Performing a Comprehensive Transesophageal Echocardiographic Examination in Children and All Patients with Congenital Heart Disease

1. Physicians wishing to perform TEE in children and all patients with CHD should:

1. TEE demonstrates a favorable safety profile and low complication rate in children and all patients with CHD.
2. Most complications are related to respiratory compromise or vascular compression. Rare serious complications include esophageal perforation, gastric laceration, and subglottic stenosis.
3. While general anesthesia is typically employed for the performance of TEE in children, TEE in the adult with CHD is routinely performed with conscious sedation. Anesthesia assistance should be considered in the adult with Fontan physiology, unrepaired or palliated cyanotic cardiac lesions or severe pulmonary hypertension.

1. TEE probe selection is primarily based upon patient weight and probe size. 2. TEE probe insertion should be performed with adequate sedation/anesthesia, and after an appropriate period of fasting. Careful monitoring for complications is essential.
3. TEE probe rinsing and high-level disinfection should be performed after each use, according to manufacturer’s instructions and institutional laboratory policy.

1. We recommend a series of 28 tomographic views be used to perform a comprehensive TEE examination in children and all patients with CHD.
2. The views are obtained from four primary positions within the esophagus/stomach: midesophageal (ME), transgastric (TG), deep transgastric (DTG), and upper esophageal (UE). All views are presented with the standard ‘‘apex up’’ orientation except for the DTG views, which are inverted to an ‘‘apex down’’ position to present an ‘‘anatomically correct’’ display.
3. The views serve as a starting point for CHD evaluation, and can be altered as needed. The exam should be structurebased, not view-based, and include a minimum of 2D imaging, color flow and spectral Doppler, obtained from multiple views.
4. 3D TEE imaging can be performed in conjunction with 2D TEE in patients > 30 kg, and involves the use of specific 3D techniques including adjustable sector, full volume, and live multiplane methods. Table 6 lists a number of clinical uses for 3D TEE in CHD.

1. Primary causes of MV dysfunction include congenital abnormalities, myxomatous changes, infectious damage from endocarditis, or inflammatory disorders. The ME views (4-Ch, 5-Ch, Mitral, 2-Ch, LAX, and AoV LAX) from 0 - 140 are most helpful to delineate MV anatomy and physiology but TG and DTG are especially valuable for congenital abnormalities. Color flow and spectral Doppler aid in return to bypass decisions following MV repair.
2. Assessment of the entire AoV complex includes the LVOT, AoV, aortic root and supravalvular region. Both ME SAX and LAX views provide complete evaluation of AoV and LV outflow tract morphology and function. TG LAX and DTG 5-Ch views are very useful for assessment of PW/CW Doppler gradients.
3. The ascending and descending thoracic aorta are best visualized systematically using a combination of ME and UE views, at different levels of the esophagus, with appropriate transducer rotation to visualize the various portions of the thoracic aorta.
4. Tricuspid valve abnormalities may be anatomical, such as Ebstein anomaly, TV dysplasia, stenosis, pacemaker leadinduced non-coaptation, flail leaflet, straddling or over-riding in some complex CHD; or functional, secondary to right or left-sided ventricular dysfunction. ME views provide assessment of TV morphology and function.
5. The pulmonary valve complex includes the sub-valvular, valvular and supra-valvular regions. ME, DTG, and UE Ao Arch SAX/PA views allow visualization of the PV complex. Color flow and spectral Doppler can be used to assess the degree of stenosis or regurgitation across this area. Due to the distance of the PV from the esophagus, 3D TEE is often limited.
6. 3D TEE provides further evaluation of valve size, pathology, mechanism and severity of valve dysfunction

1. Measurement of LV size is recommended in the TG SAX views in children because the TG LAX view does not account for the lateral motion of the LV seen in many children.
2. LV systolic function is generally assessed qualitatively but can be quantified with linear shortening fraction and 2D/3D volumetric ejection fraction.
3. Assessment of RV size and function is generally assessed qualitatively but 3D volumetric programs do allow for quantitative assessment.

