Echocardiography Handbook

Echocardiography Laboratory The Labatt Family Heart Center Sickkids Toronto

BASIC ULTRASOUND PHYSICS

Introduction
A basic understanding of the principles of ultrasound imaging is essential during data acquisition and for correct interpretation of the echocardiographic studies. Although, at times current instruments provide instantaneous images so clear and detailed that it seems as if we can directly see the heart and blood flow, in reality we always are looking at images and flow data reconstructed based on ultrasound waves reflected and backscattered from the patients body. Knowledge of the basic concepts of and especially the strengths and limitations of the different techniques used in clinical echocardiography is crucial for the correct interpretation of the images and measurements. A. What is ultrasound? Ultrasonic waves are defined to be longitudinal compression waves with a frequency above 20 kHz, as 20 kHz is the highest frequency which can be detected by the human ear. Requires a medium through which to travel (e.g. air, water, soft tissue) An acoustic wave causes particles to be displaced from an equilibrium position while the wave is traveling through the medium, causing local compression and rarefaction influenced by the elasticity and density of the medium. Ultrasound waves are generated and detected by a piezoelectric crystal which deforms under the influence of an electrical field. Physical properties of sound waves: Velocity Frequency Wavelength Amplitude

From Stoylen 2006. This shows how sound waves are transmitted through a medium by compression and rarefaction. The actual waveform is sinusoidal. Velocity The speed with which a sound wave travels through a medium Units of measure are distance/time m/sec The speed of sound is determined by the density and stiffness of the media in which it travels. Stiffness is the hardness or resistance of the material to compression. Density is the concentration of the matter. Increase in stiffness, increases speed, increase in density, decreases speed slow in air/gasses fast in solid media The speed of ultrasound transmission in the body/soft tissue is relatively constant and is about 1540 m/sec

Frequency (f) The number of cycles occurring in one second of time (cycles per second)

Units to describe frequency: Hertz = 1 cycle in one second Kilohertz (kHz) = 1,000 cycles per second (or 1,000 Hertz) Megahertz (MHz) = 1,000,000 cycles per second (or 1,000,000 Hertz) ultrasound imaging frequency range is 2-12 MHz Wavelength Length of space over which one cycle occurs (in distance) expressed in millimeter (mm) or meter (m)

Amplitude The strength/intensity/loudness of the soundwave at any given point in time Represented by the height of the wave Amplitude/intensity decreases with increasing depth Amplitude of an ultrasound wave is describe in terms of decibels

Relationships between physical properties The velocity or transmission, the frequency and wavelength are related by the formula c= f x where c= speed of sound through the medium, f = the frequency of the wave the wavelength. The frequencies used in clinical imaging vary between 2-12 mHz resulting in wavelengths 0.8-0.13 mm. These are intrinsic limitation for spatial resolution as two structures need to be separated by one wavelength. Interaction of ultrasound with tissue When an ultrasound wave is transmitted through a medium, different interactions occur. Reflection Refraction Scattering Attenuation

Reflection Reflection occurs at a boundary/interface between two media having a different acoustic density. The difference in acoustic impedance (z) between the two tissues causes reflection of the sound wave in the direction of the transducer. Acoustic impedance is dependent on the density and velocity

z = density x velocity The larger the difference in acoustic impedance between two adjacent tissues, the greater the reflection Reflection from a smooth tissue interface (specular) causes the sound wave to return to the transducer. Irregular interfaces will cause scatter in different directions. The ultrasound image is created based on the reflected waves.

Refraction The ultrasound wave which passes through the tissue is refracted based on the incidence of the beam. The change of sound direction when sound passes from one medium to another and occurs when there is oblique incidence and there is different propagation speed of two media Oblique incidence happens when the sound beam direction is not 90 degrees to the boundary of two media. This same phenomenon explains why a straight pencil that sits in a glass of water appears to have a bend in it.

Scattering Redirection of the ultrasound wave in several directions. Caused by interaction between the ultrasound beam and the irregular shape of the interface.

Scattering also does occur due to the spatial fluctuations in acoustic properties of a medium

Attenuation It is the loss of sound energy as sound propagates through a medium due to absorption of the ultrasound energy by conversion to heat, as well as by reflection and scattering. The deeper the wave travels in the body, the weaker it becomes. The amplitude/strength of the wave decreases with increasing depth. Overall attenuation is frequency dependent, such that lower ultrasound frequencies penetrate deeper into the body than higher frequencies. Lower frequency probes have better penetration but as mentioned above have lower spatial resolution. Higher frequency probes have lower penetration but higher spatial resolution. Attenuation also depends on acoustic impedance and on mismatch in impedance between adjacent structures. Since air has very high acoustic impedance, any air between the transducer and the cardiac structures of interest results in substantial signal attenuation. This is avoided on transthoracic examinations by use of water-soluble gel to form an airless contact between the transducer and the skin. The air-filled lungs are avoided by careful patient positioning and the use of acoustic windows that allow access of the ultrasound beam to the cardiac structures without intervening lung tissue. Attenuation causes decrease in amplitude as the wave passes through the tissue. This is corrected for by automatic attenuation compensation in most ultrasound systems. However, further manual depth gain or time gain compensation can be achieved by controls on the machine (sliders which correct for the automated attenuation correction at a specific image depth).

B. The Physical Principles of Ultrasound Imaging Ultrasound imaging is based on the pulse-echo principle in which a short burst of ultrasound is emitted from a transducer and directed into tissue. The pulse is partly reflected from a boundary between two tissue structures, and partially transmitted. The time difference, , between emitting and receiving a pulse is the time it takes for sound to travel the distance to the scatterer and back, i.e. twice the range, r, to the scatterer at the speed of sound, c, in the tissue. Thus:

The pulse is thus emitted, and the system is set to await the reflected signals, calculating the depth of the scatterer on the basis of the time from emission to reception of the signal. The total time for awaiting the reflected ultrasound is determined by the preset depth desired in the image.

The received energy at a certain time, i.e. from a certain depth, can be displayed as energy amplitude, A-mode. It gives information on the structures in front of the transducer and represents one line of the ultrasound image. The amplitude can also be displayed as the brightness of the certain point representing the scatterer, in a B-mode plot. And if some of the scatterers are moving, the motion curve can be traced by letting the B-mode image sweep across a screen or paper. This is called the M-mode (Motion). In a typical M-mode display the pulses are repeated about a 1000 time/sec or more resulting in a very high temporal resolution. A 2-dimensional image is built up by firing a beam vertically, waiting for the return echoes, maintaining the information and then firing a new line from a neighboring transducer along a neighboring line in a sequence of B-mode lines. This obviously is done at a lower temporal resolution determined by the frame rate.

C. Image quality
This refers to the resolution of an imaging system. Two important aspects are the temporal and spatial resolution of an imaging system. Spatial resolution refers to the capacity of a system to resolve small structures. It can be considered as the smallest distance by which the system is capable of identifying two dots as separate dots instead of individual ones. Contrast resolution is the ability of the system to distinguish differences in soft tissue density. Temporal resolution refers to the capacity of the system to resolve time differences. Finally there are many potential sources for artifacts in ultrasound images. Spatial resolution This can be defined as the combination of axial and lateral resolution. Axial Resolution - The capacity of the ultrasound system to distinguish how close together two objects can be along the axis of the beam and still be distinguished as two separate objects (depth resolution) - Wavelength affects axial resolution and it is improved by increasing the frequency - Axial resolution of the systems is much better compared to lateral resolution as it is not dependent on the beam formation

Lateral Resolution The capacity of the ultrasound system to resolve two adjacent objects that are perpendicular to the beam axis as separate objects. Beam width affects lateral resolution. The wider the beam the lower the lateral resolution. This is influenced by the focal zone which is the depth where you have the smallest beam width. The near field is the zone between the transducer and the focal zone, the far field is the region beyond the focal zone. Optimizing the focus at a certain depth optimizes lateral resolution. The limit using the focal zone is determined by transducer size and frequency of the transducer. Small transducers focus well in the near field, large transducers better in the far field Beam width is also influenced by frequency of the transducer with higher frequency probes having a better lateral resolution compared to low frequency probes. The higher frequency has however limited penetration into the tissue. Line density can be improved by decreasing the sector width resulting in better lateral resolution Optimizing lateral resolution is achieved by optimizing frequency selection, focal zone settings and sector width.

Temporal Resolution To image moving objects, structures such as blood and heart, the frame rate is important, related to the motion speed of the object. The eye generally can only see 25 FPS (video frame rate), giving a temporal resolution of about 40 ms. The temporal resolution is limited by the sweep speed of the beam. And the sweep speed is limited by the speed of sound, as the echo from the deepest part of the image has to return before the next pulse is sent out ad a different angle in the neighboring beam. The sweep speed can be increased by reducing the number of beams/increasing the beam width in the sector (frame rate control knob), or by decreasing the sector angle (width of the sector). The first option decreases the lateral resolution, the second decreases the image field, thus basically temporal resolution cannot be increased without a trade off, due to the physical limitations of echocardiography. Modern systems are capable of manipulating the frame

rates by sending out different pulses at different times but in general increasing frame rate will reduce lateral resolution. Ultrasound imaging artifacts There are many potential sources for artifacts in echocardiography. For interpretation of the images it is important to know and recognize them. 1. Drop-out of parallel structures. When structures are parallel to the ultrasound beam, there is very little reflection caused by the structure, resulting in drop-out. Typical example is the atrial septum when viewed from the apex (image) 2. Acoustic shadowing. Transmission of the ultrasound beam through the tissue is influenced by the presence of tissue with very high density. Typical examples are prosthetic valves, devices, catheters, calcifications. These structures can make it impossible to view structures behind them. 3. Reverberations can occur with lateral spread of high-intensity echos. Bright echos can have considerable width. A lot of reverberations originate from the interaction between the transducer and the ribs. 4. Mirror imaging. Appears as a display of two images, one real and one artifact, due to the sound beam interacting with a strong reflector 5. Ring down/Comet tail: ring down artifact comes from gas bubble in a fluid medium. Comet tail originates from highly reflective structures such as surgical clip or a bullet The use of harmonic imaging in children As an ultrasound wave, travels through tissue it gets distorted and harmonic frequencies are generated. The further the sound wave travels through tissue, the more harmonics are generated. An ultrasound system can take advantage of this phenomenon by transmitting in the fundamental frequency and receiving in the second harmonics, filtering out the fundamental frequencies. Based on this principle harmonic imaging has certain advantages: can be used as a kind of depth compensation boosting signals from deeper structures. The relative advantages of harmonics are therefore more limited in infants where the cardiac structures are more in the near field. For deeper structures also in infants it can have advantages. filters out near field artifacts, especially reverberations from interaction between transducer and ribs. Penetration in harmonics is less compared to fundamental imaging.

Disadvantages of harmonics are that the way the filtering is done makes the cardiac structures look thicker. Especially valve leaflets can look much thicker when using harmonics.

Image optimization in children 1. always use the highest possible transducer frequency to optimize spatial resolution. Especially for infants and to image structures in the near field (like RVOT) 2. routinely use harmonics in large children. In infants and small children harmonic imaging might not be requires and result in decreased image quality. 3. adapt depth settings at the minimum possible to include the region of interest 4. adapt overall gain settings and time-gain-compensation to get optimally image near field and far field structures. On the GE and Philips machine (I scan) there is a tool for automatic contrast optimization. This can sometimes be helpful. 5. use the narrowest sector width necessary to image the structure you are interested in to optimize resolution 6. adapt focus settings to the depth of the structure of interest

IMAGING MODES USED IN ECHOCARDIOGRAPHY

M-Mode 2-D Imaging Doppler Color Doppler

M-Mode - The M-mode is the oldest modality to display moving structures from the heart. - It is a graphic representation to measure the movement and size of the cardiac structures - It is generated from 2-D ultrasound with the imaging plane used to guide the placement of the M-Mode ultrasound beam - M-Mode of any cardiac structure can be obtained from any cardiac plane simply by obtaining a 2-D image and positioning a cursor line on the image through the structure to be interrogated. - The M-mode echocardiograms were measured from the leading edge of the anterior echo to the leading edge of the posterior echo, whereas measurements obtained from the two-dimensional echocardiogram directly are made from the tissue-blood pool interface (black-white interface).

2D Imaging In 2-D imaging different standard views can be obtained. These views will be further illustrated in the HSC standard protocol. Standard 2-D views: 1. Parasternal Views (Long & Short Axis Views). The transducer is placed in a region adjacent to the sternum in the second, third or fourth left intercostal space with the patient in the left lateral position. From this position, sector images can be obtained of the heart along its long and short axes. Long axis view of the left ventricle The long axis view of the LV is obtained with the transducer groove facing toward the patients right flank and the transducer positioned in the third or fourth left intercostal space so that the ultrasound beam is parallel with the line joining the right shoulder to the left flank. The long axis view of the LV is displayed as a sagittal section of the heart viewed from the left side of a supine patient. The long axis view of the LV allows the visualization of the aortic root and aortic valve leaflets. The chamber behind the aortic root is the left atrial (LA) cavity. The long axis view allows good visualization of the posterior and anterior leaflets of the mitral valve and their chordal and papillary muscle attachments. \

Long axis view of RV inflow With the transducer in the same intercostal space (third or fourth) and with an inferomedial tilt (towards the patients right hip), a long axis view of the RV and RA and TV is obtained. The image orientation of this view is such that the chest wall is anterior. These views allow visualization of the RA cavity, the tricuspid valve (anterior and septal leaflet) and the RV inflow up to the apex of the RV.

Long axis view of the RV outflow When the transducer is angled anteriorly from the standard parasternal long axis view towards the patients left shoulder, a parasternal long axis view through the right ventricular outflow is obtained. As the sweep is performed, the plane of sound passes way from the aortic root and sub-aortic outlet septum and through the area of the sub-pulmonic outlet septum, the outflow or the infundibular septum of the RV, the pulmonary valve and the main pulmonary artery.

Short axis views The parasternal short axis views are obtained by rotating the transducer 90 degrees clockwise from the parasternal long axis view. The groove on the transducer is pointed superiorly facing the right supraclavicular fossa, and the beam is roughly parallel with a line joining the left shoulder to the right flank. A number of short axis planes can be generated by tilting the transducer from the base of heart superiorly to the cardiac apex inferiorly. a. At the base of the heart, the right ventricular anterior wall and a portion of the right ventricular outflow tract are seen in the anterior portion of the sector scan. The tricuspid valve leaflets are seen in this view (anterior and septal leaflets). The aortic valve is seen in the center of the sector scan which appears as a circle with trileaflet aortic valve that has the appearance of a letter Y during diastole. The pulmonary valve and the proximal portion of the main pulmonary artery can be seen to the left and anterior of the aortic valve.

b. With a slight cranial and leftward angulation of the transducer from the standard parasternal short axis view at the base of the heart the distal

pulmonary artery branches can be visualized. This view can be obtained more easily if the transducer is repositioned one intercostal space higher.

c. With the transducer pointed directly more inferiorly, a cross section is obtained of the LV at the level of the MV leaflets. In this view, the mitral anterior and posterior leaflets are seen which look like a fish mouth during diastole.

d. By further inferior tilting of the transducer a cross section at the level of the papillary muscles is obtained. The two papillary muscles (the anterolateral and posteromedial), project into the LV cavity at approximately 3 and 8 oclock positions respectively.

e. From this position, the transducer is tilted inferiorly towards the LV apex, so that a transverse section of the LV apex is obtained.

2. The Apical views This view is best obtained with the patient turned in the left lateral decubitus position so that the cardiac apex is closer to the chest wall and the left lung falls downward away from the heart. The apical impulse is localized and the transducer is placed at or in immediate vicinity of the point of maximal impulse. The Apical Four Chamber View The standard apical four chamber view is obtained by orienting the plane of sound in a nearly coronal body plane through both ventricles and both atria. If the transducer is correctly aligned, the apex of the left ventricle is seen in the apex of the fan or sector scan. All four cardiac chambers, mitral and tricuspid valves, and both atrial and ventricular septa are visualized. Because all four cardiac chambers and both atrial and ventricular septa are visualized simultaneously in the apical four chamber view, this view is especially useful for determining atrial situs and the atrio-ventricular connections and determining the morphology of the four cardiac chambers.

