Additional Applications of Transesophageal Echocardiography

Wong, Pierre C.

doi:10.1007/978-1-84800-064-3_16

Pierre C. Wong MD³

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2 Citations

Abstract

While the majority of this book addresses the use of transesophageal echocardiography (TEE) for the evaluation of patients with congenital heart disease (CHD), there are a number of other conditions in which TEE can play a significant role in pediatric and young adult patients. Some conditions, such as infective endocarditis, can occur in patients with a history of CHD. Other pathologies, such as cardiac tumors, can be seen in the absence of coexisting CHD. This chapter addresses additional applications of TEE in the pediatric and young adult population.

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Other Applications, Including the Critical Care Setting

Indications and Guidelines in Pediatric and Congenital Heart Disease

Echocardiography in Patients with Adult Congenital Heart Disease

Keywords

Introduction

While the majority of this book addresses the use of transesophageal echocardiography (TEE) for the evaluation of congenital heart disease (CHD), there are other conditions in which TEE can prove beneficial in pediatric and young adult patients with and without CHD. This chapter discusses some of the more common clinical scenarios in which this might occur, and how TEE can contribute useful (and sometimes invaluable) additional information. Some situations will require TEE to be performed both in and out of the intraoperative setting.

Infective Endocarditis

Infective endocarditis (IE) is defined as a microbial infection of the endocardial (endothelial) surface of the heart, primarily involving the valves but also affecting other endocardial surfaces, including any prosthetic or foreign material in the heart. Infection can also occur on the endothelial surfaces of the great vessels (including the great arteries), and in this case it is known as infective endarteritis. Infective endocarditis can be caused by a number of different organisms, mainly bacteria and fungae (Table 16.1), and the clinical course ranges in severity from indolent to fulminant [1, 2]. Despite the fact that IE begins as an infection of the endocardial surface, the infectious process can be highly invasive, causing extensive destruction of heart valve and surrounding tissue, as well as intramyocardial abscesses. Some of the most severe complications resulting from IE include congestive heart failure (due to valvar regurgitation), systemic and pulmonary embolic phenomena, sepsis, arrhythmias, myocardial failure, and death [3].

Table 16.1 Organisms commonly associated with infectious endocarditis

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A number of conditions can predispose to IE, including mitral valve prolapse, rheumatic heart disease, indwelling catheters, intravenous drug abuse, prosthetic valves, and both unrepaired and repaired CHD [1]. The role of congenital cardiac disease as a predisposing factor for IE continues to increase in developed countries; CHD now appears to be the predominant underlying condition for IE in children over the age of 2 years [4]. Certain congenital heart lesions are recognized to have an association with IE, including ventricular septal defects, patent ductus arteriosus, aortic valve abnormalities, and tetralogy of Fallot [5]. Furthermore, increasing numbers of children with IE have had previous palliative or corrective surgery for CHD [4, 6]. However, IE can occur in the absence of any structural heart disease; this is seen in approximately 2–5 % of younger pediatric IE cases (2 months to 15 years of age), and 25–45 % of older IE patients (15–60 years of age) [1, 7].

Because of the variability in clinical presentation, the diagnosis of IE is not always straightforward. To assist in diagnosis, a set of criteria—known as the Duke criteria—were proposed in 1994 as a diagnostic schema for patients with suspected IE [8]. Several refinements were made in 2000, resulting in the modified Duke criteria [9]. These criteria incorporate clinical, laboratory, pathologic and echocardiographic criteria, and stratify patients into three main categories—definite IE, possible IE, and rejected—based upon the presence of major and minor criteria (Tables 16.2 and 16.3). “Definite” IE is defined by the presence of two major criteria, or one major and three minor, or five minor criteria. “Possible” IE is defined by one major and one minor criterion, or three minor criteria. “Rejected” indicates absence of evidence supporting the diagnosis of IE. For diagnosis, microbiologic confirmation (as demonstrated by positive blood cultures) is a principal criterion. However, the Duke criteria clearly recognize and acknowledge the importance of echocardiography (with special mention of TEE) in the diagnosis of IE, as evidenced by the inclusion of echocardiographic findings as a major criterion for diagnosis. Indeed, echocardiography is now considered essential in the diagnostic workup and continuing evaluation of IE [10]. The utility of the Duke criteria have been validated by a number of studies in both the adult and pediatric populations [11–14]. They are now widely accepted guidelines for the evaluation of possible IE, both in adults as well as children [4, 15, 16].

Table 16.2 Modified Duke criteria: definition of terms used for the diagnosis of infective endocarditis (IE)

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Table 16.3 Definition of endocarditis from modified Duke criteria

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The full diagnostic evaluation and management of IE encompasses a wide variety of aspects, including microbiology, antimicrobial therapy/duration, ongoing assessment, extracardiac complications, and indications and timing of surgical management [1, 17]. Therapy of IE is aimed at (a) eradication of the infecting microorganism; (b) treatment of cardiac complications; (c) prevention and/or treatment of extracardiac (particularly embolic) complications [1]. Antimicrobial therapy (selection/duration) is based upon the microbiology and susceptibility of the causative organism, and continues to evolve as new therapeutic agents are introduced. The role of surgery for IE also continues to evolve, and it is clear that this role has expanded; a recent European survey revealed that >50 % of adult patients with IE underwent surgery [18]. Controversy still exists as to the exact indications and timing for surgery; in most instances surgery is performed for left sided endocarditis, severe valvular involvement, and/or evidence of embolic phenomena [19]. Recently, there has also been published evidence that early surgery for severe left sided endocarditis in adults results in significantly better outcomes than conventional medical therapy [20]. Proposed indications and timing of surgery in adults are listed in Tables 16.4 and 16.5 [19]. In children, the indications for surgery are not as straightforward; surgery tends to be performed less frequently because 80–90 % of children with IE are expected to survive solely with conservative medical treatment [21, 22]. Nonetheless several published series of IE in children have reported an important percentage (between 16–67 %) of cases that required surgical intervention—in patients with and without CHD. In most published reports, the indications for cardiac surgery in pediatric IE patients tend to be similar to those of adults—valve dysfunction, congestive heart failure, septic embolization, and large vegetations. In addition, surgery is performed in CHD patients to repair the underlying cardiac defect (if still present), and also to remove or replace infected prosthetic material such as homografts and aortopulmonary shunts [7, 18, 21, 23–25].

