APPENDIX
Appendix 1. (TTE ICL DATA REVIEW FORM AND PROPOSED STANDARD
REPORTING ELEMENTS)
Study Number: _____________________
Institution Code: _____________________
Echocardiogram: Date of Study: ________/_____/________(mm/dd/yyyy)
Reviewer Initials: _______
Which coronary is anomalous? Left
Right
Where does it arise? Right Sinus
Left Sinus
Right Coronary Artery
LAD
Circumflex
Both
Non-coronary Sinus
Left Coronary Artery
Supra Sinus
AAOCA course: Interarterial
Intramural
Yes
No
Yes
No
Unsure
Unsure
Length of Intramural portion: __________mm
Intraconal
Yes
No
Unsure
Anterior to Pulmonary Artery Yes
Retro-aortic Yes
No
No
Unsure
Unsure
Ostia: Two in left sinus: Yes
No
One in left sinus
One in right sinus
Yes
High ostial take-off: Yes
No
No
Two in right sinus: Yes
Unsure
Yes
No
No
Image not obtained
Angle of take-off from anomalous sinus: Acute/angulated Yes
No
Unsure
*Color Doppler flow in diastole of intramural segment of AAOCA (if applicable): Yes
No
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Appendix 2.
BEST PRACTICE ECHOCARDIOGRAPHY PROTOCOL FOR PERFORMING TTE
AND TEE
The goal of this echocardiography protocol is to allow for diagnosis of all of the following
1.
Diagnosis of AAOCA
2.
Intramural course
3.
Interarterial course
4.
Intraconal course
5.
Position of the ostial origin relative to STJ of the aorta
Principles for image optimization
In order to best visualize and detail coronary anatomy, high spatial and temporal resolution; use
of high-frequency probes and small sector scanning is key. It is recommended to optimize use of
grey scale range with dynamic range near 50 decibels, edge enhancement, placement of transmit
focus at the region of interest and depth adjustments, adjusting color flow map settings using
high color Doppler frequency and optimization of Nyquist limit (near 20-40 cm/s). This allows
for visualization of flow in the coronary throughout the cardiac cycle while performing sweeps
rather than three beat clips and avoidance of still frame capture. Optimal positioning of the color
flow sector also allows for accurate diagnosis by demonstrating antegrade flow from the aortic
sinus into the proximal coronary. Additionally positioning the color sector over the interarterial
space allows for detection of anomalous coronaries if not noted from standard planes. AAOCA
may be difficult to diagnose in real time and obtaining a loop of several seconds and playing
them back slowly can help identify the ostia and the proximal course of the coronary arteries.
Imaging Views
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Parasternal short axis imaging (PS SAX)
Normal coronary ostia and the proximal course can be demonstrated at the level of the
aortic sinuses with the left main coronary artery (LMCA) arising approximately at 4
o’clock (Video1), and the right coronary artery (RCA) arising at approximately 10- 12 o’clock.
(Figure 1). For evaluating the interarterial course, subtle clockwise rotation of the transducer
from the standard parasternal short-axis and / or angling leftward towards the left shoulder is
generally needed (Figure 1, PSSAX-2). The course of the coronary artery in the interarterial
space is not necessarily from a standard short axis- plane (Video2, Video3, Video 6, and
Video 7). In patients with AAOCA, the ostia may arise more superiorly at or even above the STJ.
Multiple beat sweeps in an inferior to superior plane, through the aortic root; need to be
performed to demonstrate a high take off. With intramural coronaries, the proximal coronary
artery course is acute and makes a <30° angle with the aortic wall and courses parallel to the
wall. This can be well demonstrated by color Doppler focusing on the interarterial space. In
AAOCA, the anomalous coronary artery may falsely appear to have a normal origin
from the appropriate aortic sinus. This area of “takeoff” may be falsely misinterpreted as a
normal origin by still frames (Figure 1, (i), (ii), (iii)). The length of intramural segment of
AAOCA can be variable. PS SAX imaging will allow imaging the ostia of the AAOCA in
relation to the intercoronary commissure (Figure 1 (ii), (iii), (iv) and Video 2, Video 3).