With posterior angulation of the transducer in apical four chamber view, the TV and MV leaflets are no longer imaged. Instead, the plane of sound passes through the coronary sinus as it courses from left to right on the posterior surface of the heart in the atrio-ventricular groove.

The Apical Five Chamber View With anterior angulation of the transducer from apical four chamber view, the aortic root can be seen arising from the left ventricle. The left ventricular outflow tract, aortic valve and a portion of the ascending aorta come into view. In some patients, it is possible to tilt the transducer even more anterior without losing contact with the chest wall to visualize the right ventricular outflow tract, pulmonary valve and the anterior trabecular septum.

The Apical Two Chamber View This view is obtained by slight lateral and counterclockwise rotation of the transducer from the standard four chamber view where the left atrium, mitral valve, and left ventricle are visualized.

The Apical Long Axis View (three chamber view) This view is obtained by rotating the transducer 90 degrees counterclockwise from the apical four chamber view. The plane of sound passes through the MV, the cardiac apex, and the aortic valve and thus is aligned along the major axis of the left ventricle. Like the parasternal long axis view, the apical long axis view provides a plane for imaging the LA, anterior and posterior MV leaflets, the LV outflow tract, the right and noncoronary aortic valve leaflets and a portion of the ascending aorta.

3. The Subcostal Views The subcostal views are best obtained with the patient lying supine on the bed. In older patients flexion of the knees can reduce the tension of the abdominal muscles and increase the ease in obtaining the subcostal images. Also, in older patients in whom the heart can be quite distant from the transducer, subcostal imaging can be improved by having the patient hold their breath at deep inspiration. This maneuver lowers the diaphragm, brings the heart closer to the transducer, and improves the penetration of the sound beam.

Subcostal View of the Abdomen (Situs Evaluation) A cross-sectional view of the inferior vena cava and descending abdominal aorta can be obtained by placing the transducer in the subcostal space in a transverse body plane and tilting the transducer caudally until the vertebral column is seen in cross- section. The descending aorta is normally visualized in the left side of the spine, and the inferior vena cava is seen to the right of the spine.

The descending aorta is easily recognized by its prominent arterial pulsations. A considerable length of the descending aorta can be imaged by placing the transducer in the sagital body plane. From this sagital view of the descending abdominal aorta, the transducer can be tilted slowly towards the patients right side to visualize the inferior vena cava. The pulsation of the IVC is more subtle than the pulsation of the descending aorta and do not correspond to ventricular systole.

Subcostal coronal view (four-chamber) A family of subcostal coronal or four chamber views can be obtained by applying the transducer to the subcostal region and tilting the plane of sound from posterior to anterior. With slight anterior angulation, the standard four chamber view can be obtained. This view like the apical four chamber view is useful for identifying the morphologic characteristics of each cardiac chamber and for determining atrial situs and atrioventricular connections. The interatrial septum is visualized well in the subcostal view because it is perpendicular to the plane of sound and imaged in the direction of the axial resolution. It contains less artificial echo drop-out in the region of the thin fossa ovalis. With anterior angulation of the transducer from the subcostal four chamber view the left ventricular outflow tract can be imaged. This plane is similar to the apical five chamber view. With anterior angulation of the transducer, the LVOT is no longer seen, and the right ventricle and RVOT come into the picture. With even more anterior angulation of the transducer, the trabeculated and outflow portion of the RV, the pulmonary valve, and a portion of the main pulmonary artery are imaged.

Subcostal Short Axis Views

The subcostal short axis view is obtained by rotating the transducer 90 degrees clockwise from the subcostal four chamber view. A family of subcostal short axis can be obtained by tilting the plane of sound from right to left. a. If the plane of sound is tilted to the patients right, the superior and inferior vena cava and their junctions with the right atrium are imaged in a longitudinal section (bicaval view). The posterior portion of the interatrial septum is seen in this view.

b. With slightly more leftward angulation of the transducer, a view similar to the parasternal short axis view is obtained. In this view the aortic valve is seen in cross section and a portion of the anterior mitral valve leaflet is also seen

c. With extreme leftward angulation of the transducer, the entire right ventricular outflow tract , the pulmonary valve, the main pulmonary artery. By tilting the transducer even more leftward and slightly inferior, the view of the left ventricle at the level of the mitral valve and papillary muscles are obtained.

4. The Suprasternal Views In this view, the transducer is placed in the suprasternal notch and aligned as closely parallel as possible with the sternum. In order to gain access to the suprasternal notch, the patient is positioned supine with a pillow placed beneath the shoulders to extend the neck. Suprasternal Long Axis View To obtain this view, the transducer is placed in the suprasternal notch with the plane of sound oriented between the right nipple and left shoulder. In this view, the ascending, transverse and descending thoracic aorta are visualized. The first branch that arises from the aortic arch is the innominate artery, which bifurcates into right subclavian artery and right common carotid artery. The second and third arterial branch off the aortic arch is the left common carotid artery and the left subclavian artery. Beneath the aortic arch is the right pulmonary artery. If the plane of sound is tilted towards the patients left, the right pulmonary disappears and the left pulmonary artery come into the view.

Suprasternal Short Axis view To visualize this view, the transducer is placed in the suprasternal notch and aligned parallel to the sternum. In this view, the transverse aorta is visualized in cross section as an anterior circular structure. Superior to the transverse aorta is the left innominate vein which can be seen in longitudinal section coursing from left to right. On the right side of the transverse aorta is the left innominate vein joins the right innominate vein to form the superior vena cava. Inferior to the transverse aorta is the entire right pulmonary is seen in longitudinal section. Beneath the right pulmonary artery is the left atrium. If penetration of the sound beam is good, the pulmonary veins can be seen entering the LA.

The sidedness of the aortic arch can be determined by following the course of the innominate artery. In normal left aortic arch, the innominate artery courses to the patients right side where it bifurcates into the right common carotid and right subclavian arteries. This bifurcation can be imaged by tilting the plane of sound in the suprasternal short axis view from anterior to posterior until the first arterial branch is seen arising from the transverse aorta. In this position the transducer is gradually moved towards the patients right shoulder until the vessel bifurcation is seen. If the first arterial branch arising from the transverse aorta courses towards the patients left side, then a right aortic arch is present. If this vessel bifurcates into left common carotid and left subclavian arteries then there is a right aortic arch with mirror image branching.

Measurements performed on 2-D images. Measurements from two-dimensional echocardiography requires: (1) correct orientation of imaging planes with regards to internal landmarks (2) a clearly defined endocardial border. To maximize the accuracy of the 2D measurements, several technical factors should be considered as already discussed before. 1. Resolution. For optimal resolution, the highest frequency transducer that provides adequate penetration should be used. The focal point of the transducer should be set as close to the center of the structure being measured. 2. Gain Setting. Gain setting should be optimized so as to image clearly the endocardial border. Too much gain will cause blooming of the endocardial echoes, and too little gain will cause echocardiographic drop-out along the endocardial surface. 3. Depth setting. The depth setting of the displayed image can influence the precision of measurements. For example, if the left ventricle is imaged in a magnified presentation, then the information being analyzed contains more pixels. With more pixels there is less error in the measurement process. 4. Frame selection. Cardiac structures are usually measured at end-diastole and end-systole. The end diastolic frame is chosen as the frame showing initial coaptation of the mitral valve leaflets or the frame at the start of the Q wave in the ECG. The end systolic frame is chosen as the frame preceding initial early diastolic mitral valve opening or the frame with the smallest ventricular dimension.

Doppler Echocardiography
The Doppler effect Doppler Echocardiography measures blood flow velocities in the heart and great vessels and is based on the Doppler effect which was described by the Austrian physicist Christian Doppler in 1842. The Doppler effect states that the sound frequency increases as a sound source moves towards the observer and decreases as the source moves away. In the circulatory system, the moving target is the red blood cell or more recently myocardial motion in case of tissue Doppler.

When an ultrasound beam with a known frequency is transmitted to the heart or great vessels, it is reflected by the red blood cells. The frequency of the reflected ultrasound waves increases when the red blood cells are moving toward the source of ultrasound. Conversely, the frequency of reflected ultrasound waves decreases when the red blood cells are moving away from the source. The change in frequency between the transmitted sound and the reflected sound is termed the frequency shift. The Doppler shift depends on the transmitted frequency, the velocity of the moving target, and the angle between the ultrasound beam and the direction of the moving target as expressed in the Doppler equation: Doppler shift equation f = +/- 2 v.fi. cos( ) c Where: f =fr-fi

= the angle between the sound beam and velocity vector measured f = Doppler shift in Hz fi = incident frequency v = speed of the reflector (blood flow velocity) m/s C = velocity of sound in soft tissue fr = reflected frequency Based on this equation, velocity of motion can be measured. Not all echocardiography methods actually use the Doppler effect in the estimation of blood pool and tissue velocities. Doppler Modalities A. Continuous Wave Doppler Continuous Wave Doppler is the technique that actually uses a transducer with two crystals, one continuously emits ultrasound pulses and the other one receives the reflected frequencies. The information obtained from the Doppler shift is displayed as a real time image in a graphic form. A component of the ultrasound machine called the Fast Fourier transform analyses the reflected frequencies and displays it on a spectral analysis graph. The image shows the distribution of the detected Doppler frequencies as a function of time and represents the different velocities occurring simultaneously within the sound beam. The amplitude of each velocity is displayed as a dot of grey. The frequency is displayed above or below the baseline for flow towards the transducer or away from the transducer respectively.

Optimise settings of CW Doppler The CW image is an echo picture that is influenced by all the parameters that affect a normal 2D picture The gain control affects the ratio of the output signal strength to the input signal strength. The gain controls should be manipulated to produce a clean uniform profile without any 'blooming'. The gain controls should be turned up to over emphasize the image and then adjusted down. This will prevent any loss of information due to too little gain. The compress control assigns the varying amplitudes a certain shade of grey. If the compress control is very low or high the quality of the spectral analysis graph will be affected and this may lead to erroneous interpretation. The reject button eliminates the smaller amplitude signals that are below a certain threshold level. This will help to provide a cleaner image and may make measurements more obvious. The filter is used to reduce the noise that occurs from reflectors that are produced from walls and other structures that are within the range of the ultrasound beam. The volume button should be at the appropriate level to hear the frequencies.

Advantages and disadvantages Because the crystal does not have to wait for the returning signals, there is no limit to the velocities that can be recorded. The one draw back is that all the velocities along the entire length of the ultrasound beam are recorded so that only the highest velocities can be measured. One must remember that the ultrasound beam has width and length that extends beyond the scope of the 2D image and the pulsed Doppler. This is important since any flow in the range of CW beam will be recorded in the spectral analysis graph. The major disadvantage of CW Doppler is that is gives no spatial information. All velocities across the line are measured. The use of the blind pencil probes (non-imaging Doppler probe) gives a more accurate assessment of the maximum flow velocities. This probe has a small interface with the skin and is easily manipulated between the rib spaces. The major drawback is that the image is not displayed on the screen; only the spectral tracing is visible, so that the operator must be sure of what flow is being assessed. B. Pulsed Wave Doppler To give spatial information on detected velocities, pulsed wave Doppler was developed. It is important to understand that PW Doppler is NOT based on the Doppler principle but provides an output in a spectral display which looks very similar to the way CW Doppler is represented. The Doppler shift itself is however not measured by the system. In the pulsed wave system an image line is chosen along which ultrasonic pulses are transmitted at a constant rate. This rate is the pulse repetition frequency (PRF). Instead of continuously sampling the backscattered waves, only one sample of the reflected wave is taken at a fixed time after transmitting certain pulse. This time interval is the range gate. The range gate will determine the exact depth where velocities are measured. The transducer sends pulses and must receive that signal back before other pulses can be transmitted. A sample volume is positioned at the area of interest. The velocity of sound in soft tissue is a given constant of 1540m/s. and the 'go and return time' is used to determine the depth of the sample volume. The velocities that can be measured are limited by the pulse repetition frequency or number of pulses that are emitted per second. Aliasing of the Doppler signal occurs when the PRF is too low and the returning signals from one waveform are not received before the next waveform is sent. The PRF at which aliasing occurs is also called the Nyquist limit. Aliasing will result in velocities being displayed at the same time below and above the baseline. The Nyquist limit will be lower the deeper the velocity is measured as the time for the ultrasound wave to travel will be longer. The Nyquist limit depends on the frequency of the probe with higher frequency probes having lower Nyquist limits while lower frequency probes have higher Nyquist limits. A 2.5 mHz transducer will display velocities of twice the magnitude as a 5.0 mHz transducer. So adequate probe selection is

important when performing pulsed Doppler measurements. To further increase the Nyquist limits different options are possible. The first one is to shift the baseline so that velocities in only one direction are measured. A second method is to send out a new pulse before the previous one has returned, which is called the high-repetition frequency method. This results in measuring velocities in more than one site or sample volume reducing the spatial resolution but increasing the Nyquist limit. The size of the sample volume can be adjusted. A smaller sample volume will result in a shaper velocity profile as fewer velocities are sampled at the same time.

Optimize settings of PW Doppler The gain control, compress , filter settings are similar compared to CW Doppler. Scale/Baseline: shift of the baseline allows the whole display to be used for either forward or reverse flow which is useful if the flow is only in one direction. The scale should always be optimized and be set no higher than necessary to display the measured flow velocities. Gate size: increase in sample volume increases the strength of the signal at the expense of a lower spatial resolution. In general the smallest sample volume that results in adequate signal-to-noise ratio should be used.

Update: allows for simultaneous 2-D imaging to optimize the sampling position relative to the 2-D image. Simultaneous 2-D imaging reduces the temporal resolution and all PWDoppler traces should be obtained with 2-D freeze. C. Color flow mapping Color Doppler displays the direction and flow velocities of the blood superimposed on the 2D image. In this technology different pulses are sent across an image line and the phase shift between the different signals is measured in two sample points. This phase shift is proportional to the velocity of the reflecting object. A color is assigned to the direction of flow whether it is away from or towards the transducer. It may be helpful to remember the word BART (Blue Away Red Towards). The flow velocity is displayed in shades of these colors. The brighter the color the higher the velocity is. The color information represents the mean velocity of flow. When the flow is disturbed or not laminar, the pattern will be mosaic. This mosaic pattern is only produced if the variance is on. The variance is a color Doppler option that is present with all the cardiac presets. As with pulsed Doppler, maximal mean velocities which can be measured by color Doppler are limited by the Nyquist limit. Similarly to pulsed Doppler this is dependent on transducer frequency and depth. Color flow Doppler provides Doppler shift information from an entire area unlike pulsed Doppler, which samples from a specific point. Therefore, color Doppler requires more time to compute the lines of information onto the screen. Frame rate and line densities are reduced proportional to the time required. Keeping the color sector small will provide a better frame rate and produce a flicker free image. Using color flow allows the operator to visualize the blood flow in relation to the surrounding structures, and provides a method for rapid interpretation of abnormal location and direction of flow and helps to guide Doppler interrogation of abnormal flow.

Optimize settings of Color Doppler Gain: the gain should be adjusted until background noise is detected in the color image and then reducing it so that the noise just disappears. Scale: should be adapted depending on the velocities of the flows measured. When looking at high velocity flows, the scale should be adapted so the maximal Nyquist limit is chosen. When low-velocities are studied (coronary flow, venous flows), the scale needs to be lowered to allow the display of these lower velocities Color sector size: to optimize frame rates the smallest necessary color sector size should be used. Different Doppler artifacts have been described and are summarized in the next tables.

