin—Marginal, Drainage—LV
CA, CAVF Type B—Distal, Origin—Marginal, Drainage—RA
CA, CAVF Type B—Distal, Origin—Marginal, Drainage—RV
CA, CAVF Type B—Distal, Origin—Marginal, Drainage—Pulmonary artery
CA, CAVF Type B—Distal, Origin—Marginal, Drainage—SVC
CA, CAVF Type B—Distal, Origin—Marginal, Drainage—Coronary sinus

CA, CAVF Type B—Distal, Origin—RCA, NOS

CA, CAVF Type B—Distal, Origin—RCA, Drainage—LA
CA, CAVF Type B—Distal, Origin—RCA, Drainage—LV
CA, CAVF Type B—Distal, Origin—RCA, Drainage—RA
CA, CAVF Type B—Distal, Origin—RCA, Drainage—RV
CA, CAVF Type B—Distal, Origin—RCA, Drainage—Pulmonary artery
CA, CAVF Type B—Distal, Origin—RCA, Drainage—SVC
CA, CAVF Type B—Distal, Origin—RCA, Drainage—Coronary sinus

CA, CAVF Type B—Distal, Origin—Single coronary, NOS

CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—LA
CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—LV
CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—RA
CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—RV
CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—Pulmonary artery
CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—SVC
CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—Coronary sinus

CAVF additional modifiers
As mentioned in the introductory paragraph on CAVF, the etiology of these lesions may be congenital or acquired. The acquired definition may itself be broken down into infectious, traumatic, and iatrogenic. Iatrogenic may be further subdivided as surgical, postcatheterization, postangioplasty, or postendomyocardial biopsy. In an effort to keep the number of hierarchy levels to a minimum, and hence reduce the arborization process that would otherwise be cumbersome, a modifying hierarchy may be added at hierarchy level 3 as to the etiology of the acquired CAVF lesions. This CAVF modifying hierarchy could be used in addition to the main CAVF hierarchy and the descriptive hierarchy both presented above.

CAVF additional modifiers: hierarchy level 3

CA, CAVF, Acquired, NOS

CA, CAVF, Acquired, Iatrogenic
CA, CAVF, Acquired, Infectious
CA, CAVF, Acquired, Traumatic

CAVF additional modifiers: hierarchy level 4

CA, CAVF, Acquired, NOS
CA, CAVF, Acquired, Iatrogenic, NOS

CA, CAVF, Acquired, Iatrogenic, Postangioplasty
CA, CAVF, Acquired, Iatrogenic, Postcatheterization
CA, CAVF, Acquired, Iatrogenic, Postendomyocardial biopsy
CA, CAVF, Acquired, Iatrogenic, Surgery

CA, CAVF, Acquired, Infectious
CA, CAVF, Acquired, Traumatic

E. Intramyocardial course of coronary arteries (bridging)
An intramural (intramyocardial) segment of epicardial coronaries, also known as bridging, was first described by Crainicianu in 1922 [35]. The general incidence at catheterization is 0.5% to 16% [36–41]. The incidence in the general population is estimated at 5.4% to 85.7% [37, 39, 41]. In 1951, Geiringer [42] reported a 23% incidence at autopsy and proposed a first classification: type 1, LAD deep in the interventricular groove, muscle bridge surrounding it (circumferential); and type 2, muscle bridge from trigonum fibrosum investing the LAD, as it passes towards the apex. This type is more common.

Ferreira and associates proposed an improvement over the Geiringer classification in 1991 by analyzing 50 hearts [43]. Fibrous thickening of the intima was described in both types: type 1 (n = 31), superficial bridge, crossing the artery transversely, towards the apex of the heart at an acute angle or perpendicularly; and type 2 (n = 10), the bridge crosses the LAD, surrounds the vessel by a bundle, arises from RV apical trabeculae, crosses the vessel, and terminates in the interventricular septum. The path of the LAD deviates from the interventricular groove towards the RV.

The first angiographic description was given in 1960 by Portsmann and Iwig, who coined the milking/compression effect of the vessel during systole [44]. In 1976, Noble proposed a new classification, according to angiographic percentage of systolic narrowing of the LAD, during a stress test as simulated by atrial pacing: [36] group 1: less than 50%; group 2: 50% to 75%; and group 3: greater than 75%. He concluded that group 3 patients, with severe narrowing of the LAD, are at risk of myocardial ischemia at effort, and recommended that patients avoid strenuous exercise. He proposed propranolol to attenuate heart rate and contractility, and was the first to suggest a theoretical indication for surgical relief. In 1982, Kramer [45] proposed a variation of Noble’s classification, without referring to him, based also on angiography: group I: less than 30% narrowing; group II: 31% to 50%; group III: 51% to 100%. A correlation was attempted with electrocardiogram (ECG) findings and viability studies (stress thallium). In group I, no ischemia on ECG or stress thallium was found; in group II, 25% had ECG abnormalities, but normal stress thallium, and in group III, 30% showed ischemia on ECG, and 33% had a positive stress thallium [45].

The nomenclature issues involve two types of classifications. The Geiringer and Ferreira classifications are based on autopsy studies, are purely morphological, and have no proven different clinical correlations between superficial and deep types. The Noble and Kramer classifications are based on angiographic studies and signs of ischemia on ECG and stress thallium. They have different cutoff values of systolic percentage of narrowing on angiography but arrive at the same end-points and clinical correlations. Noble’s was the initial study chronologically, and the stress testing with atrial pacing, measured lactate levels, and ECG changes are simultaneous to the angiographical data in a controlled study setting. Kramer’s classification attempts retrospectively to correlate symptoms, ECG changes, and thallium studies done at various institutions and at different times than their angiographic data, and therefore represents a less powerful and/or useful classification.

Hierarchy level 3

CA, Coronary bridging, NOS

CA, Coronary bridging, Symptomatic
CA, Coronary bridging, Asymptomatic

Hierarchy level 4

CA, Coronary bridging, Symptomatic, NOS

CA, Coronary bridging, Symptomatic, Cx
CA, Coronary bridging, Symptomatic, LAD
CA, Coronary bridging, Symptomatic, RCA
CA, Coronary bridging, Symptomatic, Mixed (multiple vessels)

CA, Coronary bridging, Asymptomatic, NOS

CA, Coronary bridging, Asymptomatic, Cx
CA, Coronary bridging, Asymptomatic, LAD
CA, Coronary bridging, Asymptomatic, RCA
CA, Coronary bridging, Asymptomatic, Mixed (multiple vessels)

GUEST EDITORS’ NOTE: If CA, Coronary bridging, Symptomatic, Mixed (multiple vessels) or CA, Coronary bridging, Asymptomatic, Mixed (multiple vessels) is chosen, one must then code each involved vessel to document which vessels are involved and the severity of their stenoses.

Groups I, II, and III in hierarchy level 5 are according to Noble’s classification [36].

Hierarchy level 5

CA, Coronary bridging, Symptomatic, NOS
CA, Coronary bridging, Symptomatic, Cx, NOS

CA, Coronary bridging, Symptomatic, Cx, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, Cx, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, Cx, Group III: > 75% systolic stenosis during stress test

CA, Coronary briding, Symptomatic, LAD, NOS

CA, Coronary bridging, Symptomatic, LAD, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, LAD, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, LAD, Group III: > 75% systolic stenosis during stress test

CA, Coronary briding, Symptomatic, RCA, NOS

CA, Coronary bridging, Symptomatic, RCA, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, RCA, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, RCA, Group III: > 75% systolic stenosis during stress test

CA, Coronary briging, Symptomatic, Mixed (multiple vessels)
CA, Coronary bridging, Asymptomatic, NOS
CA, Coronary bridging, Asymptomatic, Cx, NOS

CA, Coronary bridging, Asymptomatic, Cx, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, Cx, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, Cx, Group III: > 75% systolic stenosis during stress test

CA, Coronary bridging, Asymptomatic, LAD, NOS

CA, Coronary bridging, Asymptomatic, LAD, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, LAD, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, LAD, Group III: > 75% systolic stenosis during stress test

CA, Coronary bridging, Asymptomatic, RCA, NOS

CA, Coronary bridging, Asymptomatic, RCA, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, RCA, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, RCA, Group III: > 75% systolic stenosis during stress test

F. CA, coronary bridging, asymptomatic, mixed (multiple vessels)
Coronary aneurysms
First described by Morgagni in 1761 [46] in a patient with syphilis, followed by Bougon in 1812 [47], coronary aneurysms are also synonymous with ectasia or dilating atherosclerosis. The first angiographic/radiological description was documented by Munkner in 1958 [48]. The incidence in the general population is 0.3% to 4.9% [49–54]. Aneurysms are more common in males (88.2%).

Aneurysms are defined as dilations of a coronary vessel 1.5 times the adjacent normal coronaries. There exist two forms: saccular and fusiform, fusiform being the most common. Fusiform aneurysms are typically poststenotic dilations, and the majority arise in the setting of atherosclerosis (ATS) [52]. Saccular aneurysms are more prone to rupture, thrombosis or fistulization. In both instances, these may be single or multiple.

In 1976, Markis proposed an angiographic classification [55]: type I, diffuse ectasia of two to three vessels; type II, diffuse ectasia of one vessel and localized aneurysm in one other; type III, diffuse ectasia in one vessel; and type IV, localized aneurysm in one vessel.

The Coronary Artery Surgery Study (CASS) study proposes another classification [53] of aneurysms in patients with coronary artery disease (CAD): group A, aneurysm, no CAD; group B, aneurysm and CAD less than 70% stenosis; group C, aneurysm and CAD greater than 70% stenosis.