Table 16.4 Indications for surgery in IE

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Table 16.5 Timing of Surgery

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Despite advances in medical and surgical management, IE remains a serious medical condition, with important risks of morbidity and mortality. A thorough and complete review of this multifaceted topic, including diagnosis, antimicrobial considerations, and medical/surgical therapy, would require its own separate chapter. For the purposes of this chapter, it is important to understand the integral role that echocardiography—and particularly TEE—plays in the many different aspects of the diagnosis and management of IE. This will be discussed below.

Echocardiographic Manifestations of IE

There are several ways in which IE can appear by echocardiography. The principal echocardiographic manifestations of IE include the following.

Vegetations

Vegetations are the most characteristic finding associated with endocarditis. They represent a mass of pathologic organisms nestled within a weave of platelets, red blood cells, and fibrin. Vegetations often develop in an area where the endothelium has been injured or disrupted by an abnormal high velocity jet or intravenous catheter; this usually occurs on a valvar surface, but can also be seen on a cardiac chamber wall when the endothelial surface has been injured. Vegetations also have the propensity to develop on foreign material such as a prosthetic valve or patch. The surface of the damaged endothelium or prosthetic material serves as a nidus for platelet/fibrin deposition, producing a thrombus (vegetation) at the site. This vegetation is at first sterile, but with bacteremia, circulating microorganisms can become adherent to the meshwork, resulting in an infected vegetation. Infection triggers further deposition of platelets and fibrin over the microorganisms; the organisms embedded within the vegetation are then shielded from host defense mechanisms, allowing them to proliferate rapidly and produce further growth of the vegetation [26]. Vegetations can have a number of detrimental effects: (a) they can grow and destroy adjacent tissue; (b) organisms can be released continuously into the bloodstream, leading to persistent bacteremia and hematogenous seeding of remote sites; (c) pieces of the vegetation can break off and embolize to other organs (brain, lung, kidney), sometimes producing serious and even devastating complications; (d) antibody response to the infecting organisms leads to subsequent tissue injury by immune complex deposition [1].

By echocardiography, vegetations are echogenic masses, generally irregular in shape and variable in size. They can localize anywhere on an affected valve or nonvalvar structure, though they tend to arise on a valve or endothelial surface “downstream” to a high velocity jet, e.g. adjacent to a ventricular septal defect or valvar regurgitant jet. They are usually freely mobile, oscillating with the cardiac cycle, and can move back and forth within the plane of a valve. Intracardiac vegetations are generally very well seen by TEE (Fig. 16.1, Video 16.1).

Valvar Dysfunction

The echocardiographic manifestations of valvar dysfunction include ruptured chordae with prolapsing or flail leaflets, fenestrations in the valve cusps, and torn leaflets. All of these lead to disruption of valvar function and resultant valvar regurgitation, often to a significant degree. The amount of regurgitation, and the area of origin, can be well-seen using color flow Doppler. It is not uncommon to see vegetations in association with valvar disruption, as evidence of the destructive process from IE. Such patients not only appear toxic from their infection, but might also suffer symptoms of congestive heart failure if valvar incompetence is significant. Examples of valve disruption and accompanying vegetation are shown in Figs. 16.1 and 16.2, Videos 16.1 and 16.2.

Intracardiac Abscesses

Intracardiac abscess formation results from suppurative extension of the infective process. Purulent cavities form as the infection spreads into adjacent tissue. Most commonly, this occurs with native aortic valve IE, as infection extends into the weakest portion of the annulus—the membranous septum and atrioventricular node. When this occurs, heart block is a frequent sequela. Perivalvar abscess formation occurs in 10–40 % of all native IE; as noted, it is most common in native aortic valve IE, less common with native tricuspid or mitral IE [10]. Perivalvar abscesses are seen even more frequently with prosthetic valve IE, occurring in 56–100 % of patients [10, 27]. The characteristic echocardiographic appearance of abscess formation is as an echo-free space, representing purulent fluid, either within the wall surrounding the affected valve (e.g. the aortic root in aortic valve endocarditis), or extending into the adjacent tissue. In those patients with an abscess surrounding a prosthetic mitral valve, valve dehiscence is often seen. For the evaluation of abscesses, TEE has been shown to improve sensitivity dramatically compared to transthoracic echocardiography (TTE), and it is the preferred modality for diagnosis of perivalvar abscesses [28]. An example of an abscess that formed around a prosthetic aortic valve is shown in Fig. 16.3, Video 16.3.