Spectral Doppler Artifacts

Artifact 1. Nonparallel intercept angle 2. Aliasing 3. Range ambiguity Effect Underestimation of velocity Inability to measure maximal velocity Doppler signals from more than one depth along the ultrasound beam are recorded Overlap of Doppler signals from adjacent flows Spectral display shows unidirectional flow both above and below the baseline Bandlike interference signal that obscures Doppler flow Change in the velocity of the ultrasound wave as it passes through a moving media which results in slight overestimation of Doppler shifts

4. Beam width 5. Mirror image

6. Electronic interference 7. Transit-time effect

Color Doppler Artifacts

Artifact 1. Shadowing 2. Ghosting 3. Background noise Appearance Absence of flow signal distal to strong reflector Brief flashes of color that overlay anatomic structures and do not correlate with flow patterns Speckled color pattern over 2D sector due to excessive gain Loss of true flow signals due to inadequate gain

4. Underestimation of flow signal

5. Intercept angle

Change in color (or absence at 90 degrees) due to the angle between the flowstream and and ultrasound beam across the image plane Wraparound of color display results in a variance display even for laminar flow Linear color artifacts or complex artifacts across the 2D image

6. Aliasing 7. Electronic interference

The HSC Echolab protocols 1. Standard protocol 2. Functional protocol 3. Atrial septal defects and partial abnormal pulmonary venous return 4. Ventricular septal defects 5. Atrioventricular septal defects 6. Coarctation and interrupted aortic arch 7. Transposition of the great arteries 8. Tetralogy of Fallot 9. Tetralogy of Fallot-Pulmonary atresia 10. Pulmonary atresia with intact ventricular septum 11. Hypoplastic left heart syndrome 12. Ebsteins anomaly

STANDARD ECHOCARDIOGRAPHIC PROTOCOL

This protocol is the reference protocol for image acquisition in the HSC echolab and is considered to be the reference protocol for the other studies. In the other protocols knowledge on the basic imaging views is assumed and they will focus more specifically on the specific information to obtained with the different lesions. SUBCOSTAL

Situs view in transverse plane: cross-section of spine, AOabd & IVC (acquire 2D image and colour Doppler) Abdominal aorta and IVC long axis (acquire colour flow and PW Doppler in IVC. Acquire image. PW Doppler in abdominal aorta to rule out coarctation). Acquire spectral display.

Long-axis views with sweep from anterior to posterior (figure 1). Acquire 2D and with colour flow sweep) RV inflow and RV outflow. Acquire 2D then colour Flow More anterior view for LVOT / Aortic valve. Acquire 2D then colour flow Posterior view: atrial septum, pulmonary venous connections. Acquire 2D then colour flow

Short axis subcostal views: rotate clockwise and from right to left Bicaval view SVC and IVC (acquire 2D then colour Doppler) Leftward sweep: pulmonary valve and mitral valve and LV apex (more leftward angulation). Acquire 2D then colour flow (See figure 2) Spectral Doppler at any level if pathology is suspected or turbulent/abnormal flow on colour Doppler is detected.

PARASTERNAL

Long axis (LAX) acquire image Do M-mode measurements preferably in PLAX view of LV and aorta and LA. Measure RV dimension, IVS, LV dimension and LVPW in diastole (start of the QRS in ECG tracing). Measure LV cavity in systole. Measure LVET (from aortic valve opening to aortic valve closure). Measure LA in end-systole. Acquire Mmode calculations.

Zoom on the aortic valve acquire the image. Measure the aortic valve annulus. Acquire the measurement.

Colour flow Doppler across the aortic valve to rule out stenosis and regurgitation acquire the image. Zoom on the MV. Acquire 2D image and colour flow Doppler Acquire colour flow in IVS sweeping from anterior to posterior to rule out VSDs RV inflow view (tilt posterior towards RV, TV, and RA) acquire image. Zoom on the TV. Colour Doppler across TV to rule out regurgitation and stenosis acquire image

PA long axis (tilt laterally and anterior). Zoom the PV, measure the annulus acquire image. Colour flow Doppler across the PV. Do PW Doppler in RVOT. If there is PI, measure the early diastolic flow to estimate mean PA pressure. Rotate clockwise and go higher (at least one intercostal space) to obtain the PA branches. Measure the size of the pulmonary arteries. Acquire image. Do colour flow, acquire image. Do PW on the PA branches, measure the flow and acquire the image.

Rotate counter clockwise to obtain the ductal cut. Acquire image in 2D and colour flow Doppler. Short axis: pulmonary valve level, acquire 2D image then colour and CW Doppler - acquire image. Aortic valve level, zoom and acquire image. imaging at the level of the mitral valve, papillary muscle and LV apex. 2D

Do colour sweeps of IVS from base to apex to exclude VSDs TV, check for regurgitation/stenosis. Acquire 2D image then colour flow Doppler. If there is TR, measure the velocity to estimate RVSP.

APICAL

4-CH at the level of the atrio-ventricular valves. Zoom and measure MV and TV separately at end-diastole. Acquire 2D image. Colour flow across the MV and TV valves separately at a higher frame rate (at least 20 Hz) to check for regurgitation/stenosis. Acquire color flow image. Spectral display of MV/TV inflows, TR (if any). If TR is present, do CW and measure the spectral display to estimate RVSP. Acquire the image. Do colour Doppler and PW in pulmonary veins if it is visible. Acquire colour Doppler and PW Doppler.

4-CH, tilt anterior to view LVOT / AO (5-CH view) then further anterior to view PA (if possible). Apply colour flow Doppler across the aortic valve, acquire image, do CW Doppler, measure the spectral display acquire image.

Tilt more anterior from apical 5-CH view to image the RVOT/PA if possible. Do colour flow and spectral display acquire the image and measurement. 4-chamber view sweep of IVS from posterior to anterior with colour flow to check for VSDs Counter clockwise rotation to obtain 2 chamber view (for wall motion abnormalities) acquire image. Apply colour flow across the MV acquire image.

Counter clockwise rotation: Apical long axis view (when necessary for LVOT / AO velocity measurement/MR).

SUPRASTERNAL

Place the transducer at the suprasternal notch to obtain the long axis of the ascending, transverse and descending thoracic aorta. Acquire 2D image and colour flow Doppler. Pulse the descending aorta, acquire the spectral display

Rotate transducer 45o clockwise to see bifurcating brachiocephalic trunk. If it bifurcates to the right side it is a left aortic arch, if it bifurcates to the left side, then it is a right aortic arch. Acquire image sweeping from the take off of the brachiocephalic trunk to the bifurcation.

Rotate back to left shoulder and if you see the left AOA rotate laterally and caudally to see LPA, acquire 2D image and colour flow Doppler to exclude peripheral stenosis

In frontal plane (AOA short axis) see RPA (RPA long axis) and RSVC with LBCV, acquire image in 2D imaging and colour flow Doppler and PW Doppler in SVC.

In frontal plane look for pulmonary veins (crab view, in small children), acquire 2 D image and colour flow Doppler. Do PW Doppler if turbulent flow is detected. Sweep to the left looking for LSVC

Functional assessment
In every patient: Systolic LV function M-mode from LAX or SAX if images in LAX view insufficient Measure RV/LV/LA dimensions and LV wall thickness, include LVEDD z-score on the report. Calculate %FS and EF based on M-mode If regional septal dysfunction/dyssynchrony, calculate EF using biplane Simpsons If doubt about LV function, obtain 3-D dataset for offline volumetric analysis

Diastolic LV function Pulsed Wave Doppler on: mitral inflow pattern: E wave, A wave, E/A, deceleration time, A-duration IVRT measurement Pulmonary venous velocity: S wave, D wave, A wave reversal duration, A wave reversal velocity TDI measurements at MV, septal and TV annulus

Systolic RV function visual assessment TAPSE if doubt (Tricuspid Annular Plane Systolic Excursion)

Diastolic RV Pulsed Wave Doppler on: TV inflow Hepatic venous flow/SVC flow Pulmonary artery flow (look for end-diastolic anterograde flow in PA present in inspiration and expiration) THINK 3-D and FUCTION + IF in doubt/abnormalities

Functional protocol
PLEASE USE VIVID 7 for FUNCTIONAL STUDIES Observe the following settings High frame rates necessary for colour TDI (>150fps) 2D Frame rates should be 70-100fps, Record sweep speed at 100mm/sec Study should be copied to Echopac for off-line analysis Record 4 beat loops Obtain BP before study and enter into machine to calculate wall stress Parasternal Long Axis View PLAX view Zoom LVOT and aortic valve PLAX view with colour of aortic and mitral valves M-mode aortic valve for LVET/LAd and R-R interval VCFc/ wall stress relation PLAX RV inflow 2D and colour PLAX view with colour tissue Doppler for strain (using appropriate TD Nyquist scale Narrow sector PLAX with colour tissue Doppler for strain Parasternal Short Axis View Regular PSAX sweep from base to apex M-mode at level of mitral valve leaflet tips LV(SF and EF if possible) Colour Doppler PV and TV Obtain mean PA pressure when possible PW Doppler of main PA 2D PSAX views at MV/PAP/apical levels for 2D speckle strain Corresponding colour tissue Doppler PSAX at MV/PAP/apical level for strain (using appropriate TD Nyquist scale) Apical Views (Cross sectional areas and long axis dimensions/volumes) Regular 2D 4 chamber sweep 2D 4 chamber view for bi-plane Simpsons and 2D Strain 2D 2 chamber view for bi-plane Simpsons and 2D Strain Obtain simultaneous 4,3,2 ch view with GE 3-D probe Colour MV/AoV and TV

Obtain RVsp Obtain tricuspid valve inflow Obtain pulsed Tissue Doppler traces optimizing alignment in the basal lateral LV, the basal septal and basal lateral RV segment Obtain pulsed Doppler traces in the basal anterior and posterior segments on the 2-chamber view Obtain 4-ch apical view of LA/ RA: 2D+ color TDI Obtain 2-ch view of LA: 2D+ color TDI Mitral valve Doppler/Pulmonary vein Doppler Record PW Doppler of Mitral inflow (MVe,a dt): between the valve leaflets (at tips of mitral leaflets) PW Doppler between inflow and outflow for IVRT and myocardial performance index Obtain Color-Doppler M-Mode of LV inflow with adequate baseline shift LV dp/dt: record CW Doppler of mitral regurgitation (RV dp/dt in single V) Record PW Doppler RUPV LVOT + AO valve Doppler Record PW LVOT Doppler Record CW Doppler through the aortic valve (gradient + aortic acceleration time) Colour Tissue Doppler Broad sector views for colour TDI for LV dyssynchrony: include 4C + RV, 3C and 2C---12 segments for analysis) Obtain simultaneous 4,3,2 ch view with color TDI with GE 3-D probe Narrow sector views from 4-chamber for colour TDI of LV lateral wall, IVS and RV lateral wall for strain (narrow sector width= high frame rates ), from two chamber view obtain narrow sector of anterior and posterior wall IVC/Hepatic veins Image and Doppler hepatic venous flow 3-D echocardiography Apical full volume

ATRIAL SEPTAL DEFECT AND PARTIAL ANOMALOUS PULMONARY VENOUS DRAINAGE

Preoperative/pre-interventional assessment

ANATOMY + HEMODYNAMICS Abdominal aorta (subcostal sagittal view with optimal angle for Doppler tracing) o Color flow mapping and pulsed Doppler tracing Inferior caval vein (subcostal infra-diaphragmatic sagittal and transversal view) o Look for abnormal inflow from bellow diaphragm to exclude / confirm infradiaphragmatic PAPVD (2D, CFM, PDE) Intra- atrial septum (subcostal 4 CH view and subcostal bicaval view) o Size of ASD o Location. Differentiate between: sinus venosus-, secundum-, or primum ASD o For secundum ASD: carefully observe margins, particularly antero-superior, posterior, and inferior margin. Measure aortic rim + inferior rim o Number of defects o Shunting: L-R or R-L (+ PW doppler) o (Measure RA area) Right ventricle (subcostal sagittal view for IVC, apical 4CH, parasternal SAX and inferior RV-RA view) o RV size (RVDd / M Mode) and function (qualitatively) o Septal motion (M-mode) o RVH Tricuspid valve (apical 4CH, parasternal SAX and inferior RV-RA view) o TV annulus size o TR grade (mild, moderate, severe) o RV systolic pressure calculation: TR jet gradient + Rap Mitral valve (apical 4CH, parasternal SAX and inferior RV-RA view) o MV annulus size o Mitral regurgitation assessment: color-flow o Especially in primum ASD, look for detailed anatomy of mitral valve: cleft ?

Pulmonary veins (Subcostal 4CH view, apical 4CH view, parasternal SAX view, suprasternal coronal view = crab view) o Identify all pulmonary veins (2D, color flow, pulsed Doppler) o Especially for sinus venosus defect abnormal drqinqge of right pulmonqry veins is very common o Check flow characteristics in veins abnormally connected to IVC, SVC and brachiocephalic vein to distinguish between systemic and pulmonary blood flow (PDE) Left ventricle (parasternal LAX) o Standard M-mode measurement MPA and PA Branches (low and high parasternal SAX, suprasternal frontal view for RPA and slightly oblique postero-inferior view for LPA) o Visualise proximal and distal RPA and measure the size of distal RPA prior its bifurcation o Visualise LPA and measure the size little distally from its origin: size Aortic arch (suprasternal views) o Direction: left, right (bifurcating first neck artery) o Absence of bifurcating artery most likely presence of aberrant subclavian artery SVC (suprasternal views: coronal, saggital from suprasternal notch, right and left infraclavicular approach) o Visualize right SVC and exclude / confirm LSVC o Check flow characteristics in veins abnormally connected to IVC, SVC and brachiocephalic vein to distinguish between systemic and pulmonary blood flow to exclude / confirm supracardiac PAPVD (PDE)

Functional assessment in ASD patients

LV systolic function - Ps LAX M-mode: LV + RV dimensions - Visual assessment and biplane Simpsons if concern - LV diastolic function - PW mitral valve - PW pulmonary veins - Tissue Doppler lateral and septal basal segment - RV systolic function - Visual assessment - RV diastolic function

- TV inflow - Hepatic veins - TDI basal lateral RV wall

3-D acquisition
in every pre-operative/pre-interventional patient with good windows - Subcostal views + Apical 4-chamber view
Postoperative/post intervention assessment

ANATOMY + HEMODYNAMICS Intra- atrial septum (subcostal 4 CH view and subcostal bicaval view) o Device (or patch) in place? o Relationship of device to other cardiac structures: pulmonary veins, mitral valve, tricuspid valve, coronary sinus, IVC, SVC, atrial roof o Any residual shunt across ASD: location, size, direction of shunt Right ventricle (subcostal sagittal view, apical 4CH, parasternal SAX and inferior RV-RA view) o RV size and systolic function (subjective, M-mode) Tricuspid valve (apical 4CH, parasternal SAX and inferior RV-RA view) o TR grade o RV systolic pressure calculation: TR jet gradient + Rap Mitral valve (apical 4CH, parasternal SAX and inferior RV-RA view) o MV annulus size o Mitral regurgitation assessment: color-flow o Especially in primum ASD, look for detailed post-operative anatomy of mitral valve Superior / Inferior caval veins (subcostal infra-diaphragmatic sagittal view, subcostal 45 CH view, subcostal bicaval view, short axis view, suprasternal views: coronal, saggital from suprasternal notch, right and left infraclavicular approach) o Demonstrate flow characteristics in SVC / IVC to exclude / confirm systemic venous obstruction (color flow, pulsed Doppler)

Pulmonary veins (Subcostal 4CH view, apical 4CH view, parasternal SAX view, suprasternal coronal view = crab view) o Identify all pulmonary veins (2D, color flow, pulsed Doppler) o Check flow characteristics in veins abnormally connected to IVC, SVC and brachiocephalic vein to distinguish between systemic and pulmonary blood flow (PDE) Left ventricle (parasternal LAX) o Standard M-mode measurement Effusions (subcostal transversal view, standard 4CH, SAX and LAX views) o Pericardial o Pleural

Functional assessment in ASD patients

- LV systolic function - Ps LAX M-mode: LV + RV dimensions, FS - LV diastolic function - PW mitral valve - PW pulmonary veins - Tissue Doppler lateral and septal basal segment - RV systolic function - Visual assessment - RV diastolic function - TV inflow - Hepatic veins - TDI basal lateral RV wall

