Imaging Atrial Septal Defects by Real-Time 3D ... Member Pages/Muhamed/Publications/2010_JAS… ·...

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Imaging Atrial Septal Defects by Real-Time 3D Transesophageal Echocardiography: Step-by-Step Approach Muhamed Saric, MD, PhD, FASE, Gila Perk, MD, FASE, Jan R. Purgess, MD, and Itzhak Kronzon, MD, FASE, New York, New York Background: There are currently no standardized three-dimensional (3D) transesophageal echocardiographic (TEE) views of the interatrial septum and atrial septal defects (ASDs). Without a standardized approach, it is difficult to ascertain the important anatomic relationships (such as the location of the aortic rim of an ASD), to perform relevant measurements (such as the size of an ASD or the size of its rims), or to guide the deploy- ment of catheters and devices during atrial septal closure. Methods: Using a 3D TEE matrix-array transducer, 706 TEE studies were performed over a 14-month period. The purpose of the study was to develop a standardized protocol for anatomically correct orientation of 3D TEE images of the interatrial septum and ASDs. Results: Among 706 TEE studies, there were 23 patients with ASDs, representing 3.3% of the study popula- tion. Eighteen patients had secundum ASDs, two had primum ASDs, and three had sinus venosus ASDs of the superior vena cava. A protocol for properly orienting 3D TEE images of the interatrial septum and ASDs was developed. When the images are acquired at an angle of 0 , the septum is properly oriented by the tilt-up-then- left maneuver. The initial 3D TEE image in first tilted up to reveal the right atrial side of the septum. Then the image is tilted 180 around its vertical axis to reveal the left atrial side of the septum; the aortic rim is on the left, the superior vena cava on the top, and the right-sided pulmonary vein ostia on the right side of the screen. For acquisitions at a higher angle, the rotate-left-in-z-axis maneuver is used. The image is first tilted up to reveal the right atrial side of the septum, as in the tilt-up-then-left maneuver. The image is then rotated coun- terclockwise in the z axis until the superior vena cave is at 12 o’clock. Finally, the image is tilted 180 around its vertical axis to reveal the left atrial side of the septum. Conclusions: The use of standardized tilt-up-then-left and rotate-left-in-z-axis maneuvers enhances the diag- nosis of ASDs, ascertains the important anatomic relationships of ASDs to surrounding structures, and facil- itates communication between echocardiographers obtaining 3D TEE images and interventional cardiologists or cardiac surgeons performing ASD closures. (J Am Soc Echocardiogr 2010;-:---.) Keywords: Real-time three-dimensional echocardiography, Transesophageal, Atrial septal defect, Interatrial septum, Device closure Three-dimensional (3D) transesophageal echocardiographic (TEE) imaging has been revolutionized by the introduction of a 3D-TEE probe with a matrix-array transducer with 3,000 elements. This is ap- proximately a 50-fold increase in the number of imaging elements compared with a standard two-dimensional transesophageal echocar- diographic probe, which typically has 64 elements. The basic princi- ples and history of 3D echocardiography have been presented in detail elsewhere. 1-3 What is the major difference between 2D and 3D TEE image ac- quisition? Briefly, a 2D TEE probe acquires a sector image whose di- mensions are as follows: width of up to 90 in the lateral (azimuth) direction, depth of up to approximately 16 cm in the axial direction, and thickness (elevation) that is negligible. The 3D TEE matrix-array probe expands this concept by acquiring not just one but a series of 2D sector images along the elevation axis to create a 3D pyramidal data set referred to as a frustum. By convention, the lateral (azimuth) direction is encoded in red, the elevation direction in green, and the depth direction in blue. Aside from the three axes of the pyramidal data set itself, there are also three axes of the 2D image used to display the pyramidal set on the screen. The horizontal axis runs from the left to the right edge of the screen. The vertical axis runs from the top to the bottom of the screen. Additionally, the axis that is perpendicular to the computer From the Division of Cardiology, New York University Langone Medical Center, New York, New York (M.S., G.P., I.K.); and the Department of Anesthesiology, New York University School of Medicine and New York Veterans Affairs Hospital, New York, New York (J.R.P.). Dr. Kronzon receives speakers’ bureau honoraria from Philips Medical Systems (Andover, MA). Reprint requests: Muhamed Saric, MD, PhD, FASE, New York University Medical Center, Noninvasive Cardiology Laboratory, 560 First Avenue, New York, NY 10016 (E-mail: [email protected]). 0894-7317/$36.00 Copyright 2010 by the American Society of Echocardiography. doi:10.1016/j.echo.2010.08.008 1 FLA 5.1.0 DTD ĸYMJE2303_proofĸ6September2010ĸ7:20pmĸceJK 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 96 97 98 99 100 101 102 103 104 105 106 107 108 109 110 111 112 113 114 115 116 117 118 119 120 121 122

