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Two-dimensional and Doppler transesophageal echocardiographic delineation and flow characterization of anomalous coronary arteries in adults
Two-dimensional and Doppler Transesophageal Echocardiographic Delineation and Flow Characterization of Anomalous Coronary Arteries in Adults Buddhadeb Dawn, MD, J. David Talley, MD, Charles R. Prince, MD, Azizul Hoque, MD, Glenn T. Morris, DO, Nicholaos P. Xenopoulos, MD, and Marcus F. Stoddard, MD, Louisville, Kentucky
Objectives: The purpose of this study was to examine the use of transesophageal echocardiography (TEE) in the identification and flow characterization of congenital coronary anomalies. Background: Congenital coronary anomalies in adults are rare but may cause serious cardiac complications. The use of TEE in evaluation of this entity has not been well defined. Very little is known regarding flow patterns in anomalous coronaries assessed by Doppler TEE. Methods: A total of 32 consecutive adult patients were studied using TEE to define the origin, course, and proximal flow pattern of suspected coronary anomalies. Results: Coronary anomalies identified using TEE included anomalous origin from the pulmonary trunk (n ⴝ 2), right sinus (n ⴝ 18), left sinus (n ⴝ 9), single coronary (n ⴝ 2), and left main coronary fistula (n ⴝ 1). Multiplane TEE performed in 20 cases simplified the delineation of more complex coronary anomalies. The origin was identified in all patients, proximal course delineated in 31, and
Congenital coronary artery anomalies may be
benign or potentially serious leading to myocardial ischemia, infarction, and sudden cardiac death.1-4 Although the overall incidence of congenital coronary artery anomalies is as high as 0.5% to 1.3%,5,6 certain specific types are exceptionally rare yet potentially fatal.1,7 A premortem diagnosis of this clinically significant entity is difficult. The specific origin and the proximal course of an anomalous coronary are major factors From the Division of Cardiology, Department of Medicine, University of Louisville, and the Jewish Hospital Heart and Lung Institute, Louisville, Kentucky. Reprint requests: Marcus F. Stoddard, MD, 530 S Jackson St, ACB, 3rd Floor, Division of Cardiology, University of Louisville, Louisville, KY 40292 (E-mail: [email protected]). Copyright 2003 by the American Society of Echocardiography. 0894-7317/2003/$30.00 ⫹ 0 doi:10.1067/S0894-7317(03)00554-6
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proximal flow pattern characterized by pulsed Doppler in 23 of 32 patients. In 16 anomalous left main, left anterior descending, or left circumflex coronary arteries, an abnormal systolic flow pattern (ie, systolic/diastolic time-velocity integral ratio >1) occurred exclusively (P < .001) when the anomalous artery had an intermediate (100%; 5/5) versus anterior or posterior course (0%; 0/11) relative to the aortic and pulmonary artery trunks. A systolic flow pattern was also evident in 4 (80%) of 5 patients with an anomalous right coronary artery with an intermediate course. Conclusions: TEE, particularly with a multiplane probe, has an important complementary role to coronary angiography in delineating the proximal course and pattern of flow in anomalous coronaries. Predominant systolic flow pattern in anatomically left proximal anomalous coronaries signifies an intermediate course between the aorta and the pulmonary trunk and may be clinically useful for risk stratification. (J Am Soc Echocardiogr 2003;16: 1274-86.)
in predicting a clinically significant or benign outcome.1-4,7-10 Although cardiac catheterization remains the gold standard for evaluation of coronary anatomy, recent smaller studies suggest a complementary role of transesophageal echocardiography (TEE) for the same.11-13 The ostia and the proximal portions of normal coronary arteries can be visualized with TEE.14 Delineation of coronary anomalies by TEE, primarily using monoplane TEE probes, has been reported.11-13,15-19 Multiplane TEE promises to further simplify the delineation of the variable proximal course of coronary anomalies20 and 3-dimensional TEE shows promise.21 Importantly, very little is known regarding the significance of flow pattern in the proximal segment of anomalous coronaries. The objectives of this study were to highlight the technical details involved in the TEE detection of coronary anomalies, and to characterize their
Journal of the American Society of Echocardiography Volume 16 Number 9
flow patterns. We report the largest series, to date, of coronary artery anomalies diagnosed or confirmed by TEE. To our knowledge, TEE evaluation of several of these rare coronary anomalies has never been reported.
METHODS Patients In all, 32 consecutive patients (22 men, 10 women) with a mean age of 49 ⫾ 12 (SD) years were included in the study. Presence of a coronary anomaly detected during a clinically indicated cardiac catheterization or TEE was the primary inclusion criterion. Specifically, 21 (65%) patients underwent TEE after an angiographic diagnosis of the anomaly, 5 (16%) underwent angiography after a TEE diagnosis of the anomaly, and 6 (19%) did not undergo angiography. The TEE operator and the angiographer were aware of these anomalies diagnosed by the alternative modalities before the procedure. An informed consent was obtained in all patients. The study protocol was approved by the human studies committee at our institution. TEE A 5.0-MHz monoplane (n ⫽ 7), biplane (n ⫽ 5), or multiplane (n ⫽ 20) TEE probe and echocardiographic machine (77020A, Sonos 1500 or Sonos 2500, HewlettPackard, Andover, Mass; HDI 5000, ATL, Bothell, Wash) were used. A comprehensive sequence of TEE views was obtained to define the origin and course of the coronary anomalies. Two-dimensional views were guided using color flow Doppler echocardiography. The origin and course of the anomalous coronary artery were identified. Normal coronaries were identified by previously described methods.22 Pulsed Doppler echocardiography was performed at relevant sites of the anomalous coronaries. Peak systolic and diastolic velocity of the coronary flow and the time-velocity integral were obtained. A systolic to diastolic time-velocity integral ratio ⬎ 1.0 was defined as a predominant systolic flow pattern. Takeoff of an anomalous artery was considered acute when the angle between the aortic wall and the proximal segment of the artery was ⬍45 degrees.8 TEE was performed and interpreted by the same experienced echocardiographer (M.F.S). Of 32 patients, 26 also underwent coronary angiography by conventional techniques.23 Statistical Analysis Fisher exact test was used to determine any significant difference in coronary flow pattern observed in different patient subgroups. A 2-tailed P value of ⬍.05 was considered statistically significant.
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RESULTS The specific coronary artery anomalies (32 cases) and the flow pattern in the proximal segment (23 cases) as demonstrated by TEE and coronary angiography are summarized in Table. Cases were divided into 5 groups on the basis of a modified classification2 as follows: (1) anomalous origin from the pulmonary trunk (n ⫽ 2); (2) anomalous origin from the right sinus or right coronary artery (RCA) (n ⫽ 18); (3) anomalous origin from the left sinus or left coronary system (n ⫽ 9); (4) single coronary artery (n ⫽ 2); and (5) coronary fistula (n ⫽ 1). Anomalous Origin from the Pulmonary Trunk Origin of a coronary artery from the pulmonary trunk was seen in 2 cases (cases 1 and 2). In case 1, the origin of the left main (LM) coronary artery could be easily identified by alternate clockwise and counterclockwise rotation of the multiplane probe while scanning the pulmonary trunk in a long-axis plane with color flow Doppler echocardiography (Figure 1, A). The LM had a posterior origin from the pulmonary trunk and branched into left anterior descending (LAD) and left circumflex (LCx), both of which had a normal proximal course. A normal origin of RCA was visualized. Biphasic retrograde flow from the LM into the pulmonary artery (PA) with diastolic predominance was documented by color flow and pulsed Doppler echocardiography (Figures 1 and 2, A and B). TEE findings were confirmed by coronary angiography (Figure 2, D) and at the time of bypass operation. In case 2, delineation of an anomalous RCA origin from the posteromedial pulmonary trunk and its course anterior to the aortic root (Figure 3) required scanning with color flow Doppler echocardiography. A maximal leftward flexion of the horizontal plane was necessary to delineate the course of the anomalous RCA. Color flow and pulsed Doppler echocardiography confirmed the communication between the PA and RCA by demonstrating a biphasic retrograde flow with diastolic predominance into the pulmonary trunk. Angiography confirmed TEE findings (Figure 4). Anomalous Origin from the Right Sinus or RCA In 18 patients, anomalous origin of a coronary artery from the right sinus (n ⫽ 16) and/or the RCA (n ⫽ 5) was noted involving the LM (n ⫽ 3), LAD (n ⫽ 7), LCx (n ⫽ 6), and RCA (n ⫽ 5). TEE was useful in delineating the origin of an anomalous LM from the right sinus with a proximal course between the aortic root and the pulmonary trunk (case 3) (Figures 5 and 6), or anterior to PA (case 5) (Figure 7). Multiplane imaging from an intermediate 45-degree plane simplified delineation of the intermediate
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Table Different subgroups of coronary anomalies and Doppler analysis of proximal coronary flow No.
Sex/age (y)
Coronary anomaly
Peak S (cm/s)
Peak D (cm/s)
Peak S/D
Si/Di
Flow predominance
Angio
36 39
34 50
1.06 0.77
0.54 0.73
Diastolic Diastolic
Yes Yes
117 50
31 50
3.80 1.00
5.94 1.24
Systolic Systolic (LM)
Yes Yes
45
30
1.50
1.49
Anterior
14
21
0.67
0.72
Systolic (RCA) Diastolic
Yes
LAD - intermediate
36
13
2.77
2.51
Systolic
Yes
LCx - posterior LAD - anterior
16 23
26 48
0.62 0.48
0.44 0.15
Diastolic Diastolic
Yes
28
40
0.70
0.29
Diastolic
Course*
Anomalous pulmonary origin 1. F/46 LM from PA Anterior 2. M/43 RCA from PA Anterior Anomalous origin from the right sinus or right coronary artery 3. F/55 LM from right sinus Intermediate 4. M/45 LM from right sinus from a Intermediate common ostium with RCA
5.