Markis’ angiographic classification (I, II, III, IV) deals with morphology and number of aneurysmal areas and vessels (ie, single-multiple aneurysms, in how many coronaries, and diffuse ectasia or localized). The CASS study classification (A, B, C) deals with the association with CAD and the degree of CAD; it attempts a more prognostic/surgical classification that does not vary from standard ischemic CADV criteria for bypass operation. Combining the two classifications would not appear necessary, as aneurysms do develop in the absence of CAD. Markis [55] morphological classification appears to be more useful in explaining the extent of disease.

Hierarchy level 3

CA, Coronary aneurysm (CAn), NOS

CA, Coronary aneurysm (CAn), Congenital
CA, Coronary aneurysm (CAn), Acquired

Hierarchy level 4

CA, CAn, Congenital, NOS

CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels
CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other
CA, CAn, Congenital, Type III—Diffuse ectasia in one vessel
CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel

CA, CAn, Acquired, NOS

CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels
CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other
CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel
CA, CAn, Acquired, Type IV—Localized aneurysm in one vessel

Hierarchy level 5

CA, CAn, Congenital, NOS
CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, NOS

CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, Cx and LAD
CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, Cx and RCA
CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, LAD and RCA
CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, Cx, LAD and RCA

CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, NOS

CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx and LAD
CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx and RCA
CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, LAD and RCA
CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx, LAD and RCA

CA, CAn, Congenital, Type III—Diffuse ectasia in one vessel, NOS

CA, CAn, Congenital, Type III—Diffuse ectasia in one vessel, Cx
CA CAn, Congenital, Type III—Diffuse ectasia in one vessel, LAD
CA, CAn, Congenital, Type III—Diffuse ectasia in one vessel, RCA

CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel, NOS

CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel, Cx
CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel, LAD
CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel, RCA

CA, CAn, Acquired, NOS
CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, NOS

CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, Cx and LAD
CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, Cx and RCA
CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, LAD and RCA
CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, Cx, LAD and RCA

CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, NOS

CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx and LAD
CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx and RCA
CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, LAD and RCA
CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx, LAD and RCA

CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel, NOS

CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel, Cx
CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel, LAD
CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel, RCA

CA, CAn, Acquired, Type IV—Localized aneurysm in one vessel, NOS

CA, CAn, Acquired, Type IV—Localized aneurysm in one vessel, Cx
CA, CAn, Acquired, Type IV—Localized aneurysm in one vessel, LAD
CA, CAn, Acquired, Type IV—Localized aneurysm in one vessel, RCA

Additional modifiers for coronary aneurysms
The etiologies of coronary aneurysms may be multiple, but are not included as a basis for nomenclature. Aneurysms may be divided at hierarchy level 3 into congenital and acquired. Based on the most common etiologies of coronary aneurysms, the acquired group may further be subdivided into atherosclerotic, Kawasaki’s disease, traumatic, iatrogenic, infectious, and systemic diseases (other than Kawasaki’s). Again, iatrogenic may be broken down into surgical, postangioplasty, postcatheterization, or postendomyocardial biopsy. Systemic diseases may include Takayasu’s disease, polyarteritis nodosa, syphilis, Ehlers-Danlos, Marfan’s disease, and scleroderma, to name a few. In an effort to keep the number of hierarchy levels to a minimum, and hence reduce the arborization process which would otherwise be cumbersome, a modifying annotation may be added at hierarchy level 3 as to the etiology of the coronary aneurysm lesion.

Aneurysms caused by Kawasaki’s disease have been classified differently. The following definitions [56] pertaining to Kawasaki’s disease are generally accepted in the literature: segmental stenosis = a braid-like lesion with multiple tortuosities of the vessel which represents recanalization of an occlusion; localized stenosis = a discrete, wedge-like narrowing at the inlet or outlet of an aneurysm; ectasia = 1.5 times larger than normal adjacent coronaries [57, 58]; dilation/small aneurysm = greater than or equal to 3 mm (± irregular lumen) [58]; aneurysms = 4 to 8 mm [58]; and giant aneurysms = greater than 8 mm [57–65].

Moderate coronary involvement with aneurysms is present in 12.8% to 25% of patients with untreated Kawasaki’s disease [57–65]. Kato and associates [66] proposed an angiographic classification based on anatomic and clinical findings at a follow-up of 4 years post acute disease: I (50%), regression of aneurysm, regular arterial wall, no symptoms, no ECG changes, no thallium study abnormality; II (24%), aneurysm without obstruction; III (17%), aneurysm and obstruction; and IV (9%), regression of aneurysm, irregular arterial wall without stenosis.

Coronary aneurysm additional modifiers: hierarchy level 3

CA, Coronary aneurysm, Acquired, NOS

CA, Coronary aneurysm, Acquired, Atherosclerotic
CA, Coronary aneurysm, Acquired, Kawasaki’s disease
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s)
CA, Coronary aneurysm, Acquired, Iatrogenic
CA, Coronary aneurysm, Acquired, Infectious
CA, Coronary aneurysm, Acquired, Traumatic

Coronary aneurysm additional modifiers: hierarchy level 4

CA, Coronary aneurysm, Acquired, NOS
CA, Coronary aneurysm, Acquired, Atherosclerotic
CA, Coronary aneurysm, Acquired, Kawasaki’s disease, NOS

CA, Coronary aneurysm, Acquired, Kawasaki’s disease, Aneurysm only
CA, Coronary aneurysm, Acquired, Kawasaki’s disease, Aneurysm and stenosis
CA, Coronary aneurysm, Acquired, Kawasaki’s disease, Stenosis only

CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), NOS

CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Takayasu’s disease
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Polyarteritis Nodosa
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Syphilis
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Ehlers-Danlos
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Marfan’s disease
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Scleroderma
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Other

CA, Coronary aneurysm, Acquired, Iatrogenic, NOS

CA, Coronary aneurysm, Acquired, Iatrogenic, Postangioplasty
CA, Coronary aneurysm, Acquired, Iatrogenic, Postcatheterization
CA, Coronary aneurysm, Acquired, Iatrogenic, Postendomyocardial biopsy
CA, Coronary aneurysm, Acquired, Iatrogenic, Surgery

CA, Coronary aneurysm, Acquired, Infectious
CA, Coronary aneurysm, Acquired, Traumatic

G. Coronary stenosis
Coronary stenosis hierarchy level 3

CA, Coronary stenosis, NOS

CA, Coronary stenosis, Congenital
CA, Coronary stenosis, Acquired

Coronary stenosis hierarchy level 4

CA, Coronary stenosis, NOS
CA, Coronary stenosis, Congenital, NOS

CA, Coronary stenosis, Congenital, Ostial
CA, Coronary stenosis, Congenital, Peripheral

CA, Coronary stenosis, Acquired, NOS

CA, Coronary stenosis, Acquired, Ostial
CA, Coronary stenosis, Acquired, Peripheral

Coronary stenosis hierarchy level 5

CA, Coronary stenosis, NOS
CA, Coronary stenosis, Congenital, NOS
CA, Coronary stenosis, Congenital, Ostial, NOS

CA, Coronary stenosis, Congenital, Ostial, Left
CA, Coronary stenosis, Congenital, Ostial, Right

CA, Coronary stenosis, Congenital, Peripheral, NOS

CA, Coronary stenosis, Congenital, Peripheral, Left main
CA, Coronary stenosis, Congenital, Peripheral, LAD
CA, Coronary stenosis, Congenital, Peripheral, Diagonal 1
CA, Coronary stenosis, Congenital, Peripheral, Diagonal 2
CA, Coronary stenosis, Congenital, Peripheral, Ramus intermedius
CA, Coronary stenosis, Congenital, Peripheral, Obtuse marginal 1
CA, Coronary stenosis, Congenital, Peripheral, Obtuse marginal 2
CA, Coronary stenosis, Congenital, Peripheral, Obtuse marginal 3
CA, Coronary stenosis, Congenital, Peripheral, Left Ventricular Branch
CA, Coronary stenosis, Congenital, Peripheral, Right main
CA, Coronary stenosis, Congenital, Peripheral, Acute marginal
CA, Coronary stenosis, Congenital, Peripheral, Posterior Descending Artery

CA, Coronary stenosis, Acquired, NOS
CA, Coronary stenosis, Acquired, Ostial, NOS

CA, Coronary stenosis, Acquired, Ostial, Left
CA, Coronary stenosis, Acquired, Ostial, Right

CA, Coronary stenosis, Acquired, Peripheral, NOS

CA, Coronary stenosis, Acquired, Peripheral, Left main
CA, Coronary stenosis, Acquired, Peripheral, LAD
CA, Coronary stenosis, Acquired, Peripheral, Diagonal 1
CA, Coronary stenosis, Acquired, Peripheral, Diagonal 2
CA, Coronary stenosis, Acquired, Peripheral, Ramus intermedius
CA, Coronary stenosis, Acquired, Peripheral, Obtuse marginal 1
CA, Coronary stenosis, Acquired, Peripheral, Obtuse marginal 2
CA, Coronary stenosis, Acquired, Peripheral, Obtuse marginal 3
CA, Coronary stenosis, Acquired, Peripheral, LVB
CA, Coronary stenosis, Acquired, Peripheral, Right main
CA, Coronary stenosis, Acquired, Peripheral, Acute marginal
CA, Coronary stenosis, Acquired, Peripheral, PDA

Coronary stenosis hierarchy level 6

CA, Coronary stenosis, Acquired, NOS
CA, Coronary stenosis, Acquired, Ostial, NOS
CA, Coronary stenosis, Acquired, Ostial, Left, NOS

CA, Coronary stenosis, Acquired, Ostial, Left, S/P arterial switch procedure
CA, Coronary stenosis, Acquired, Ostial, Left, S/P Ross procedure
CA, Coronary stenosis, Acquired, Ostial, Left, Other