1. Determination of atrial situs can be performed by identification of the atrial appendages and key systemic venous structures. While the ME views are most commonly used, all TEE positions and views should be employed.
2. TEE can identify unusual systemic venous anomalies such as left SVC to coronary sinus or left atrium, interrupted hepatic portion of the IVC, and atrial anomalies such as juxtaposed atrial appendages and cor triatriatum.
3. TEE provides excellent visualization of normal left and right pulmonary venous return and enables accurate pulmonary venous Doppler assessment, which can be utilized in the evaluation of stenosis within individual veins or the pulmonary venous confluence.
4. TEE can also be used to evaluate partial and total anomalous pulmonary venous return. However with the latter, care must be taken because hemodynamic compromise has been described with TEE probe insertion/manipulation.

1. Atrial septal defect (ASD) subtypes include secundum, primum, sinus venosus, and coronary sinus defects.
2. Ventricular septal defect (VSD) subtypes include perimembranous, inlet, muscular, outlet, and malalignment (the last is associated with conotruncal defects).
3. TEE plays an important role in monitoring of ASD and VSD closures in the catheterization laboratory.
4. TEE is important for postoperative assessment of VSD closure, especially for determination of significant residual VSDs, outflow tract obstruction, and aortic regurgitation, as well as whether return to bypass is indicated.
5. TEE is indicated preoperatively in essentially all patients with AVSD, but particularly in the older infant/child in whom surgical decision-making might require delineation of the degree and mechanism of AV valve regurgitation, mechanism of outflow tract obstruction, and or suitability for biventricular correction.
6. 3D TEE is useful for assessment of ASD and VSD morphology, and for monitoring of ASD/VSD closure in the catheterization laboratory. 3D TEE can also facilitate preoperative assessment of atrioventricular septal defects.

1. Imaging of the coronary artery origins is primarily done in the ME SAX views.
2. The demonstration of anomalous origin of a coronary artery from the opposite sinus of Valsalva requires careful interrogation with both 2D imaging and color Doppler.
3. In ALCAPA and ARCAPA, the anomalous origin of the coronary artery from the pulmonary artery can be visualized using the ME views.
4. Segmental wall motion abnormalities, ventricular dysfunction and mitral regurgitation should be assessed.

1. Conotruncal defects include TOF, DORV, truncus arteriosus, d-TGA and l-TGA.
2. Anatomic assessment should include great artery positioning (anteroposterior, mediolateral), as well as conal septum location (sub-pulmonary, subaortic or bilateral), position (anterior or posterior), and size (hypoplastic or absent). Associated VSDs can be classified as subaortic, subpulmonary, remote or doubly committed.
3. For all conotruncal defects, TEE allows for pre- and post-operative evaluation of outflow tract patency/obstruction, residual VSDs (including intramural VSDs), residual atrial level communications, AV or semilunar valve insufficiency, and ventricular function. A combination of ME, TG, and DTG views should be used for evaluation.
4. 3D TEE can be used to provide further elucidation of the VSD/outflow tract anatomy and semilunar valve morphology/function. Where applicable, intra-atrial baffle obstructions or leaks can also be evaluated by narrow sector live 3D, biplane echo, or full volume acquisition.

1. The role of TEE in the complex single ventricle will depend upon the underlying anatomy and also the stage of surgical palliation: (1) Damus-Kaye-Stansel (DKS) or Norwood procedure with modified Blalock-Taussig shunt or Sano shunt; (2) superior cavopulmonary (Glenn) procedure; (3) total cavopulmonary (Fontan) operation.
2. The DKS anastomosis is best viewed in the ME AoV LAX and ME Asc Ao LAX views and lies just above the semilunar valves. Visualization and assessment of the shunt and branch PAs is often difficult, but can be achieved using the UE Ao arch LAX, Ao arch SAX, and PA views.
3. Visualization of the Glenn anastomosis is often difficult with TEE but it does offer the ability to assess for any narrowing or turbulence at the anastomotic site to the PA. The best views are in the ME Asc Ao LAX and UE Ao arch SAX.
4. TEE in the Fontan patient allows for assessment of thrombus, residual Fontan fenestration, AV valve function and ventricular function. It is important to assess the Fontan pathway in both ME 4-Ch view and ME Bicaval view so that the entire conduit is visualized from IVC to PA.
5. Interventional device closure or creation of a Fontan fenestration is guided with both 2D and 3D TEE. 3D TEE can also be used to assess atrial and Fontan conduit thrombus and AV valve function.