Transcript of Imaging Atrial Septal Defects by Real-Time 3D ... Member Pages/Muhamed/Publications/2010_JAS… ·...

Page 1: Imaging Atrial Septal Defects by Real-Time 3D ... Member Pages/Muhamed/Publications/2010_JAS… · Imaging Atrial Septal Defects by Real-Time 3D Transesophageal Echocardiography:

Imaging Atrial Septal Defects by Real-Time 3DTransesophageal Echocardiography: Step-by-Step

Approach

Muhamed Saric, MD, PhD, FASE, Gila Perk, MD, FASE, Jan R. Purgess, MD,

and Itzhak Kronzon, MD, FASE, New York, New York

Background: There are currently no standardized three-dimensional (3D) transesophageal echocardiographic(TEE) views of the interatrial septum and atrial septal defects (ASDs). Without a standardized approach, it isdifficult to ascertain the important anatomic relationships (such as the location of the aortic rim of an ASD),to perform relevant measurements (such as the size of an ASD or the size of its rims), or to guide the deploy-ment of catheters and devices during atrial septal closure.

Methods: Using a 3D TEE matrix-array transducer, 706 TEE studies were performed over a 14-month period.The purpose of the study was to develop a standardized protocol for anatomically correct orientation of 3DTEE images of the interatrial septum and ASDs.

Results: Among 706 TEE studies, there were 23 patients with ASDs, representing 3.3% of the study popula-tion. Eighteen patients had secundumASDs, two had primum ASDs, and three had sinus venosus ASDs of thesuperior vena cava. A protocol for properly orienting 3D TEE images of the interatrial septum and ASDs wasdeveloped.When the images are acquired at an angle of 0�, the septum is properly oriented by the tilt-up-then-left maneuver. The initial 3D TEE image in first tilted up to reveal the right atrial side of the septum. Then theimage is tilted 180� around its vertical axis to reveal the left atrial side of the septum; the aortic rim is on theleft, the superior vena cava on the top, and the right-sided pulmonary vein ostia on the right side of the screen.For acquisitions at a higher angle, the rotate-left-in-z-axis maneuver is used. The image is first tilted up toreveal the right atrial side of the septum, as in the tilt-up-then-left maneuver. The image is then rotated coun-terclockwise in the z axis until the superior vena cave is at 12 o’clock. Finally, the image is tilted 180� around itsvertical axis to reveal the left atrial side of the septum.

Conclusions: The use of standardized tilt-up-then-left and rotate-left-in-z-axis maneuvers enhances the diag-nosis of ASDs, ascertains the important anatomic relationships of ASDs to surrounding structures, and facil-itates communication between echocardiographers obtaining 3D TEE images and interventional cardiologistsor cardiac surgeons performing ASD closures. (J Am Soc Echocardiogr 2010;-:---.)

Keywords: Real-time three-dimensional echocardiography, Transesophageal, Atrial septal defect, Interatrialseptum, Device closure

Three-dimensional (3D) transesophageal echocardiographic (TEE)

imaging has been revolutionized by the introduction of a 3D-TEE

probe with a matrix-array transducer with 3,000 elements. This is ap-

proximately a 50-fold increase in the number of imaging elements

compared with a standard two-dimensional transesophageal echocar-

diographic probe, which typically has 64 elements. The basic princi-

ples and history of 3D echocardiography have been presented in

detail elsewhere.1-3

What is the major difference between 2D and 3D TEE image ac-

quisition? Briefly, a 2D TEE probe acquires a sector image whose di-

mensions are as follows: width of up to 90� in the lateral (azimuth)

direction, depth of up to approximately 16 cm in the axial direction,

and thickness (elevation) that is negligible. The 3D TEE matrix-array

probe expands this concept by acquiring not just one but a series of

2D sector images along the elevation axis to create a 3D pyramidal

data set referred to as a frustum. By convention, the lateral (azimuth)

direction is encoded in red, the elevation direction in green, and the

depth direction in blue.