ATRIAL SEPTAL DEFECT AND PARTIAL ANOMALOUS PULMONARY VENOUS DRAINAGE- SONOGRAPHER

Preoperative assessment
Subcostal Sagittal views Abdominal aorta/IVC o Color flow mapping and pulsed Doppler tracing Inferior caval vein (subcostal infra-diaphragmatic sagittal and transversal view) o Look for abnormal inflow from below diaphragm to exclude / confirm infra-diaphragmatic PAPVD (2D, CFM, PDE) Subcostal 4 chamber view and subcostal bicaval views on intra-atrial septum o Size of ASD o Location. Differentiate between: sinus venosus-, secundum-, or primum ASD o For secundum ASD: carefully observe margins, particularly anterosuperior, posterior, and inferior margin. Measure aortic rim + inferior rim o Number of defects o Shunting: L-R or R-L (+ PW doppler) o (Measure RA area) Ps long axis view Right ventricle (parasternal LAX and inferior RV-RA view) o o o RV size (RVDd / M Mode) Septal motion (M-mode) RVH

Left ventricle (parasternal LAX) o Standard M-mode measurement

Mitral valve o o Mitral prolapse? Regurgitation assessment by color-flow Especially in primum ASD, look for detailed anatomy of mitral valve:cleft

Tricuspid valve (RV inflow view)

o Tricuspid regurgitation assessment by color-flow, measure gradient to estimate RVSP Ps Short- axis views o Especially in primum ASD, look for detailed anatomy of mitral valve:cleft ? o High ps short axis view at the level of the aortic valve identifies the intraatrial septum and also the ASD and the relationship towards the aorta. For secundum ASD this is good view to identify the presence/absence of aortic rim. Pulmonary valve, MPA and PA Branches (low and high parasternal SAX, suprasternal frontal view for RPA and slightly oblique postero-inferior view for LPA) o Visualize LPA and measure the size little distally from its origin: size Apical Views o Exclude pulmonary valve stenosis associated with ASD: measure CWDoppler across RVOT. Flow gradient is common. o Measure early diastolic PI-jet velocity and estimate mean PA-pressure

Apical 4-Ch, 2-chamber and 3-chamber views o Mitral regurgitation assessment: color-flow; cleft/prolapse o Tricuspid regurgitation: degree and systolic gradient; estimate RVSp o Check pulmonary vein flow from 4 chamber view o Check ASD-size, location and rims from 4 chamber view taking into account echo drop-out o Measure LVOT velocity using CW-Doppler o Assess RV function qualitatively . Suprasternal views (short-axis and long-axis views) Aortic arch o Direction: left, right (bifurcating first neck artery) o Absence of bifurcating artery most likely presence of aberrant subclavian artery o Check flow characteristics in veins abnormally connected to SVC and

brachiocephalic vein to distinguish between systemic and pulmonary blood flow (PDE) SVC (suprasternal views: coronal, saggital from suprasternal notch, right and left infraclavicular approach) o Visualise right SVC and exclude / confirm LSVC coronal view = crab view o Check flow characteristics in veins abnormally connected to IVC, SVC and brachiocephalic vein to distinguish between systemic and pulmonary blood flow to exclude / confirm supracardiac PAPVD (PDE) Pulmonary veins Check drainage of all pulmonary veins: use crab view and other windows to identify the pulmonary veins Color-Doppler the pulmonary veins and if possible PW-Doppler in each pulmonary vein

!! 3-D acquisition in every pre-operative/pre-interventional patient with good windows - Subcostal views - Apical 4-chamber views Postoperative/post intervention assessment
Subcostal 4 chamber view and subcostal bicaval view Intra- atrial septum o Device (or patch) in place? o Relationship of device to other cardiac structures: pulmonary veins, mitral valve, tricuspid valve, coronary sinus, IVC, SVC, atrial roof o Any residual shunt across ASD: location, size, direction of shunt Superior / Inferior caval veins o Demonstrate flow characteristics in SVC / IVC to exclude / confirm systemic venous obstruction (color flow, pulsed Doppler) Parasternal Long axis view

Left ventricle (parasternal LAX) o Standard M-mode measurement Right ventricle (parasternal LAX and inferior RV-RA view) o RV size and systolic function (subjective, M-mode) Tricuspid valve (parasternal LAX and inferior RV-RA view) o TR grade o RV systolic pressure calculation: TR jet gradient + Rap Mitral valve (parasternal LAX and inferior RV-RA view) o MV annulus size o Mitral regurgitation assessment: color-flow o Especially in primum ASD, look for detailed post-operative anatomy of mitral valve Ps Short- axis views o Especially in primum ASD, look for detailed anatomy of mitral valve: residual cleft. Mitral regurgitation? o High ps short axis view at the level of the aortic valve identifies the intraatrial septum patch/device and the relationship towards the aorta. o Pericardial effusion? Pulmonary valve, MPA and PA Branches (low and high parasternal SAX, suprasternal frontal view for RPA and slightly oblique postero-inferior view for LPA) o Visualize proximal and distal RPA and measure the size of distal RPA prior its bifurcation o Visualize LPA and measure the size little distally from its origin: size Apical Views Exclude pulmonary valve stenosis associated with ASD: measure CW- Doppler across RVOT. Flow gradient is common.

Apical 4-Ch, 2-chamber and 3-chamber views o Mitral regurgitation assessment: color-flow; cleft/prolapse o Tricuspid regurgitation: degree and systolic gradient; estimate RVSp o Check pulmonary venous flow from 4 chamber view o Check for residual ASD: size, location and rims from 4 chamber view taking into account echo drop-out

o .

Measure LVOT velocity using CW-Doppler

Suprasternal views (short-axis and long-axis views) SVC (suprasternal views: coronal, saggital from suprasternal notch, right and left infraclavicular approach) o measure flow in SVC after ASD closure espc after sinus venosus defect closure

VENTRICULAR SEPTAL DEFECT

Preoperative/pre-interventional assessment

ANATOMY AND HEMODYNAMICS

Intra- atrial septum (subcostal 4 CH view and subcostal bicaval view) o Associated PFO/ASD o Shunting across IAS: L-R or R-L + Doppler gradient Tricuspid valve (apical 4CH, parasternal SAX and inferior RV-RA view) o Any aneurysmal tissue occluding the VSD? o TR? (Grade, RV systolic pressure calculation: TR jet gradient + Rap) o Jet from VSD through tricuspid valve? Right ventricle (subcostal sagittal view for IVC, apical 4CH, parasternal SAX and inferior RV-RA view) o RV size (z-score) and systolic function (qualitative) o RVH? o Assess RVOT for RVOT obstruction and presence of muscle bundles Left atrium (parasternal LAX view) o Size (standard M-Mode measurement) + z-score Pulmonary veins o Check drainage of all pulmonary veins: use crab view and other windows to identify the pulmonary veins o Color-Doppler the pulmonary veins Left ventricle (apical 4 CH view, parasternal SAX view) o LV size and systolic function (standard M-mode measurement) o LVEDD: diameter + z-score o Assess the LVOT for the presence of LVOT obstruction Mitral valve (apical 4CH, parasternal SAX and inferior RV-RA view)

o Mitral regurgitation: assess degree of MR qualitatively o Associated mitral valve anomalies, espc when inlet VSD (cleft mitral valve) Intra ventricular septum (subcostal sagittal + 4 CH + SAX view, all apical and parasternal views) o Size of VSD (2-D echo, two different views) o Location of VSD o perimembranous: indicate extension towards inlet or outlet o muscular: describe location in IVS o doubly committed o Malalignment of the IVS (best evaluated by long-axis view) o Anterior malalignment of outlet septum is associated with RVOT obstruction (tetralogy of Fallot) o Posterior malalignment is associated with LVOT obstruction and arch anomalies (coarcatation of the aorta, IAA) o Shunting: L-R, R-L or bidirectional + PW/CW Doppler gradient. Estimate RV systolic pressure (RVSP= SBP (VSD peak velocity2) Aortic valve (apical 3 + 5 CH view, parasternal LAX view) o Bicuspid or tricuspid? o Aortic valve prolapse? (usually of the right and non-coronary cusp) o Aortic insufficiency? (Grade qualitatively) Aortic arch (suprasternal views) o Direction: left, right (bifurcating first neck artery) o Absence of bifurcating artery most likely presence of aberrant subclavian artery o Associated coarctation? Abdominal aorta (subcostal sagittal view with optimal angle for Doppler tracing): o Size at the level of diaphragm SVC (suprasternal views) o Visualise right SVC and exclude / confirm LSVC Pulmonary veins o Check drainage of all pulmonary veins: use crab view and other windows to identify the pulmonary veins o Color-Doppler the pulmonary veins

PDA (subcostal sagital view, suprasternal aortic arch view, modified frontal views) o Exclude presence/absence of PDA o If present look at PDA: size, flow and course: short, long and tortuous

3-D ACQUISITION
- obtain apical 4-chamber view - any view capable of imaging the VSD en face FUNCTIONAL ASSESSMENT - LV systolic function - Ps LAX M-mode: LV + RV dimensions, FS - LV diastolic function - PW mitral valve - PW pulmonary veins - Tissue Doppler lateral and septal basal segment - RV systolic function - Visual assessment - RV diastolic function - TV inflow - Hepatic veins - TDI basal lateral RV wall Postoperative/post-interventional assessment

ANATOMY AND HEMODYNAMICS

VSD patch (subcostal 4 CH view, apical and parasternal views)

o Patch in place? o Residual shunting? L-R or R-L, or LV-RA + Doppler gradient o Exclude presence of other VSDs VSD device o Device in place? o Residual shunt through device?

Tricuspid valve (apical 4CH, parasternal SAX and inferior RV-RA view) o Tricuspid regurgitation/stenosis? Are leaflets moving freely? o TR? (Grade, RV systolic pressure calculation: TR jet gradient + Rap) o Residual jet from VSD through tricuspid valve? Right ventricle (subcostal sagittal view for IVC, apical 4CH, parasternal SAX and inferior RV-RA view) o RV size (z-score) and systolic function (qualitative) o Assess RVOT for RVOT obstruction and presence of muscle bundles Left atrium (parasternal LAX view) o Size (standard M-Mode measurement) + z-score Left ventricle (apical 4 CH view, parasternal SAX view, LAX view) o LV size and systolic function (standard M-mode measurement) o LVEDD: diameter + z-score o Assess the LVOT for the presence of LVOT obstruction Mitral valve (apical 4CH, parasternal SAX and inferior RV-RA view) o Mitral regurgitation: assess degree of MR qualitatively o Associated mitral valve anomalies, espc when inlet VSD (cleft mitral valve) Intra ventricular septum (subcostal sagittal + 4 CH + SAX view, all apical and parasternal views) o Residual VSD? Location + size o Shunting: L-R, R-L or bidirectional + PW/CW Doppler gradient. Estimate RV systolic pressure (RVSP= SBP (VSD peak velocity2) Aortic valve (apical 3 + 5 CH view, parasternal LAX view) o Aortic insufficiency? (Grade qualitatively) o After device placement: relationship of device to the aortic valve

3-D ACQUISITION
- obtain 3-D apical 4-chamber view FUNCTIONAL ASSESSMENT

- LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - LV diastolic function - PW mitral valve - PW pulmonary veins - Tissue Doppler lateral and septal basal segment - RV systolic function - Visual assessment - RV diastolic function - TV inflow - Hepatic veins - TDI basal lateral RV wall

ATRIOVENTRICULAR SEPTAL DEFECT

Preoperative assessment ANATOMY AND HEMODYNAMICS
DETERMINE SITUS AND VENOUS ANOMALIES as per standard protocolexclude isomerism/other situs anomalies Interatrial septum (subcostal + apical 4CH, PSAX, bicaval view) - Determine presence and size of primum defect - Determine size and direction of shunt (Colour Doppler, PW Doppler) - Exclude additional defects (PFO/secundum defects) Interventricular septum (subcostal + apical 4CH, PLAX, PSAX) - Determine size and extension of ventricular communication - Determine direction and velocity of shunt (Colour Doppler, CW Doppler) - Exclude additional VSDs (sweeps of the IVS) Atrioventricular valve (subcostal + apical 4CH, PLAX, PSAX) - Confirm common AV valve at the AV-junction (2D) inserted at one level (no offset between RAVV and LAVV. - Determine anatomy of CAVV - Number of leaflets - Size of anterior and inferior bridging leaflets - Size of left mural leaflet usually found between the LV papillary muscles, (the distance between these will help you determine the size of the leaflet) - Describe zone of apposition (cleft) in left AV valve - Locate attachment of valve chordae of the superior bridging leaflet - Determine number and position of papillary muscles - Determine location and severity of AV valve regurgitation (colour Doppler, subjective grading) - Specify location of regurgitant jet (zone of apposition, left AV valve coaptation, right AV valve coaptation, LV-RA shunt, isolated, multiple)

- Measure right AV valve regurgitant jet gradient to calculate RV pressure and/or to distinguish between TR jet and LV-RA shunt (PW/CW Doppler) - Exclude valvar stenosis - mean and peak gradient (Colour Doppler, PW/CW) - Balanced vs. unbalanced AVSD - Measure the R and L AVV areas - Obtain 3-D full volumes Left atrium and ventricle (Apical 4CH, PLAX, PSAX) - LA-dimension (2-D) - LV size / volume, wall thickness and function (SAX M-mode and/or 2D) - Obtain 3-D volumes when appropriate Pulmonary veins - Check drainage of all pulmonary veins: use crab view and other windows to identify the pulmonary veins - Color-Doppler the pulmonary veins Right atrium and ventricle (Apical 4CH, PLAX, PSAX) - RA-size (2-D) - RV dimension (M-mode, 2D) and function (subjective) Outflow tracts (Apical 5+3CH, PLAX, PSAX) - Assess LVOT for possible LVOTO: typical goose neck appearance with / without accessory chordal attachment beneath aortic valve (2/3D) - Measure significance of obstruction if present (Colour Doppler, PW/CW Doppler) Aortic valve (Apical 5+3CH, PLAX, PSAX) - Exclude stenosis (CW/PW Doppler) - Severity of valvar regurgitation (colour Doppler) SVC and AOA (Suprasternal) - Confirm RSVC and bridging vein - Exclude LSVC - Assess sidedness of aortic arch !!! THINK OF ASSOCIATED DEFECTS

PDA, Tetralogy of Fallot, Isomerism, ASD, VSD, LSVC -> CS, PS, COA

3-D Echocardiography
ALWAYS Obtain 3-D datasets for offline analysis Apical 4-chamber view Subcostal views FUNCTIONAL ASSESSMENT - LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - RV systolic function - Visual assessment

Post-operative Assessment
ANATOMY AND HEMODYNAMICS ASD/VSD (Apical + subcostal 4CH, PSAX) - Evaluate integrity of patch/es (2D) - Residual shunt - direction and velocity: between LA and RA, between LV and RV, between LV and RA (PW/CW/colour Doppler) AV VALVES (Apical + subcostal 4CH, PSAX, PLAX) - Evaluate integrity of valve repair (PW/CW/colour Doppler) - Determine location and severity of residual R and L AV valve regurgitation (Colour Doppler, semi objective grading). Specify location of regurgitant jet (zone of apposition, left AV valve coaptation, right AV valve coaptation, LV-RA shunt, isolated, multiple) - Measure residual right AV valve regurgitant jet gradient to calculate RV pressure and/or to distinguish between TR jet and LV-RA shunt (PW/CW Doppler) - Exclude valvar stenosis - mean and peak gradient (Colour Doppler, PW/CW Doppler) OUTFLOW TRACTS (Apical 5+3CH, PLAX, PSAX) - Detailed LVOT observation for possible LVOT obstruction (2/3D, similarly to preoperative assessment). Determine mechanism of obstruction if present: membrane, AV-valve tissue, other.