Aneurysm Formation/Fistulous Tracts

If there is extension of the infection to the adjacent vessel wall, destruction of wall tissue can occur, leading to thinning and aneurysm formation. This can be seen especially with aortic valve IE, in which fistulous tracts can form between the sinus of Valsalva and an adjacent cardiac structure (i.e. an infected sinus of Valsalva aneurysm), or a fistulous tract can form into the pericardial space [29, 30]. In this setting, TEE will demonstrate the aneurysmal dilation of the vessel wall, and color flow Doppler will show systolic/diastolic (or continuous) flow between the aorta and the receiving chamber (Figs. 16.3 and 16.4, Videos 16.3 and 16.4). Infective endarteritis and pseudoaneurysm formation can also occur, particularly in areas of foreign material such as suture lines and biologic grafts [31, 32] (Fig. 16.5, Video 16.5).

Congestive Heart Failure/Pericardial Effusion

Congestive heart failure (CHF) is a known complication of IE, and one associated with a poor prognosis [33–35]. It can occur as a result of several different complicating processes associated with IE: native valve destruction/perforation/chordal rupture, prosthetic valve dehiscence, abscess formation causing heart block, sudden intracardiac shunts due to fistulous tracts, and septic emboli to the coronaries causing myocardial ischemia/infarction [10, 19]. In addition, myocardial dysfunction can be seen with IE, either from myocardial toxicity due to the IE process (e.g. large abscess), ventricular decompensation from valvar regurgitation, or the overall septic process causing generalized depression of myocardial function (particularly with a virulent organism such as Staphylococcus aureus). In native valve IE, acute CHF is more frequently seen with left sided infections—aortic (29 %) and mitral (20 %)—than with tricuspid infections (8 %) [10]. A pericardial effusion can also be seen in patients with IE; it can be infectious, resulting from hematogenous seeding of the pericardium or as a direct extension from intracardiac IE (e.g. perforation of a perivalvar abscess). Rarely, it can occur as a reactive/serous effusion [36].

Use of TEE for Evaluation of Infective Endocarditis

As noted above, echocardiography is considered essential in the diagnosis and management of IE. The major question is whether to perform TTE or use TEE to evaluate for vegetations. In adults, a number of studies have confirmed the superior diagnostic sensitivity of TEE over TTE for IE [37–40]. This is particularly true in those patients with intracardiac abscesses and prosthetic valve endocarditis, in whom TEE appears clearly superior for diagnosis [28, 41]. In children, the advantages of TEE over TTE are less apparent, particularly with the infant and younger child, in whom TTE generally provides excellent imaging. For younger pediatric patients, TTE is usually adequate for the diagnostic evaluation of IE [42]. In most pediatric patients, particularly the younger age groups, the evidence suggests that TEE can be reserved for those patients in whom imaging quality is felt to be suboptimal. A recent study in pediatric patients ages 10 days to 17.5 years (over half of whom had CHD) showed that TTE, when used in conjunction with the Duke criteria, was useful for the diagnosis of IE; the authors recommended TEE only for an “inadequate” TTE study or when there was an organism with a high association of aortic root abscess [43].

The evaluation of IE by TEE can occur in several settings. It can be performed in the ICU or ambulatory setting, serving as either diagnostic evaluation for suspected IE or as a monitoring procedure for a patient receiving treatment for known IE. It also plays a vital role in the operating room. Preoperatively, TEE is used to assess not only the vegetation or abscess, but also valvar function and the surrounding cardiac structures. When applicable, prosthetic valve dehiscence and pseudoaneurysm formation can also be evaluated. Postoperatively, TEE is used to assess the results of operative repair or valve replacement, and to guide perioperative hemodynamic management [19, 36].

If TEE is performed to evaluate for IE, a complete study should be performed using all major TEE views/windows, as outlined in Chap. 4. Assessment should focus on a number of details. If a vegetation is present, its appearance and motion should be evaluated in multiple planes, and linear measurements can be obtained. The risk of embolic events appears to be greatest with vegetations >10 mm on the anterior leaflet of mitral valve [44]. Valve leaflet anatomy and motion should also be evaluated both by imaging and color flow Doppler, with attention paid to any valvar perforation or chordal disruption. In the case of aortic valve endocarditis, a careful evaluation should also be made for possible abscess or aneurysm formation. If a prosthetic valve is in place, a thorough assessment should be performed for vegetations, valve leaflet motion, and possible perivalvar abscess/dehiscence. Color flow Doppler is useful to determine valve competency and flow profile. A complete evaluation should be performed of other cardiac structures to rule out structural defects and/or other potential sites of IE, including the aorta and pulmonary artery (when the pulmonary artery can be visualized by TEE). Rarely, cases of endarteritis can also be found by TEE (Fig. 16.5, Video 16.5). Finally, myocardial function should also be assessed.

Three caveats are important to consider. First, even with TEE, not all vegetations will be visible, particularly if the vegetations are smaller than the resolution limits of the TEE probe and/or TEE imaging is suboptimal. This is an important consideration in patients with operated and unoperated CHD, who can have vegetations located in areas not readily visible by TEE (e.g. a Blalock-Taussig shunt). Studies have shown that—irrespective of whether TTE or TEE is used—patients with CHD and IE are less likely to have visible vegetations [5]. Thus, the echocardiographic data should be considered in the context of the entire clinical picture, as noted with the Duke criteria listed above. In some cases, if IE is still suspected, a TTE or TEE can be performed 7–10 days later to determine if a vegetation or abscess has appeared [10]. The second important caveat is that not all echogenic masses represent vegetations. Sterile thrombi, tumors, irregular valve excrescences, and foreign material (such as suture material) can sometimes resemble vegetations. Again, the echocardiogram should be reviewed in conjunction with the entire clinical picture. If previous echocardiograms are available (either transthoracic or transesophageal), these can be very useful to make direct comparisons to determine whether an abnormal finding is new or longstanding. New findings are much more suspicious for IE. The last important caveat is that not all vegetations are infectious. A number of medical conditions can produce sterile vegetations adherent to valvar surfaces. Examples of these include systemic lupus erythematosus (Libman-Sachs endocarditis), and nonbacterial thrombotic endocarditis (NBTE, also known as marantic endocarditis). The latter can occur as complication of malignancy, uremia, burns, hypercoagulable states, or autoimmune diseases, and it has been found in approximately 1.2 % of all autopsy patients, although the reported incidence is between 0.3–9.3 % [1, 45]. In fact, Libman-Sachs endocarditis is felt to be a form of NBTE [46]. These vegetations are usually seen on the valve closure contact line of the atrial surface of the atrioventricular (AV) valves and ventricular surface of the semilunar valves. In many cases, the vegetations are benign and clinically inapparent. However, systemic embolization has been described in up to 30–50 % of patients [45–47], with a tendency towards embolization to the brain, kidney, spleen, mesenteric bed, or extremities [46, 48].