Aside from the three axes of the pyramidal data set itself, there are

also three axes of the 2D image used to display the pyramidal set on

the screen. The horizontal axis runs from the left to the right edge of

the screen. The vertical axis runs from the top to the bottom of the

screen. Additionally, the axis that is perpendicular to the computer

From the Division of Cardiology, New York University Langone Medical Center,

New York, New York (M.S., G.P., I.K.); and the Department of Anesthesiology,

New York University School of Medicine and New York Veterans Affairs

Hospital, New York, New York (J.R.P.).

Dr. Kronzon receives speakers’ bureau honoraria from Philips Medical Systems

(Andover, MA).

Reprint requests: Muhamed Saric, MD, PhD, FASE, New York University Medical

Center, Noninvasive Cardiology Laboratory, 560 First Avenue, New York, NY

10016 (E-mail: [email protected]).

0894-7317/$36.00

Copyright 2010 by the American Society of Echocardiography.

doi:10.1016/j.echo.2010.08.008

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monitor on the ultrasound sys-

tem and comes out of the screen

toward the user at the time of im-

age review is referred to as the z

axis. Rotations along the z axis

move in clockwise or counter-

clockwise direction and allow

for placing 3D images into con-

ventional views (such as the sur-

gical view of the mitral valve or

the anatomic orientation of the

interatrial septum).

There are currently no stan-

dardized 3D TEE views of the in-

teratrial septum and atrial septal

defects (ASDs). Without a standardized approach, it is difficult to

ascertain the important anatomic relationships (such as the location

of the aortic rim of an ASD), to perform relevant measurements

(such as the size of the ASD or the size of its rims), or to guide the

deployment of catheters and devices during atrial septal closure.

The purpose of this study was to develop a standardized protocol

for the placement of 3D TEE images in anatomically correct orienta-

tion.

METHODS

Three-dimensional TEE studies were recorded with a commercially

available ultrasound system (Philips iE33; Philips Medical Systems,

Andover, MA) using a matrix-array 3D TEE probe (X7-2 t; Philips

Medical Systems). Over a 14-month period, 3DTEE studies were per-

formed in 706 individuals.

Three-dimensional TEE images were obtained in the following four

modalities: (1) biplane imaging (a side-by-side display of a pair of 2D

TEE images that are 90� apart), (2) full-volume imaging (the frustum is

automatically subdivided by the ultrasound system in several slices;

each slice is acquired over one cardiac cycle; individual slices are

stitched together and displayed in a delayed nonlive fashion), (3)

narrow-angle live 3D imaging (live imaging of a system-selected frus-

tum segment measuring approximately 60� 30� in lateral x eleva-

tion axes and having a full depth in the depth axis), and (4) wide-angle

3D zoom imaging (live imaging of a user-selected frustum segment

measuring up to 85� 85� in lateral x elevation axes but with a sys-

tem-limited slice thickness in the depth axis).

Wide-angle 3D zoom appears to be the most useful 3D modality

for visualizing the anatomy of the interatrial septum because it pro-

vides instantaneous (live) images of almost the entire interatrial sep-

tum. Although full-volume 3D imaging provides a wider view of

the interatrial septum than the 3D zoom, it is not instantaneous and

often suffers from stitching artifacts (misalignment of image slices

obtained in separate cardiac cycles). Narrow-angle live 3D imaging

is used extensively during percutaneous atrial septal closure proce-

dures to visualize the tips and trajectories of various catheters and

devices used in closing defects.4

With reference to the manipulation of the 3D image on the ultra-

sound system, the word ‘‘tilt’’ is used in this report to refer to image

movements around either the horizontal or the vertical axis of the im-

age and the word ‘‘rotation’’ to refer to imagemovements in the z axis.

Because the raw 3DTEE images are often nonintuitive, a standard-

ized approach for image acquisition and display of the interatrial sep-

tum and ASDs is needed. In this article, we propose two simple

maneuvers for rapid orientation of 3D TEE images of the interatrial

septum in proper anatomic orientations.