- Assess presence of aortic valve regurgitation and if present assess importance of degree of regurgitation - Detailed RVOT observation for possible RVOT obstruction (2/3D, similarly to preoperative assessment). Determine mechanism (muscular, valvar, supravalvar) - Measure significance of LVOT/ RVOT obstruction if present (Colour Doppler, PW/CW Doppler) LEFT AND RIGHT VENTRICLE - Right and left heart size, wall thickness and LV function (PS SAX M-mode) - Visual assessment of RV function PULMONARY ARTERIES - assess pulmonary artery size and exclude AORTIC ARCH - assess aortic flow in abdominal aorta

3-D Echocardiography
ALWAYS Obtain 3-D datasets for offline analysis Apical 4-chamber view Subcostal views FUNCTIONAL ASSESSMENT - LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - RV systolic function - Visual assessment

COARCTATION OF THE AORTA Interruption of the aortic arch (IAA)

PREOPERATIVE ASSESSMENT ANATOMY + HEMODYNAMICS Abdominal aorta (subcostal sagittal view, with angle optimised for Doppler) - Color flow Doppler + pulsed Doppler to assess pulsatility and presence of antegrade diastolic flow, or retrograde diastolic flow if there is a large PDA Intracardiac (subcostal 4 & 5ch, SAX, apical 4 & 5 ch, parasternal LAX & SAX) - Look for any associated lesions, left heart obstructive lesions (most commonly bicuspid aortic valve), VSD etc, and assess these fully LVOT + Aortic valve (Ps short axis, long-axis, apical 4CH, 3CH, subcostal views) - 2-D short axis pictures looking for bicuspid valve or other aortic valve anomalies - measure aortic valve annulus and LVOT dimension - carefully assess LVOT for sub-aortic stenosis; if present describe the mechanism of sub-AS - describe aortic valve with commissural anatomy (fusion left-right cusp, left, NC cusp) - assess aortic valve stenosis/regurgitation - colour Doppler, CW Doppler aortic valve SAX, LAX, apical views, CW Doppler also from supra-sternal windows Mitral valve (subcostal views, 4CH, 2CH, 3CH, PS LAS, PS SAX) measure size of mitral annulus color Doppler, PW Doppler mitral inflow (CW Doppler if mitral stenosis) assess degree of mitral insufficiency (mild-moderate, severe) exclude mitral valve anomalies like parachute mitral valve, supravalvular stenosis, arcade mitral valve: look at supravalvar level, valve leaflets and subvalvular level

Left ventricle - Assess LV size (SAX dimension, long axis dimension)

- Assess degree of any LVH (Z scores), measure wall thickness on m-mode or 2D echo - Assess LV ventricular systolic function (M-mode) - Assess LV diastolic function (mitral inflow, pulmonary veins)

Right ventricle - Assess degree of any RVH or RV dilatation - Qualitative assessment of RV function - Assess RV pressures by quantifying TR jet velocity Interventricular septum - Exclude presence/absence of associated ventricular septal defect. Especially exclude presence of malalignment defect with posterior malalignment - Exclude presence of muscular spetal defects using the different sweeps mentioned in the VSD protocol PDA (modified frontal views, ductal cut, suprasternal views) - PDA size, flow and course (2D echo, color Doppler, PW Doppler, CW Doppler) - Direction of flow, assess if PDA is restrictive Aortic arch (Suprasternal views, modified L or R high parasternal SAX) - Side of arch: left or right (bifurcating innominate artery), double aortic arch? - Size of transverse arch. On 2D echo, measure the diameter of the ascending aorta, proximal aortic arch, distal aortic arch, aortic isthmus, coarctation and descending aorta, in early systole. Measure the diameter of the transverse arch between LCCA and LSCA - Assess site of CoA- preductal (usual neonatal site), post ductal - Assess nature of CoA- tubular hypoplasia, waist-like or shelf-like narrowing? - Confirm presence of posterior shelf (an isolated anterior ductal shelf just proximal to the aortic origin of the duct or ductal ligament is a normal finding, not CoA) - Use CFM to visualise high velocity jet in CoA region, and the proximal acceleration zone. - Use PW Doppler to measure Vmax just proximal to CoA, to decide if it is appropriate to use the expanded Bernouilli equation. This is necessary in multilevel left heart obstructive lesions, when velocity just proximal to the CoA is

greater than 1m/sec. - Use CW Doppler to record the flow profile across the narrowing. CW Doppler tracing in severe CoA normally shows high velocity flow in systole, with antegrade flow extending into diastole. Note- if there is a large L to R shunt at ductal level, antegrade diastolic flow may be present in proximal desc Ao due to steal into pulmonary vascular bed, when there is no evidence of CoA. Also, mild CoA may not have antegrade diastolic flow. In patients with a CoA and a large R to L shunt by way of a PDA, the descending Ao Doppler profile may be normal). - Do not measure mean gradient in CoA- the diastolic flow component would lead to an underestimation of the true gradient across the obstruction.

3-D Echocardiography
- Obtain 3-D images of the LV from the apical 4ch view for LV volume quantification - Obtain images for studying mitral valve and aortic valve anatomy, when judged abnormal on 2-D FUNCTIONAL ASSESSMENT - LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - LV diastolic function - PW mitral valve - PW pulmonary veins - RV systolic function - Visual assessment - RV diastolic function - TV inflow - Hepatic veins

Interrupted Aortic Arch

PREOPERATIVE ASSESSMENT ANATOMY + HEMODYNAMICS Abdominal aorta (subcostal sagittal view, with angle optimised for Doppler) CFM adjust colour scale as necessary- may be very low velocity flow present PWD to confirm pulsatility of abdominal aorta, measure Vmax Intracardiac (subcostal 4 & 5ch, SAX views, apical 4 & 5ch, parasternal L and SAX)

Look for any associated lesions, i.e, left heart obstructive lesions, VSDs, A-P window etc, Invariably there is a VSD (in types B & C), so assess size and position of VSD (doubly committed VSD, perimembranous outlet VSD). A common association is posterior deviation of the outlet septum causing LVOTO, so store clips of the 2D images of the LVOT from multiple views. Measure the LVOT diameter and AoV annulus from parasternal LAX in early systole (Z score), and document the appearance of the LVOT effective orifice from SAX views. Also confirm direction of shunting Assess cardiac chamber dimensions, LVH/RVH & measure wall thickness on mmode or 2D echo, and assess ventricular function PDA (modified frontal views, ductal cut, suprasternal views) PDA size, flow and course (2D echo, CFM, CW Doppler, PW Doppler) Confirm direction of flow, and note if PDA is restrictive It is rare to find IAA with no PDA, but when it does occur there will be profuse collaterals between the two segments of the aorta, so it is important to document these using colour Doppler Aortic arch (Suprasternal views, modified L or R high parasternal SAX views, ductal cut Side of arch: left or right (bifurcating innominate artery) *note- it is rare to have interrupted right aortic arch, unless there is a double aortic arch On 2D echo, measure size of aortic arch proximal and distal to the interruption, and measure the distance between the proximal and distal parts of the aorta Note- it will not be possible to measure the transverse arch between LCCA and LSCA in type B or C Use CFM and PW Doppler to demonstrate discontinuity of flow across interruption Ascertain level of interruption: TYPE A interruption at isthmus,with duct supplying lower body TYPE B interruption between LCCA and LSCA TYPE C between innominate artery and LCCA Check for aberrant origin or isolation of subclavian artery

Interruption between LCCA and LCSA (B) is most common form, and is more frequently associated with VCFS Interruption at isthmus (A) is less common, but is more often associated with A-P window Interruption between carotid arteries (C) is rare

3-D Echocardiography
- Obtain 3-D images of the LV from the apical 4ch view for LV volume quantification FUNCTIONAL ASSESSMENT - LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - LV diastolic function - PW mitral valve - PW pulmonary veins - RV systolic function - Visual assessment - RV diastolic function - TV inflow COARCTATION AND IAA POSTOPERATIVE ASSESSMENT

ANATOMY AND HEMODYNAMICS

Abdominal aorta (subcostal sagittal view, with angle optimised for Doppler)

Pulsatility- use CFM and PW Doppler to identify Vmax in systole and presence of any antegrade diastolic flow CoA/IAA repair site (Suprasternal views, modified L or R high parasternal SAX views) Appearance on 2D echo and CFM: If there is any evidence of narrowing proximal to CoA or IAA repair site, use PW Doppler to assess whether it is necessary to use expanded Bernouilli equation when calculating the gradient across the repair site, and use CW Doppler to rule out/confirm any residual obstruction. Measure Vmax in systole and note presence or absence of antegrade flow in diastole Size of transverse arch. On 2D echo, measure the diameter of the ascending aorta, proximal aortic arch, distal aortic arch, aortic isthmus, coarctation and descending aorta, in early systole. Measure the diameter of the transverse arch between LCCA and LSCA Is there any evidence of dissection/ aneurysmal dilatation at surgical or balloon sit Intracardiac (subcostal 4 & 5ch, SAX, apical 4 & 5 ch, parasternal LAX & SAX) - Look for any associated lesions, left heart obstructive lesions (most commonly bicuspid aortic valve), VSD etc, and assess these fully LVOT + Aortic valve (Ps short axis, long-axis, apical 4CH, 3CH, subcostal views) - 2-D short axis pictures looking for bicuspid valve or other aortic valve anomalies - measure aortic valve annulus and LVOT dimension - carefully assess LVOT for sub-aortic stenosis; if present describe the mechanism of sub-AS - describe aortic valve with commissural anatomy (fusion left-right cusp, left, NC cusp) - assess aortic valve stenosis/regurgitation - colour Doppler, CW Doppler aortic valve SAX, LAX, apical views, CW Doppler also from supra-sternal windows Mitral valve (subcostal views, 4CH, 2CH, 3CH, PS LAS, PS SAX) measure size of mitral annulus color Doppler, PW Doppler mitral inflow (CW Doppler if mitral stenosis) assess degree of mitral insufficiency (mild-moderate, severe) exclude mitral valve anomalies like parachute mitral valve, supravalvular stenosis, arcade mitral valve: look at supravalvar level, valve leaflets and subvalvular level

Left ventricle

- Assess LV size (SAX dimension, long axis dimension) - Assess degree of any LVH (Z scores), measure wall thickness on m-mode or 2D echo - Assess LV ventricular systolic function (M-mode) - Assess LV diastolic function (mitral inflow, pulmonary veins) Right ventricle - Assess degree of any RVH or RV dilatation - Qualitative assessment of RV function - Assess RV pressures by quantifying TR jet velocity

FUNCTIONAL ASSESSMENT - LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - LV diastolic function - PW mitral valve - PW pulmonary veins - Tissue Doppler lateral and septal basal segment - RV systolic function - Visual assessment - RV diastolic function - TV inflow - Hepatic veins - TDI basal lateral RV wall

ADDENDUM for SONOGRAPHERS

Subcostal Window
Abdominal Aorta - 2-D image of the abdominal aorta - 2-D image with colour with soundbeam as parallel as possible (insert image here) - Pulsed Wave Doppler signal aligned as parallel as possible to interpret flow pattern (show picture of Doppler Profile) IVC

- Image IVC and collapse in 2-D and one with colour of the hepatic vein (interrogate for high RV pressures. - Pulse Wave Doppler hepatic vein RVOT - Sagittal plane, image the RVOT to rule out RV muscle bundles. (insert image) LVOT - In subcostal long axis angle anterior to see LVOT and rule out any LVOT obstruction. (insert image)

Parasternal Long Axis

- Long Axis image of mitral valve, aortic valve, left ventricle, and right ventricle (insert image) - Align ventricles as perpendicular as possible to cursor line to complete m-mode measurement of chamber dimensions and LV wall thickness; assess for LVH secondary to coarctation. - obtain LV mass and index vs. height. (rely on chart and calculation if it is not done automatically by the machine). - Zoom LVOT area to rule out sub aortic ridge, or any sort of dynamic obstruction. In the presence of a sub aortic ridge or fixed obstruction, measure distance of ridge/membrane to the valve annulus. Use colour Doppler to demonstrate origin of turbulent flow. (insert image) - Zoom aortic valve to rule out AS/AI/BAV. Repeat with colour Doppler. (insert image) - Measure aortic root at the level of sinus, ST junction, ascending aorta (rule out any supravalvular narrowing or post stenotic dilatation) (insert image here) - Zoom mitral valve to rule out any mitral valve anomalies ie. Prolapse, parachute mitral valve, etc. Repeat with colour (insert image here) - Angle anterior to image RVOT in long axis. - Zoom pulmonary valve to rule out any pulmonary valve anomalies ie. Tethering, doming, Repeat with colour. - Angle towards patients hip to image RVIT in long axis. - Zoom tricuspid valve to rule out any tricuspid valve anomalies. - Image with colour to see tricuspid regurgitation. If soundbeam is parallel with TR jet obtain highest gradient to estimate RVSP (insert image)

Parasternal Short Axis

- Image aortic valve to denote accurate number of leaflets. - Zoom aortic valve, repeat with colour to rule out regurgitation. (insert image here) - Zoom/concentrate on the MPA and branch PAs to rule out supravalvular narrowing of the pulmonary root, or branch PA stenosis. Repeat with colour - Angle towards basal portion of ventricle to visualize mitral valve. Assess for any wall motion abnormalities/LVH. - Zoom mitral valve/repeat with colour. Rule out any cleft valves or associated lesions. (insert image) - Angle towards mid ventricle to denote two papillary muscles. Assess for any wall motion abnormalities/LVH. - Angle towards apex of heart. Assess for any wall motion abnormalities/LVH.

Ductal Cut
- Image LPA and descending aorta to rule out posterior shelf at the level of the isthmus. Rule out PDA. (insert image) - Zoom region of the isthmus. Pulse the descending aorta to rule out coarctation.

Apical Imaging
- Apical 4 chamber view to assess tricuspid valve, mitral valve, and LV wall thickness/and function. Evaluate each valve with colour. - Doppler pulmonary vein to assess a wave reversal. - Angle anterior to image apical 5 chamber and zoom on area of LVOT to get close look at any anomalies. Repeat with colour to see where turbulent flow begins. - Apical 2 chamber view to assess function and wall thickness. Repeat with colour on the mitral valve to rule out mitral regurgitation. - Apical 3 chamber view to assess function and wall thickness. Zoom LVOT, and repeat with colour.

Suprasternal Notch
- Determine arch sidedeness if undefined (beginning with transducer in a long axis cut and angling anteriorly to see where the first branch arrives. - Image entire aortic arch (candy cane view) - Zoom area of interest : where coarctation begins. - Make appropriate measurements at the transverse arch, isthmus region, site of narrowing/Coarctation, descending aorta (index measurement vs. BSA). (insert picture here with measurements). - Use colour Doppler to determine where turbulent velocity begins. - Use pulsed wave Doppler to acquire initial gradient before coarctation/narrowing occurs. - Continue walking PW Doppler along the arch to see where flow gradient truly begins. - Use Continuous wave to obtain max velocity and calculate net gradient V2 - V1

Blood Pressure Reading

- Obtain blood pressure from right arm and leg.

POST OPERATIVE ASSESSMENT OF COARCTATION

Subcostal Imaging
Abdominal aorta - Image abdominal aorta and align soundbeam as parallel as possible to obtain most accurate Doppler signal.

Subcostal long axis - Image LVOT to check for any residual subaortic obstruction.

Parasternal Imaging
Parasternal Long Axis - M-mode through ventricle to assess LV wall thickness and chamber dimensions - Zoom aortic, mitral, pulmonary and tricuspid valve to rule out any residual anomalies. Repeat with colour to rule out stenosis or regurgitation. Parasternal Short Axis - Zoom aortic, pulmonary, tricuspid and mitral valve and repeat with colour. - Angle at each level of the ventricle to assess function and LV wall thickness. Ductal Cut - Image LPA and descending aorta to rule out residual shelves. - Pulse wave Doppler the descending aorta to rule out residual coarctation.