CA, Coronary stenosis, Acquired, Ostial, Right, NOS

CA, Coronary stenosis, Acquired, Ostial, Right, S/P arterial switch procedure
CA, Coronary stenosis, Acquired, Ostial, Right, S/P Ross procedure
CA, Coronary stenosis, Acquired, Ostial, Right, Other

III. A unified coronary anomalies nomenclature system

Coronary anomalies hierarchy level 1

Coronary Anomalies (CA)

Coronary anomalies hierarchy level 2

Coronary Anomalies (CA), NOS

Coronary Anomalies (CA), Anomalous pulmonary origins of the coronaries (APOC)
Coronary Anomalies (CA), Anomalous aortic origins of the coronaries (AAOC)
Coronary Anomalies (CA), Congenital atresia of the left main (CALM)
Coronary Anomalies (CA), Coronary arteriovenous fistulas (CAVF)
Coronary Anomalies (CA), Coronary bridging
Coronary Anomalies (CA), Coronary aneurysms (CAn)
Coronary Anomalies (CA), Coronary stenosis

Coronary anomalies hierarchy level 3

CA, APOC, NOS

CA, APOC, Anomalous left coronary from the pulmonary artery (ALCAPA)
CA, APOC, Anomalous right coronary from the pulmonary artery (ARCAPA)
CA, APOC, Anomalous circumflex from the pulmonary artery (ACxPA)
CA, APOC, Anomalous left and right coronaries from the pulmonary artery (Both ALCAPA and ARCAPA)

CA, AAOC, NOS

CA, AAOC, Origin of left main from the right aortic sinus of Valsalva (LMCA from RASV)
CA, AAOC, Origin of right coronary artery from the left aortic sinus of Valsalva (RCA from LASV)
CA, AAOC, Origin of left anterior descending coronary artery from the right aortic sinus of Valsalva (LAD from RASV)
CA, AAOC, Origin of left anterior descending coronary artery from the right coronary artery (LAD from RCA)
CA, AAOC, Origin of circumflex coronary artery from the right aortic sinus of Valsalva (Cx from RASV)
CA, AAOC, Origin of circumflex coronary artery from the right coronary artery (Cx from RCA)
CA, AAOC, Single coronary artery
CA, AAOC, Inverted coronary arteries
CA, AAOC, Other anomalous aortic origin of coronary artery, specify

CA, Congenital atresia of the left main (CALM)
CA, CAVF, NOS

CA, CAVF, Congenital
CA, CAVF, Acquired

CA, Coronary bridging, NOS

CA, Coronary bridging, Symptomatic
CA, Coronary bridging, Asymptomatic

CA, Coronary aneurysm (CA), NOS

CA, Coronary aneurysm (CA), Congenital
CA, Coronary aneurysm (CA), Acquired

CA, Coronary stenosis, NOS

CA, Coronary stenosis, Congenital
CA, Coronary stenosis, Acquired

Coronary anomalies hierarchy level 4

CA, APOC, NOS
CA, APOC, ALCAPA, NOS

CA, APOC, ALCAPA, Origin sinus 1 (right-handed pulmonary sinus)
CA, APOC, ALCAPA, Origin sinus 2 (left-handed pulmonary sinus)
CA, APOC, ALCAPA, Origin nonfacing pulmonary sinus
CA, APOC, ALCAPA, Origin from commissure between sinus 1 and nonfacing sinus
CA, APOC, ALCAPA, Origin from commissure between sinus 2 and nonfacing sinus
CA, APOC, ALCAPA, Origin from commissure between sinus 1 and sinus 2
CA, APOC, ALCAPA, Origin from left pulmonary artery
CA, APOC, ALCAPA, Origin from right pulmonary artery

CA, APOC, ARCAPA, NOS

CA, APOC, ARCAPA, Origin sinus 1 (right-handed pulmonary sinus)
CA, APOC, ARCAPA, Origin sinus 2 (left-handed pulmonary sinus)
CA, APOC, ARCAPA, Origin nonfacing pulmonary sinus
CA, APOC, ARCAPA, Origin from commissure between sinus 1 and nonfacing sinus
CA, APOC, ARCAPA, Origin from commissure between sinus 2 and nonfacing sinus
CA, APOC, ARCAPA, Origin from commissure between sinus 1 and sinus 2
CA, APOC, ARCAPA, Origin from left pulmonary artery
CA, APOC, ARCAPA, Origin from right pulmonary artery

CA, APOC, ACxPA, NOS

CA, APOC, ACxPA, Origin sinus 1 (right-handed pulmonary sinus)
CA, APOC, ACxPA, Origin sinus 2 (left-handed pulmonary sinus)
CA, APOC, ACxPA, Origin nonfacing pulmonary sinus
CA, APOC, ACxPA, Origin from commissure between sinus 1 and nonfacing sinus
CA, APOC, ACxPA, Origin from commissure between sinus 2 and nonfacing sinus
CA, APOC, ACxPA, Origin from commissure between sinus 1 and sinus 2
CA, APOC, ACxPA, Origin from left pulmonary artery
CA, APOC, ACxPA, Origin from right pulmonary artery

CA, APOC, Both ALCAPA and ARCAPA
CA, AAOC, NOS
CA, AAOC, LMCA from RASV, NOS

CA, AAOC, LMCA from RASV, Origin anterior to pulmonary artery
CA, AAOC, LMCA from RASV, Origin posterior to aorta
CA, AAOC, LMCA from RASV, Origin between great vessels
CA, AAOC, LMCA from RASV, Septal course through conal septum

CA, AAOC, RCA from LASV, NOS

CA, AAOC, RCA from LASV, Origin anterior to aorta
CA, AAOC, RCA from LASV, Origin between great vessels

CA, AAOC, LAD from RASV
CA, AAOC, LAD from RCA
CA, AAOC, Cx from RASV, NOS

CA, AAOC, Cx from RASV, Origin anterior great vessels
CA, AAOC, Cx from RASV, Origin posterior great vessels

CA, AAOC, Cx from RCA
CA, AAOC, Single coronary artery, NOS

CA, AAOC, Single coronary artery, Left
CA, AAOC, Single coronary artery, Right

CA, AAOC, Inverted coronary arteries, NOS
CA, AAOC, Other anomalous aortic origin of coronary artery, specify
CA, Congenital atresia of the left main (CALM)
CA, CAVF, NOS
CA, CAVF, Congenital, NOS

CA, CAVF, Congenital, Single
CA, CAVF, Congenital, Multiple

CA, CAVF, Acquired, NOS

CA, CAVF, Acquired, Single
CA, CAVF, Acquired, Multiple

CA, Coronary bridging, NOS
CA, Coronary bridging, Symptomatic, NOS

CA, Coronary bridging, Symptomatic, Cx
CA, Coronary bridging, Symptomatic, LAD
CA, Coronary bridging, Symptomatic, RCA
CA, Coronary bridging, Symptomatic, Mixed (multiple vessels)

CA, Coronary bridging, Asymptomatic, NOS

CA, Coronary bridging, Asymptomatic, Cx
CA, Coronary bridging, Asymptomatic, LAD
CA, Coronary bridging, Asymptomatic, RCA
CA, Coronary bridging, Asymptomatic, Mixed (multiple vessels)

CA, Coronary aneurysm (CAn), NOS
CA, CAn, Congenital, NOS

CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels
CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other
CA, CAn, Congenital, Type III—Diffuse ectasia in one vessel
CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel

CA, CAn, Acquired, NOS

CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels
CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other
CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel
CA, CA, Acquired, Type IV—Localized aneurysm in one vessel

CA, Coronary stenosis, NOS
CA, Coronary stenosis, Congenital, NOS

CA, Coronary stenosis, Congenital, Ostial
CA, Coronary stenosis, Congenital, Peripheral

CA, Coronary stenosis, Acquired, NOS

CA, Coronary stenosis, Acquired, Ostial
CA, Coronary stenosis, Acquired, Peripheral

Coronary anomalies hierarchy level 5

CA, APOC, NOS
CA, APOC, ALCAPA, NOS
CA, APOC, ALCAPA, Origin sinus 1 (right-handed pulmonary sinus)
CA, APOC, ALCAPA, Origin sinus 2 (left-handed pulmonary sinus)
CA, APOC, ALCAPA, Origin nonfacing pulmonary sinus
CA, APOC, ALCAPA, Origin from commissure between sinus 1 and nonfacing sinus
CA, APOC, ALCAPA, Origin from commissure between sinus 2 and nonfacing sinus
CA, APOC, ALCAPA, Origin from commissure between sinus 1 and sinus 2
CA, APOC, ALCAPA, Origin from left pulmonary artery
CA, APOC, ALCAPA, Origin from right pulmonary artery
CA, APOC, ARCAPA, NOS
CA, APOC, ARCAPA, Origin sinus 1 (right-handed pulmonary sinus)
CA, APOC, ARCAPA, Origin sinus 2 (left-handed pulmonary sinus)
CA, APOC, ARCAPA, Origin nonfacing pulmonary sinus
CA, APOC, ARCAPA, Origin from commissure between sinus 1 and nonfacing sinus
CA, APOC, ARCAPA, Origin from commissure between sinus 2 and nonfacing sinus
CA, APOC, ARCAPA, Origin from commissure between sinus 1 and sinus 2
CA, APOC, ARCAPA, Origin from left pulmonary artery
CA, APOC, ARCAPA, Origin from right pulmonary artery
CA, APOC, ACxPA, NOS
CA, APOC, ACxPA, Origin sinus 1 (right-handed pulmonary sinus)
CA, APOC, ACxPA, Origin sinus 2 (left-handed pulmonary sinus)
CA, APOC, ACxPA, Origin nonfacing pulmonary sinus
CA, APOC, ACxPA, Origin from commissure between sinus 1 and nonfacing sinus
CA, APOC, ACxPA, Origin from commissure between sinus 2 and nonfacing sinus
CA, APOC, ACxPA, Origin from commissure between sinus 1 and sinus 2
CA, APOC, ACxPA, Origin from left pulmonary artery
CA, APOC, ACxPA, Origin from right pulmonary artery
CA, APOC, Both ALCAPA and ARCAPA
CA, AAOC, NOS
CA, AAOC, LMCA from RASV, NOS
CA, AAOC, LMCA from RASV, Origin anterior to pulmonary artery
CA, AAOC, LMCA from RASV, Origin posterior to aorta
CA, AAOC, LMCA from RASV, Origin between great vessels
CA, AAOC, LMCA from RASV, Septal course through conal septum
CA, AAOC, RCA from LASV, NOS
CA, AAOC, RCA from LASV, Origin anterior to aorta
CA, AAOC, RCA from LASV, Origin between great vessels
CA, AAOC, LAD from RASV
CA, AAOC, LAD from RCA
CA, AAOC, Cx from RASV, NOS
CA, AAOC, Cx from RASV, Origin anterior great vessels
CA, AAOC, Cx from RASV, Origin posterior great vessels
CA, AAOC, Cx from RCA
CA, AAOC, Single coronary artery, NOS
CA, AAOC, Single coronary artery, Left, NOS