RESULTS

Among 706 3D TEE studies performed, we identified 23 patients

with ASDs (3.3% of the study population). There were 18 patients

with secundum ASDs, two with primum ASDs, and three with sinus

venosus ASDs of the superior vena cava (SVC). After a trial-and-error

optimization, we developed a simple protocol for the placement of

3DTEE images of the interatrial septum in anatomically correct orien-

tation. We then used this protocol for imaging a variety of ASDs.

Protocol Development

Preparations for 3DTEE imaging start with the acquisition of good 2D

TEE images of the interatrial septum. In principle, the interatrial sep-

tum can be imaged at any 2D angle. Image acquisition at 0� and

90� is described first. Then the impact of image acquisition at interme-

diate 2D angles on the subsequent 3D TEE images is discussed. All

image acquisition described in this article were performed on 3D

TEE systems from Philips Medical Systems. However, the general

principles should apply to 3D TEE systems that are being developed

by other manufacturers.

Image Acquisition at 0� (Tilt-Up-Then-Left [TUPLE]

Maneuver). After obtaining a good midesophageal view of the in-

teratrial septum at 0� in 2D mode, the 3D zoom mode is selected.

Initially, a pair of biplane images appears on the screen. The left image

represents the lateral (azimuth or red) plane, while the right image

represents the elevation (green) plane. In each image, there is a region-

of-interest selection box; the user can select the sector width in azi-

muth or elevation planes by changing the left and right borders of

the box using the track ball. Additionally, by moving the selection

box up and down the screen, the user determines which portion of

the depth (blue) plane will be displayed in the subsequent 3D view.

After the region of interest is selected, the 3D zoom view is

obtained. The image of the interatrial septum appears in the same ori-

entation as the 2D image at 0� (Figure 1A); that is, the interatrial sep-

tum is seen in its long axis rather than en face. We refer to this initial

image as the ‘‘opening scene.’’

In the next step, the image is manipulated using to obtain the en

face view of the interatrial septum from either the left or the right

atrial perspective. This view, which is unobtainable in real time by

any imaging technique other than 3D echocardiography, demon-

strates the interatrial septum the way an anatomist or a surgeonwould

view it.5

One way to obtain the en face view of the interatrial septum from

the opening scene 3D zoom image is to tilt the image down around its

horizontal axis. Although this maneuver indeed provides an en face

view of the interatrial septum from the left atrial perspective, it places

the superior rim of the interatrial septum at the bottom of the screen.

In essence, it creates an upside-down image of the interatrial septum

(Figure 1B).

Instead, we propose the TUPLE maneuver to provide more intui-

tive en face images of the interatrial septum. In this maneuver, the ini-

tial 3D image of the interatrial septum seen in Figure 1A is first tilted

up along its horizontal axis to reveal an en face view of the interatrial

septum from the right atrial perspective. The superior portion of the

interatrial septum is now on the top of the screen and the anterior por-

tion on the right side of the screen (Figure 1C).

Abbreviations

ASD = Atrial septal defect

IVC = Inferior vena cava

ROLZ = Rotate-left-in-z-axis

SVC = Superior vena cava

TEE = Transesophagealechocardiographic

3D = Three-dimensional

TUPLE = Tilt-up-then-left

2D = Two-dimensional

2 Saric et al Journal of the American Society of Echocardiography

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The SVC and the ascending aorta are themost important landmark

in this view; by identifying and properly orienting these two vessels,

one ensures proper orientation of the interatrial septum. The SVC

is at the top of the screen, and the aortic valve and the ascending aorta

are on the right side of the screen. The fossa ovalis, located in the mid-

dle of the image, is another important landmark, because its visualiza-

tion is essential for guiding transseptal puncture during various

percutaneous cardiac interventions.

In the next step, the image of the interatrial septum is tilted to the

left around the vertical axis by approximately 180� until the en face

view of the interatrial septum from the left atrial perspective is

obtained. In this view, the superior rim of the interatrial septum

remains at the top of the screen; the anterior (aortic) rim is now on

the left side and the ostia of the right-sided pulmonary veins on the

right side of the monitor (Figure 1D). The imaging of a normal intera-

trial septum using the TUPLE maneuver is illustrated in Video 1.

Image Acquisition at 90� (TUPLE Plus Rotate-Left-in-z-Axis

[ROLZ] Maneuver). Using the same principle of initial imaging (se-

lection of region of interest in the biplane midesophageal view), the

opening scene 3D zoom image is obtained. Again, this initial 3D im-

age has the same orientation as the equivalent 2D image, which rep-

resents the bicaval view.