Apical Imaging
Apical 4 chamber to image mitral and tricuspid valve. Repeat with colour. PW Doppler of mitral valve and pulmonary veins to assess diastolic function Apical 5 chamber - zoom LVOT area to rule out obstruction. Apical 2 and 3 chamber - Assess function and valvular regurgitation and stenosis.

Suprasternal Imaging
- Image aortic arch in its with probe held in a sagittal position. - Rule out residual coarctation - Make appropriate measurements

Blood Pressures
- Obtain right arm and leg BP to rule out any residual gradient.

TRANSPOSITION OF THE GREAT ARTERIES

Preoperative Assessment ANATOMY AND HEMODYNAMICS
Abdominal Aorta (subcostal sagittal view with optimal imaging for Doppler tracing) o Color Doppler and pulsed Doppler

Inferior vena cava o Check connection of IVC to heart (beware of interrupted IVC esp. when considering balloon septostomy

Atrial Septum defect assessment (subcostal 4CH + SAX, parasternal SAX) o o o Presence/size of atrial communication Direction of shunt: L>R (normally), R>L restrictive vs. non restrictive, Doppler flow analysis (PDE/CW): mean gradient between atria

Atrioventricular Valves (subcostal SAX, apical 4CH) o o o Measure size of tricuspid valve and mitral valve annulus (z-scores) Identify where tricuspid and mitral valve chordal attachments insert: look for valve abnormalities esp. straddling of tricuspid valve (inlet VSD), additional mitral valve attachments Color Doppler flow analysis to detect presence/degree of regurgitation

Interventricular septum (subcostal LAX sweeps, SAX sweeps, apical sweeps, SAX and LAX) o o o o o 2-D imaging and color flow mapping: detect presence of VSD. Beware that shunt normally goes from RV to LV location and size of VSD: perimembranous, muscular, outlet, inlet malalignment of VSD (esp. anterior malalignment of outlet part vs. muscular trabecular septum) Doppler flow analysis to measure gradient across VSD Beware of additional muscular VSDs

Great arteries (subcostal coronal + SAX, apical 5CH, parasternal LAX + SAX) o Define spatial relationship of great vessels to each other: normally aorta is positioned right anterior to PA. Other variants are: side by side, posterior aorta,

o o o o o

Structure of each semilunar valve and function of cusps: stenosis or regurgitation Describe the presence of commissural malaligment on SAX view Determine the diameter of the annulus of both great arteries Assess outflow tracts espc. LVOT for obstruction/stenosis, describe mechanism (membrane, rigde, abnormal attachments from mitral/tricuspid valve,) Doppler flow assessment (PDE, CW) for subvalvar, valvar stenosis (particularly LVOT). Beware that due to pulmonary hypertension the gradient across the LVOT can initially be low and 2-D assessment of the LVOT and pulmonary valve is important.

Coronary Arteries (subcostal anterior 4CH, high parasternal SAX + modified LAX) o Use Leiden convention for describing coronary artery anatomy with in case of right anterior aorta the left coronary sinus = sinus 1 from which the LCA originates and the right coronary sinus= sinus 2 from which the RCA originates. Most common variants are described in included figures. Visualize origins of LCA and RCA using 2-D and color flow mapping Course of LCA: look for bifurcation of LCA into LAD and LCX, or separate origin of circumflex Look for intramural proximal course of the coronary arteries. subcostal 4CH looking for LCX; in LCX originating from sinus 2, the LCX runs posterior to the pulmonary artery looping of coronaries: anterior looping/posterior looping in relationship to great vessels

o o o o o

Branch Pulmonary Arteries (low and high parasternal SAX, suprasternal frontal views) o o o Visualize proximal and distal RPA and LPA Measure the size of distal RPA and LPA Look with color flow Doppler to look for proximal branch stenosis

Ventricular size and function o o o Assess size of both ventricles: normally RV is somewhat larger compared to LV at birth Global LV function (FS and EF by M=mode) Qualitative assessment of RV function

PDA (subcostal sagittal, high parasternal modified frontal views, suprasternal aortic arch views) o o Size, flow and course of PDA Color Doppler + pulse Doppler of flow across the duct: L>R or R>L during systole and diastole

Aortic Arch (suprasternal views) o o o o Direction of arch (branching pattern of innominate artery-absence of bifurcating artery is most likely an aberrant subclavian artery) Confirm presence of right SVC and brachiocephalic vein Exclude/confirm left SVC Exclude presence of coarctation of the aorta; this can be difficult in case of patent arterial duct

3-D echocardiography
o Could be useful for measuring ventricular volumes and function

Coronary patterns in TGA

PASQUINI et al. 1987

Gremmels et al JASE 2004

Postoperative Assessment after the arterial switch ANATOMY AND HEMODYNAMICS

PFO/ASD (subcostal 4CH + SAX, parasternal SAX) o Residual shunt: direction, Doppler gradient

VSD Patch (subcostal 5CH + SAX, apical 5CH, parasternal LAX + SAX) o Residual shunt: direction, Doppler gradient

Left Ventricle (apical 4CH, 2CH, 3CH, parasternal LAX + SAX) o o o o Standard M-mode assessment for LV size, hypertrophy and fractional shortening Look for regional wall motion abnormalities Mitral regurgitation: grade LA size on standard M-mode

Right Ventricle (subcostal sagittal view for IVC, apical 4CH, parasternal SAX + LAX inferior RA-RV view) o o o RV size and systolic function (qualitative assessment) Tricuspid regurgitation: grade, estimate right ventricular systolic pressure-TR gradient + right atrial pressure RA size

Great Arteries (subcostal 5CH + SAX, apical 5CH, parasternal LAX + SAX) o o o Color, PW + CW Doppler flow assessment across neo-aortic valve and pulmonary valve for valvar stenosis/regurgitation Look for supravalvar stenosis caused by suture lines particularly supravalvar pulmonary stenosis after the Lecompte maneuver (see branch pulmonary arteries) Measure diameter of aortic valve anulus, sinus of Valsalva and sinotubular junction (z-scores). Progressive neo-aortic root dilatation is common after the switch procedure.

Coronary Arteries (subcostal anterior 4CH, high parasternal SAX + modified LAX) o o Determine the position of the reimplanted coronaries Look for antegrade diastolic flow in the origin of LCA and RCA

Branch Pulmonary Arteries (low and high parasternal SAX, suprasternal frontal view)

o o

Visualize the origin of LPA and RPA (after Lecompte maneuver RPA has a proximal course to the right of the aorta and the LPA to the left of the aorta. Evaluate for the presence of stenosis (PDE/CW) at the origins and distally. Obtain peak gradients with the best possible alignation

Aortic Arch (suprasternal) o Confirm unobstructed flow in the descending aorta

3-D ECHOCARDIOGRAPHY
o o Could be considered for analyzing LV and RV volumes and function Could be considered for analysis of aortic root dimensions and aortic regurgitation

FUNCTIONAL ASSESSMENT - LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - Look for regional wall motion abnormalities - IF RWMA or global dysfunction, perform full functional protocol !!! - LV diastolic function - PW mitral valve - PW pulmonary veins - Tissue Doppler lateral and septal basal segment - RV systolic function - Visual assessment - RV diastolic function - TV inflow - Hepatic veins - TDI basal lateral RV wall

TETRALOGY OF FALLOT
Preoperative Assessment ANATOMY AND HEMODYNAMICS
Abdominal aorta (subcostal sagital view with optimal angle for Doppler tracing) o CFM (look for collaterals) o Pulsed Doppler to detect possible PDA / collaterals VSD (subcostal 5CH + SAX, parasternal LAX and parasternal SAX) o o o o o o Size and position (perimembranous, outlet, doubly-committed) Shunting: R-L or L-R (pink Fallot) Degree of overriding of aorta (>50% = DORV) Aortic-mitral continuity TV: chordal attachments Look for additional VSDs: can be very difficult!!

RVOT + Pulmonary trunk (subcostal SAX and anterior 4CH, low and high parasternal SAX + modified high parasternal LAX) o Exclude presence of pulmonary valve atresia o Anatomy of RVOT obstruction: low (DCRV), high, localized, tubular, presence / absence of infundibular chamber o Ventriculo-arterial junction (annulus): size (z-score) o Valvar morphology (if possible) o Supravalvar narrowing (diameter of MPA at narrowest point) o Doppler flow analysis (PDE, CW): level and flow characteristics (dynamic, fixed) Coronary Arteries (subcostal anterior 4CH, high parasternal SAX + modified LAX) o Origins of RCA and LCA: look for LAD coming from RCA crossing the RVOT o Course of RCA: look for bifurcating RCA which suspects conal branch o Course of LCA: look for bifurcating LCA which confirm presence of LAD PA Branches (low and high parasternal SAX, suprasternal frontal view for RPA and slightly oblique postero-inferior view for LPA) o Confirm presence of PA-bifurcation and continuity of pulmonary branches from the central PA. Exclude discontinuous PAs. o Visualize proximal and distal RPA and measure the size of distal RPA prior its bifurcation: size (z-score)

o Visualize LPA and measure the size close to its origin: size (z-score) PDA + Collaterals (subcostal sagital view, suprasternal aortic arch view, modified frontal views) o PDA: size, flow and course: short, long, and tortuous o Collaterals: check brachiocephalic trunk, left (right) carotid artery Aortic arch (suprasternal views) o Direction: left, right (bifurcating first neck artery) o Absence of bifurcating artery most likely presence of aberrant subclavian artery SVC (suprasternal views) o Visualize right SVC exclude / confirm LSVC

3-D ECHOCARDIOGRAPHY
o Could be considered useful for RVOT anatomy o Could be useful for LV and RV function FUNCTIONAL ASSESSMENT - LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - RV systolic function - Visual assessment - RV diastolic function - TV inflow - Hepatic veins - TDI basal lateral RV wall

Postoperative assessment ANATOMY AND HEMODYNAMICS

PFO/ASD (subcostal 4CH + SAX, parasternal SAX)

Residual shunt: direction, Doppler gradient

Right ventricle (subcostal sagital view for IVC, apical 4CH, parasternal SAX and inferior RV-RA view) RV dimension from 2-D short-axis M-mode TV annulus size (z-score) TR grade (CFM) RV systolic pressure calculation: TR jet gradient + RAp RV diastolic dysfunction (check restrictive physiology): IVC size + collapse, RA size (RA), RV inflow (pulsed Doppler at breath hold if possible), anterograde PA flow (PDE, CW) at end-diastole during inspiration and expiration o RV systolic function (subjective, M-mode) o Optional: tricuspid annulus systolic excursion (TAPSE), tissue-Doppler velocities at in RV free wall basal segment, acquisition for strain calculation o o o o o

Left ventricle (parasternal LAX)

o Standard M-mode measurements o Functional assessment

VSD Patch (subcostal SAX + 5CH, parasternal LAX and SAX)

o Residual shunt: direction, Doppler gradient (peak) RVOT / Pulmonary trunk (subcostal SAX and anterior 4CH, low and high Parasternal SAX + modified high parasternal LAX) o Residual RVOTO/PS: morphology (subvalvar, valvar, supravalvar), Doppler gradient (peak, mean) o RVOT aneurysm o Pulmonary regurgitation: grade o Based on color flow mapping: Gr I from main AP, GrII from bifurcation, GR III from main right and left branches, Gr IV from distal branches o Based on PDE: deceleration time o Measure vena contracta PA Branches (low and high parasternal SAX, suprasternal frontal view for RPA and slightly oblique postero-inferior view for LPA) o Residual RPA / LPA stenosis: morphology + Doppler gradient (try to optimize angle) Aortic valve and arch

o o o o

Measure aortic annulus Assess presence of aortic regurgitation and grade based on CFM Measure aorta at level of sinuses of Valsalva and sinotubular junction Assess for presence of collaterals

3-D ECHOCARDIOGRAPHY
o Could be considered useful for RVOT anatomy o Obtain for LV and RV volumes FUNCTIONAL ASSESSMENT - LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - LV diastolic function - PW mitral valve - PW pulmonary veins - Tissue Doppler lateral and septal basal segment - RV systolic function - Visual assessment - RV diastolic function - PA flow (PW Doppler) - TV inflow - Hepatic veins - TDI basal lateral RV wall

Sonographer Addendum (Pre-operative Assessment)

Subcostal Window Abdominal aorta - Image abdominal aorta by 2-D and colour - Spectral Doppler to interrogate the flow profile (optimize angle as parallel to soundbeam as possible - Detect for presence of any collaterals or large PDA (flow reversal below baseline) Subcostal Sagital Cut Begin sweeping from Right atrium towards apex of heart Interrogate with colour to determine the location of the VSD and the amount of pulmonary stenosis, aortic override, intact septum? Optimize Doppler angle to obtain peak gradient across RVOT Use Colour Doppler to assess shunting physiology and degree/presence of insufficiency, flow turbulence.

Subcostal Coronal Cut Begin with the probe angled posteriorly to image both atria, and slowly angle anterior to show ventricular function and relationship of the great arteries. Repeat with colour to determine presence/location of VSD. Angle anterior to visualize aorta and degree of override. Repeat with colour to assess VSD physiology. Determine whether or not aorta is more than 50% committed to right ventricle. Angle slightly anterior to image the RVOT and pulmonary valve. Align Doppler cursor to obtain a peak gradient across RVOT. Qualitatively assess for RVH.

Parasternal Long Axis Determine presence and location of VSD and assess the degree of aortic override (> 50% qualifies as double outlet right ventricle) Assess VSD physiology using colour Doppler and Spectral Doppler. Measure size of VSD in an anterior to posterior orientation. M-Mode line perpendicular through the basal LV to obtain accurate measurements of chambers and wall thickness (important to look for RVH if present, and septal curvature).

Colour Doppler mitral valve and aortic valve to assess for any insufficiency or stenosis. Angle probe towards tricuspid valve to image by 2-D and colour, to obtain peak TR jet to estimate RVsp. Angle probe towards pulmonary valve to image by 2-D and colour. Obtain peak flow across pulmonary valve using CW Doppler.

Parasternal Short Axis Image great vessels to determine relationship and orientation. Zoom aortic valve and determine coronary artery pattern. Determine size and location of VSD by 2-D first and repeat with colour Doppler and spectral Doppler to determine VSD physiology (if VSD is doubly committed there will be aortic and pulmonary continuity) Image pulmonary valve and obtain peak velocity across RVOT. Pay attention to RV function and RVH. Angle slightly anterior, or move probe one rib space higher to try and image branch PAs by 2- D. Measure branch PAs and Doppler to rule out any branch PA stenosis. Begin to sweep towards apex starting at the level of the branch Pas (may have to move probe one or two rib spaces lower to image apex clearly) Repeat sweep with colour to rule out additional muscular VSDs.

Ductal Cut Determine the size and presence of a PDA. Assess with colour and spectral Doppler to determine shunting physiology. Rule out coarctation and posterior shelves in the descending aorta.

Apical 4 and 5 Chamber Sweep posterior to anterior with 2-D and with colour to rule out additional VSDs and view extent of VSD. Rule out inlet extension. Determine the degree of aortic override. Rule out any LVOT obstruction, aortic insufficiency/stenosis. Rule out mitral valve anomalies. Image tricuspid valve and align sound beam as parallel to TR jet to obtain peak RVsp. Angle anterior to image RVOT. Assess with colour and spectral Doppler if angle is sufficient.

Apical 3 Chamber Image LVOT and rule out any obstruction Assess for any aortic insufficiency, subaortic obstruction, mitral valve insufficiency/stenosis.

Suprasternal Short Axis Image entire aortic arch to rule out any coarctation Angle towards LPA to measure size and to rule out LPA stenosis Use colour and spectral Doppler in your assessment to obtain peak gradients and determine where flow acceleration starts.

Suprasternal Long Axis Image SVC and measure size of RPA and LPA

Sonographer Post Operative Assessment

Subcostal Sagital Cut Image abdominal aorta by 2-D and colour. Align soundbeam as parallel as possible to evaluate flow profile. Angle towards SVC and begin to sweep towards apex in a long recorded loop to conceptualize ventricular function and patch integrity. Obtain peak gradient using CW Doppler across RVOT to determine whether or not there is any residual RVOT obstruction. Assess for insufficiency as well.