M/50
6.
F/37
7.
M/31
LM from right sinus from a common ostium with RCA LAD and LCx with separate ostium from right sinus LAD from RCA and LCx from right sinus
LCx - posterior LAD from RCA and LCx from right LAD - intermediate, sinus LCx - posterior 9. M/37 LAD from RCA Intermediate 10. F/55 LAD from right sinus from common Intermediateostium with RCA 11. F/62 LAD from right sinus from common Anterior ostium with RCA 12. F/53 LAD from right sinus Anterior 13. M/43 LCx from right sinus Posterior 14. M/53 LCx from RCA Intermediate 15. M/48 LCx from RCA Anterior 16. M/44 Anterior takeoff of RCA from right Intermediate sinus 17. M/43 RCA from extreme left side of right Intermediate sinus 18. M/53 High and anterior takeoff of RCA Intermediate from right sinus 19. M/39 High takeoff of RCA from right Anterior sinus 20. M/54 High takeoff of RCA from right Not delineated sinus Anomalous origin from the left sinus or the left coronary system 21. M/44 High takeoff of RCA from left sinus Intermediate 22. F/64 RCA from left sinus, anterior to LM Intermediate 23. M/56 RCA from LCx Posterior 24. M71 RCA from left sinus, common Intermediate ostium with LM 25. M/50 Separate ostia for LAD and LCx LAD - anterior, LCx - posterior
8.
F/47
course of the coronary anomaly between the aorta and PA by obtaining a cross section of the coronary artery (Figure 5, B). Spectral Doppler echocardiography showed a predominant systolic flow pattern (Figure 6, B). However, a predominant diastolic flow pattern was observed when the anomalous LM coursed anterior to the pulmonary trunk (case 5). TEE findings were confirmed by angiography. In one unique case (case 6), LAD, LCx, and RCA originated from separate ostia from the right sinus. LCx coursed around the aorta in a rightward and
Yes 45 -
41 -
1.10 -
1.03 -
Systolic
Yes Yes
28
60
0.47
0.34
Diastolic
Yes
38 54 83
41 25 15
0.93 2.15 5.53
0.62 2.26 3.73
Diastolic Systolic Systolic
Yes Yes Yes Yes Yes
38
29
1.31
1.87
Systolic
No
90
20
4.50
3.63
Systolic
Yes
42
0
Systolic
Yes
-
-
-
Yes
36 96
80 62
No Yes Yes No
-
-
Diastolic Systolic (RCA) -
-
-
0.45 1.55
0.33 1.12
-
-
Yes
subsequent posterior direction. The vessel was readily seen using a modified approach of withdrawing the multiplane probe above the level of the aortic valve and using extreme retroflexion to reach the posterior aspect of the aortic sinuses in a tangential plane (Figure 8). The LAD had an acute takeoff from the aorta and coursed in between the aorta and PA. The ostia for LAD and LCx were to the left and to the right, respectively, of the normal RCA. Spectral Doppler of the proximal LAD and LCx demonstrated predominant systolic and diastolic
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Table continued No.
Sex/age (y)
26.
F/74
Coronary anomaly
Separate ostia for LAD and LCx
27.
M/23
Separate ostia for LAD and LCx
28.
M/75
Separate ostia for LAD and LCx
29.
M/23
LM from noncoronary sinus
Single coronary artery from the aorta 30. M/48 Single coronary artery from the noncoronary sinus 31. F/59 LM from RCA
Coronary fistula 32. M/42 LM fistula continuing as LCx fistula
Peak S (cm/s)
Course*
LAD - anterior, LCx - posterior
Peak D (cm/s)
Peak S/D
Si/Di
19
51
0.37
0.40
12
33
0.36
0.32
-
-
0.60
0.50
-
-
LAD - anterior, LCx - posterior LAD - anterior, LCx - posterior Anterior
-
-
12
20
-
-
Anterior
19
23
0.82
0.85
Posterior
36
75
0.48
0.31
86
44
1.95
1.51
160
153
1.05
0.75
Posterior
Flow predominance
Diastolic (LAD) Diastolic (LCx) -
Angio
No
Yes
Diastolic (LAD) -
No
Diastolic (LCx) Diastolic (LM) Systolic (RCA)
Yes
Diastolic, (LCx fistula)
Yes
No
Yes
F, Female; LAD, left anterior descending coronary artery; LCx, left circumflex artery; LM, left main artery; M, male; PA, pulmonary artery; RCA, right coronary artery; S, systolic velocity; D, diastolic velocity; Si, Systolic time-velocity integral; Di, diastolic time-velocity integral; Angio, coronary angiography. *Relative to aortic and pulmonary trunk.
flow patterns, respectively. Coronary angiography confirmed separate ostia from the right sinus. Distinguishing a specific coronary anomaly often depended on defining the anatomy of the remaining coronaries (cases 9-12). For example, in case 9 the anomalous coronary originating from the RCA with a proximal course between the aorta and PA (Figure 9) could only be identified as the LAD after further demonstrating a normal LCx in the atrioventricular groove (Figure 10, A) and the absence of a normal LAD (Figure 10, B). Angiography confirmed the TEE findings (Figure 11). Anomalous LAD from the right sinus with a common ostium with RCA could be identified in the horizontal and vertical planes as illustrated by case 10 (Figures 12 and 13) and case 11 (Figures 14 and 15), respectively. The proximal course of an anomalous LCx with origin from RCA could be demonstrated between the aorta and PA (case 14) (Figure 16) or anterior to the PA (case 15) by monoplane or biplane TEE, respectively. In case 16, anomalous origin of RCA from an extreme leftward or anterior aspect of the right sinus with resultant compression of the vessel between the aorta and the right ventricular infundibulum was demonstrable with multiplane imaging. Spectral Doppler demonstrated a systolic flow predominance. Coronary anomalies with a takeoff significantly higher than at the normal sinus of Valsalva level (cases 19 and 20) were particularly challenging to locate and required a meticulous search of the aortic trunk, which was greatly aided by color flow Doppler echocardiogra-
phy. The long-axis and intermediate planes provided by multiplane TEE were especially well suited for this specific anomaly as imaging from the horizontal plane was unreliable. Anomalous Origin from the Left Sinus or Left Coronary System Anomalous origin of a coronary artery from the left sinus or left coronary system was present in 9 cases (Table). The complexity of delineating a high takeoff of RCA from the right sinus (eg, cases 19 and 20) was compounded by the high takeoff of the same vessel from the left sinus and its intermediate course between the aorta and PA (case 21) (Figures 17 and 18). Importantly, echocardiographic dropout of the vessel and the PA wall at the site of potential continuity with PA could lead to a misinterpretation of the RCA as having an origin from the pulmonary trunk (Figure 18), further emphasizing the role of color flow and pulsed Doppler echocardiography to confirm or to refute communication between a coronary anomaly and PA. Delineation of RCA from the left sinus (case 22) (Figure 19) or from LCx (case 23) posed unique challenges. To adequately demonstrate the intermediate course between the aorta and PA of the RCA originating from the left sinus, the immediate superior course of the vessel toward the right ventricular atrioventricular groove must be anticipated requiring appropriate degrees of anteflexion and slight withdrawal of the TEE probe. Exceptionally complex was delineation of RCA with origin from LCx with a subsequent posterior course
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Figure 1 Color flow Doppler from long-axis transesophageal echocardiographic plane demonstrating communication between left main (LM) (leftward arrow) and pulmonary artery (PA). Retrograde flow was evident in LM (arrowhead) (A). Pulsed Doppler echocardiography of distal LM showed higher peak systolic (S) compared with diastolic (D) velocity (B). EKG, Electrocardiogram; LA, left atrium.
Journal of the American Society of Echocardiography December 2003
Figure 3 Horizontal 2-dimensional transesophageal echocardiographic (TEE) view of right coronary artery (arrow) from main pulmonary trunk (PA) coursing anterior to aorta (AO) (A). Retrograde flow at communication with PA was biphasic with similar systolic (S) and diastolic (D) peak velocities but greater D versus S time-velocity integral by pulsed Doppler TEE (B). EKG, Electrocardiogram; LA, left atrium.
tion of separate ostia of LAD and LCx from the left sinus with a diastolic flow pattern was readily feasible (cases 25-28). Anomalous origin of LM from the noncoronary sinus was seen in 1 case (case 29). Single Coronary Artery from the Aorta
Figure 2 Color flow (A) and pulsed (B) Doppler of immediate proximal (Prox.) left main (LM) from long-axis transesophageal echocardiographic view. Peak systolic (S) was greater than peak diastolic (D) flow. Normal takeoff of right coronary artery (RCA) was demonstrable (C). Results were confirmed by coronary angiography demonstrating retrograde flow (arrow) from LM into pulmonary artery during RCA injection (left anterior oblique view) (D). EKG, Electrocardiogram; LA, left atrium; LV, left ventricle; RV, right ventricle.