CA, AAOC, Single coronary artery, Left, Type 1: one artery supplies entire heart
CA, AAOC, Single coronary artery, Left, Type 2: divides in two coronaries, right and left
CA, AAOC, Single coronary artery, Left, Type 3: other

CA, AAOC, Single coronary artery, Right, NOS

CA, AAOC, Single coronary artery, Right, Type 1: one artery supplies entire heart
CA, AAOC, Single coronary artery, Right, Type 2: divides in two coronaries, right and left
CA, AAOC, Single coronary artery, Right, Type 3: other

CA, AAOC, Inverted coronary arteries, NOS
CA, AAOC, Other anomalous aortic origin of coronary artery, specify
CA, Congenital atresia of the left main (CALM)
CA, CAVF, NOS
CA, CAVF, Congenital, NOS
CA, CAVF, Congenital, Single
CA, CAVF, Congenital, Multiple
CA, CAVF, Acquired, NOS
CA, CAVF, Acquired, Single
CA, CAVF, Acquired, Multiple
CA, Coronary bridging, NOS
CA, Coronary bridging, Symptomatic, NOS
CA, Coronary bridging, Symptomatic, Cx, NOS

CA, Coronary bridging, Symptomatic, Cx, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, Cx, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, Cx, Group III: > 75% systolic stenosis during stress test

CA, Coronary bridging, Symptomatic, LAD, NOS

CA, Coronary bridging, Symptomatic, LAD, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, LAD, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, LAD, Group III: > 75% systolic stenosis during stress test

CA, Coronary bridging, Symptomatic, RCA, NOS

CA, Coronary bridging, Symptomatic, RCA, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, RCA, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, RCA, Group III: > 75% systolic stenosis during stress test

CA, Coronary bridging, Symptomatic, Mixed (multiple vessels)
CA, Coronary bridging, Asymptomatic, NOS
CA, Coronary bridging, Asymptomatic, Cx, NOS

CA, Coronary bridging, Asymptomatic, Cx, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, Cx, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, Cx, Group III: > 75% systolic stenosis during stress test

CA, Coronary bridging, Asymptomatic, LAD, NOS

CA, Coronary bridging, Asymptomatic, LAD, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, LAD, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, LAD, Group III: > 75% systolic stenosis during stress test

CA, Coronary bridging, Asymptomatic, RCA, NOS

CA, Coronary bridging, Asymptomatic, RCA, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, RCA, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, RCA, Group III: > 75% systolic stenosis during stress test

CA, Coronary bridging, Asymptomatic, Mixed (multiple vessels)
CA, Coronary aneurysm (CAn), NOS
CA, CAn, Congenital, NOS
CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, NOS

CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, Cx and LAD
CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, Cx and RCA
CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, LAD and RCA
CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, Cx LAD and RCA

CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, NOS

CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx and LAD
CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx and RCA
CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, LAD and RCA
CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx LAD and RCA

CA, CAn, Congenital, Type III—Diffuse ectasia in one vessel, NOS

CA, CAn, Congenital, Type III—Diffuse ectasia in one vessel, Cx
CA, CAn, Congenital, Type III—Diffuse ectasia in one vessel, LAD
CA, CAn, Congenital, Type III—Diffuse ectasia in one vessel, RCA

CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel, NOS

CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel, Cx
CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel, LAD
CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel, RCA

CA, CAn, Acquired, NOS
CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, NOS

CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, Cx and LAD
CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, Cx and RCA
CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, LAD and RCA
CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, Cx LAD and RCA

CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, NOS

CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx and LAD
CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx and RCA
CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, LAD and RCA
CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx LAD and RCA

CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel, NOS

CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel, Cx
CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel, LAD
CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel, RCA

CA, CAn, Acquired, Type IV—Localized aneurysm in one vessel, NOS

CA, CAn, Acquired, Type IV—Localized aneurysm in one vessel, Cx
CA, CAn, Acquired, Type IV—Localized aneurysm in one vessel, LAD
CA, CAn, Acquired, Type IV—Localized aneurysm in one vessel, RCA

CA, Coronary stenosis, NOS
CA, Coronary stenosis, Congenital, NOS
CA, Coronary stenosis, Congenital, Ostial, NOS

CA, Coronary stenosis, Congenital, Ostial, Left
CA, Coronary stenosis, Congenital, Ostial, Right

CA, Coronary stenosis, Congenital, Peripheral, NOS

CA, Coronary stenosis, Congenital, Peripheral, Left main
CA, Coronary stenosis, Congenital, Peripheral, LAD
CA, Coronary stenosis, Congenital, Peripheral, Diagonal 1
CA, Coronary stenosis, Congenital, Peripheral, Diagonal 2
CA, Coronary stenosis, Congenital, Peripheral, Ramus intermedius
CA, Coronary stenosis, Congenital, Peripheral, Obtuse marginal 1
CA, Coronary stenosis, Congenital, Peripheral, Obtuse marginal 2
CA, Coronary stenosis, Congenital, Peripheral, Obtuse marginal 3
CA, Coronary stenosis, Congenital, Peripheral, LVB
CA, Coronary stenosis, Congenital, Peripheral, Right main
CA, Coronary stenosis, Congenital, Peripheral, Acute marginal
CA, Coronary stenosis, Congenital, Peripheral, PDA

CA, Coronary stenosis, Acquired, NOS
CA, Coronary stenosis, Acquired, Ostial, NOS

CA, Coronary stenosis, Acquired, Ostial, Left
CA, Coronary stenosis, Acquired, Ostial, Right

CA, Coronary stenosis, Acquired, Peripheral, NOS

CA, Coronary stenosis, Acquired, Peripheral, Left main
CA, Coronary stenosis, Acquired, Peripheral, LAD
CA, Coronary stenosis, Acquired, Peripheral, Diagonal 1
CA, Coronary stenosis, Acquired, Peripheral, Diagonal 2
CA, Coronary stenosis, Acquired, Peripheral, Ramus intermedius
CA, Coronary stenosis, Acquired, Peripheral, Obtuse marginal 1
CA, Coronary stenosis, Acquired, Peripheral, Obtuse marginal 2
CA, Coronary stenosis, Acquired, Peripheral, Obtuse marginal 3
CA, Coronary stenosis, Acquired, Peripheral, LVB
CA, Coronary stenosis, Acquired, Peripheral, Right main
CA, Coronary stenosis, Acquired, Peripheral, Acute marginal
CA, Coronary stenosis, Acquired, Peripheral, PDA

Coronary anomalies hierarchy level 6

CA, APOC, NOS
CA, APOC, ALCAPA, NOS
CA, APOC, ALCAPA, Origin sinus 1 (right-handed pulmonary sinus)
CA, APOC, ALCAPA, Origin sinus 2 (left-handed pulmonary sinus)
CA, APOC, ALCAPA, Origin nonfacing pulmonary sinus
CA, APOC, ALCAPA, Origin from commissure between sinus 1 and nonfacing sinus
CA, APOC, ALCAPA, Origin from commissure between sinus 2 and nonfacing sinus
CA, APOC, ALCAPA, Origin from commissure between sinus 1 and sinus 2
CA, APOC, ALCAPA, Origin from left pulmonary artery
CA, APOC, ALCAPA, Origin from right pulmonary artery
CA, APOC, ARCAPA, NOS
CA, APOC, ARCAPA, Origin sinus 1 (right-handed pulmonary sinus)
CA, APOC, ARCAPA, Origin sinus 2 (left-handed pulmonary sinus)
CA, APOC, ARCAPA, Origin nonfacing pulmonary sinus
CA, APOC, ARCAPA, Origin from commissure between sinus 1 and nonfacing sinus
CA, APOC, ARCAPA, Origin from commissure between sinus 2 and nonfacing sinus
CA, APOC, ARCAPA, Origin from commissure between sinus 1 and sinus 2
CA, APOC, ARCAPA, Origin from left pulmonary artery
CA, APOC, ARCAPA, Origin from right pulmonary artery
CA, APOC, ACxPA, NOS
CA, APOC, ACxPA, Origin sinus 1 (right-handed pulmonary sinus)
CA, APOC, ACxPA, Origin sinus 2 (left-handed pulmonary sinus)
CA, APOC, ACxPA, Origin nonfacing pulmonary sinus
CA, APOC, ACxPA, Origin from commissure between sinus 1 and nonfacing sinus
CA, APOC, ACxPA, Origin from commissure between sinus 2 and nonfacing sinus
CA, APOC, ACxPA, Origin from commissure between sinus 1 and sinus 2
CA, APOC, ACxPA, Origin from left pulmonary artery
CA, APOC, ACxPA, Origin from right pulmonary artery
CA, APOC, Both ALCAPA and ARCAPA
CA, AAOC, NOS
CA, AAOC, LMCA from RASV, NOS
CA, AAOC, LMCA from RASV, Origin anterior to pulmonary artery
CA, AAOC, LMCA from RASV, Origin posterior to aorta
CA, AAOC, LMCA from RASV, Origin between great vessels
CA, AAOC, LMCA from RASV, Septal course through conal septum
CA, AAOC, RCA from LASV, NOS
CA, AAOC, RCA from LASV, Origin anterior to aorta
CA, AAOC, RCA from LASV, Origin between great vessels
CA, AAOC, LAD from RASV
CA, AAOC, LAD from RCA
CA, AAOC, Cx from RASV, NOS
CA, AAOC, Cx from RASV, Origin anterior great vessels
CA, AAOC, Cx from RASV, Origin posterior great vessels
CA, AAOC, Cx from RCA
CA, AAOC, Single coronary artery, NOS
CA, AAOC, Single coronary artery, Left, NOS
CA, AAOC, Single coronary artery, Left, Type 1: one artery supplies entire heart
CA, AAOC, Single coronary artery, Left, Type 2: divides in two coronaries, right and left, NOS