In the first step, the image is tilted up to reveal the right atrial side of

the interatrial septum. In comparison with the image obtained at 0�,

the SVC now appears on the right side of the screen. In effect, chang-

ing the 2D angle at time of image acquisition leads to 3D zoom image

rotation in the z axis. Therefore, to orient this 3D image to the proper

anatomic position, one must rotate the image to the left (counter-

clockwise) by 90� in the z axis.

Once the image of interatrial septum from the right atrial perspec-

tive properly oriented, it is tilted to the left around its vertical axis to

obtain the view of the interatrial septum from the left atrial perspec-

tive. We refer to this manipulation of the image as the TUPLE-plus-

ROLZ maneuver. It is illustrated in Video 2.

Imaging at Intermediate Angles. As a general rule, the larger the

2D angle used at the time of image acquisition, the more rotation of

the 3D image in the counterclockwise direction in the z axis will be

needed. Images that were acquired at 0� in two dimensions will re-

quire no rotation in the z axis of the 3D images, images acquired at

75� will require a 75� counterclockwise rotation in the z axis, images

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Figure 1 Imaging of a normal interatrial septum at 0�. (A) The initial image (opening scene) of the interatrial septum acquired at 0�. Theasterisk denotes the left atrial aspect of the interatrial septum. (B) The result of tilting the initial image down to fully reveal the left atrialaspect of the interatrial septum. Such a maneuver places the cranial portion of the interatrial septum counterintuitively to the bottomof the screen. (C) The first step of the TUPLEmaneuver, namely, tilting up of the image to reveal the right atrial aspect of the interatrialseptum. (D) The second step of the TUPLE maneuver, namely, tilting of the image along its vertical axis to reveal the left atrial aspectof the interatrial septum in proper anatomic orientation. RUPV, Right upper pulmonary vein.

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Figure 2 Imaging at intermediate angles. The impact of various acquisition angles on the 3D images of the interatrial septum is dem-onstrated. Each image demonstrates the right atrial aspect of the interatrial septum. Note that as the angle of image acquisition in-creases, the position of the SVC rotates progressively in the clockwise rotation.

Journal of the American Society of Echocardiography

Volume- Number-

Saric et al 3

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acquired at 100� will require a 100� counterclockwise rotation in the z

axis, and so forth. The impact of image acquisition at various 2D an-

gles is shown in Figure 2.

The TUPLE and ROLZmaneuvers were successful in obtaining di-

agnostic images in all 23 patients with ASDs. On average, the TUPLE

maneuver with or without the ROLZ maneuver takes 1 to 2 minutes

to perform.

3D TEE Imaging of Secundum ASDs

In our series, there were 18 patients with secundum ASDs, 14 of

whom were women (72%; all secundum ASDs). Using the TUPLE

and ROLZmaneuvers, one places a secundumASD in the proper an-

atomic orientation. Figure 3 and Video 3 demonstrate imaging of a se-

cundum ASD using the TUPLE maneuver and a 0� acquisition angle.

Complete resorption of the septum primum over the fossa ovalis

leads to the classic appearance of the secundum ASD (Video 4).

Three-dimensional TEE imaging clearly demonstrates that these

ASDs are frequently not perfectly circular, as has been commonly as-

sumed in 2D echocardiography, but often oval or irregular in shape.

In addition, what is assumed to be an ASD’s diameter on 2D TEE im-

aging is often a geometric chord rather than the true ASD diameter.

(A geometric chord is a line that connects two points of a circle but

does not pass the circle’s center, as its diameter does.)

With 3D TEE imaging, one can also appreciate that the size of an

ASD varies throughout the cardiac cycle, being maximal during ven-

tricular systole and minimal during atrial systole.6 In our laboratory,

we report the size of the ASD at the time of its maximal diameters.

The precise measurement of ASD size helps avoid inappropriate pa-

tient selection for percutaneous ASD closure.

A properly oriented 3D TEE image of a secundum ASD allows for

evaluation of the size of the ASD tissue rims and their relationship to

the aortic valve and the ascending aorta (Figure 4). Knowledge of the

defect size and the size of tissue rims is of utmost importance for per-

cutaneous device closure of secundum ASDs. Percutaneous closure

of a secundum ASD is usually considered feasible is the largest

ASD diameter is <38 mm, its aortic rim is >3 mm, and other rims

are >7 mm.7 Using the TUPLE and ROLZ maneuvers, the location

and the size of the each rim in general and the aortic rim in particular

can usually be determined.