(case 23). In case 24, RCA originated from the left sinus from a common ostium with LM (Figure 20). The subsequent course of RCA was intermediate between the aorta and PA. Flow in the proximal RCA showed a systolic predominance pattern. Delinea-
Single coronary artery with origin from the posterior aortic sinus was seen in 2 cases (cases 30 and 31). In case 30, branching of the single coronary into vessels analogous to RCA, LM, LAD, and LCx could be definitively demonstrated despite the use of a monoplane probe (Figure 21). This potentially challenging coronary anomaly was surprisingly easy to visualize by leftward flexion because the proximal branches were not in dissimilar planes. The course of the vessel analogous to LCx was anterior to PA with a subsequent posterior course into the atrioventricular groove (Figure 22, A). A predominant systolic flow pattern was observed (Figure 22, B). TEE findings were corroborated by angiography in cases 30 and 31. Coronary Fistula A LM fistula with origin from the left sinus and continuing as a LCx fistula was demonstrated in 1 patient and was confirmed by angiography (case 32) (Figures 23 to 25). The demonstration of the circuitous route of the fistula from its origin to the entry into the right atrium near the coronary sinus was greatly facilitated by multiplane views that varied from 0 to 135 degrees (Figure 23, A, and Figure 24). It is doubtful that the complex anatomy of this coronary fistula could have been fully demonstrated
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Figure 4 Coronary angiogram from right anterior oblique projection showing contrast injection into left main (LM) with flow from left anterior descending (LAD) to right coronary artery (RCA). Retrograde flow (arrow) from RCA into main pulmonary artery was evident. Angiogram confirmed transesophageal echocardiographic findings in Figure 3.
Figure 6 Color flow Doppler transesophageal echocardiographic (TEE) demonstrating laminar flow at origin (leftward arrow) and intermediate (upward arrows) course between aorta and pulmonary artery (PA) (A). Peak systolic (S) flow was greater than diastolic (D) flow at coronary origin by pulsed Doppler TEE (B). LA, Left atrium.
Figure 5 Horizontal (A) and longitudinal (B) transesophageal echocardiographic views of left main (LM) originating from right sinus and coursing (upward arrows) in between aorta and pulmonary artery (PA). Horizontal plane was useful in delineating angle of takeoff of anomalous coronary and longitudinal view was helpful in assessing potential extrinsic coronary compression. LA, Left atrium.
Figure 7 Sequential horizontal transesophageal echocardiographic views of anomalous left main showing common ostium (arrow) with right coronary artery from right sinus (A) and course (arrow) anterior to pulmonary artery (PA) (B) and bifurcation into left anterior descending (C) and left circumflex (arrows) (D). AO, Aorta.
in sequential views with biplane TEE, and it is unlikely to have been fully appreciated by monoplane TEE. Spectral Doppler echocardiography demonstrated high systolic and diastolic velocities.
(Table). Of the 16 anomalous LM, LAD, or LCx coronary arteries in 15 patients, 5 had an intermediate course between the aorta and PA, whereas 11 coursed anterior or posterior to the great vessels. In this group, a predominant systolic flow pattern was observed exclusively in arteries with an intermediate course (5/5, P ⬍ .001), with the usual diastolic flow pattern observed consistently (11/11) in arteries with an anterior or posterior
Proximal Coronary Flow Pattern by Pulsed Doppler Proximal flow pattern was examined by pulsed Doppler echocardiography in 23 of 32 patients
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Figure 8 Multiplane transesophageal echocardiographic views from horizontal (A) and vertical (B) planes demonstrating anomalous circumflex (downward arrows) coursing posterior to aorta (AO) at level of aortic valve (AV). Vessel and left anterior (LA) descending artery originated from separate ostia in right sinus. LV, Left ventricle; RA, right atrium; RV, right ventricle.
Figure 9 Transesophageal echocardiographic illustrating anomalous left anterior descending with origin from right coronary artery (arrow) and course (arrowhead) between aorta (AO) and pulmonary artery (PA). LA, Left atrium; RA, right atrium.
course. Of these 5 anomalous left-sided arteries with an intermediate course, 2 originated from another artery, and 3 originated from the right sinus. All 3 arteries with a sinus origin had an acute angulation at takeoff. Of the 6 anomalous RCAs, 5 were interposed between the great vessels, and 1 followed an anterior course. Of 5 (80%) anomalous RCAs with an intermediate course, 4 showed a predominant systolic flow pattern.
Figure 10 Horizontal (A) and vertical (B) transesophageal echocardiographic views of left circumflex (LCx) in same patient as that of Figure 9. Definitive identification of anomalous vessel in this patient as left anterior descending (LAD) was confirmed by demonstrating absence of anatomically normal LAD. No LAD was present at normal anatomic site (upward arrows). AO, Aorta; LA, left atrium; PA, pulmonary artery.
DISCUSSION Clinical outcome of adults with anomalous coronary arteries greatly depends on the anatomy of the anomalous vessel.1-4,7-10 Coronary anomalies with an origin from the PA or having a proximal course between the PA and aortic trunk are frequently associated with a more adverse outcome.9,10,24,25
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Figure 11 Coronary angiogram from right anterior oblique projection from same patient as in Figures 9 and 10 showing origin of left anterior descending (LAD) from right coronary artery (RCA). Arrows demonstrate course of LAD in anterior interventricular sulcus.
Figure 13 Coronary angiogram from right anterior oblique view confirming transesophageal echocardiographic findings seen in Figure 12. Left anterior descending (LAD) has common ostium with right coronary artery (RCA).
Figure 12 Horizontal transesophageal echocardiographic view of anomalous left anterior descending (upward arrow) with common ostium with right coronary artery (downward arrow) and course between aorta (AO) and pulmonary artery (PA). LA, Left atrium.
Figure 14 Vertical transesophageal echocardiographic view with color flow Doppler illustrating origin of normal right coronary artery (RCA) (downward arrow) (A) and anomalous left anterior (LA) descending (LAD) (leftward arrow) (B) from right sinus. Anomalous LAD was visualized to have common ostium with RCA by slight counterclockwise rotation after visualizing RCA.
This study includes diverse and rare coronary anomalies, with several evaluated by TEE for the first time. The findings demonstrate a very effective adjunctive role of multiplane TEE to coronary angiography in identifying the origin and delineating the proximal course of anomalous coronary anatomy in adults. Examination of the flow patterns in the proximal segments of anomalous coronaries revealed additional findings of clinical importance. A predominant systolic flow pattern, as opposed to the usual predominant diastolic flow, was observed in anomalous coronaries with a proximal course between the aorta and PA. This observation provides novel
insights into the dynamic flow characteristics in anomalous coronary arteries subjected to reduced diastolic flow as a result of the shape of ostium and/or compression between the aorta and PA. Anomalous Coronary from the Pulmonary Trunk Origin of LM or RCA from the pulmonary trunk may result in myocardial infarction or sudden death.2,25 Survival depends on the reversal of flow to retrograde flow into the PA within the first 2 to 12
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Figure 15 Coronary angiogram from left anterior oblique view showing common ostium of left anterior descending (LAD) and right coronary artery (RCA) in same patient as in Figure 14.
Figure 16 Horizontal transesophageal echocardiographic view at level of aorta and pulmonary artery (PA) demonstrating anomalous left circumflex (LCx) with origin from right coronary artery (RCA) and proximal course in between aorta and PA (A). Accompanying angiogram from right anterior oblique projection confirms origin of LCx from RCA. RCA had normal course in atrioventricular groove (horizontal arrow) (B). Cath, Catheter; LA, left atrium.
months of life.2 In this study, retrograde flow from the LM and RCA into the PA was biphasic. A diastolic predominant pattern despite a higher systolic velocity was noted in the LM. This high systolic velocity can be explained on the basis of higher retrograde driving pressures generated during left ventricular systole as compared with diastole. However, the distribution of this biphasic flow in a LM from the pulmonary trunk will likely be influenced by aortic versus PA systolic pressures, and the length of diastole. Retrograde flow in the anomalous RCA
Journal of the American Society of Echocardiography December 2003
Figure 17 Intermediate transesophageal echocardiographic view of proximal aorta (AO) demonstrating high takeoff of anomalous right coronary artery (RCA) from left sinus (arrow) (A). Analogous view with color flow Doppler demonstrates color aliasing flow pattern in proximal RCA (B). Counterclockwise rotation of probe delineated intermediate course between great vessels (C and D). LA, Left atrium; RA, right atrium.
Figure 18 Transesophageal echocardiographic view in same patient as that of Figure 17 showing anomalous right coronary artery course (arrows) anterior to aorta. Echocardiographic dropout of anomalous vessel and pulmonary artery (PA) wall at site of apposition simulated origin of anomalous vessel from PA. Left main coronary artery (LM) had normal origin from left sinus. LA, Left atrium; RA, right atrium.
from the PA also showed a diastolic predominance with similar velocities in systole and diastole. This can be explained by a less pronounced difference between systolic and diastolic pressures generated by the right ventricle. These observations suggest that the relative predominance of systolic or diastolic flow by pulsed Doppler may be useful in the assessment of the anomalous coronary artery from
Journal of the American Society of Echocardiography Volume 16 Number 9
Figure 19 Horizontal transesophageal echocardiographic view of anomalous right coronary artery (RCA) form left sinus with intermediate course between aorta and pulmonary artery (PA) (A). Left main (LM) was normal. Coronary angiogram confirmed origin of anomalous RCA (B). Cath, Catheter; LA, left atrium.