CA, AAOC, Single coronary artery, Left, Type 2a: divides in two coronaries, Right branch anterior to great vessels
CA, AAOC, Single coronary artery, Left, Type 2b: divides in two coronaries, Right branch between great vessels
CA, AAOC, Single coronary artery, Left, Type 2c: divides in two coronaries, Right branch posterior to great vessels

CA, AAOC, Single coronary artery, Left, Type 3: other
CA, AAOC, Single coronary artery, Right, NOS
CA, AAOC, Single coronary artery, Right, Type 1: one artery supplies entire heart
CA, AAOC, Single coronary artery, Right, Type 2: divides in two coronaries, right and left, NOS

CA, AAOC, Single coronary artery, Right, Type 2a: divides in two coronaries, Left branch anterior to great vessels
CA, AAOC, Single coronary artery, Right, Type 2b: divides in two coronaries, Left branch between great vessels
CA, AAOC, Single coronary artery, Right, Type 2c: divides in two coronaries, Left branch posterior to great vessels

CA, AAOC, Single coronary artery, Right, Type 3: other
CA, AAOC, Inverted coronary arteries, NOS
CA, AAOC, Other anomalous aortic origin of coronary artery, specify
CA, Congenital atresia of the left main (CALM)
CA, CAVF, NOS
CA, CAVF, Congenital, NOS
CA, CAVF, Congenital, Single
CA, CAVF, Congenital, Multiple
CA, CAVF, Acquired, NOS
CA, CAVF, Acquired, Single
CA, CAVF, Acquired, Multiple
CA, Coronary bridging, NOS
CA, Coronary bridging, Symptomatic, NOS
CA, Coronary bridging, Symptomatic, Cx, NOS
CA, Coronary bridging, Symptomatic, Cx, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, Cx, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, Cx, Group III: > 75% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, LAD, NOS
CA, Coronary bridging, Symptomatic, LAD, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, LAD, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, LAD, Group III: > 75% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, RCA, NOS
CA, Coronary bridging, Symptomatic, RCA, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, RCA, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, RCA, Group III: > 75% systolic stenosis during stress test
CA, Coronary bridging, Symptomatic, Mixed (multiple vessels)
CA, Coronary bridging, Asymptomatic, NOS
CA, Coronary bridging, Asymptomatic, Cx, NOS
CA, Coronary bridging, Asymptomatic, Cx, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, Cx, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, Cx, Group III: > 75% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, LAD, NOS
CA, Coronary bridging, Asymptomatic, LAD, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, LAD, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, LAD, Group III: > 75% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, RCA, NOS
CA, Coronary bridging, Asymptomatic, RCA, Group I: < 50% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, RCA, Group II: 50% to 75% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, RCA, Group III: > 75% systolic stenosis during stress test
CA, Coronary bridging, Asymptomatic, Mixed (multiple vessels)
CA, Coronary aneurysm (CAn), NOS
CA, CAn, Congenital, NOS
CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, NOS
CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, Cx and LAD
CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, Cx and RCA
CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, LAD and RCA
CA, CAn, Congenital, Type I—Diffuse ectasia of two to three vessels, Cx, LAD and RCA
CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, NOS
CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx and LAD
CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx and RCA
CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, LAD and RCA
CA, CAn, Congenital, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx, LAD and RCA
CA, CAn, Congenital, Type III—Diffuse ectasia in one vessel, NOS
CA, CAn, Congenital, Type III—Diffuse ectasia in one vessel, Cx
CA, CAn, Congenital, Type III—Diffuse ectasia in one vessel, LAD
CA, CAn, Congenital, Type III—Diffuse ectasia in one vessel, RCA
CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel, NOS
CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel, Cx
CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel, LAD
CA, CAn, Congenital, Type IV—Localized aneurysm in one vessel, RCA
CA, CAn, Acquired, NOS
CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, NOS
CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, Cx and LAD
CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, Cx and RCA
CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, LAD and RCA
CA, CAn, Acquired, Type I—Diffuse ectasia of two to three vessels, Cx, LAD and RCA
CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, NOS
CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx and LAD
CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx and RCA
CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, LAD and RCA
CA, CAn, Acquired, Type II—Diffuse ectasia of one vessel and localized aneurysm in one other, Cx LAD and RCA
CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel, NOS
CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel, Cx
CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel, LAD
CA, CAn, Acquired, Type III—Diffuse ectasia in one vessel, RCA
CA, CAn, Acquired, Type IV—Localized aneurysm in one vessel, NOS
CA, CAn, Acquired, Type IV—Localized aneurysm in one vessel, Cx
CA, CAn, Acquired, Type IV—Localized aneurysm in one vessel, LAD
CA, CAn, Acquired, Type IV—Localized aneurysm in one vessel, RCA
CA, Coronary stenosis, NOS
CA, Coronary stenosis, Congenital, NOS
CA, Coronary stenosis, Congenital, Ostial, NOS
CA, Coronary stenosis, Congenital, Ostial, Left
CA, Coronary stenosis, Congenital, Ostial, Right
CA, Coronary stenosis, Congenital, Peripheral, NOS
CA, Coronary stenosis, Congenital, Peripheral, Left main
CA, Coronary stenosis, Congenital, Peripheral, LAD
CA, Coronary stenosis, Congenital, Peripheral, Diagonal 1
CA, Coronary stenosis, Congenital, Peripheral, Diagonal 2
CA, Coronary stenosis, Congenital, Peripheral, Ramus intermedius
CA, Coronary stenosis, Congenital, Peripheral, Obtuse marginal 1
CA, Coronary stenosis, Congenital, Peripheral, Obtuse marginal 2
CA, Coronary stenosis, Congenital, Peripheral, Obtuse marginal 3
CA, Coronary stenosis, Congenital, Peripheral, LVB
CA, Coronary stenosis, Congenital, Peripheral, Right main
CA, Coronary stenosis, Congenital, Peripheral, Acute marginal
CA, Coronary stenosis, Congenital, Peripheral, PDA
CA, Coronary stenosis, Acquired, NOS
CA, Coronary stenosis, Acquired, Ostial, NOS
CA, Coronary stenosis, Acquired, Ostial, Left, NOS

CA, Coronary stenosis, Acquired, Ostial, Left, S/P arterial switch procedure
CA, Coronary stenosis, Acquired, Ostial, Left, S/P Ross procedure
CA, Coronary stenosis, Acquired, Ostial, Left, Other

CA, Coronary stenosis, Acquired, Ostial, Right, NOS

CA, Coronary stenosis, Acquired, Ostial, Right, S/P arterial switch procedure
CA, Coronary stenosis, Acquired, Ostial, Right, S/P Ross procedure
CA, Coronary stenosis, Acquired, Ostial, Right, Other

CA, Coronary stenosis, Acquired, Peripheral, NOS
CA, Coronary stenosis, Acquired, Peripheral, Left main
CA, Coronary stenosis, Acquired, Peripheral, LAD
CA, Coronary stenosis, Acquired, Peripheral, Diagonal 1
CA, Coronary stenosis, Acquired, Peripheral, Diagonal 2
CA, Coronary stenosis, Acquired, Peripheral, Ramus intermedius
CA, Coronary stenosis, Acquired, Peripheral, Obtuse marginal 1
CA, Coronary stenosis, Acquired, Peripheral, Obtuse marginal 2
CA, Coronary stenosis, Acquired, Peripheral, Obtuse marginal 3
CA, Coronary stenosis, Acquired, Peripheral, LVB
CA, Coronary stenosis, Acquired, Peripheral, Right main
CA, Coronary stenosis, Acquired, Peripheral, Acute marginal
CA, Coronary stenosis, Acquired, Peripheral, PDA

Coronary anomalies: additional hierarchy

CAVF description: hierarchy level 3

CA, CAVF Type A—Proximal
CA, CAVF Type B—Distal

CAVF description: hierarchy level 4

CA, CAVF Type A—Proximal, NOS

CA, CAVF Type A—Proximal, Origin—Cx
CA, CAVF Type A—Proximal, Origin—Diagonal
CA, CAVF Type A—Proximal, Origin—LAD
CA, CAVF Type A—Proximal, Origin—Marginal
CA, CAVF Type A—Proximal, Origin—RCA
CA, CAVF Type A—Proximal, Origin—Single coronary