Cardiac Masses

Aside from infective endocarditis, the most common pathologic cardiac masses are thrombus or tumors. The role of TEE in the diagnostic assessment of cardiac masses is generally complementary to TTE, particularly in pediatric patients. In most cases, TTE is more than adequate to evaluate the location and extent of a cardiac mass. However in older patients, and in those with poor echocardiographic windows, TEE might be called upon to assist in evaluation. For instance, TEE has become well established in adults as a requisite tool for evaluation of the etiology of ischemic strokes; approximately 15–20 % are caused by cardiogenic emboli (thrombi, tumors, vegetations, etc.) [49].

Cardiac Thrombi

Thrombi can vary considerably in their echocardiographic appearance and location. They are generally echogenic, relatively homogeneous in density, and irregular in shape. In some instances there can be calcification within the thrombus. They are generally attached to some structure in the heart, whether it is an endocardial surface, AV valve, or some type of foreign material. The attachment point is usually broad-based, but it can also be thin and pedunculated in appearance. In most clinical settings, thrombi are associated with an indwelling catheter, although in certain pathologic situations—e.g. where there is stasis of blood—they can arise spontaneously. An important example of this is dilated cardiomyopathy, in which reduced cardiac motion leads to stasis of blood flow and a propensity for thrombus formation, particularly near the apex of the left ventricle (Fig. 16.6, Video 16.6). In those situations in which a catheter is present, usually the superior vena cava (SVC) or right atrium, TEE can be used to evaluate the attachment point, size, and extent of the thrombus. Mid to upper esophageal views, with the probe rotated rightward and multiplane angle 80–95° (mid esophageal bicaval view), permit a sagittal visualization of the length of the SVC as it returns to the right atrium (Fig. 16.7, Video 16.7). If an indwelling catheter is present, it can be visualized and evaluated for a possible attached thrombus. It is important not to misinterpret normal structures or variants, such as the crista terminalis (the muscular ridge in the interior of the right atrium separating right atrial appendage from the remainder of the atrium), as an intracardiac thrombus. Color flow Doppler can evaluate the flow returning through the SVC to the right atrium. If there is obstruction to SVC return, a mean gradient can sometimes be obtained using the deep transgastric views (long axis and sagittal), rightward turning of the probe, and anteflexion to achieve posterior angulation (see Chap. 4). These maneuvers enable visualization of SVC flow as it enters the right atrium, and also provide an excellent angle of insonation for spectral Doppler interrogation.

Thrombi sometimes develop in the left (and less frequently, the right) atrial appendage in patients, particularly adults with severe mitral stenosis and/or atrial fibrillation [50, 51]. In adult studies, the incidence of left atrial thrombus in patients with atrial fibrillation ranges between 10–15 %, and right atrial thrombus 0.4–7.5 % [52]. These thrombi are often challenging to visualize by TTE [53]. It can also be difficult to distinguish these thrombi from pectinate muscles. In this setting, TEE proves invaluable because of the close proximity of the esophagus to both atria, providing excellent visualization of all atrial structures, including the atrial appendages [54, 55]. The left atrial appendage can be well visualized by TEE using the mid esophageal views and leftward rotation, with slight anteflexion to bring the appendage into view (Fig. 16.8, Video 16.8). Rotating the multiplane angle between 0° and 90° affords different views of the structure. The right atrial appendage is best seen from the mid esophageal bicaval view, multiplane angle 85–90°; it is seen anterior to the SVC/right atrial junction. The multiplane angle can also be rotated to 0° to visualize the right atrial appendage from a different plane. Again, it is important to inspect carefully both the right and left atrial appendages, and to distinguish pectinate muscles and multiple appendage lobations [56] from actual thrombus [54, 57].

There are certain normal anatomic structures that can resemble a thrombus, and must therefore be inspected carefully by TEE. As previously noted, the crista terminalis can occasionally be mistaken as a thrombus. A Chiari network can sometimes be confused for a thin thrombus or vegetation. This structure, an embryologic remnant of the right valve of the sinus venosus, is usually very mobile and can exhibit considerable anatomic variation [58]. It is attached to the Eustachian and Thebesian valves; it is thin and redundant and often displays a porous, fenestrated or filamentous appearance. A related structure, a prominent Eustachian valve, can be very prominent and extend into the right atrium. It can also be very mobile, and in some cases has the appearance of long vegetation or thrombus. Both a Chiari network and Eustachian valve are well seen using the mid esophageal bicaval view, with multiplane angle approximately 80–90°. In the left atrium, a prominent ridge between left upper pulmonary vein and left atrial appendage (sometimes informally called the “coumadin ridge”) can, on occasion, be mistaken for a thrombus. Another important artifact that may resemble an intracardiac thrombus is the presence of spontaneous echo contrast, or “smoke”, in an atrial chamber [50, 59, 60]. This represents red cell aggregation from sluggish blood flow, such as that seen in a low-flow Fontan circuit (Chap. 10). When dense and echogenic, it can produce the appearance of thrombus. However, the swirling and circular movements differentiate this type of finding from a thrombus (Fig. 16.9, Video 16.9).