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Figure 21 Horizontal transesophageal echocardiographic view demonstrating single coronary artery with origin (arrow) from noncoronary sinus (NCS) that branched into vessels analogous to right coronary artery (RCA) and left main (LM) (A). Vessels analogous to left anterior descending (LAD) and left circumflex (LCx) coronary arteries could be delineated branching from LM (B). LA, Left atrium; LCS, left coronary sinus; PA, pulmonary artery; RA, right atrium; RCS, right coronary sinus.
vessel orifice or extrinsic compression was seen in only 1 case, TEE observations at rest do not exclude the possibility of compression or dynamic narrowing at the orifice during exercise. Doppler interrogation of proximal flow revealed the usual diastolic pattern in LAD and LCx originating from separate ostia. Single Coronary Artery
Figure 20 Horizontal transesophageal echocardiographic view of anomalous right coronary artery (R) originating from left sinus with common ostium (arrow) with left main (L). R coursed between aorta (AO) and pulmonary artery. L gave rise to left circumflex (C) and continued its course as left anterior descending. LA, Left atrium; RVOT, right ventricular outflow tract.
the PA. Prior TEE studies identifying similar anomalies in 4 patients did not evaluate the spectral Doppler flow patterns.11-13,26 Anomalous Origin from the Right or Left Coronary Systems Multiplane as compared with monoplane TEE allowed for a more expeditious localization of coronary anomalies with a high takeoff from the right sinus. Although overt dynamic narrowing of the
TEE diagnosis of a single coronary artery requires an especially meticulous technique in that exclusion of other possible coronaries from the aorta or pulmonary trunk is mandatory. In addition, the definitive diagnosis by TEE alone would appear to require coronary anatomy with a distribution reminiscent of the LAD, LCx, and RCA. However, in certain circumstances, the single coronary anatomy may be extremely varied and may not follow the usual coronary distribution,2 which would complicate the TEE diagnosis. To our knowledge, this study is the first description of spectral Doppler TEE flow patterns of a single coronary artery. Coronary Artery Fistula Although the larger size of a coronary fistula makes its identification relatively easy, delineating its contiguous course from its origin to the end is far more challenging and emphasizes the importance of multiplane TEE. The velocity of blood flow was high in both systole and diastole and can be explained by communication of a high-pressure (ie, aorta) with a low-pressure (ie, right atrium) chamber.
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Figure 22 Horizontal transesophageal echocardiographic view demonstrating continuation of anomalous circumflex (arrows) from single coronary artery coursing lateral to pulmonary artery (PA) and toward atrioventricular groove (A). Spectral Doppler in midportion of vessel demonstrated predominantly systolic flow (B) with peak velocity of 43 cm/s (arrow). LA, Left atrium; p, electrocardiographic P wave.
Figure 23 Horizontal transesophageal echocardiographic view of left main coronary (LM) fistula, which gave rise to normal size left anterior descending (arrow) and continued as left circumflex (LCx) fistula (A). Contrast angiography demonstrated proximal origin of LM fistula from left sinus (B). LA, Left atrium; NC, noncoronary sinus; RC, right coronary sinus.
Examination of the Proximal Coronary Flow Pattern The features of a coronary anomally that predispose to adverse outcomes include an acute angle of takeoff,30 passage between the aorta and PA,8 a slit-like or hypoplastic ostium,1 and presence of a ridge or flap at origin.1,6 An acute takeoff is more
Journal of the American Society of Echocardiography December 2003
Figure 24 Sequential transesophageal echocardiographic (TEE) views demonstrating circuitous route of coronary fistula from same patient as that of Figure 23. Circumflex portion of fistula (arrows) began in atrioventricular groove as shown by horizontal TEE views (A). Fistula continued posteriorly in serpiginous manner (arrows) as demonstrated in vertical view with color flow Doppler (B). Further course of fistula inferior to left ventricle (LV) and left atrium (LA) was delineated with color Doppler (arrow) from long-axis view (C). Further clockwise rotation of TEE probe demonstrated entry of fistula into right atrium (RA) (D).
likely to occur when an anomalous artery originates from the opposite sinus and takes an intermediate course between the aorta and PA.1 An acute angle of takeoff may prevent blood from entering the artery in diastole with consequent myocardial ischemia resulting in fatality.1,6,8 An adverse outcome is more likely when an anomalous artery of the left system takes an intermediate course.1 Although pulsed Doppler interrogation of anomalous coronaries has been reported,28 this study provides a systematic analysis of coronary flow pattern in relation to the origin and proximal course of anomalous coronaries and their significance for the first time. Doppler flow analysis in the proximal segments of 5 left-sided anomalous coronaries with an intermediate course between the aorta and PA showed a predominant systolic flow pattern. Because the leftsided coronary arteries usually have diastolic predominant flow, this finding indicates that flow in diastole is compromised leading to a relative systolic flow predominance in these anomalous arteries. Interestingly, all 3 arteries with origin from the opposite (ie, right) sinus in this group showed acute angulation at takeoff. This novel echocardiographic observation supports the notion that anomalous coronaries with an acute takeoff, and an intermediate course relative to the aorta and PA, are more susceptible to restricted flow during diastole.1,6,8,27 All anomalous left-sided coronaries with an intermediate course showed an abnormal systolic flow
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Dawn et al 1285
suggests a possible interposition of the proximal segment between the aorta and the pulmonary trunk. This abnormal flow pattern is also more likely to be associated with an acute angulation at takeoff of the coronary from the opposite sinus. Because the intermediate course of an anomalous coronary is associated with a worse prognosis,1,8 this novel Doppler TEE observation may potentially be used for noninvasive risk stratification in patients with anomalous coronaries. Larger studies will be necessary to further elucidate this issue.
REFERENCES Figure 25 Contrast angiography from shallow left anterior oblique projection of coronary fistula described in Figures 23 and 24 confirming transesophageal echocardiographic findings. Arrows delineate circuitous route of fistula from its origin from aorta until its drainage into right atrium.
pattern, suggesting the possibility of ostial compromise or luminal compression irrespective of specific site of origin. Analysis of proximal flow in anomalous RCAs with an intermediate course also showed a consistent systolic predominance. However, flow in a normal RCA is determined by pressures in the right-sided chambers, and the difference between systolic and diastolic pressure gradients that drive coronary flow in systole and diastole, respectively, are not as pronounced as in the left-sided chambers. Thus, although considered diastolic predominant,29 this predominance is rather marginal, and RCA flow pattern can be easily affected by other factors including heart rate, presence of pulmonary hypertension, and right ventricular hypertrophy.30 Notwithstanding these possible confounding variables, pronounced systolic predominance in the anomalous RCAs interposed between the aorta and the PA suggests that this systolic flow pattern can serve as a reliable marker of an intermediate proximal course for RCAs also. Study Limitations The relatively small patient population is a limitation, but to date, this is the largest reported series of coronary anomalies delineated by TEE. Coronary angiography was not performed in 6 of 32 cases. Conclusions TEE, particularly with a multiplane probe, is extremely useful in accurately delineating the origin, proximal course, and pattern of flow in anomalous coronaries. Identification of a predominant systolic flow pattern by TEE in an anomalous coronary artery
1. Cheitlin MD, De Castro CM, McAllister HA. Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva, a not-so-minor congenital anomaly. Circulation 1974;50:780-7. 2. Roberts WC. Major anomalies of coronary arterial origin seen in adulthood. Am Heart J 1986;111:941-63. 3. Liberthson RR, Dinsmore RE, Fallon JT. Aberrant coronary artery origin from the aorta: report of 18 patients, review of literature and delineation of natural history and management. Circulation 1979;59:748-54. 4. Chaitman BR, Lesperance J, Saltiel J, Bourassa MG. Clinical, angiographic, and hemodynamic findings in patients with anomalous origin of the coronary arteries. Circulation 1976; 53:122-31. 5. Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Catheter Cardiovasc Diagn 1990;21:28-40. 6. Lipsett J, Cohle SD, Berry PJ, Russell G, Byard RW. Anomalous coronary arteries: a multicenter pediatric autopsy study. Pediatr Pathol 1994;14:287-300. 7. Mahowald JM, Blieden LC, Coe JI, Edwards JE. Ectopic origin of a coronary artery from the aorta: sudden death in 3 of 23 patients. Chest 1986;89:668-72. 8. Taylor AJ, Rogan KM, Virmani R. Sudden cardiac death associated with isolated congenital coronary artery anomalies. J Am Coll Cardiol 1992;20:640-7. 9. Kragel AH, Roberts WC. Anomalous origin of either the right or left main coronary artery from the aorta with subsequent coursing between aorta and pulmonary trunk: analysis of 32 necropsy cases. Am J Cardiol 1988;62:771-7. 10. Roberts WC, Kragel AH. Anomalous origin of either the right or left main coronary artery from the aorta without coursing of the anomalistically arising artery between aorta and pulmonary trunk. Am J Cardiol 1988;62:1263-7. 11. Gaither NS, Rogan KM, Stajduhar K, Banks AK, Hull RW, Whitsitt T, et al. Anomalous origin and course of coronary arteries in adults: identification and improved imaging utilizing transesophageal echocardiography. Am Heart J 1991;122: 69-75. 12. Fernandes F, Alam M, Smith S, Khaja F. The role of transesophageal echocardiography in identifying anomalous coronary arteries. Circulation 1993;88:2532-40. 13. Giannoccaro PJ, Sochowski RA, Morton BC, Chan KL. Complementary role of transoesophageal echocardiography to coronary angiography in the assessment of coronary artery anomalies. Br Heart J 1993;70:70-4. 14. Tardif JC, Vannan MA, Taylor K, Schwartz SL, Pandian NG. Delineation of extended lengths of coronary arteries by mul-
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15.
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tiplane transesophageal echocardiography. J Am Coll Cardiol 1994;24:909-19. Samdarshi TE, Hill DL, Nanda NC. Transesophageal color Doppler diagnosis of anomalous origin of left circumflex coronary artery. Am Heart J 1991;122:571-3. Koh KK. Confirmation of anomalous origin of the right coronary artery from the left sinus of Valsalva by means of transesophageal echocardiography. Am Heart J 1991;122: 851-4. Henson KD, Geiser EA, Billett J, Alexander JA, Akins EW, Bopitiya C. Use of transesophageal echocardiography to visualize an anomalous right coronary artery arising from the left main coronary artery (single coronary artery). Clin Cardiol 1992;15:462-5. Zwicky P, Daniel WG, Mugge A, Lichtlen PR. Imaging of coronary arteries by color-coded transesophageal Doppler echocardiography. Am J Cardiol 1988;62:639-40. Salloum JA, Thomas D, Evans J. Transoesophageal echocardiography in diagnosis of aberrant coronary artery. Int J Cardiol 1991;32:106-8. Sasson Z, Grande P, Lorette I, McEwan P. Proximal narrowing of anomalous right coronary artery from the left coronary sinus: delineation by omniplane transesophageal echocardiogram. Can J Cardiol 1996;12:529-31. Nanda NC, Bhambore MM, Jindal A, Misra VK, Ansingkar K, Puri V, et al. Transesophageal three-dimensional echocardiographic assessment of anomalous coronary arteries. Echocardiography 2000;17:53-60. Pearce FB, Sheikh KH, deBruijn NP, Kisslo J. Imaging of the coronary arteries by transesophageal echocardiography. J Am Soc Echocardiogr 1989;2:276-83.