CA, CAVF Type B—Distal, NOS

CA, CAVF Type B—Distal, Origin—Cx
CA, CAVF Type B—Distal, Origin—Diagonal
CA, CAVF Type B—Distal, Origin—LAD
CA, CAVF Type B—Distal, Origin—Marginal
CA, CAVF Type B—Distal, Origin—RCA
CA, CAVF Type B—Distal, Origin—Single coronary

CAVF description: hierarchy level 5

CA, CAVF Type A—Proximal, NOS
CA, CAVF Type A—Proximal, Origin—Cx, NOS

CA, CAVF Type A—Proximal, Origin—Cx, Drainage—LA
CA, CAVF Type A—Proximal, Origin—Cx, Drainage—LV
CA, CAVF Type A—Proximal, Origin—Cx, Drainage—RA
CA, CAVF Type A—Proximal, Origin—Cx, Drainage—RV
CA, CAVF Type A—Proximal, Origin—Cx, Drainage—Pulmonary artery
CA, CAVF Type A—Proximal, Origin—Cx, Drainage—SVC
CA, CAVF Type A—Proximal, Origin—Cx, Drainage—Coronary sinus

CA, CAVF Type A—Proximal, Origin—Diagonal, NOS

CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—LA
CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—LV
CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—RA
CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—RV
CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—Pulmonary artery
CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—SVC
CA, CAVF Type A—Proximal, Origin—Diagonal, Drainage—Coronary sinus

CA, CAVF Type A—Proximal, Origin—LAD, NOS

CA, CAVF Type A—Proximal, Origin—LAD, Drainage—LA
CA, CAVF Type A—Proximal, Origin—LAD, Drainage—LV
CA, CAVF Type A—Proximal, Origin—LAD, Drainage—RA
CA, CAVF Type A—Proximal, Origin—LAD, Drainage—RV
CA, CAVF Type A—Proximal, Origin—LAD, Drainage—Pulmonary artery
CA, CAVF Type A—Proximal, Origin—LAD, Drainage—SVC
CA, CAVF Type A—Proximal, Origin—LAD, Drainage—Coronary sinus

CA, CAVF Type A—Proximal, Origin—Marginal, NOS

CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—LA
CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—LV
CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—RA
CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—RV
CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—Pulmonary artery
CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—SVC
CA, CAVF Type A—Proximal, Origin—Marginal, Drainage—Coronary sinus

CA, CAVF Type A—Proximal, Origin—RCA, NOS

CA, CAVF Type A—Proximal, Origin—RCA, Drainage—LA
CA, CAVF Type A—Proximal, Origin—RCA, Drainage—LV
CA, CAVF Type A—Proximal, Origin—RCA, Drainage—RA
CA, CAVF Type A—Proximal, Origin—RCA, Drainage—RV
CA, CAVF Type A—Proximal, Origin—RCA, Drainage—Pulmonary artery
CA, CAVF Type A—Proximal, Origin—RCA, Drainage—SVC
CA, CAVF Type A—Proximal, Origin—RCA, Drainage—Coronary sinus

CA, CAVF Type A—Proximal, Origin—Single coronary, NOS

CA, CAVF Type A—Proximal, Origin—Single coronary, Drainage—LA
CA, CAVF Type A—Proximal, Origin—Single coronary, Drainage—LV
A, CAVF Type A—Proximal, Origin—Single coronary, Drainage—RA
CA, CAVF Type A—Proximal, Origin—Single coronary, Drainage—RV
CA, CAVF Type A—Proximal, Origin—Single coronary, Drainage—Pulmonary artery
CA, CAVF Type A—Proximal, Origin—Single coronary, Drainage—SVC
CA, CAVF Type A—Proximal, Origin—Single coronary, Drainage—Coronary sinus

CA, CAVF Type B—Distal, NOS
CA, CAVF Type B—Distal, Origin—Cx, NOS

CA, CAVF Type B—Distal, Origin—Cx, Drainage—LA
CA, CAVF Type B—Distal, Origin—Cx, Drainage—LV
CA, CAVF Type B—Distal, Origin—Cx, Drainage—RA
CA, CAVF Type B—Distal, Origin—Cx, Drainage—RV
CA, CAVF Type B—Distal, Origin—Cx, Drainage—Pulmonary artery
CA, CAVF Type B—Distal, Origin—Cx, Drainage—SVC
CA, CAVF Type B—Distal, Origin—Cx, Drainage—Coronary sinus

CA, CAVF Type B—Distal, Origin—Diagnoal, NOS

CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—LA
CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—LV
CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—RA
CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—RV
CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—Pulmonary artery
CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—SVC
CA, CAVF Type B—Distal, Origin—Diagonal, Drainage—Coronary sinus

CA, CAVF Type B—Distal, Origin—LAD, NOS

CA, CAVF Type B—Distal, Origin—LAD, Drainage—LA
CA, CAVF Type B—Distal, Origin—LAD, Drainage—LV
CA, CAVF Type B—Distal, Origin—LAD, Drainage—RA
CA, CAVF Type B—Distal, Origin—LAD, Drainage—RV
CA, CAVF Type B—Distal, Origin—LAD, Drainage—Pulmonary artery
CA, CAVF Type B—Distal, Origin—LAD, Drainage—SVC
CA, CAVF Type B—Distal, Origin—LAD, Drainage—Coronary sinus

CA, CAVF Type B—Distal, Origin—Marginal, NOS

CA, CAVF Type B—Distal, Origin—Marginal, Drainage—LA
CA, CAVF Type B—Distal, Origin—Marginal, Drainage—LV
CA, CAVF Type B—Distal, Origin—Marginal, Drainage—RA
CA, CAVF Type B—Distal, Origin—Marginal, Drainage—RV
CA, CAVF Type B—Distal, Origin—Marginal, Drainage—Pulmonary artery
CA, CAVF Type B—Distal, Origin—Marginal, Drainage—SVC
CA, CAVF Type B—Distal, Origin—Marginal, Drainage—Coronary sinus

CA, CAVF Type B—Distal, Origin—RCA, NOS

CA, CAVF Type B—Distal, Origin—RCA, Drainage—LA
CA, CAVF Type B—Distal, Origin—RCA, Drainage—LV
CA, CAVF Type B—Distal, Origin—RCA, Drainage—RA
CA, CAVF Type B—Distal, Origin—RCA, Drainage—RV
CA, CAVF Type B—Distal, Origin—RCA, Drainage—Pulmonary artery
CA, CAVF Type B—Distal, Origin—RCA, Drainage—SVC
CA, CAVF Type B—Distal, Origin—RCA, Drainage—Coronary sinus

CA, CAVF Type B—Distal, Origin—Single Coronary, NOS

CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—LA
CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—LV
CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—RA
CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—RV
CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—Pulmonary artery
CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—SVC
CA, CAVF Type B—Distal, Origin—Single coronary, Drainage—Coronary sinus

Coronary anomalies: additional modifiers

Coronary anomalies: additional modifiers: hierarchy level 3

CA, CAVF, Acquired, NOS

CA, CAVF, Acquired, Iatrogenic
CA, CAVF, Acquired, Infectious
CA, CAVF, Acquired, Traumatic

CA, Coronary aneurysm, Acquired, NOS

CA, Coronary aneurysm, Acquired, Atherosclerotic
CA, Coronary aneurysm, Acquired, Kawasaki’s disease
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s)
CA, Coronary aneurysm, Acquired, Iatrogenic
CA, Coronary aneurysm, Acquired, Infectious
CA, Coronary aneurysm, Acquired, Traumatic

Coronary anomalies: additional modifiers: hierarchy level 4

CA, CAVF, Acquired, NOS
CA, CAVF, Acquired, Iatrogenic, NOS

CA, CAVF, Acquired, Iatrogenic, Postangioplasty
CA, CAVF, Acquired, Iatrogenic, Postcatheterization
CA, CAVF, Acquired, Iatrogenic, Postendomyocardial biopsy
CA, CAVF, Acquired, Iatrogenic, Surgery

CA, CAVF, Acquired, Infectious
CA, CAVF, Acquired, Traumatic
CA, Coronary aneurysm, Acquired, NOS
CA, Coronary aneurysm, Acquired, Atherosclerotic
CA, Coronary aneurysm, Acquired, Kawasaki’s disease, NOS

CA, Coronary aneurysm, Acquired, Kawasaki’s disease, Aneurysm only
CA, Coronary aneurysm, Acquired, Kawasaki’s disease, Aneurysm and stenosis
CA, Coronary aneurysm, Acquired, Kawasaki’s disease, Stenosis only

CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), NOS

CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Takayasu’s disease
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Polyarteritis Nodosa
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Syphilis
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Ehlers-Danlos
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Marfan’s disease
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Scleroderma
CA, Coronary aneurysm, Acquired, Systemic diseases (other than Kawasaki’s), Other

CA, Coronary aneurysm, Acquired, Iatrogenic

CA, Coronary aneurysm, Acquired, Iatrogenic, Postangioplasty
CA, Coronary aneurysm, Acquired, Iatrogenic, Postcatheterization
CA, Coronary aneurysm, Acquired, Iatrogenic, Postendomyocardial biopsy
CA, Coronary aneurysm, Acquired, Iatrogenic, Surgery

CA, Coronary aneurysm, Acquired, Infectious
CA, Coronary aneurysm, Acquired, Traumatic

IV. Treatment options for coronary artery anomalies

Coronary anomaly treatment hierarchy level 1

Coronary anomaly treatment

Coronary anomaly treatment hierarchy level 2

Coronary anomaly treatment, NOS

ALCAPA repair
ARCAPA repair
ACxPA repair
Anomalous aortic origin of the coronary repair
Congenital atresia of the left main repair
Coronary arteriovenous fistula repair
Coronary bridging repair
Coronary aneurysms repair
Coronary stenosis repair