Cardiac Tumors

Cardiac tumors are divided into two categories: primary and secondary/metastatic. Primary cardiac tumors originate in the heart, and most of these are benign [61–64]. However despite their “benign” histopathology, some primary tumors can still cause significant clinical sequelae due to arrhythmias, mass effect (obstruction), or embolization. Rarely, primary cardiac tumors are malignant and, if so, they are usually sarcomas and are almost always associated with a poor outcome [62]. The other class of cardiac tumors comprises the secondary/metastatic tumors that are, by definition, malignant. Secondary cardiac tumors occur mostly as distant metastases, but they can also invade the heart by direct extension, for example via the inferior vena cava (IVC). Secondary malignant cardiac tumors are seen much more frequently than primary cardiac tumors, both in adults and children. Indeed, in the adult literature metastatic tumors outnumber primary cardiac tumors by a 20–30 to 1 ratio, and in children, by a 3–4 to 1 ratio [61, 62]. In adults, the most common primary sites for metastasis to the heart include lung, breast, lymphoma/leukemia, esophageal, uterine and melanoma. Most of these metastases are to the pericardium, and 90 % are clinically silent [63]. In children, the most common tumors with metastasis to the heart are non-Hodgkin’s lymphoma, neuroblastoma, Wilms tumor, and soft tissue/bone sarcomas [61]. Direct extension of a tumor can occur from the IVC to the right atrium in patients with Wilms tumor [65], renal myosarcoma, or adrenal and hepatocellular carcinoma (Fig. 16.10, Video 16.10). As with adults, overt clinical signs and symptoms of secondary tumors are uncommon [61].

The echocardiographic presentation of cardiac tumors is highly variable, depending upon the type of tumor and the site of involvement. Cardiac tumors can involve only endocardium, only myocardium, only pericardium, or various combinations thereof [62]. There can be one or multiple foci, again depending on tumor type. An extensive discussion of cardiac tumors is beyond the scope of this chapter, and there are other excellent references available on the subject [62, 63, 66]. The remainder of this section will discuss a few of the more common primary cardiac tumors and provide examples of their evaluation by TEE. The method of TEE evaluation for these particular tumors can be applied to the evaluation of cardiac tumors of any etiology.

Rhabdomyoma

Rhabdomyomas are the most commonly encountered primary cardiac tumors in the pediatric age group, comprising approximately 60–80 % [67, 68]. Typically, multiple tumors are located within the free wall of the right and left ventricle, as well as the interventricular septum. They can also be present in the right atrium. There is a strong association (at least 50 %) with tuberous sclerosis. These tumors are histologically benign but nevertheless patients can be clinically symptomatic in a number of different ways. Depending upon location, atrial or ventricular arrhythmias can occur. Tumors of sufficient size adjacent to an area of inflow or outflow can cause significant obstruction, which occasionally necessitates surgical resection. However with time, spontaneous regression of these tumors usually occurs, so surgery is not indicated in most patients unless the tumors cause significant mass effect.

Given the generally benign nature of cardiac rhabdomyomas, and the young age that these tumors are first diagnosed, it is very rare that a TEE is necessary for evaluation; TTE generally suffices. The major indication for TEE is in patients undergoing surgery for relief of inflow or outflow tract obstruction. Rhabdomyomas are characteristically homogenous, echogenic, and well circumscribed; they are easily distinguished from the surrounding myocardium. The mid esophageal four chamber (ME 4 Ch) view (multiplane angle 0°) is the best view from which to begin evaluation of the tumors, both ventricular and (if present) atrial. Once located, they can be visualized more closely with variations in multiplane angle, along with anteflexion and retroflexion to bring the tumor(s) into view. If a large tumor is causing possible obstruction to AV inflow, color flow and spectral Doppler should be used to evaluate for disturbed flow patterns around the tumor, and to determine a mean gradient across the area. If the tumor is causing possible outflow tract obstruction, mid esophageal views such as the ME 4 Ch, mid esophageal long axis (ME LAX), and mid esophageal right ventricular inflow-outflow (ME RV In-Out) with multiplane angles varying between 0° to 110° are best to evaluate the anatomic extent of the tumor and any turbulence as seen by color flow Doppler (Fig. 16.11, Video 16.11). Deep transgastric long axis (DTG LAX) and deep transgastric sagittal (DTG Sagittal) views with probe anteflexion/retroflexion provide the best angle of spectral Doppler interrogation of the ventricular outflow tracts. The transgastric views also provide other perspectives from which to visualize both right and left sided tumors. These views include the transgastric basal short axis (TG Basal SAX), mid short axis (TG Mid SAX), two chamber (TG 2 Ch), long axis (TG LAX), and right ventricular inflow (TG RV In).

Fibroma

Fibromas are the second most common cardiac tumor encountered in the pediatric age group (10–30 %) [69]. They are generally solitary and intramural, located within the ventricular septum or left ventricular free wall [62]. When large, they can impinge significantly upon the adjacent cardiac chamber (usually the left ventricle), causing symptomatology such as congestive heart failure and cyanosis. Rarely, they can be multiple and involve other parts of the heart including the ventricular conduction system, right ventricle and right ventricular free wall. They frequently cause ventricular arrhythmias [70]. Because these tumors do not regress spontaneously and sometimes can grow significantly, surgical resection is recommended.