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23. Douglas JS Jr, Franch RH, King SB III. Coronary artery anomalies. In: King SB III, Douglas JS Jr, editors. Coronary arteriography and angioplasty. New York: McGraw-Hill; 1985. 24. Thompson SI, Vieweg WV, Alpert JS, Hagan AD. Anomalous origin of the right coronary artery from the left sinus of Valsalva with associated chest pain: report of two cases. Catheter Cardiovasc Diagn 1976;2:397-402. 25. Cieslinski G, Rapprich B, Kober G. Coronary anomalies: incidence and importance. Clin Cardiol 1993;16:711-5. 26. O’Rourke DJ, Flanagan M, Berman N, Southworth JB, Palac RT. Stenosis at the origin of an anomalous left main coronary artery arising from the pulmonary artery in a symptom-free adolescent girl: transesophageal echocardiographic findings. J Am Soc Echocardiogr 1996;9:724-6. 27. Virmani R, Chun PK, Goldstein RE, Robinowitz M, McAllister HA. Acute takeoffs of the coronary arteries along the aortic wall and congenital coronary ostial valve-like ridges: association with sudden death. J Am Coll Cardiol 1984;3:766-71. 28. Kasprzak JD, Kratochwil D, Peruga JZ, Drozdz J, Rafalska K, Religa W, et al. Coronary anomalies diagnosed with transesophageal echocardiography: complementary clinical value in adults. Int J Card Imaging 1998;14:89-95. 29. Ofili EO, Labovitz AJ, Kern MJ. Coronary flow velocity dynamics in normal and diseased arteries. Am J Cardiol 1993; 71:3-9D. 30. Watanabe N, Awa S, Akagi M, Ando Y, Oki N, Waragai T, et al. Effects of heart rate and right ventricular pressure on right coronary arterial flow and its systolic versus diastolic distribution in a variety of congenital heart diseases in children. Pediatr Int 2000;42:476-82.
Objectives: The purpose of this study was to examine the use of transesophageal echocardiography (TEE) in the identification and flow characterization of congenital coronary anomalies. Background: Congenital coronary anomalies in adults are rare but may cause serious cardiac complications. The use of TEE in evaluation of this entity has not been well defined. Very little is known regarding flow patterns in anomalous coronaries assessed by Doppler TEE. Methods: A total of 32 consecutive adult patients were studied using TEE to define the origin, course, and proximal flow pattern of suspected coronary anomalies. Results: Coronary anomalies identified using TEE included anomalous origin from the pulmonary trunk (n ⴝ 2), right sinus (n ⴝ 18), left sinus (n ⴝ 9), single coronary (n ⴝ 2), and left main coronary fistula (n ⴝ 1). Multiplane TEE performed in 20 cases simplified the delineation of more complex coronary anomalies. The origin was identified in all patients, proximal course delineated in 31, and
Congenital coronary artery anomalies may be
benign or potentially serious leading to myocardial ischemia, infarction, and sudden cardiac death.1-4 Although the overall incidence of congenital coronary artery anomalies is as high as 0.5% to 1.3%,5,6 certain specific types are exceptionally rare yet potentially fatal.1,7 A premortem diagnosis of this clinically significant entity is difficult. The specific origin and the proximal course of an anomalous coronary are major factors From the Division of Cardiology, Department of Medicine, University of Louisville, and the Jewish Hospital Heart and Lung Institute, Louisville, Kentucky. Reprint requests: Marcus F. Stoddard, MD, 530 S Jackson St, ACB, 3rd Floor, Division of Cardiology, University of Louisville, Louisville, KY 40292 (E-mail: [email protected]). Copyright 2003 by the American Society of Echocardiography. 0894-7317/2003/$30.00 ⫹ 0 doi:10.1067/S0894-7317(03)00554-6
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proximal flow pattern characterized by pulsed Doppler in 23 of 32 patients. In 16 anomalous left main, left anterior descending, or left circumflex coronary arteries, an abnormal systolic flow pattern (ie, systolic/diastolic time-velocity integral ratio >1) occurred exclusively (P < .001) when the anomalous artery had an intermediate (100%; 5/5) versus anterior or posterior course (0%; 0/11) relative to the aortic and pulmonary artery trunks. A systolic flow pattern was also evident in 4 (80%) of 5 patients with an anomalous right coronary artery with an intermediate course. Conclusions: TEE, particularly with a multiplane probe, has an important complementary role to coronary angiography in delineating the proximal course and pattern of flow in anomalous coronaries. Predominant systolic flow pattern in anatomically left proximal anomalous coronaries signifies an intermediate course between the aorta and the pulmonary trunk and may be clinically useful for risk stratification. (J Am Soc Echocardiogr 2003;16: 1274-86.)
in predicting a clinically significant or benign outcome.1-4,7-10 Although cardiac catheterization remains the gold standard for evaluation of coronary anatomy, recent smaller studies suggest a complementary role of transesophageal echocardiography (TEE) for the same.11-13 The ostia and the proximal portions of normal coronary arteries can be visualized with TEE.14 Delineation of coronary anomalies by TEE, primarily using monoplane TEE probes, has been reported.11-13,15-19 Multiplane TEE promises to further simplify the delineation of the variable proximal course of coronary anomalies20 and 3-dimensional TEE shows promise.21 Importantly, very little is known regarding the significance of flow pattern in the proximal segment of anomalous coronaries. The objectives of this study were to highlight the technical details involved in the TEE detection of coronary anomalies, and to characterize their
Journal of the American Society of Echocardiography Volume 16 Number 9
flow patterns. We report the largest series, to date, of coronary artery anomalies diagnosed or confirmed by TEE. To our knowledge, TEE evaluation of several of these rare coronary anomalies has never been reported.
METHODS Patients In all, 32 consecutive patients (22 men, 10 women) with a mean age of 49 ⫾ 12 (SD) years were included in the study. Presence of a coronary anomaly detected during a clinically indicated cardiac catheterization or TEE was the primary inclusion criterion. Specifically, 21 (65%) patients underwent TEE after an angiographic diagnosis of the anomaly, 5 (16%) underwent angiography after a TEE diagnosis of the anomaly, and 6 (19%) did not undergo angiography. The TEE operator and the angiographer were aware of these anomalies diagnosed by the alternative modalities before the procedure. An informed consent was obtained in all patients. The study protocol was approved by the human studies committee at our institution. TEE A 5.0-MHz monoplane (n ⫽ 7), biplane (n ⫽ 5), or multiplane (n ⫽ 20) TEE probe and echocardiographic machine (77020A, Sonos 1500 or Sonos 2500, HewlettPackard, Andover, Mass; HDI 5000, ATL, Bothell, Wash) were used. A comprehensive sequence of TEE views was obtained to define the origin and course of the coronary anomalies. Two-dimensional views were guided using color flow Doppler echocardiography. The origin and course of the anomalous coronary artery were identified. Normal coronaries were identified by previously described methods.22 Pulsed Doppler echocardiography was performed at relevant sites of the anomalous coronaries. Peak systolic and diastolic velocity of the coronary flow and the time-velocity integral were obtained. A systolic to diastolic time-velocity integral ratio ⬎ 1.0 was defined as a predominant systolic flow pattern. Takeoff of an anomalous artery was considered acute when the angle between the aortic wall and the proximal segment of the artery was ⬍45 degrees.8 TEE was performed and interpreted by the same experienced echocardiographer (M.F.S). Of 32 patients, 26 also underwent coronary angiography by conventional techniques.23 Statistical Analysis Fisher exact test was used to determine any significant difference in coronary flow pattern observed in different patient subgroups. A 2-tailed P value of ⬍.05 was considered statistically significant.
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RESULTS The specific coronary artery anomalies (32 cases) and the flow pattern in the proximal segment (23 cases) as demonstrated by TEE and coronary angiography are summarized in Table. Cases were divided into 5 groups on the basis of a modified classification2 as follows: (1) anomalous origin from the pulmonary trunk (n ⫽ 2); (2) anomalous origin from the right sinus or right coronary artery (RCA) (n ⫽ 18); (3) anomalous origin from the left sinus or left coronary system (n ⫽ 9); (4) single coronary artery (n ⫽ 2); and (5) coronary fistula (n ⫽ 1). Anomalous Origin from the Pulmonary Trunk Origin of a coronary artery from the pulmonary trunk was seen in 2 cases (cases 1 and 2). In case 1, the origin of the left main (LM) coronary artery could be easily identified by alternate clockwise and counterclockwise rotation of the multiplane probe while scanning the pulmonary trunk in a long-axis plane with color flow Doppler echocardiography (Figure 1, A). The LM had a posterior origin from the pulmonary trunk and branched into left anterior descending (LAD) and left circumflex (LCx), both of which had a normal proximal course. A normal origin of RCA was visualized. Biphasic retrograde flow from the LM into the pulmonary artery (PA) with diastolic predominance was documented by color flow and pulsed Doppler echocardiography (Figures 1 and 2, A and B). TEE findings were confirmed by coronary angiography (Figure 2, D) and at the time of bypass operation. In case 2, delineation of an anomalous RCA origin from the posteromedial pulmonary trunk and its course anterior to the aortic root (Figure 3) required scanning with color flow Doppler echocardiography. A maximal leftward flexion of the horizontal plane was necessary to delineate the course of the anomalous RCA. Color flow and pulsed Doppler echocardiography confirmed the communication between the PA and RCA by demonstrating a biphasic retrograde flow with diastolic predominance into the pulmonary trunk. Angiography confirmed TEE findings (Figure 4). Anomalous Origin from the Right Sinus or RCA In 18 patients, anomalous origin of a coronary artery from the right sinus (n ⫽ 16) and/or the RCA (n ⫽ 5) was noted involving the LM (n ⫽ 3), LAD (n ⫽ 7), LCx (n ⫽ 6), and RCA (n ⫽ 5). TEE was useful in delineating the origin of an anomalous LM from the right sinus with a proximal course between the aortic root and the pulmonary trunk (case 3) (Figures 5 and 6), or anterior to PA (case 5) (Figure 7). Multiplane imaging from an intermediate 45-degree plane simplified delineation of the intermediate
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1276 Dawn et al
Table Different subgroups of coronary anomalies and Doppler analysis of proximal coronary flow No.