At present, no measurements of ASD size can be obtained directly

from 3D TEE images. Instead, the size of an ASD can be determined

(1) semiquantitatively by superimposing a rectangular grid of known

dimensions over the 3D TEE ASD image (Figure 5A) and (2) quanti-

tatively by tracing the outlines and diameters of the ASD using offline

software for multiplanar reconstruction.

When there is only partial resorption of the septum primum over

the fossa ovalis, secundum ASDs appear fenestrated. In such ASDs,

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Figure 3 Imaging of a secundumASDat 0�. The TUPLEmaneuver, described in Figure 2, is here applied to the imaging of a secundumASD. (A) The initial 3D TEE image (opening scene). (B) The right atrial aspect of the ASD. (C) The left atrial aspect of the ASD.

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Figure 4 Rims of a secundum ASD. (A) The right atrial aspect and (B) the left atrial aspect of a secundum ASD (asterisk). The dottedline follows the aortic rim and the solid line follows other rims of the secundum ASD. The size of the aortic rim (arrow) is of utmostimportance in percutaneous ASD closure. AV, Aortic valve.

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there are bands of tissue (remnants of the septum primum) traversing

the fossa ovalis.5 Although the anatomy of such bands is difficult to

conceptualize on 2D echocardiography, their appearance is readily

ascertained by 3D TEE imaging (Figure 5).

Proper orientation of the 3D TEE images of an ASD, such as

through the TUPLE and ROLZ maneuvers, is also essential for guid-

ing the percutaneous device closure of an ASD (Figure 6).

After the septal defect is sized and deemed amenable to percuta-

neous repair, a collapsed closure device is deployed via the guiding

catheter. Three-dimensional TEE imaging allows for continuous visu-

alization of the intracardiac portions of the guiding catheters, balloons,

and closure devices.8

Once the closure device has been expanded and deployed, 3D

TEE imaging is used to ascertain its proper positioning. Both the left

atrial and the right atrial plate of the device are easily visualized. In ad-

dition, 3D TEE imaging helps determine if sufficient tissue rim is

caught between the two plates of the device. In case of device malpo-

sitioning, 3D TEE imaging can be used to guide repositioning of the

device (Figures 6C and 6D). After the device is fully deployed, 3D

TEE color Doppler imaging is used to verify that no significant residual

blood flow around the device is present.

3D TEE Imaging of Primum ASDs

In general, some 15% of all ASDs are in the region of the ostium pri-

mum, located adjacent to the interventricular septum. They are

termed primum defects and account for about 15% of all ASDs.

PrimumASDs are usually part of the endocardial cushion defect spec-

trum, ranging from isolated primum ASDs to complete atrioventricu-

lar canal defects. In our series, two patients had primum ASD (1 man,

1 woman).

Using the TUPLE and ROLZ maneuvers, a primum ASD is visual-

ized as an often ovoid communication between the two atria adjacent

to the atrioventricular valves. The relationship between a primum

ASD and the surrounding structures, such as the mitral and tricuspid

valves, is easily discernible from 3D TEE images (Figure 7, Video 5).

Frequently, primum ASDs are associated with a cleft anterior

mitral leaflet and the attendant eccentric mitral regurgitation. The

anatomy of a cleft leaflet can easily be visualized by 3D TEE imaging

from both the atrial and ventricular perspectives (Figures 7C and 7D,

Video 6).

3D TEE Imaging of Sinus Venosus ASDs

When there is a communication between the sinus venosus portion of

the right atrium (which is adjacent to the venae cavae) and the left

atrium, the defect is called a sinus venosus ASD. It is more common

to observe such a defect adjacent to the ostium of the SVC than the

inferior vena cava (IVC). Both sinus venosus ASD types are usually

associated with partial anomalous pulmonary venous return.

In an SVC-type sinus venosus ASD, the right upper and the right

middle pulmonary vein commonly drain into the SVC instead of

the left atrium. In an IVC-type sinus venosus ASD, commonly the

lower right pulmonary vein drains into the IVC. Because of the char-

acteristic appearance of the course of this anomalous pulmonary vein

on chest x-rays, the condition is referred to as the scimitar syndrome.