The TEE evaluation of fibromas generally occurs in the operating room setting, and is similar to that involved with the evaluation of other cardiac tumors. The tumors are best seen using the mid esophageal views, with a combination of multiplane angles between 0° and 100°. Color flow and spectral Doppler should be employed to evaluate for potential compromise of ventricular inflow or outflow due to the tumor. The echocardiographic appearance of a fibroma is that of a single, bright, echo-dense intramural mass with calcifications and cystic areas within the tumor (Fig. 16.12, Video 16.12).

Myxoma

Myxomas represent the most common primary cardiac tumor in adults [62, 71]. On occasion, they can also be seen in children. They are most commonly located in the left atrial cavity (75–90 %), but can also be found in the right atrium [72], as well as the ventricles [62, 73]. Rarely, they can be present in more than one cavity [74]. Clinical manifestations of a left atrial myxoma are quite variable and can include embolic phenomena and/or obstruction to blood flow causing breathlessness, syncope, and congestive heart failure. Other constitutional symptoms such as fever, malaise, weight loss, and myalgias/arthralgias can also be present. Familial occurrence is reported in approximately 7–10 % of all myxomas, generally in younger patients. These are associated with multiple endocrine syndromes including LAMB (lentigines, atrial myxoma, mucocutaneous myxoma, and blue nevi) and NAME (nevi, atrial myxoma, neurofibromata, and ephelides).

Myxomas are gelatinous in consistency and they can be pedunculated. By echocardiography, they have a globular, lobulated, or fimbriated appearance, and a pedicle can be present. There may be small lucencies and calcifications in the tumor, resulting in an inhomogeneous appearance. Left atrial myxomas characteristically have attachments to the atrial septum, but they can also attach to other portions of the left atrium or to the mitral valve. If large enough, the myxoma can cause obstruction to mitral inflow. Given the numerous potential complications, including embolus, the treatment for atrial myxomas is surgery, and TEE plays an important role in the pre and postoperative assessment [75]. The attachment point, the extent of the tumor, the number of tumors, and relationship to surrounding structures, should all be evaluated carefully prior to surgical resection. Following surgery, it is important for TEE assessment to include evaluation of the left atrium and interatrial septum, in order to determine completeness of tumor resection as well as the integrity of the mitral valve. Atrial myxomas are best visualized by TEE with the mid esophageal views, using several multiplane angles to determine the location and extent of the tumor (Fig. 16.13, Video 16.13). Color flow and spectral Doppler should be used to evaluate any obstruction to mitral valve inflow, and/or mitral regurgitation resulting from damage to the mitral valve leaflets or valve interference by the tumor.

Other Cardiac Tumors

Hemangiomas and teratomas are two other primary tumors that are occasionally encountered in the pediatric population. Hemangiomas can be located anywhere within the heart, but have a predilection for the right atrium and ventricular septum [76]. By echocardiography, there are numerous echolucent spaces, and color flow Doppler often demonstrates multiple vascular channels (Fig. 16.14, Video 16.14). Teratomas are usually detected in the fetus and neonate. They most commonly occur in the pericardial space, but can also arise within the heart, attached to the atrial or ventricular wall. When in the pericardial space, they are often attached to the aortic root or pulmonary trunk, and they can grow to a large size. There may be an associated pericardial effusion. If sufficiently large, either the tumor or the effusion (or both) can compress the heart, leading to tamponade physiology. When intracardiac in location, these can cause clinical signs and symptoms similar to other intracardiac tumors. By echocardiography, the tumors appear heterogeneous and encapsulated [67].

Role of TEE in the Evaluation of Cardiac Tumors

Most cardiac tumors are well seen by TTE, particularly in younger patients, and therefore TTE serves as the principal modality for their evaluation and follow-up. Cardiac magnetic resonance imaging has become increasingly utilized for diagnostic evaluation of cardiac tumors [77–79]. The role of TEE is primarily for two settings: (a) for those patients in whom TTE imaging is poor or incomplete, and (b) for intraoperative assessment. Specifics of optimal TEE probe location and multiplane angles, along with methods of evaluation, will depend upon the type and location of the tumor (as detailed above). In adults, TEE has been reported to provide more precise depiction of tumor attachment sites and extent of myocardial/pericardial involvement [80, 81]. Intraoperatively, the preoperative TEE study is used to confirm the nature and extent of tumor involvement [82, 83]. The postoperative TEE study evaluates the results of cardiac surgery—focusing on completeness of tumor resection residual obstruction (if previously present), whether any adjacent cardiac structures (e.g. cardiac valves) might have been affected by the resection, and assessment of ventricular function [84, 85].

Evaluation of Prosthetic Valves

Prosthetic valves are integral to the medical and surgical management of pediatric and adult patients with CHD. These valves fall into two major categories—mechanical and biologic—based upon valve composition (Table 16.6).