Coronary anomaly treatment hierarchy level 3

Coronary anomaly treatment, NOS
ALCAPA repair, NOS

ALCAPA repair, Ligation
ALCAPA repair, Reimplantation (Translocation from PA to aorta)
ALCAPA repair, Takeuchi operation (Intrapulmonary tunnel)
ALCAPA repair, Coronary artery bypass graft (CABG)

ARCAPA repair, NOS

ARCAPA repair, Ligation
ARCAPA repair, Reimplantation (Translocation from PA to aorta)
ARCAPA repair, Takeuchi operation (Intrapulmonary tunnel)
ARCAPA repair, Bypass graft (CABG)

ACxPA repair, NOS

ACxPA repair, Ligation
ACxPA repair, Reimplantation (Translocation from PA to aorta)
ACxPA repair, Takeuchi operation (Intrapulmonary tunnel)
ACxPA repair, Bypass graft (CABG)

Anomalous aortic origin of the coronary repair, NOS

Anomalous aortic origin of the coronary repair, Bypass graft (CABG)
Anomalous aortic origin of the coronary repair, Reimplantation

Congenital atresia of the left main repair, NOS

Congenital atresia of the left main repair, Proximal arterioplasty
Congenital atresia of the left main repair, Bypass graft (CABG)

Coronary arteriovenous fistula repair, NOS

Coronary arteriovenous fistula repair, Ligation
Coronary arteriovenous fistula repair, Ligation and bypass graft (CABG)

Coronary bridging repair, NOS

Coronary bridging repair, Unroofing (myotomy)
Coronary bridging repair, Bypass graft (CABG)
Coronary bridging repair, Unroofing and bypass graft (CABG)

Coronary aneurysm repair, NOS

Coronary aneurysm repair, Bypass graft (CABG)
Coronary aneurysm repair, Aneurysmorraphy
Coronary aneurysm repair, Aneurysmorraphy and bypass graft (CABG)
Coronary aneurysm repair, Thromboendarterectomy
Coronary aneurysm repair, Other

Coronary stenosis repair, NOS

Coronary stenosis repair, Bypass graft (CABG)
Coronary stenosis repair, Endarterectomy
Coronary stenosis repair, Bypass graft (CABG) and endarterectomy

Coronary anomaly treatment hierarchy level 4

Coronary anomaly treatment, NOS
ALCAPA repair, NOS
ALCAPA repair, Ligation
ALCAPA repair, Reimplantation (Translocation from PA to aorta)
ALCAPA repair, Takeuchi operation (Intrapulmonary tunnel)
ALCAPA repair, Bypass graft (CABG), NOS

ALCAPA repair, Bypass graft (CABG), With CPB
ALCAPA repair, Bypass graft (CABG), Without CPB

ARCAPA repair, NOS
ARCAPA repair, Ligation
ARCAPA repair, Reimplantation (Translocation from PA to aorta)
ARCAPA repair, Takeuchi operation (Intrapulmonary tunnel)
ARCAPA repair, Bypass graft (CABG), NOS

ARCAPA repair, Bypass graft (CABG), With CPB
ARCAPA repair, Bypass graft (CABG), Without CPB

ACxPA repair, NOS
ACxPA repair, Ligation
ACxPA repair, Reimplantation (Translocation from PA to aorta)
ACxPA repair, Takeuchi operation (Intrapulmonary tunnel)
ACxPA repair, Bypass graft (CABG), NOS

ACxPA repair, Bypass graft (CABG), With CPB
ACxPA repair, Bypass graft (CABG), Without CPB

Anomalous aortic origin of the coronary repair, NOS
Anomalous aortic origin of the coronary repair, Bypass graft (CABG)
Anomalous aortic origin of the coronary repair, Reimplantation
Congenital atresia of the left main repair, NOS
Congenital atresia of the left main repair, Proximal arterioplasty, NOS

Congenital atresia of the left main repair, Proximal arterioplasty, Autologous tissue
Congenital atresia of the left main repair, Proximal arterioplasty, Homograft tissue
Congenital atresia of the left main repair, Proximal arterioplasty, Other bioprosthetic material
Congenital atresia of the left main repair, Bypass graft (CABG), Nonbioprosthetic prosthetic material

Coronary arteriovenous fistula repair, NOS
Coronary arteriovenous fistula repair, Ligation, NOS

Coronary arteriovenous fistula repair, Ligation, With CPB
Coronary arteriovenous fistula repair, Ligation, Without CPB

Coronary arteriovenous fistula repair, Ligation and bypass graft (CABG), NOS

Coronary arteriovenous fistula repair, Ligation and bypass graft (CABG), With CPB
Coronary arteriovenous fistula repair, Ligation and bypass graft (CABG), Without CPB

Coronary bridging repair, NOS
Coronary bridging repair, Unroofing (myotomy)
Coronary bridging repair, Bypass graft (CABG)
Coronary bridging repair, Unroofing and bypass graft (CABG)
Coronary aneurysm repair, NOS
Coronary aneurysm repair, Bypass graft (CABG)
Coronary aneurysm repair, Aneurysmorraphy
Coronary aneurysm repair, Aneurysmorraphy and bypass graft (CABG)
Coronary aneurysm repair, Thromboendarterectomy
Coronary aneurysm repair, Other
Coronary stenosis repair, NOS
Coronary stenosis repair, Bypass graft (CABG)
Coronary stenosis repair, Endarterectomy
Coronary stenosis repair, Bypass graft (CABG) and endarterectomy

Coronary anomaly treatment hierarchy modifier
All patients receiving CABG will also need the following conduit data coded:

Coronary anomaly treatment, Bypass graft, NOS
Coronary anomaly treatment, Bypass graft, Left Internal Thoracic Artery (LITA)
Coronary anomaly treatment, Bypass graft, Right Internal Thoracic Artery (RITA)
Coronary anomaly treatment, Bypass graft, Subclavian artery, NOS
Coronary anomaly treatment, Bypass graft, Subclavian artery, Left
Coronary anomaly treatment, Bypass graft, Subclavian artery, Right
Coronary anomaly treatment, Bypass graft, Radial artery, NOS
Coronary anomaly treatment, Bypass graft, Radial artery, Left
Coronary anomaly treatment, Bypass graft, Radial artery, Right
Coronary anomaly treatment, Bypass graft, Saphenous vein
Coronary anomaly treatment, Bypass graft, Prosthesis

V. Diagnosis and procedure short lists

Diagnosis Short List
Anomalous origin of coronary artery from pulmonary artery
Coronary artery fistula
Coronary artery anomaly

Procedure Short List
Anomalous origin of coronary artery from pulmonary artery repair
Coronary artery bypass
Coronary artery fistula ligation
Coronary artery procedure, other