In general, sinus venous ASDs account for 5% to 10% of all ASDs. In

our series, three patients had primum ASDs (all men).

print&

web4C=FPO

Figure 5 Fenestrated secundum ASDs. Three-dimensional TEE images of tissue bands (arrow) stretched across secundum ASDs(fenestrated ASDs) from four different patients. (A–C) Three-dimensional TEE images of ASDs from the left atrial perspective. (D)Two ASDs (asterisks) separated by a band of tissues as viewed from the right atrial perspective.

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Using the TUPLE and ROLZ maneuvers, a sinus venosus ASD is

first placed in the correct anatomic orientation and visualized both

from the right and left atrial perspectives (Figures 8A and 8B).

Video 7 demonstrate an SVC-type sinus venosus ASD with an irreg-

ularly shaped orifice between the left atrium and the cranial portion of

the right atrium adjacent to the orifice of the SVC.

Once the defect is visualized from the right atrial perspective, the

image is rotated along its vertical axis using the track ball to reveal

the ostia of the right upper and the right middle pulmonary vein

anomalously draining into the terminal portion of the SVC (Figures

8C and 8D, Video 8).

DISCUSSION

ASDs usually develop through three mechanisms: (1) excessive re-

sorption of an interatrial septal membrane that covers an ostium

(e.g., partial or complete resorption of the septum primum in the re-

gion of the foramen ovale leading to ostium secundum ASDs), (2)

persistence of an ostium that normally closes (e.g., ostium primum

ASD), and (3) abnormal development (such as sinus venosus ASDs).

The two major objectives of this study were (1) to develop a stan-

dardized approach that all health care providers involved in image ac-

quisition, interpretation, and clinical use can follow and (2) to place

the 3D TEE images in an anatomically correct orientation. The proto-

col we propose takes only minutes to complete. Although there may

be more rapid methods, any loss of time in orienting 3D TEE images

using our method is rewarded by a standardized, anatomically correct

image orientation that facilitates interaction between echocardiog-

raphers, interventionalists, and cardiac surgeons. We believe that

with a meticulous attention to detail, one can obtain 3D TEE images

of the interatrial septum using the proposed technique.

In our laboratory, we have tried various approaches to orienting

3D TEE images of the interatrial septum and found out that the tilt-

up approach from the opening scene provides on average more ana-

tomic landmarks (the SVC, aorta, and even IVC and coronary sinus)

than the tilt-down method (whose major anatomic landmarks are the

fossa ovalis and occasionally the right upper pulmonary vein). In ad-

dition, the SVC with a clear distinction between its longitudinal and

short axes appears to provide more orienting information that the

much smaller and circular fossa ovalis.

Three-dimensional TEE imaging provides excellent real-time

images of the interatrial septum and the atrial septum defects. It pro-

vides an en face view of the septum not obtainable by any other

imaging modality in real time. For proper anatomic orientation of

3D TEE images of the interatrial septum and the atrial septum, we

propose the TUPLE and ROLZ maneuvers. We hope that standard-

ized maneuvers such TUPLE and ROLZ described in this article

will enhance the diagnosis of ASDs and facilitate communication

between echocardiographers obtaining 3D TEE images and interven-

tional cardiologists or cardiac surgeons performing ASD closures.

Limitations

Current 3DTEE systems suffer from several limitations. Occasionally,

there is a dropout in the region of the fossa ovalis in both normal

print&

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Figure 6 Percutaneous closure of a secundum ASD. (A) The left atrial aspect of the interatrial septum after the TUPLE maneuver. Acatheter is being delivered from the IVC through the secundum ASD (asterisk) into the left atrium. (B) Cross-section of the interatrialseptum. An Amplatzer ASD occluder (AGA Medical, Plymouth, MN) is being delivered to close the secundum ASD; the device is stillattached to the delivery catheter (arrow). (C) The left atrial disk (LA) of an Amplatzer ASD occluder. Insufficient capture of the posteriorASD rim is noted (arrow). (D) The left atrial disk after repositioning; now all the ASD rims are fully captured. RA, Right atrial disk of theASD occluder; RUPV, right upper pulmonary vein.