Mechanical heart valves contain nonbiologic materials (polymers, metal, carbon) in all parts of the prosthesis: the valve ring, sewing cuff, and orifice occluder. A number of mechanical valves have been developed over the past 50 years, essentially of three major types. The first type to be developed was the caged ball valve, consisting of a silastic ball with a circular sewing ring and a cage formed by three metal arches. The most notable of these was the Starr-Edwards valve (Fig. 16.15c), though similar valves have been produced including the Smeloff-Cutter valve. While these valves are no longer implanted, thousands of patients who received these valves are still alive, and continue to be followed regularly. The second type of mechanical valve is the monoleaflet valve, in which a single disk is secured by lateral or central metal struts, and surrounded by a sewing ring. The disk, generally made of extremely hard carbon (pyrolytic carbon), opens by tilting at an angle (about 60°–80°), resulting in two orifices of different sizes. Typical examples of this include the Bjork-Shiley (discontinued), and the Medtronic-Hall valve (Fig. 16.15b). The third major type of mechanical valve is the bileaflet tilting disk valve, made of two semicircular pyrolytic carbon disks attached by hinges to a rigid valve sewing ring. In the open position the valve leaflets tilt to an opening angle of 75°–90°, resulting in three orifices: a small slit-like orifice centrally between the two leaflets, and two semicircular orifices laterally. Of the three types of mechanical valves, the bileaflet tilting disk valve provides the most natural blood flow, greater effective orifice area for a given valve size, and it is also the least thrombogenic. Currently, they are the most commonly implanted mechanical valves, notably the St. Jude Medical (Fig. 16.15a) and Carbomedics bileaflet tilting disk valves. The latter two valves are available in a variety of sizes (from 16–33 mm) suitable for both pediatric and adult patients. The mechanical valves have a proven record of durability, however they require ongoing anticoagulation therapy, and there are ever-present risks of thrombosis and endocarditis of the valve. In many types of mechanical valves, separate aortic and mitral versions are available; nonetheless for a number of the mechanical valves, implantation has been performed in any of the four valve positions.

Table 16.6 Typical biologic and mechanical valves

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Biologic heart valves are derived from human or animal tissue, and certain types contain nonbiologic material as well, such as metal and fabric. Human tissue valves fall into two categories: homografts (allografts) and autografts. Homograft valves are cryopreserved human cadaveric aortic and pulmonary valves, generally used as pulmonary or aortic valve replacements (Fig. 16.16). They come in a variety of sizes, depending upon donor availability. In contrast, an autograft represents the patient’s own valve translocated from one site to another. Usually, the autograft is the patient’s pulmonary valve translocated to the aortic position (Ross procedure) or rarely the mitral position (Ross II), with a homograft valve being placed in the original pulmonary position [86–88]. Biologic valves derived from animal tissue are known as xenograft (or heterograft) valves; the most commonly used animal tissues are porcine aortic valve and bovine pericardium, and the tissues are fixed with glutaraldehyde. These valves come in two major forms. Stented biologic valves contain a sewing ring and struts composed of nonbiologic material (metal, cloth), and valve tissue is sewn onto the fabric covering the struts. Both porcine valve (Fig. 16.15d) and bovine pericardium (Fig. 16.15e) are used with these types of valves. Stentless biologic valves contain no struts or sewing ring, which leaves more room for blood flow. Stentless xenograft valves derive primarily from harvested porcine aortic valves (Fig. 16.15f). Of note, human homograft and autograft valves also fall into the category of unstented biologic valves, since they contain no sewing ring or struts. This is because the entire homograft/allograft root (containing the valves) is harvested, thus the intrinsic structural support for the valve leaflets remains intact. Another category of bioprosthesis that has gained popularity is the Contegra pulmonary valve conduit. The Contegra conduit is a bovine jugular vein preserved in glutaraldehyde, and it contains a valve with three leaflets; the leaflets are similar to a human semilunar valve (Fig. 16.17). Since it is derived from a venous vascular structure, it is felt to be best suited for conditions of lower pressure such as the pulmonary circuit, and therefore it is used primarily for congenital heart surgeries in which a right ventricle to pulmonary artery conduit is needed such as the Ross procedure, tetralogy of Fallot, truncus arteriosus, etc. [89]. Thus it serves as an alternative to the homograft, and has achieved comparable short to intermediate term results [90–92]. Strictly speaking, it is a valved conduit (not solely a biologic valve), but it is used in a number of operations in which a valve is necessary. The Contegra conduit is available in both a supported and unsupported model; the supported model contains two external cloth covered polypropylene rings that encircle the valve annulus and commissure to provide additional support (Fig. 16.17a), and is utilized in those cases in which sternal compression could be an issue. As will be discussed below, the bovine jugular valve is also used for transcatheter valve technology.

Over the years, a large number of different valve prostheses (both mechanical and biologic) have been developed, in a variety of valve sizes and profiles. Development continues at a rapid pace, with novel alternatives currently in development or advanced clinical trials. A list of some of the better-known biologic and mechanical valves is given in Table 16.6; this list is by no means exhaustive, and new models and types are periodically being introduced. A full discussion and elaboration of the many individual valves (including details about durability and complications) would require a separate chapter. For further information, the reader is referred to a number of references providing more detailed coverage of the topic [93–97].

Both mechanical and biologic valves can be used to replace a stenotic or regurgitant valve in any of the four valve positions. The preference for valve replacement type varies depending upon the desired site of implantation, and includes considerations such as age of the patient, evidence-based effectiveness of valve prosthesis alternatives for the intended valvar position, valve durability, valve size availability, and the need for ongoing medical therapy. Mechanical prostheses boast greater durability, but this must be balanced with the need for constant anticoagulation and the ever-present risks of bleeding, thrombosis, and endocarditis. Conversely, biologic valves do not generally require significant anticoagulation, but their durability can be much more variable. While there are multiple options for each valve site, some generalizations can be made [17]. For pulmonary valve replacement, biologic valves—notably homograft (allograft) and heterograft (porcine, bovine pericardial, contegra)—are generally preferred, though some investigators have advocated for mechanical valves [98]. For tricuspid, either a mechanical valve or stented porcine valve is generally used. For the aortic valve, both biologic (homograft, heterograft, pulmonary autograft) and mechanical prosthetic valves are utilized. A number of stented and unstented biosprosthetic xenograft valves have been developed for the aortic valve position. For the mitral valve, mechanical prostheses predominate in children and adults, although stented biologic valves (porcine and bovine pericardial) are selectively used in the adult group due to other important considerations (such as pregnancy and the risk of warfarin embryopathy) [17].