Subcostal Coronal Cut Begin with probe angled posterior to visualize right and left atria and with a long recorded loop angle anterior to see the great arteries, the ventricular function at each level, and the integrity of the patch with colour Doppler. Rule out any LVOT/aortic insufficiency. Rule out any RVOT/pulmonary insufficiency. Check for pericardial effusion when sweeping posterior to anterior.

Parasternal Long Axis Beginning at the classic parasternal long axis image, use a long recorded loop ( one with and without colour) angle towards the pulmonary valve and then towards the tricuspid valve to evaluate integrity of the VSD patch and the ventricular function. Obtain perpendicular angle to measure chamber dimensions and wall thicknesses. Look for any wall motion abnormalities, particularly paradoxical septal wall motion. Check for any MR/AR/LVOTO/AS. Angle towards Tricuspid valve to try and obtain an accurate RVsp.

Angle towards Pulmonary valve to rule out any residual RVOTO, pulmonary gradients, pulmonary insufficiency or conduit insufficiency.

Parasternal Short Axis Sweep from great arteries towards apex to check the integrity of the VSD patch. Look for any residual leaks. Measure branch PAs and rule out any branch PA stenosis. Try and obtain peak RVsp from TR jet. Assess mitral and aortic valve for any insufficiency. Rule out any pericardial effusion at each level.

Apical 4 and 5 Chamber Sweep posterior to anterior to check for any residual VSD patch leaks/residual VSDs. Rule out any LVOTO. Check for any aortic insufficiency or mitral regurgitation Check for any wall motion abnormalities. If abnormal septal motion, obtain appropriate images to calculate a reliable ejection fraction by Simpsons rule. Rule out any pericardial effusion.

Suprasternal Short Axis Image aortic arch and rule out coarctation Angle towards RPA and LPA to measure size

Suprasternal Long Axis Image SVC and RPA by 2-D and colour

Tetralogy of Fallot - PULMONARY ATRESIA

Pre-operative assessment ANATOMY AND HEMODYNAMICS
Abdominal aorta (subcostal sagital view with optimal angel for Doppler tracing): o CFM (look for collaterals) o PWD to detect retrograde flow to duct/collaterals Right ventricle (subcostal sagital view for IVC, apical 4CH, parasternal SAX and inferior RV-RA view) o o o o o RV hypertrophy, size and systolic function (subjective, M-mode) RVH severity TV annulus size TR grade Ascertain absence of flow from the RVOT to the PA

Left ventricle (parasternal SAX) o Standard M-mode measurement VSD (subcostal 5CH + SAX, parasternal LAX and parasternal SAX) o o o o o Size and position () Shunting: R-L (obiligatory) Aorto-mitral continuity TV: chordal attachment Look for additional VSDs (!)

RVOT + Pulmonary trunk (subcostal SAX and anterior 4CH, low and high parasternal SAX + modified high parasternal LAX) o Anatomy of RVOT obstruction: level of atresia and distance of RV from pulmonary artery, presence of imperforate membrane at valvar level o PV annulus diameter (if detectable) o Doppler flow analysis (PWD, CW): to determine if antegrade flow Coronary Arteries (subcostal anterior 4CH, high parasternal SAX + modified LAX)

o Origins of RCA and LCA: look for LAD <RCA crossing RVOT o Course of RCA: look for bifurcating RCA which suspects infundibular branch o Course of LCA: look for bifurcating LCA which confirm presence of LAD

PA Branches (low and high parasternal SAX, suprasternal frontal view for RPA and slightly oblique postero-inferior view for LPA) o Look for the presence of central PAs o If present visualize proximal and distal RPA and measure the size of distal RPA prior its bifurcation: size o Visualize LPA and measure the size at its origin Aortic arch (suprasternal views) o Direction: left, right (bifurcating first neck artery) o Absence of bifurcating artery most likely presence of aberrant subclavian artery SVC (suprasternal views) o Visualize right SVC and exclude / confirm LSVC PDA + Collaterals (subcostal sagital view, suprasternal aortic arch view, modified frontal views) o PDA: size, flow and course: short, long and tortuous o Look for collaterals originating from the aorta, neck vessels, coronaries

3-D ECHOCARDIOGRAPHY
o Could be considered useful for RVOT anatomy o Obtain for LV and RV volumes FUNCTIONAL ASSESSMENT - LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - RV systolic function - Visual assessment

Postoperative Assessment
Right ventricle (subcostal sagital view for IVC, apical 4CH, parasternal SAX and inferior RV-RA view) o TR grade o RV systolic pressure calculation: TR jet gradient + RAp o RV diastolic dysfunction (check restrictive physiology): IVC size + collapse, RA size (ARA), RV inflow (PDE at breath hold if possible), antegrade PA flow (PDE, CW) with presence / absence of atrial wave o RV hypertrophy, size and systolic function (subjective, M-mode) Left ventricle (parasternal LAX) o Standard M-mode measurement VSD Patch (subcostal SAX + 5CH, parasternal LAX and SAX) o Residual shunt: direction, Doppler gradient (peak) RVOT / Pulmonary trunk (subcostal SAX and anterior 4CH, low and high Parasternal SAX + modified high parasternal LAX) o Residual RVOTO/PS: morphology (subvalvar, valvar, supravalvar), Doppler gradient (peak, mean) o RVOT aneurysm o Pulmonary regurgitation: grade and doppler flow analysis (PW or CW) PA Branches (low and high parasternal SAX, suprasternal frontal view for RPA and slightly oblique postero-inferior view for LPA) o Residual RPA / LPA stenosis: morphology + Doppler gradient (try to optimise angle) o Visualise proximal and distal RPA and measure the size of distal RPA prior its bifurcation o Visualise LPA and measure the size little distally from its origin Aortic arch + descending aorta (suprasternal views) o Collaterals?

3-D ECHOCARDIOGRAPHY
o Could be considered useful for RVOT anatomy o Obtain for LV and RV volumes

FUNCTIONAL ASSESSMENT - LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - LV diastolic function - PW mitral valve - PW pulmonary veins - Tissue Doppler lateral and septal basal segment - RV systolic function - Visual assessment - RV diastolic function - PA flow (PW Doppler) - TV inflow - Hepatic veins - TDI basal lateral RV wall

Sonographer Addendum (Pre-operative Assessment)

Subcostal Window IVC Determine size of IVC and flow physiology of hepatic veins. Using pulsed wave Doppler optain flow profile of HV. Determine whether or not IVC is collapsing with respiration.

Abdominal aorta - Image abdominal aorta by 2-D and colour - Spectral Doppler to interrogate the flow profile (optimize angle as parallel to soundbeam as possible - Detect for presence of any collaterals or large PDA (flow reversal seen below the baseline would be a strong indicator for the presence of a large PDA, collaterals, etc.) Subcostal Sagital Cut - Begin sweeping from Right atrium towards apex of heart - Interrogate with colour to determine the location and size of the VSD and the amount, aortic override, intact septum? - Use Colour Doppler to assess shunting physiology Subcostal Coronal Cut - Begin with the probe angled posteriorly to image both atria, and slowly angle anterior to show ventricular function and relationship of the great arteries. Repeat with colour to determine presence/location of VSD. - Rule out any LVOTO, aortic insufficiency/stenosis. - Determine whether or not there is flow from LV to aorta using spectral Doppler. - Measure size and extent of VSD. Repeat with colour to assess VSD physiology. - Determine whether or not aorta is more than 50% committed to right ventricle. - Angle slightly anterior to image the RVOT and assess flow physiology from RV to the VSD to aorta. Ductal Cut (EXTREMELY CRUCIAL) - Determine the size and presence of a PDA. Assess with colour and spectral Doppler to determine shunting physiology. Rule out coarctation and posterior shelves in the descending aorta. - Check for any aorta-pulmonary collaterals.

Parasternal Long Axis - Determine presence and location of VSD and assess the degree of aortic override (> 50% qualifies as double outlet right ventricle) - Assess VSD physiology using colour Doppler and Spectral Doppler. - Measure size of VSD in an anterior to posterior orientation. - M-Mode line perpendicular through the basal LV to obtain accurate measurements of chambers and wall thickness (important to look for RVH if present, and septal curvature). - Colour Doppler mitral valve and aortic valve to assess for any insufficiency or stenosis. - Angle probe towards tricuspid valve to image by 2-D and colour, to obtain peak TR jet to estimate RVsp (pressures will mostly be systemic). Parasternal Short Axis - Zoom aortic valve and determine coronary artery pattern. - Determine size and location of VSD by 2-D first and repeat with colour Doppler and spectral Doppler to determine VSD physiology - Image branch PAs by 2- D. Measure branch PAs to determine whether or not they are of good size or hypoplastic. - Doppler to determine whether or not there is sufficient antegrade flow supplying them. - Begin to sweep towards apex starting at the level of the aorta (may have to move probe one or two rib spaces lower to image apex clearly) - Repeat sweep with colour to rule out additional muscular VSDs.

Apical 4 and 5 Chamber - Sweep posterior to anterior with 2-D and with colour to rule out additional VSDs and view extent of VSD. Rule out inlet extension. - Determine the degree of aortic override. - Rule out any LVOT/RVOT obstruction through VSD, - Rule out aortic insufficiency/stenosis. - Rule out mitral valve anomalies. Apical 3 Chamber - Image LVOT and rule out any obstruction - Assess for any aortic insufficiency, subaortic obstruction, mitral valve insufficiency/stenosis. Suprasternal Short Axis - Image entire aortic arch to rule out any coarctation - Angle towards LPA to measure size and to rule out LPA stenosis - Use colour and spectral Doppler in your assessment to confirm the presence of antegrade flow. -

Suprasternal Long Axis - Image SVC and measure size of RPA

Post Operative Assessment

Subcostal Sagital Cut - Image abdominal aorta by 2-D and colour. Align soundbeam as parallel as possible to evaluate flow profile. - Angle towards SVC and begin to sweep towards apex in a long recorded loop to conceptualize ventricular function and patch integrity. - Obtain peak gradient using CW Doppler across RV stent/conduit. Subcostal Coronal Cut - Begin with probe angled posterior to visualize right and left atria and with a long recorded loop angle anterior to see the great arteries, the ventricular function at each level, and the integrity of the patch with colour Doppler. - Rule out any LVOT/aortic insufficiency. - Rule out any RVOT/pulmonary insufficiency. - Check for pericardial effusion when sweeping posterior to anterior. Parasternal Long Axis - Beginning at the classic parasternal long axis image, use a long recorded loop one with and without colour) angle towards the RV conduit and then towards the tricuspid valve to evaluate integrity of the VSD patch and the ventricular function. - Obtain perpendicular angle to measure chamber dimensions (RV end diastolic dimension and function is of utmost importance). - Look for any wall motion abnormalities, particularly paradoxical septal wall motion. - Check for any MR/AR/LVOTO/AS. - Angle towards Tricuspid valve to try and obtain an accurate RVsp. - Angle towards Conduit to obtain peak gradient across and determine the degree of insufficiency (Free vs. Mild) Parasternal Short Axis - Sweep from great arteries towards apex to check the integrity of the VSD patch. Look for any residual leaks. - Assess conduit from short axis to determine peak velocity (note in long segments degree of stenosis/obstruction is overestimated) - Measure branch PAs and rule out any branch PA stenosis. - Try and obtain peak RVsp from TR jet. - Assess mitral and aortic valve for any insufficiency. - Rule out any pericardial effusion at each level.

Apical 4 and 5 Chamber - Sweep posterior to anterior to check for any residual VSD patch leaks/residual VSDs. - Rule out any LVOTO. - Check for any aortic insufficiency or mitral regurgitation - Check for any wall motion abnormalities. If abnormal septal motion, obtain appropriate images to calculate a reliable ejection fraction by Simpsons rule. - Rule out any pericardial effusion. Suprasternal Short Axis - Image aortic arch and rule out coarctation - Angle towards LPA to determine size Suprasternal Long Axis - Image SVC and RPA by 2-D and colour Ductal Cut - Rule out any residual PDA - Rule out coarctation

PULMONARY ATRESIA WITH INTACT IVS

Pre-operative/pre-interventional assessment ANATOMY AND HEMODYNAMICS
Abdominal aorta (subcostal sagittal view with optimal angle for Doppler tracing) o CFM (look for collaterals) o PDE to look for flow reversal Intra- atrial septum (subcostal 4 CH view and subcostal bicaval view) o Size of PFO/ASD o Shunting: L-R or R-L + doppler gradient Right ventricle (subcostal sagittal view for IVC, apical 4CH, parasternal SAX and inferior RV-RA view) o o o o RV dimensions (2-D, M-mode): z-score RVH severity RV function (subjective) Look for sinusoidal connections between RV and coronary arteries (2D and CFM)

Tricuspid valve (apical 4CH, parasternal SAX and inferior RV-RA view) o TV annulus size (z-score) o TR grade o RV systolic pressure calculation: TR jet gradient + RAp Left ventricle (parasternal LAX) o Standard M-mode measurement RVOT + Pulmonary trunk (subcostal SAX and anterior 4CH, low and high parasternal SAX + modified high parasternal LAX) o Anatomy of RVOT obstruction: presence of imperforate membrane at valvar level. Infundibular atresia? o PV annulus diameter o Doppler flow analysis (PDE, CW): to determine antegrade flow Coronary Arteries (subcostal anterior 4CH, high parasternal SAX + modified LAX)

o o o o o

Origins of RCA and LCA Course of RCA: look for bifurcating RCA which suspects infundibular branch Course of LCA: look for bifurcating LCA which confirm presence of LAD Size of proximal LCA and RCA; dilated, tortuous. Check connections between coronaries and RV (CFM)

PA Branches (low and high parasternal SAX, suprasternal frontal view for RPA and slightly oblique postero-inferior view for LPA) o Visualise proximal and distal RPA and measure the size of distal RPA prior its bifurcation o Visualise LPA and measure the size little distally from its origin Aortic arch (suprasternal views) o Direction: left, right (bifurcating first neck artery) o Absence of bifurcating artery most likely presence of aberrant subclavian artery SVC (suprasternal views) o Visualise right SVC exclude / confirm LSVC PDA + Collaterals (subcostal sagital view, suprasternal aortic arch view, modified frontal views) o PDA: size, flow and course: short, long and tortuous o Look for MAPCAs

3-D ECHOCARDIOGRAPHY
o Obtain for LV and RV volumes FUNCTIONAL ASSESSMENT - LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - RV systolic function - Visual assessment

Management
o Blalock-Taussig shunt o Transcatheter treatment. Pulmonary valve perforation + balloon dilatation +/ductal stenting o Surgical treatment. o Bidirectional Glenn shunt>TCPC

Postoperative Assessment for all procedures

Right ventricle (subcostal sagital view for IVC, apical 4CH, parasternal SAX and inferior RV-RA view) o TR grade o RV systolic pressure calculation: TR jet gradient + RAp o RV diastolic dysfunction (check restrictive physiology): IVC size + collapse, RA size (ARA), RV inflow (PDE at breath hold if possible), antegrade PA flow (PDE, CW) with presence / absence of atrial wave o RV hypertrophy, size and systolic function (subjective, M-mode) Left ventricle (parasternal LAX) o Standard M-mode measurement PA Branches (low and high parasternal SAX, suprasternal frontal view for RPA and slightly oblique postero-inferior view for LPA) o Residual RPA / LPA stenosis: morphology + Doppler gradient (try to optimise angle) o Visualise proximal and distal RPA and measure the size of distal RPA prior its bifurcation o Visualise LPA and measure the size little distally from its origin

3-D ECHOCARDIOGRAPHY
o Could be considered useful for RVOT anatomy o Obtain for LV and RV volumes FUNCTIONAL ASSESSMENT - LV systolic function