Color Doppler evaluation of the ostium and the proximal course should be obtained. The linear
diastolic flow parallel or “with in” the aortic wall is an important indicator for an intramural
course. (Figure 1 (iv), (v)). Color Doppler can also be helpful in identifying which coronary
artery is anomalous. With AAOLCA a blue color Doppler signal will be seen in the anomalous
coronary as flow moves away from the right sinus towards the more posterior positioned left
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sinus of Valsalva (Video 3). This is in contrast to AAORCA, where a red color Doppler signal
will be seen in the anomalous coronary as flow moves anteriorly towards the right sinus from its
origin in the left sinus (Video 2).
An intraconal left main coronary artery (LMCA) or left anterior descending (LAD) can generally
be well demonstrated from a parasternal short-axis with the appearance of “muscle” surrounding
the proximal coronary artery (Figure 1 (vi), Video 7).
Left parasternal long axis imaging (PS LAX)
From the PS LAX view, the anomalous coronary coursing between the great arteries can
be seen as a discrete circle anterior to the aortic root (Figure 2 (i)). This view also allows
demonstration of the coronary origin of an AAOCA in relation to the sinutubular junction via a
sweep (Fig 2 (ii), Video 4a, Video 4b, Video 4c, Video 6). Color Doppler flow into the high
ostial takeoff should be performed (Video 4b, Video 4c). For intraconal course of the left main
coronary (LMCA) or left anterior descending (LAD) a PS LAX sweep angling to the left
shoulder demonstrates the intraconal course of the coronary inferior to the pulmonary
valve (Video 7). This PS LAX imaging plane may be one or two interspaces lower than usual.
Modified Apical
From a typical apical four-chamber view, moving the transducer medially and superiorly
and tilting it anteriorly (imaging window between a typical parasternal and apical window) will
demonstrate the course and length of the anomalous coronary between the two great arteries.
(Video 12). This view also will show an intraconal coronary artery inferior to the pulmonary
valve annulus (Video 5). The “hammock sign” of the LMCA from the right sinus of Valsalva (as
seen on RAO projection by coronary angiography or CTA) can also be obtained from modified
apical imaging. Intraconal LMCA coronary artery course can be associated with stenosis, and
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this should be evaluated with Color Doppler (Figure3 (ii)). Intraconal LAD can be associated
with a posterior course of the circumflex coronary artery (Video 10). Imaging the branching
pattern and origin in single coronary can also be demonstrated from this view (Figure 3(i)).
Sub-xiphoid imaging
Children who have good subcostal windows, a sweep from the aortic root more anteriorly
to the pulmonary root can help to identify a length of the anomalous coronary running between
the great arteries (Video 11).
The transesophageal imaging of AAOCA
This modality can be particularly useful in adolescents and adults when TTE cannot
adequately define the coronary origins. The above principles of imaging equally apply to TEE
imaging. The objective is to demonstrate the origin of the AAOCA from the aortic sinus and its
proximal course. Optimization of 2D and color Doppler as mentioned above apply. The imaging
planes in TEE should be similar to those in TTE in AAOCA. These planes are often
modifications of typical TEE imaging planes. Short axis imaging planes to visualize the ostium
and its relation to the commissure may be obtained between 25° to 50° (figure 11a, 11b). Long
axis imaging of the aorta and STJ at 100°-135° will demonstrate the origin of the anomalous
coronary artery in relation to the STJ and its interarterial course (Video 8c, Video 9b).
Color Doppler interrogation of the aortic root and interarterial space should be performed
as in TTEs. Since the probe is posterior to the heart in TEE compared to anterior in TTE the
correspondence between flow and color will be reversed in the anomalous coronary
artery. With AAOLCA red color Doppler signal (Video 9a, Video 9b) will be seen in contrast
to AAORCA where a blue color Doppler signal will be seen in the anomalous coronary as flow
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moves away from the TEE transducer towards the right sinus from its origin in the left sinus.
(Video 8a, Video 8b, Video 8c).
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