Sex/age (y)
Coronary anomaly
Peak S (cm/s)
Peak D (cm/s)
Peak S/D
Si/Di
Flow predominance
Angio
36 39
34 50
1.06 0.77
0.54 0.73
Diastolic Diastolic
Yes Yes
117 50
31 50
3.80 1.00
5.94 1.24
Systolic Systolic (LM)
Yes Yes
45
30
1.50
1.49
Anterior
14
21
0.67
0.72
Systolic (RCA) Diastolic
Yes
LAD - intermediate
36
13
2.77
2.51
Systolic
Yes
LCx - posterior LAD - anterior
16 23
26 48
0.62 0.48
0.44 0.15
Diastolic Diastolic
Yes
28
40
0.70
0.29
Diastolic
Course*
Anomalous pulmonary origin 1. F/46 LM from PA Anterior 2. M/43 RCA from PA Anterior Anomalous origin from the right sinus or right coronary artery 3. F/55 LM from right sinus Intermediate 4. M/45 LM from right sinus from a Intermediate common ostium with RCA
5.
M/50
6.
F/37
7.
M/31
LM from right sinus from a common ostium with RCA LAD and LCx with separate ostium from right sinus LAD from RCA and LCx from right sinus
LCx - posterior LAD from RCA and LCx from right LAD - intermediate, sinus LCx - posterior 9. M/37 LAD from RCA Intermediate 10. F/55 LAD from right sinus from common Intermediateostium with RCA 11. F/62 LAD from right sinus from common Anterior ostium with RCA 12. F/53 LAD from right sinus Anterior 13. M/43 LCx from right sinus Posterior 14. M/53 LCx from RCA Intermediate 15. M/48 LCx from RCA Anterior 16. M/44 Anterior takeoff of RCA from right Intermediate sinus 17. M/43 RCA from extreme left side of right Intermediate sinus 18. M/53 High and anterior takeoff of RCA Intermediate from right sinus 19. M/39 High takeoff of RCA from right Anterior sinus 20. M/54 High takeoff of RCA from right Not delineated sinus Anomalous origin from the left sinus or the left coronary system 21. M/44 High takeoff of RCA from left sinus Intermediate 22. F/64 RCA from left sinus, anterior to LM Intermediate 23. M/56 RCA from LCx Posterior 24. M71 RCA from left sinus, common Intermediate ostium with LM 25. M/50 Separate ostia for LAD and LCx LAD - anterior, LCx - posterior
8.
F/47
course of the coronary anomaly between the aorta and PA by obtaining a cross section of the coronary artery (Figure 5, B). Spectral Doppler echocardiography showed a predominant systolic flow pattern (Figure 6, B). However, a predominant diastolic flow pattern was observed when the anomalous LM coursed anterior to the pulmonary trunk (case 5). TEE findings were confirmed by angiography. In one unique case (case 6), LAD, LCx, and RCA originated from separate ostia from the right sinus. LCx coursed around the aorta in a rightward and
Yes 45 -
41 -
1.10 -
1.03 -
Systolic
Yes Yes
28
60
0.47
0.34
Diastolic
Yes
38 54 83
41 25 15
0.93 2.15 5.53
0.62 2.26 3.73
Diastolic Systolic Systolic
Yes Yes Yes Yes Yes
38
29
1.31
1.87
Systolic
No
90
20
4.50
3.63
Systolic
Yes
42
0
Systolic
Yes
-
-
-
Yes
36 96
80 62
No Yes Yes No
-
-
Diastolic Systolic (RCA) -
-
-
0.45 1.55
0.33 1.12
-
-
Yes
subsequent posterior direction. The vessel was readily seen using a modified approach of withdrawing the multiplane probe above the level of the aortic valve and using extreme retroflexion to reach the posterior aspect of the aortic sinuses in a tangential plane (Figure 8). The LAD had an acute takeoff from the aorta and coursed in between the aorta and PA. The ostia for LAD and LCx were to the left and to the right, respectively, of the normal RCA. Spectral Doppler of the proximal LAD and LCx demonstrated predominant systolic and diastolic
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Dawn et al 1277
Table continued No.
Sex/age (y)
26.
F/74
Coronary anomaly
Separate ostia for LAD and LCx
27.
M/23
Separate ostia for LAD and LCx
28.
M/75
Separate ostia for LAD and LCx
29.
M/23
LM from noncoronary sinus
Single coronary artery from the aorta 30. M/48 Single coronary artery from the noncoronary sinus 31. F/59 LM from RCA
Coronary fistula 32. M/42 LM fistula continuing as LCx fistula
Peak S (cm/s)
Course*
LAD - anterior, LCx - posterior
Peak D (cm/s)
Peak S/D
Si/Di
19
51
0.37
0.40
12
33
0.36
0.32
-
-
0.60
0.50
-
-
LAD - anterior, LCx - posterior LAD - anterior, LCx - posterior Anterior
-
-
12
20
-
-
Anterior
19
23
0.82
0.85
Posterior
36
75
0.48
0.31
86
44
1.95
1.51
160
153
1.05
0.75
Posterior
Flow predominance
Diastolic (LAD) Diastolic (LCx) -
Angio
No
Yes
Diastolic (LAD) -
No
Diastolic (LCx) Diastolic (LM) Systolic (RCA)
Yes
Diastolic, (LCx fistula)
Yes
No
Yes
F, Female; LAD, left anterior descending coronary artery; LCx, left circumflex artery; LM, left main artery; M, male; PA, pulmonary artery; RCA, right coronary artery; S, systolic velocity; D, diastolic velocity; Si, Systolic time-velocity integral; Di, diastolic time-velocity integral; Angio, coronary angiography. *Relative to aortic and pulmonary trunk.
flow patterns, respectively. Coronary angiography confirmed separate ostia from the right sinus. Distinguishing a specific coronary anomaly often depended on defining the anatomy of the remaining coronaries (cases 9-12). For example, in case 9 the anomalous coronary originating from the RCA with a proximal course between the aorta and PA (Figure 9) could only be identified as the LAD after further demonstrating a normal LCx in the atrioventricular groove (Figure 10, A) and the absence of a normal LAD (Figure 10, B). Angiography confirmed the TEE findings (Figure 11). Anomalous LAD from the right sinus with a common ostium with RCA could be identified in the horizontal and vertical planes as illustrated by case 10 (Figures 12 and 13) and case 11 (Figures 14 and 15), respectively. The proximal course of an anomalous LCx with origin from RCA could be demonstrated between the aorta and PA (case 14) (Figure 16) or anterior to the PA (case 15) by monoplane or biplane TEE, respectively. In case 16, anomalous origin of RCA from an extreme leftward or anterior aspect of the right sinus with resultant compression of the vessel between the aorta and the right ventricular infundibulum was demonstrable with multiplane imaging. Spectral Doppler demonstrated a systolic flow predominance. Coronary anomalies with a takeoff significantly higher than at the normal sinus of Valsalva level (cases 19 and 20) were particularly challenging to locate and required a meticulous search of the aortic trunk, which was greatly aided by color flow Doppler echocardiogra-
phy. The long-axis and intermediate planes provided by multiplane TEE were especially well suited for this specific anomaly as imaging from the horizontal plane was unreliable. Anomalous Origin from the Left Sinus or Left Coronary System Anomalous origin of a coronary artery from the left sinus or left coronary system was present in 9 cases (Table). The complexity of delineating a high takeoff of RCA from the right sinus (eg, cases 19 and 20) was compounded by the high takeoff of the same vessel from the left sinus and its intermediate course between the aorta and PA (case 21) (Figures 17 and 18). Importantly, echocardiographic dropout of the vessel and the PA wall at the site of potential continuity with PA could lead to a misinterpretation of the RCA as having an origin from the pulmonary trunk (Figure 18), further emphasizing the role of color flow and pulsed Doppler echocardiography to confirm or to refute communication between a coronary anomaly and PA. Delineation of RCA from the left sinus (case 22) (Figure 19) or from LCx (case 23) posed unique challenges. To adequately demonstrate the intermediate course between the aorta and PA of the RCA originating from the left sinus, the immediate superior course of the vessel toward the right ventricular atrioventricular groove must be anticipated requiring appropriate degrees of anteflexion and slight withdrawal of the TEE probe. Exceptionally complex was delineation of RCA with origin from LCx with a subsequent posterior course
1278 Dawn et al
Figure 1 Color flow Doppler from long-axis transesophageal echocardiographic plane demonstrating communication between left main (LM) (leftward arrow) and pulmonary artery (PA). Retrograde flow was evident in LM (arrowhead) (A). Pulsed Doppler echocardiography of distal LM showed higher peak systolic (S) compared with diastolic (D) velocity (B). EKG, Electrocardiogram; LA, left atrium.