- Ps SAX M-mode: LV + RV dimensions, FS - Look for regional wall motion abnormalities - LV diastolic function - PW mitral valve - PW pulmonary veins - Tissue Doppler lateral and septal basal segment - RV systolic function - Visual assessment - RV diastolic function - PA flow (PW Doppler) - TV inflow - Hepatic veins - TDI basal lateral RV wall Postoperative Assessment BT shunt/ductal stent BT shunt (suprasternal views) o o o o o o Look at proximal and distal origin of shunt Look for any narrowing of shunt (CFM, 2D) Color Doppler + CWD flow through shunt Look at flow in RPA and LPA Measure RPA and LPA size Doppler abdominal aortic flow for detection of flow reversal

Stent in duct (suprasternal views) o o o o o Position of stent: pulmonary and aortic side Look for any narrowing on stent (CFM, 2D) Look for narrowing on pulmonary or aortic side (CFM, 2D) CW Doppler flow through stent Doppler abdominal aortic flow for detection of flow reversal

Postoperative Assessment Pulmonary post fulguaration/valvotomy/valvectomy or transannular patch

RVOT / Pulmonary trunk (subcostal SAX and anterior 4CH, low and high Parasternal SAX + modified high parasternal LAX)

o Residual RVOTO/PS: morphology (subvalvar, valvar, supravalvar), Doppler gradient (peak, mean) o RVOT aneurysm o Pulmonary regurgitation: grade and Doppler flow analysis (CFM, PW or CW)

Bidirectional Glenn shunt (cf separate protocol)

HYPOPLASTIC LEFT HEART SYNDROME

Pre-operative/pre-interventional assessment ANATOMY AND HEMODYNAMICS
Abdominal aorta (subcostal sagital view with optimal angel for pulsed Doppler tracing) o Colour Doppler and PWD at the level of the aorta Pulmonary venous connection (subcostal and apical 4CH view) o Look for all four pulmonary veins o Flow: Laminar, turbulent (color Doppler, PWD of the veins)

Atrial communication (subcostal 4CH and bicaval view)

o o o o Size Not seen Restrictive (L-R mean gradient over respiratory cycle 3 cardiac cycles) No atrial or restrictive ASD, think of presence of levocardinal vain decompressing the LA to the innominate vein

Mitral (left AV) valve (apical 4CH view) o Absent connection o Atresia o Stenosis (MV annulus in borderline ventricle, z-score) Tricuspid (right AV) valve (apical 4CH view) o Anatomy o Annulus (TV annulus in borderline ventricle) o Tricuspid regurgitation (grading) Left ventricle (apical 4CH view) o o o o Length (LV-l : RV-l ratio in borderline ventricle) LV Volume (mod. Simpson in borderline ventricle) Fibroelastosis (EFE) Coronary sinusoids

Right ventricle (apical 4CH + parasternal LAX/SAX view) o Length (LV-l : RV-l) o Systolic function (qualitative) Aorta (parasternal LAX) and AO arch (suprasternal) o o o o o Absent ventriculo-arterial connection Atresia Stenosis (AO annulus) Arch size (ascending aorta, transverse arch, isthmus) and flow direction Look for coarctation (posterior shelve)

Pulmonary artery + PDA o o o o Pulmonary regurgitation PDA patency (restrictive, non-restrictive) and shunting (systolic R-L) Proximal RPA size + flow to distal RPA Proximal LPA size + flow to distal LPA

3-D ECHOCARDIOGRAPHY
o Obtain for LV volumes in borderline case + RV volumes and function (apical 4Ch view) FUNCTIONAL ASSESSMENT - RV systolic function - Visual assessment - RV diastolic function - TV inflow - Hepatic veins - TDI basal lateral RV wall

Postoperative assessment
I. Hybrid procedure

ANATOMY AND HEMODYNAMICS

Abdominal aorta (subcostal sagital view with optimal angel for pulsed Doppler tracing) o PW antegrade systolic + retrograde diastolic steal from duct Tricuspid (right AV) valve (apical 4CH view) o Tricuspid regurgitation (grading) Right ventricular systolic function (apical 4CH + parasternal LAX/SAX view) o Systolic function (qualitative) o Tissue Doppler Imaging (apical 4CH, 3 cardiac cycles, FR>160 fps) One clip to be stored for off-line analyses SR and Strain Pulsed TDI from right lateral and septal annulus Stent within PDA o Evaluate position of stent in the duct o View entire stent within PDA: especially look at aortic and pulmonary end o Use CFM, PW or CW Doppler to assess direction of flow through the duct and gradient Pulmonary branches (parasternal LAX/SAX view, espec. suprasternal views)

o Identify position of band of pulmonary arteries (2-D, CFM) o Assess gradients across the bands (LPA and RPA) by CW Aorta (parasternal LAX) and AO arch (suprasternal) o Look at retrograde flow from descending aorta to arch o If there is a reversed shunt from PA to aorta, assess flow through the shunt, gradient (CFM, CW)

3-D ECHOCARDIOGRAPHY
o RV volumes and function (apical 4-Ch view) FUNCTIONAL ASSESSMENT - RV systolic function - Visual assessment - RV diastolic function

- TV inflow - Hepatic veins - TDI basal lateral RV wall

Stage I NORWOOD ASSESSMENT

ANATOMY AND HEMODYNAMICS

Abdominal aorta (subcostal sagital view with optimal angel for pulsed Doppler tracing) o Antegrade systolic + retrograde diastolic steal (in mB-T) Tricuspid (right AV) valve (apical 4CH view) o Tricuspid regurgitation (grading) Right ventricular systolic function (apical 4CH + parasternal LAX/SAX view) o Systolic function (eyeballing, M-mode) o Tissue Doppler Imaging (apical 4CH, 3 cardiac cycles, FR>160 fps) One clip to be stored for off-line analyses SR and Strain) Pulsed TDI from right lateral and septal annulus Neo-aorta (parasternal LAX/SAX view, suprasternal views) o o o o o Neo-aortic regurgitation (grading) Coronary aorta size Aortic arch size (z-score) Aortic isthmus (distal patch) size Antegrade Doppler flow velocity: peak and mean systolic (only) gradient

Pulmonary branches and B-T shunt / RV-PA conduit (parasternal LAX/SAX view, suprasternal views)

B-T shunt velocity (peak and mean systolic-diastolic gradient) RV PA conduit (Sano) CW Doppler gradient Distal RPA size + flow Proximal RPA (medial to B-T shunt or just right and left from Sano shunt) size + flow o Distal LPA: size + flow o o o o

3-D ECHOCARDIOGRAPHY
o RV volumes and function (apical 4-Ch view) FUNCTIONAL ASSESSMENT - RV systolic function - Visual assessment - Clip for 2-D strain - TDI clip for TVI - RV diastolic function

III. POST- STAGE II (Bidirectional Glenn) ASSESSMENT

Abdominal aorta (subcostal sagital view with optimal angel for pulsed Doppler tracing) o Abdominal aortic flow (CFM + PWD) Tricuspid (right AV) valve (apical 4CH view) o Tricuspid regurgitation (grading) Right ventricular systolic function (apical 4CH + parasternal LAX/SAX view) o Systolic function (eyeballing, M-mode) o Tissue Doppler Imaging (apical 4CH, 3 cardiac cycles, FR>160 fps) One clip to be stored for off-line analysis SR and Strain Pulsed TDI from right lateral and septal annulus Neo-aorta (parasternal LAX/SAX view, suprasternal views)

o o o o o

Regurgitation Coronary aorta size Aortic arch size Aortic isthmus (distal patch) size Antegrade Doppler flow velocity: peak and mean systolic (only) gradient

Pulmonary branches (parasternal LAX/SAX view, suprasternal views)

o Distal RPA o Proximal RPA (medial to Glenn anastomosis) o Distal LPA

o Glenn anastomosis patency, SVC flow characteristics

3-D ECHOCARDIOGRAPHY
o RV volumes and function (apical 4-Ch view) FUNCTIONAL ASSESSMENT - RV systolic function - Visual assessment - Clip for 2-D strain - TDI clip for TVI - RV diastolic function

EBSTEINS ANOMALY
Preoperative assessment ANATOMY AND HEMODYNAMICS
Determine situs and venous anomalies as per standard protocol- exclude isomerism/other situs anomalies. Be aware that Ebsteins anomaly may affect the systemic AV-valve in double discordance. IVC, SVC, HV Assess flow in IVC, SVC, HV using CFM and PWD. Note: this may not provide an estimation of the degree of TI if TI- jet is directed to the posterior wall of RA

Abdominal aorta Assess aortic flow in abdominal aorta

Tricuspid valve (apical 4CH, subcostal, PSLAX-RV inflow, PSAX) Measure the distance between the offset of TV and MV ( apical 4-CH). Confirm displacement of the septal TV leaflet by calculating the displacement index. A value >0.8cm/m2 BSA is diagnostic for Ebsteins anomaly In Ebsteins anomaly part of the TV (septal /posterior leaflet) are displaced into the RV. The portion of the RV between the true (original) tricuspid annulus and the functional annulus (orifice of the displaced TV) is called the atrialized portion of the RV. In the severe end of the spectrum, the functional RV consists only of trabecular and outflow components. In TV dysplasia the valve is not displaced. If this is not feasible or if the septal TV leaflet is missing confirm displacement of the posterior TV leaflet (PSLAX-RV-inflow). Assess septal TV leaflet (apical 4-CH) Degree of displacement (see above) Dysplasia and thickening Tethering (abnormally short chordae ) Rare variants: - Just cauliflower like remnants of septal TV leaflet - Absence of septal TV-leaflet - Assess posterior TV-leaflet ( PSLAX-RV-inflow, subcostal SAX) -

- Check for the same as mentioned for septal TV leaflet - Be aware that an adequate size of this leaflet allows at least a bifoliate repair - Assess anterior TV leaflet ( PSLAX-RV-inflow, PSAX, subcostal SAX) regarding - Proximal attachment ( PSLAX-RV-inflow, apical 4-CH) - Distal attachments ( subcostal SAX) to anterior papillary muscle focal attachment or to short chordae inserting at the RV-wall if 3 or more tethering of the anterior TV-leaflet or to a muscular shelf between inlet and trabecular part of the RV linear attachment - Elongation, sail-like appearance (PSAX) - Mobility (PSLAX-RV-inflow) - usually restricted in case of either tethering or linear attachment - Be aware that mobility of the anterior leaflet is crucial for TV repair - Presence of fenestrations (=accessory orifices)(subcostal SAX colour) - Assess TV function (PLAX-RV-inflow/outflow, PSAX, apical 4-CH, subcostal 4-CH) - The opening of the tricuspid valve is often displaced towards the RVOT. This is important when assessing TV stenosis/regurgitation - Measure the size of the true (= original) tricuspid annulus (apical 4-CH) - Measure the size of the mitral valve annulus (apical 4-CH) - Measure area of true RA, atrialized RV and true RV - Grade TI - Note: use low PRF and Nyquist limits to optimize visualization and measurement of low-velocity regurgitation jets - Look also for regurgitation jets from fenestrations - Measure velocity across TV - Note: take into account that velocity may be also increased because of concomitant TI - Calculate peak and mean diastolic transvalvular pressure gradient - Calculate systolic RV-RA gradient and right ventricular pressure - Note: in right heart failure mean atrial pressure will be increased

RA (apical 4CH, PLAX-RV-inflow, PSAX) - RA-size (2-D) - Think of thrombi in RA ( right atrial appendage) as there is usually low flow in the RA

ASD /PFO (subcostal 4-CH, bicaval view, PSAX) - Determine presence of intra-atrial communication: location + size - Determine direction of shunt (L>R or R>L) Assess RV ( PLAX, apical 4-CH, PSAX, subcostal short axis) RV dimension (M-mode, 2D) and function (subjective) Check for aneurysmal dilatation of RVOT Check for right ventricular thinning Check for dyskinesis Check for non-compaction

Pulmonary valve, main pulmonary artery and branches ( PLAX-RV-outflow, PSAX, suprasternal, ductal-view) - Measure PV annulus and proximal RPA and LPA diameters - Exclude stenosis (neonates may present with either true or functional atresia of PV-in case of latter look for PI-Doppler trace indicating that the valve is open) - Exclude PDA Left atrium and ventricle (Apical 4CH, PLAX, PSAX), LVOT, AoV Usually on the smaller side in Ebsteins anomaly LA-dimension (2-D) LV size / volume, wall thickness and function (SAX M-mode and/or 2D) Describe movement of IVS ( M-mode)-usually paradoxical Be aware that RV measurements on M-mode are in fact measurements of the atrialized RV Obtain 3-D volumes when appropriate Exclude non-compaction Exclude MV prolapse, mitral stenosis Assess LVOT and Ao Valve

Pulmonary veins

- Check drainage of all pulmonary veins: use crab view and other windows to identify the pulmonary veins - Color-Doppler the pulmonary veins SVC and AOA (Suprasternal) Confirm RSVC and bridging vein Exclude LSVC Assess sidedness of aortic arch and arch vessels Exclude CoA

Rule out other associated lesions: VSD, mitral valve disease ( prolapse, mitral stenosis), AVSD ( double orifice mitral valve). In l-loop transposition Ebsteins anomaly affects the systemic atrioventricular valve, therefore having important prognostic relevance.

3-D Echocardiography
ALWAYS Obtain 3-D datasets for offline analysis Apical 4-chamber view Subcostal views FUNCTIONAL ASSESSMENT - LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - RV systolic function - Visual assessment

Post-operative Assessment
ANATOMY AND HEMODYNAMICS Surgical options in Ebsteins anomaly range from simple ASD closure to either repair or replacement of TV with/without RV plication. Depending on RV-size and function a concomitant bidirectional cavopulmonary shunt may be performed. Assess flow in abdominal aorta

Abdominal aorta Assess aortic flow in abdominal aorta

ASD-closure (subcostal -4CH, bicaval view) - Evaluate integrity of interatrial septum (2D) - Residual shunt - direction and velocity: between LA and RA (PW/CW/colour Doppler) Right atrium - Measure RA size (2-D) - Exclude thrombi in RA Tricuspid valve (Apical + subcostal 4CH, PSAX, PLAX-RV inflow) TV-repair - Evaluate integrity of valve repair (PW/CW/colour Doppler) - Determine location and severity of residual TV regurgitation (Colour Doppler). Specify location of regurgitant jet(s) (isolated, multiple) - Measure residual TV regurgitant jet gradient to calculate RV pressure (PW/CW Doppler) - Exclude TV stenosis - mean and peak gradient (Colour Doppler, PW/CW Doppler) TV-replacement - Measure transvalvular velocity, calculate peak and (PW/CW/colour Doppler) in at least three consecutive cycles - Exclude paravalvular leaks - Exclude thrombi - Exclude iatrogenic membranousVSD mean gradient

RIGHT AND LEFT VENTRICLE (PLAX, apical 4-CH, subcostal short axis, PSAX- M-mode) Right and left heart size, wall thickness and LV function Visual assessment of RV function Exclude dyskinesis ( RV, IVS, LV) Describe thinning if present Describe aneurysmatic RVOT if present

Mitral valve (Apical + subcostal 4CH, PSAX, PLAX) - Measure transvalvar velocity (PW/CW/colour Doppler) - Exclude MV regurgitation (Colour Doppler, semi objective grading) OUTFLOW TRACTS (Apical 5+3CH, PLAX, PSAX) - Assess RVOT (Colour Doppler, PW/CW Doppler) - Assess LVOT (dynamic obstruction due to septal movement?) - Measure velocity across AoV PULMONARY ARTERIES - Assess pulmonary artery size - Measure velocity across PV (PWD) - Demonstrate flow in RPA, LPA (colour Doppler), measure proximal RPA and LPA - In case of BCPC: assess as described AORTIC ARCH - assess aortic flow (colour Doppler) - In case of BT-shunt; assess as described there

3-D Echocardiography
ALWAYS Obtain 3-D datasets for offline analysis Apical 4-chamber view Subcostal views FUNCTIONAL ASSESSMENT - LV systolic function - Ps SAX M-mode: LV + RV dimensions, FS - RV systolic function - Visual assessment