References

  1. Ogden J.A. Congenital anomalies of the coronary arteries. Am J Cardiology 1969;70:474-479.
  2. Brooks S.J. Two cases of abnormal coronary artery of the heart arising from the pulmonary artery. J Anat Physiol 1886;20:26.
  3. Bland E.F., White P.D., Garland J. Congenital anomalies of the coronary arteries. Am Heart J 1933;8:787-801.
  4. Smith A., Arnold R., Anderson R.H., Wilkinson J.L., Qureshi S.A., McKay R. Anomalous origin of the left coronary artery from the pulmonary trunk. J Thorac Cardiovasc Surg 1989;98:16-24.[Abstract]
  5. Cohen A.J., Grishkin B.A., Helsel R.A., Head H.D. Surgical therapy in the management of coronary anomalies. Ann Thorac Surg 1989;47:630-637.[Abstract]
  6. Taylor A.J., Rogan K.M., Virmani R. Sudden cardiac death associated with isolated congenital coronary artery anomalies. J Am Coll Cardiol 1992;20:640-647.[Abstract]
  7. Barth C.W., III, Roberts W.C. Left main coronary artery originating from the right sinus of Valsalva and coursing between the aorta and pulmonary trunk. J Am Coll Cardiol 1986;7:366-373.[Abstract]
  8. Mustafa I., Gula G., Radley-Smith R., Durrer S., Yacoub M. Anomalous origin of the left coronary artery from the anterior aortic sinus. J Thorac Cardiovasc Surg 1981;82:297-300.[Medline]
  9. Moodie D.S., Gill C., Loop F.D., Sheldon W.C. Anomalous left main coronary artery originating from the right sinus of Valsalva. J Thorac Cardiovasc Surg 1980;80:198-205.[Medline]
  10. Kimbiris D. Anomalous origin of the left main coronary artery from the right sinus of Valsalva. Am J Cardiol 1985;55:765-769.[Medline]
  11. Liberthson R.R., Dinsmore R.E., Fallon J.T. Aberrant coronary artery origin from the aorta; report of 18 patients, review of literature and delineation of natural history, and management. Circulation 1979;59:748-754.[Abstract/Free Full Text]
  12. Benge W., Martins J.B., Funk D.C. Morbidity associated with anomalous origin of the right coronary artery from the left sinus of Valsalva. Am Heart J 1980;99:96-100.[Medline]
  13. Bett J.H.N., O’Brien M.F., Murray P.J.S. Surgery for anomalous origin of the right coronary artery. Br Heart J 1985;53:459-461.[Abstract/Free Full Text]
  14. Roberts W.C., Siegel R.J., Zipes D.P. Origin of the right coronary artery from the left sinus of Valsalva and it’s functional consequences. Am J Cardiol 1982;49:863-868.[Medline]
  15. Bloomfield P., Ehrtich C., Folland E.D., Bianco J.A., Tow D.E., Parisi A.F. Anomalous right coronary artery. Am J Cardiol 1983;51:1235-1237.[Medline]
  16. Jokl C., McClellan J.T., Ross G.D. Congenital anomaly of left coronary artery in young athletes. JAMA 1962;182:572-573.[Abstract/Free Full Text]
  17. Sharbaugh A.H., White R.S. Single coronary artery. JAMA 1974;230:243-246.[Abstract/Free Full Text]
  18. Smith J. Review of single coronary artery with report of two cases. Circulation 1950;1:1168-1174.[Medline]
  19. Lurie P.R. Abnormalities and diseases of the coronary vessels. In: Moss A.J., Adams F.H., Emmanoulides G.C., eds. Heart disease in infants, children, and adolescents, 2nd ed. Baltimore: Williams and Wilkins, 1977:487-489.
  20. Ghosh P.K., Friedman M., Vidne B.A. Isolated congenital atresia of the left main coronary artery and atherosclerosis. Ann Thorac Surg 1993;55:1564-1565.[Abstract]
  21. Musiani A., Cernigliaro C., Sansa M., Maselli D., DeGasperis, C Left main coronary artery atresia. Eur J Cardiothorac Surg 1997;11:505-514.[Abstract]
  22. Koh E., Nakagania M., Hamaoka K., Sawada T., Oga K. Congenital atresia of the left coronary ostium. Pediatr Cardiol 1989;10:159-162.[Medline]
  23. Rosenkranz E.R., Murphy D.J., Cosgrove D.M., III Surgical management of left coronary artery ostial atresia and supravalvar aortic stenosis. Ann Thorac Surg 1995;54:779-787.[Abstract]
  24. Byrum C.J., Blackman M.S., Schneider B., Sondheimer H.M., Kavey R.E.W. Congenital atresia of the left coronary ostium and hypoplasia of the left main coronary artery. Am Heart J 1980;99:354-358.[Medline]
  25. Krause W. Ueber den Ursprung einer Accessorischen A. Coronaria Cordis aus der A. Pulmonalis. Z Rat Med 1865;24:225-227.
  26. Fernandes E.D., Kadivar H., Hallman G.L., Reul G.J., Ott D.A., Cooley D.A. Congenital malformations of the coronary arteries. Ann Thorac Surg 1992;54:732-740.[Abstract]
  27. Olearchyk A.S., Runk D.M., Alavi M., Grosso M.A. Congenital bilateral coronary-to-pulmonary artery fistulas. Ann Thorac Surg 1997;64:233-235.[Abstract/Free Full Text]
  28. Urrutia-S C.O., Falaschi G., Ott D.A., Cooley D.A. Surgical management of 56 patients with congenital coronary artery fistulas. Ann Thorac Surg 1983;35:300-307.[Abstract]
  29. Harris W.O., Andrews J.C., Nichols D.A., Holmes D.R. Percutaneous transcatheter embolization of coronary arteriovenous fistulas. Mayo Clin Proc 1996;71:37-42.[Abstract]
  30. Lowe J.E., Oldham H.N., Sabiston D.C. Surgical management of congenital coronary artery fistulas. Ann Surg 1981;194:373-380.[Medline]
  31. Schumacher G., Roithmaier A., Lonrenz H.P., et al. Congenital coronary artery fistula in infancy and childhood. Thorac Cardiovasc Surgeon 1997;45:287-294.[Medline]
  32. Mavroudis C., Backer C.L., Rocchini A.P., Muster A.J., Gevitz M. Coronary artery fistulas in infants and children. Ann Thorac Surg 1997;63:1235-1242.[Abstract/Free Full Text]
  33. Tkebuchava T., von Segesser L.K., Vogt P.R., Jenni R., Arbenz U., Turina M. Congenital coronary fistulas in children and adults. J Cardiovasc Surg 1996;37:29-34.[Medline]
  34. Sakakibara S., Yokoyama M., Takao A., Nogi M., Gomi H. Coronary arteriovenous fistula. Am Heart J 1966;72:307-314.[Medline]
  35. Crainicianu A. Antomisce Studien über die Coronararterien und Experimentelle Untersuchungen über ihre Durchgangigkeit. Virchows Arch (Pathol Anat) 1922;238:1-8.
  36. Noble J., Bourassa M.G., Petitclerc R., Dyrda I. Myocardial bridging and milking effect of the left anterior descending coronary artery. Am J Cardiol 1976;37:993-999.[Medline]
  37. Angelini P., Trivellato M., Doris J., Leachman R.D. Myocardial bridges. Prog Cardiovasc Dis 1983;26:75-88.[Medline]
  38. Pey J., Martin de Dios R., Epeldegui A. Myocardial bridging and hypertrophic cardiomyopathy. Int J Cardiol 1985;8:327-330.[Medline]
  39. Den Dulk K., Brugada P., Braat S., Heddle B., Wellens H.J.J. Myocardial bridging as a cause of paroxysmal atrioventricular block. J Am Coll Cardiol 1983;1:965-969.[Abstract]
  40. Vasan R.S., Bahl V.K., Rajani M. Myocardial infarction associated with myocardial bridge. Int J Cardiol 1989;25:240-241.[Medline]
  41. Schwartz E.R., Klues H.G., Vom Dahl J., Klein I., Krebs W., Hanrath P. Functional, angiographic and intracoronary Doppler flow characteristics in symptomatic patients with myocardial bridging. J Am Coll Cardiol 1996;27:1637-1645.[Abstract]
  42. Geiringer E. The mural coronary. Am Heart J 1951;41:359-368.[Medline]
  43. Ferreira A.G., Jr, Trotter S.E., Konig B., Jr, Decourt L.V., Fox K., Olsen E.G.F. Myocardial bridges. Br Heart J 1991;66:364-367.[Abstract/Free Full Text]
  44. Portsmann W., Iwig J. Die Intramurale Koronarie im Angiogramm. Fortschr Rontgenstr 1960;92:129-132.
  45. Kramer J.R., Kitazume H., Proudfit W.L. Clinical significance of isolated coronary bridges; benign and frequent condition involving the left anterior descending artery. Am Heart J 1982;103:283-288.[Medline]
  46. Morgagni JB. DeSedibus, et causis morbotum per anatomen indegatis. Tomus primus, Liber 11 Epist 27, Article 28, Venetiis 1761.
  47. Bougon M. Bibliot Med 1812;37:183.
  48. Munkner T., Petersen O., Vesterdal J. Congenital aneurysm of the coronary artery with an arteriovenous fistula. Acta Radiol 1958;50:333-340.
  49. Befeler B., Aranda J.M., Emdi A., Mullin F.L., El-Sherif N., Lazzara R. Coronary artery aneurysm. Am J Med 1977;62:597-607.[Medline]
  50. Falsetti H.L., Carroll R.J. Coronary artery aneurysm. Chest 1976;69:630-636.[Abstract/Free Full Text]
  51. Analstawi I.N., deLeon J.A. Arteriosclerotic aneurysms of the coronary arteries. J Thorac Cardiovasc Surg 1974;68:226-228.[Medline]
  52. Aintablian A., Hamby R.I., Hoffman I., Kramer R.J. Coronary ectasia. Am Heart J 1978;96:309-315.[Medline]
  53. Swaye P.S., Fisher L.D., Litwin P., et al. Aneurysmal coronary artery disease. Circulation 1983;67:134-138.[Abstract/Free Full Text]
  54. Hartnell G.G., Parnell B.M., Pridie R.B. Coronary artery ectasia; its prevalence and clinical significance in 4993 patients. Br Heart J 1985;54:392-395.[Abstract/Free Full Text]
  55. Markis J.E., Joffe D.C., Cohn P.F., Feen D.J., Herman M.V., Gorlin R. Clinical significance of coronary ectasia. Am J Cardiol 1976;37:217-222.[Medline]
  56. Suzuki A., Kamiya T., Ono Y., Okuno M., Yagihara T. Aortocoronary bypass surgery for coronary arterial lesions resulting from Kawasaki disease. J Pediatr 1990;116:567-573.[Medline]
  57. Dajani A.S., Taubert K.A., Gerber M.A., et al. Diagnosis and therapy of Kawasaki disease in children. Circulation 1993;87:1776-1780.[Free Full Text]
  58. Akagi T., Rose V., Benson L.N., Newman A., Freedom R.M. Outcome of coronary artery aneurysms after Kawasaki disease. J Pediatr 1992;121:689-694.[Medline]
  59. Beiser A.S., Takahashi M., Baker A.L., Sundel R.P., Newburger J.W. A predictive instrument for coronary artery aneurysms in Kawasaki Disease. Am J Cardiol 1998;81:116-120.
  60. Nakamura Y., Yanagawa H., Ojima T., Kawasaki T., Kato H. Cardiac sequelae of Kawasaki disease among recurrent cases. Arch Dis Child 1998;78:163-165.[Abstract/Free Full Text]
  61. Gersony W.M. Predicting coronary aneurysms in Kawasaki disease. Am J Cardiol 1998;81:1162-1164.[Medline]
  62. Pahl E. Kawasaki disease. Pediatr Ann 1997;26:112-115.[Medline]
  63. Burns J.C., Shike H., Gordon J.B., Malhotra A., Schoenwetter M., Kawasaki T. Sequelae of Kawasaki disease in adolescents and young adults. J Am Coll Cardiol 1996;28:253-257.[Abstract]
  64. Suzuki A., Kamiya T., Kuwahara N., et al. Coronary arterial lesions of Kawasaki disease. Pediatr Cardiol 1986;7:3-9.[Medline]
  65. Kato H., Ichinosa E., Yoshioka F., et al. Fate of coronary aneurysms in Kawasaki disease. Am J Cardiol 1982;42:1758-1766.
  66. Kato H., Kolke S., Yamamoto M., Ito Y., Yano E. Coronary aneurysms in infants and young children with acute febrile MCLNS. J Pediatr 1975;86:892-898.[Medline]



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ANN THORAC SURG ASIAN CARDIOVASC THORAC ANN EUR J CARDIOTHORAC SURG
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