Journal of the American Society of Echocardiography December 2003
Figure 3 Horizontal 2-dimensional transesophageal echocardiographic (TEE) view of right coronary artery (arrow) from main pulmonary trunk (PA) coursing anterior to aorta (AO) (A). Retrograde flow at communication with PA was biphasic with similar systolic (S) and diastolic (D) peak velocities but greater D versus S time-velocity integral by pulsed Doppler TEE (B). EKG, Electrocardiogram; LA, left atrium.
tion of separate ostia of LAD and LCx from the left sinus with a diastolic flow pattern was readily feasible (cases 25-28). Anomalous origin of LM from the noncoronary sinus was seen in 1 case (case 29). Single Coronary Artery from the Aorta
Figure 2 Color flow (A) and pulsed (B) Doppler of immediate proximal (Prox.) left main (LM) from long-axis transesophageal echocardiographic view. Peak systolic (S) was greater than peak diastolic (D) flow. Normal takeoff of right coronary artery (RCA) was demonstrable (C). Results were confirmed by coronary angiography demonstrating retrograde flow (arrow) from LM into pulmonary artery during RCA injection (left anterior oblique view) (D). EKG, Electrocardiogram; LA, left atrium; LV, left ventricle; RV, right ventricle.
(case 23). In case 24, RCA originated from the left sinus from a common ostium with LM (Figure 20). The subsequent course of RCA was intermediate between the aorta and PA. Flow in the proximal RCA showed a systolic predominance pattern. Delinea-
Single coronary artery with origin from the posterior aortic sinus was seen in 2 cases (cases 30 and 31). In case 30, branching of the single coronary into vessels analogous to RCA, LM, LAD, and LCx could be definitively demonstrated despite the use of a monoplane probe (Figure 21). This potentially challenging coronary anomaly was surprisingly easy to visualize by leftward flexion because the proximal branches were not in dissimilar planes. The course of the vessel analogous to LCx was anterior to PA with a subsequent posterior course into the atrioventricular groove (Figure 22, A). A predominant systolic flow pattern was observed (Figure 22, B). TEE findings were corroborated by angiography in cases 30 and 31. Coronary Fistula A LM fistula with origin from the left sinus and continuing as a LCx fistula was demonstrated in 1 patient and was confirmed by angiography (case 32) (Figures 23 to 25). The demonstration of the circuitous route of the fistula from its origin to the entry into the right atrium near the coronary sinus was greatly facilitated by multiplane views that varied from 0 to 135 degrees (Figure 23, A, and Figure 24). It is doubtful that the complex anatomy of this coronary fistula could have been fully demonstrated
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Dawn et al 1279
Figure 4 Coronary angiogram from right anterior oblique projection showing contrast injection into left main (LM) with flow from left anterior descending (LAD) to right coronary artery (RCA). Retrograde flow (arrow) from RCA into main pulmonary artery was evident. Angiogram confirmed transesophageal echocardiographic findings in Figure 3.
Figure 6 Color flow Doppler transesophageal echocardiographic (TEE) demonstrating laminar flow at origin (leftward arrow) and intermediate (upward arrows) course between aorta and pulmonary artery (PA) (A). Peak systolic (S) flow was greater than diastolic (D) flow at coronary origin by pulsed Doppler TEE (B). LA, Left atrium.
Figure 5 Horizontal (A) and longitudinal (B) transesophageal echocardiographic views of left main (LM) originating from right sinus and coursing (upward arrows) in between aorta and pulmonary artery (PA). Horizontal plane was useful in delineating angle of takeoff of anomalous coronary and longitudinal view was helpful in assessing potential extrinsic coronary compression. LA, Left atrium.
Figure 7 Sequential horizontal transesophageal echocardiographic views of anomalous left main showing common ostium (arrow) with right coronary artery from right sinus (A) and course (arrow) anterior to pulmonary artery (PA) (B) and bifurcation into left anterior descending (C) and left circumflex (arrows) (D). AO, Aorta.
in sequential views with biplane TEE, and it is unlikely to have been fully appreciated by monoplane TEE. Spectral Doppler echocardiography demonstrated high systolic and diastolic velocities.
(Table). Of the 16 anomalous LM, LAD, or LCx coronary arteries in 15 patients, 5 had an intermediate course between the aorta and PA, whereas 11 coursed anterior or posterior to the great vessels. In this group, a predominant systolic flow pattern was observed exclusively in arteries with an intermediate course (5/5, P ⬍ .001), with the usual diastolic flow pattern observed consistently (11/11) in arteries with an anterior or posterior
Proximal Coronary Flow Pattern by Pulsed Doppler Proximal flow pattern was examined by pulsed Doppler echocardiography in 23 of 32 patients
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Figure 8 Multiplane transesophageal echocardiographic views from horizontal (A) and vertical (B) planes demonstrating anomalous circumflex (downward arrows) coursing posterior to aorta (AO) at level of aortic valve (AV). Vessel and left anterior (LA) descending artery originated from separate ostia in right sinus. LV, Left ventricle; RA, right atrium; RV, right ventricle.
Figure 9 Transesophageal echocardiographic illustrating anomalous left anterior descending with origin from right coronary artery (arrow) and course (arrowhead) between aorta (AO) and pulmonary artery (PA). LA, Left atrium; RA, right atrium.
course. Of these 5 anomalous left-sided arteries with an intermediate course, 2 originated from another artery, and 3 originated from the right sinus. All 3 arteries with a sinus origin had an acute angulation at takeoff. Of the 6 anomalous RCAs, 5 were interposed between the great vessels, and 1 followed an anterior course. Of 5 (80%) anomalous RCAs with an intermediate course, 4 showed a predominant systolic flow pattern.
Figure 10 Horizontal (A) and vertical (B) transesophageal echocardiographic views of left circumflex (LCx) in same patient as that of Figure 9. Definitive identification of anomalous vessel in this patient as left anterior descending (LAD) was confirmed by demonstrating absence of anatomically normal LAD. No LAD was present at normal anatomic site (upward arrows). AO, Aorta; LA, left atrium; PA, pulmonary artery.
DISCUSSION Clinical outcome of adults with anomalous coronary arteries greatly depends on the anatomy of the anomalous vessel.1-4,7-10 Coronary anomalies with an origin from the PA or having a proximal course between the PA and aortic trunk are frequently associated with a more adverse outcome.9,10,24,25
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Figure 11 Coronary angiogram from right anterior oblique projection from same patient as in Figures 9 and 10 showing origin of left anterior descending (LAD) from right coronary artery (RCA). Arrows demonstrate course of LAD in anterior interventricular sulcus.
Figure 13 Coronary angiogram from right anterior oblique view confirming transesophageal echocardiographic findings seen in Figure 12. Left anterior descending (LAD) has common ostium with right coronary artery (RCA).
Figure 12 Horizontal transesophageal echocardiographic view of anomalous left anterior descending (upward arrow) with common ostium with right coronary artery (downward arrow) and course between aorta (AO) and pulmonary artery (PA). LA, Left atrium.
Figure 14 Vertical transesophageal echocardiographic view with color flow Doppler illustrating origin of normal right coronary artery (RCA) (downward arrow) (A) and anomalous left anterior (LA) descending (LAD) (leftward arrow) (B) from right sinus. Anomalous LAD was visualized to have common ostium with RCA by slight counterclockwise rotation after visualizing RCA.
This study includes diverse and rare coronary anomalies, with several evaluated by TEE for the first time. The findings demonstrate a very effective adjunctive role of multiplane TEE to coronary angiography in identifying the origin and delineating the proximal course of anomalous coronary anatomy in adults. Examination of the flow patterns in the proximal segments of anomalous coronaries revealed additional findings of clinical importance. A predominant systolic flow pattern, as opposed to the usual predominant diastolic flow, was observed in anomalous coronaries with a proximal course between the aorta and PA. This observation provides novel
insights into the dynamic flow characteristics in anomalous coronary arteries subjected to reduced diastolic flow as a result of the shape of ostium and/or compression between the aorta and PA. Anomalous Coronary from the Pulmonary Trunk Origin of LM or RCA from the pulmonary trunk may result in myocardial infarction or sudden death.2,25 Survival depends on the reversal of flow to retrograde flow into the PA within the first 2 to 12
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Figure 15 Coronary angiogram from left anterior oblique view showing common ostium of left anterior descending (LAD) and right coronary artery (RCA) in same patient as in Figure 14.
Figure 16 Horizontal transesophageal echocardiographic view at level of aorta and pulmonary artery (PA) demonstrating anomalous left circumflex (LCx) with origin from right coronary artery (RCA) and proximal course in between aorta and PA (A). Accompanying angiogram from right anterior oblique projection confirms origin of LCx from RCA. RCA had normal course in atrioventricular groove (horizontal arrow) (B). Cath, Catheter; LA, left atrium.
months of life.2 In this study, retrograde flow from the LM and RCA into the PA was biphasic. A diastolic predominant pattern despite a higher systolic velocity was noted in the LM. This high systolic velocity can be explained on the basis of higher retrograde driving pressures generated during left ventricular systole as compared with diastole. However, the distribution of this biphasic flow in a LM from the pulmonary trunk will likely be influenced by aortic versus PA systolic pressures, and the length of diastole. Retrograde flow in the anomalous RCA
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Figure 17 Intermediate transesophageal echocardiographic view of proximal aorta (AO) demonstrating high takeoff of anomalous right coronary artery (RCA) from left sinus (arrow) (A). Analogous view with color flow Doppler demonstrates color aliasing flow pattern in proximal RCA (B). Counterclockwise rotation of probe delineated intermediate course between great vessels (C and D). LA, Left atrium; RA, right atrium.