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subjects and patients with ASDs. In normal subjects, the dropout cre-

ates the false appearance of an ASD. In patients with ASDs, the appar-

ent ASD size may appear larger than it is in reality. The dropouts can

be minimized by adequate gain controls and by the use of color

Doppler (which demonstrates color flow through real septal defects

but not through the dropouts). In addition, because the IVC enters

the right atrium at different angles in different subjects, the inferior

rim is sometimes hard to visualize and may represent a ‘‘blind spot.’’

REFERENCES

1. Houck RC, Cooke JE, Gill EA. Live 3D echocardiography: a replacement for

traditional 2Dechocardiography?AJRAm J Roentgenol 2006;187:1092-106.

2. Salcedo EE, Quaife RA, Seres T, Carroll JD. A framework for systematic

characterization of the mitral valve by real-time three-dimensional transeso-

phageal echocardiography. J Am Soc Echocardiogr 2009;22:1087-99.

3. Sugeng L, Shernan SK, Salgo IS, Weinert L, Shook D, Raman J, et al. Live

3-dimensional transesophageal echocardiography initial experience using

the fully-sampled matrix array probe. J Am Coll Cardiol 2008;52:446-9.

4. Perk G, Ruiz C, Saric M, Kronzon I. Real-time three-dimensional transeso-

phageal echocardiography in transcutaneous, catheter-based procedures

for repair of structural heart diseases. Curr Cardiovasc Imaging Rep

2009;2:363-74.

5. McCarthy KP, Ho SY, Anderson RH. Defining the morphologic pheno-

types of atrial septal defects and interatrial communications. Images Pae-

diatr Cardiol 2003;15:1-24.

6. Acar P, Saliba Z, Bonhoeffer P, Aggoun Y, Bonnet D, Sidi D, et al. In-

fluence of atrial septal defect anatomy in patient selection and assess-

ment of closure with the Cardioseal device; a three-dimensional

transoesophageal echocardiographic reconstruction. Eur Heart J 2000;

21:573-81.

7. Misra M, Sadiq A, Namboodiri N, Karunakaran J. The ‘‘aortic rim’’ recount:

embolization of interatrial septal occluder into the main pulmonary artery

bifurcation after atrial septal defect closure. Interact Cardiovasc Thorac

Surg 2007;6:384-6.

8. Taniguchi M, Akagi T, Watanabe N, Okamoto Y, Nakagawa K, Kijima Y,

et al. Application of real-time three-dimensional transesophageal echocardi-

ography using a matrix array probe for transcatheter closure of atrial septal

defect. J Am Soc Echocardiogr 2009;22:1114-20. Q1

print&web4C=F

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Figure 7 Primum ASD. (A) The left atrial perspective of a primum ASD (asterisk) on a full-volume 3D TEE image. (B) A left-to-rightshunt across the primum ASD (arrow) on a 2D color Doppler TEE image. (C) Three-dimensional zoom TEE image showing the leftatrial aspect of themitral valve (MV) having a cleft anterior leaflet (white arrow). The black arrowheadmarks the location of the primumASD. (D) Three-dimensional zoom TEE image showing the left ventricular aspect of the MV having a cleft anterior leaflet (arrow). AV,Aortic valve; LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

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print&web4C=F

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Figure 8 SVC-type sinus venosus ASD on 3D TEE imaging. (A) The right atrial aspect and (B) the left atrial (LA) aspect of an SVC-typesinus venosus ASD (asterisk) visualized by the 3D zoom technique. (C,D) Full-volume 3D TEE images of another patient with an SVC-type sinus venosus ASD (asterisk). (C) The right atrial aspect of the defect (asterisk). (D) The LA aspect of the defect (asterisk). The rightupper pulmonary vein (RUPV) and right middle pulmonary vein (RMPV) drain anomalously into the SVC. AV, Aortic valve; FO, fossaovalis.

Video 1 Imaging of the normal interatrial septum using the TU-PLE maneuver.

Video 2 Imaging of the normal interatrial septum using the TU-PLE and ROLZmaneuvers. This videowas postprocessed usinga source clip kindly provided by Dr. Robert Donnino of the Man-hattan Campus of the Veterans Administration New York HarborHealthcare System (New York, NY).

Video 3 Imaging of a secundum ASD using the TUPLE maneu-ver.

Video 4 Details of a secundum ASD.

Video 5 Primum ASD.

Video 6 Cleft mitral valve and primum ASD.

Video 7 SVC-type sinus venosus ASD.

Video 8 Partial anomalous pulmonary venous return in a sinusvenosus ASD.

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