Figure 18 Transesophageal echocardiographic view in same patient as that of Figure 17 showing anomalous right coronary artery course (arrows) anterior to aorta. Echocardiographic dropout of anomalous vessel and pulmonary artery (PA) wall at site of apposition simulated origin of anomalous vessel from PA. Left main coronary artery (LM) had normal origin from left sinus. LA, Left atrium; RA, right atrium.
from the PA also showed a diastolic predominance with similar velocities in systole and diastole. This can be explained by a less pronounced difference between systolic and diastolic pressures generated by the right ventricle. These observations suggest that the relative predominance of systolic or diastolic flow by pulsed Doppler may be useful in the assessment of the anomalous coronary artery from
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Figure 19 Horizontal transesophageal echocardiographic view of anomalous right coronary artery (RCA) form left sinus with intermediate course between aorta and pulmonary artery (PA) (A). Left main (LM) was normal. Coronary angiogram confirmed origin of anomalous RCA (B). Cath, Catheter; LA, left atrium.
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Figure 21 Horizontal transesophageal echocardiographic view demonstrating single coronary artery with origin (arrow) from noncoronary sinus (NCS) that branched into vessels analogous to right coronary artery (RCA) and left main (LM) (A). Vessels analogous to left anterior descending (LAD) and left circumflex (LCx) coronary arteries could be delineated branching from LM (B). LA, Left atrium; LCS, left coronary sinus; PA, pulmonary artery; RA, right atrium; RCS, right coronary sinus.
vessel orifice or extrinsic compression was seen in only 1 case, TEE observations at rest do not exclude the possibility of compression or dynamic narrowing at the orifice during exercise. Doppler interrogation of proximal flow revealed the usual diastolic pattern in LAD and LCx originating from separate ostia. Single Coronary Artery
Figure 20 Horizontal transesophageal echocardiographic view of anomalous right coronary artery (R) originating from left sinus with common ostium (arrow) with left main (L). R coursed between aorta (AO) and pulmonary artery. L gave rise to left circumflex (C) and continued its course as left anterior descending. LA, Left atrium; RVOT, right ventricular outflow tract.
the PA. Prior TEE studies identifying similar anomalies in 4 patients did not evaluate the spectral Doppler flow patterns.11-13,26 Anomalous Origin from the Right or Left Coronary Systems Multiplane as compared with monoplane TEE allowed for a more expeditious localization of coronary anomalies with a high takeoff from the right sinus. Although overt dynamic narrowing of the
TEE diagnosis of a single coronary artery requires an especially meticulous technique in that exclusion of other possible coronaries from the aorta or pulmonary trunk is mandatory. In addition, the definitive diagnosis by TEE alone would appear to require coronary anatomy with a distribution reminiscent of the LAD, LCx, and RCA. However, in certain circumstances, the single coronary anatomy may be extremely varied and may not follow the usual coronary distribution,2 which would complicate the TEE diagnosis. To our knowledge, this study is the first description of spectral Doppler TEE flow patterns of a single coronary artery. Coronary Artery Fistula Although the larger size of a coronary fistula makes its identification relatively easy, delineating its contiguous course from its origin to the end is far more challenging and emphasizes the importance of multiplane TEE. The velocity of blood flow was high in both systole and diastole and can be explained by communication of a high-pressure (ie, aorta) with a low-pressure (ie, right atrium) chamber.
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Figure 22 Horizontal transesophageal echocardiographic view demonstrating continuation of anomalous circumflex (arrows) from single coronary artery coursing lateral to pulmonary artery (PA) and toward atrioventricular groove (A). Spectral Doppler in midportion of vessel demonstrated predominantly systolic flow (B) with peak velocity of 43 cm/s (arrow). LA, Left atrium; p, electrocardiographic P wave.
Figure 23 Horizontal transesophageal echocardiographic view of left main coronary (LM) fistula, which gave rise to normal size left anterior descending (arrow) and continued as left circumflex (LCx) fistula (A). Contrast angiography demonstrated proximal origin of LM fistula from left sinus (B). LA, Left atrium; NC, noncoronary sinus; RC, right coronary sinus.
Examination of the Proximal Coronary Flow Pattern The features of a coronary anomally that predispose to adverse outcomes include an acute angle of takeoff,30 passage between the aorta and PA,8 a slit-like or hypoplastic ostium,1 and presence of a ridge or flap at origin.1,6 An acute takeoff is more
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Figure 24 Sequential transesophageal echocardiographic (TEE) views demonstrating circuitous route of coronary fistula from same patient as that of Figure 23. Circumflex portion of fistula (arrows) began in atrioventricular groove as shown by horizontal TEE views (A). Fistula continued posteriorly in serpiginous manner (arrows) as demonstrated in vertical view with color flow Doppler (B). Further course of fistula inferior to left ventricle (LV) and left atrium (LA) was delineated with color Doppler (arrow) from long-axis view (C). Further clockwise rotation of TEE probe demonstrated entry of fistula into right atrium (RA) (D).
likely to occur when an anomalous artery originates from the opposite sinus and takes an intermediate course between the aorta and PA.1 An acute angle of takeoff may prevent blood from entering the artery in diastole with consequent myocardial ischemia resulting in fatality.1,6,8 An adverse outcome is more likely when an anomalous artery of the left system takes an intermediate course.1 Although pulsed Doppler interrogation of anomalous coronaries has been reported,28 this study provides a systematic analysis of coronary flow pattern in relation to the origin and proximal course of anomalous coronaries and their significance for the first time. Doppler flow analysis in the proximal segments of 5 left-sided anomalous coronaries with an intermediate course between the aorta and PA showed a predominant systolic flow pattern. Because the leftsided coronary arteries usually have diastolic predominant flow, this finding indicates that flow in diastole is compromised leading to a relative systolic flow predominance in these anomalous arteries. Interestingly, all 3 arteries with origin from the opposite (ie, right) sinus in this group showed acute angulation at takeoff. This novel echocardiographic observation supports the notion that anomalous coronaries with an acute takeoff, and an intermediate course relative to the aorta and PA, are more susceptible to restricted flow during diastole.1,6,8,27 All anomalous left-sided coronaries with an intermediate course showed an abnormal systolic flow
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suggests a possible interposition of the proximal segment between the aorta and the pulmonary trunk. This abnormal flow pattern is also more likely to be associated with an acute angulation at takeoff of the coronary from the opposite sinus. Because the intermediate course of an anomalous coronary is associated with a worse prognosis,1,8 this novel Doppler TEE observation may potentially be used for noninvasive risk stratification in patients with anomalous coronaries. Larger studies will be necessary to further elucidate this issue.
REFERENCES Figure 25 Contrast angiography from shallow left anterior oblique projection of coronary fistula described in Figures 23 and 24 confirming transesophageal echocardiographic findings. Arrows delineate circuitous route of fistula from its origin from aorta until its drainage into right atrium.
pattern, suggesting the possibility of ostial compromise or luminal compression irrespective of specific site of origin. Analysis of proximal flow in anomalous RCAs with an intermediate course also showed a consistent systolic predominance. However, flow in a normal RCA is determined by pressures in the right-sided chambers, and the difference between systolic and diastolic pressure gradients that drive coronary flow in systole and diastole, respectively, are not as pronounced as in the left-sided chambers. Thus, although considered diastolic predominant,29 this predominance is rather marginal, and RCA flow pattern can be easily affected by other factors including heart rate, presence of pulmonary hypertension, and right ventricular hypertrophy.30 Notwithstanding these possible confounding variables, pronounced systolic predominance in the anomalous RCAs interposed between the aorta and the PA suggests that this systolic flow pattern can serve as a reliable marker of an intermediate proximal course for RCAs also. Study Limitations The relatively small patient population is a limitation, but to date, this is the largest reported series of coronary anomalies delineated by TEE. Coronary angiography was not performed in 6 of 32 cases. Conclusions TEE, particularly with a multiplane probe, is extremely useful in accurately delineating the origin, proximal course, and pattern of flow in anomalous coronaries. Identification of a predominant systolic flow pattern by TEE in an anomalous coronary artery
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23. Douglas JS Jr, Franch RH, King SB III. Coronary artery anomalies. In: King SB III, Douglas JS Jr, editors. Coronary arteriography and angioplasty. New York: McGraw-Hill; 1985. 24. Thompson SI, Vieweg WV, Alpert JS, Hagan AD. Anomalous origin of the right coronary artery from the left sinus of Valsalva with associated chest pain: report of two cases. Catheter Cardiovasc Diagn 1976;2:397-402. 25. Cieslinski G, Rapprich B, Kober G. Coronary anomalies: incidence and importance. Clin Cardiol 1993;16:711-5. 26. O’Rourke DJ, Flanagan M, Berman N, Southworth JB, Palac RT. Stenosis at the origin of an anomalous left main coronary artery arising from the pulmonary artery in a symptom-free adolescent girl: transesophageal echocardiographic findings. J Am Soc Echocardiogr 1996;9:724-6. 27. Virmani R, Chun PK, Goldstein RE, Robinowitz M, McAllister HA. Acute takeoffs of the coronary arteries along the aortic wall and congenital coronary ostial valve-like ridges: association with sudden death. J Am Coll Cardiol 1984;3:766-71. 28. Kasprzak JD, Kratochwil D, Peruga JZ, Drozdz J, Rafalska K, Religa W, et al. Coronary anomalies diagnosed with transesophageal echocardiography: complementary clinical value in adults. Int J Card Imaging 1998;14:89-95. 29. Ofili EO, Labovitz AJ, Kern MJ. Coronary flow velocity dynamics in normal and diseased arteries. Am J Cardiol 1993; 71:3-9D. 30. Watanabe N, Awa S, Akagi M, Ando Y, Oki N, Waragai T, et al. Effects of heart rate and right ventricular pressure on right coronary arterial flow and its systolic versus diastolic distribution in a variety of congenital heart diseases in children. Pediatr Int 2000;42:476-82.