One of the most important and exciting new areas in prosthetic valve technology has been the development of catheter-based, implantable prosthetic valves, also known as transcatheter heart valves or THVs. Valve leaflets composed of biologic tissue are mounted in an expandable metal frame, which can then be delivered using various transcatheter techniques and precisely placed in the location of the diseased or absent valve. Several of these THVs are well known, having already gained a large clinical experience. The Melody valve (Fig. 16.18) is a bovine jugular valve mounted on a platinum iridium stent, and delivered by a 22 F balloon in balloon catheter delivery system [99]. It is primarily designed for pulmonary valve replacement, and in the United States it is currently used only in an existing right ventricular outflow conduit, though in Europe it has also been used in patients with tetralogy of Fallot and a right ventricular outflow tract patch (using pre-stenting techniques) [100]. It has also been used in other positions such as failed AV valve bioprostheses (also known as “valve-in-valve” replacement) [99, 101, 102], native aortic valve replacement [101], and in the branch pulmonary arteries [103]. For transcatheter aortic valve replacement/implantation (also known as TAVR, or TAVI), there are two major devices currently available. The Edwards SAPIEN valve contains bovine pericardial leaflets sewn inside a stainless steel or cobalt chromium alloy frame. The inflow of the frame is covered with fabric to provide an annulus seal (Figs. 16.15g and 16.19a, b). The valve is positioned through a sheath (22–24 F for the SAPIEN, 16–19 F for the SAPIEN XT) either from the femoral artery, ascending aorta, or through the left ventricular apex (the latter two methods utilizing a hybrid surgical approach). Once positioned, the frame and valve are balloon expanded within the diseased native aortic valve, displacing the native leaflets (Fig. 16.19). Rapid ventricular pacing is performed during implantation to reduce cardiac contraction during valve implantation [104]. This valve is also used for valve-in-valve replacement (mitral, tricuspid), and is undergoing trials for use in transcatheter pulmonary valve replacement (similar to the Melody valve) [99, 105]. The other major valve for TAVR/TAVI is the Medtronic CoreValve system. This valve is composed of porcine pericardial leaflets and annular seal, mounted in a self-expanding nitinol frame (Fig. 16.15h). It is delivered within an 18 F sheath, introduced percutaneously via femoral or subclavian artery access. Once the sheathed device is located in the desired position, the device expands (and becomes deployed) by retraction of the sheath. Deployment does not require rapid ventricular pacing. To date, it has limited utility for valve-in-valve therapy.

It should be noted that research and development in THVs continues at a very rapid pace; in the near future one can expect to see a number of new valves in various stages of clinical trials [106].

TEE Evaluation of Prosthetic Valves

Echocardiography is very important for the evaluation of both mechanical and biologic valves. This is particularly true in the intraoperative setting, in which TEE is used preoperatively to visualize the diseased valve location, size and pathology, and postoperatively to assess the function and anatomic appearance of the newly implanted prosthetic valve. However it is important to remember that the intraoperative setting presents unique challenges for valvar assessment due to physiologic alterations from changing preload/afterload, inotropic support, open sternum, positive pressure ventilation, and general anesthesia (also discussed in Chaps. 11 and 15).

The TEE evaluation of prosthetic valves utilizes the same principles applied to native valve pathology: one should perform a combination of careful 2D imaging, as well as color flow and spectral Doppler evaluation of flow across the valve. For imaging, a prosthetic valve should be examined from multiple views, with emphasis on valve occluder/leaflet motion, appearance of the sewing ring, and presence of an abnormal echo density attached to any part of the prosthesis. The examiner should establish that the valve is well seated, and without excessive movement or “rocking”, which could be a sign of dehiscence [107]. The imaging findings should always be paired with color flow and spectral Doppler evaluation, which gives important information regarding valve function, including possible paravalvar leaks as well as areas of stenosis/abnormal flow. The examiner should understand the type of prosthetic valve implanted, and whether there are any unique imaging/flow characteristics expected with that particular prosthesis. For example, imaging artifacts produced by certain prosthetic valves can sometimes interfere with their assessment. In this case the use of multiple imaging planes and views can help to circumvent these artifacts, and this is particularly applicable to TEE.

For mechanical prosthetic valves, echocardiography (both TTE and TEE) serves as an essential tool for evaluation [107]. Normal flow across a mechanical valve will be different than that of the corresponding native valve; increased spectral Doppler velocities can be anticipated, depending upon valve manufacturer and valve location and size. These “normal” or expected Doppler velocities have been well documented for the different valve types and sizes (discussed below) [95, 107, 108]. Mechanical valves can be difficult to image by TTE due to their nonbiologic material composition (metal and pyrolytic carbon) that produces significant acoustic shadowing and reverberation, as well as side lobe artifacts. As such, limited information is available in those areas of the valve prosthesis distal to the ultrasound transducer. Thus for certain mechanical valves, TEE can play a very important role in their evaluation because of the proximity of the TEE probe to the heart, with a location proximal to the prosthesis, resulting in superior overall imaging (Fig. 16.20). Excellent visualization of mechanical valve motion, as well as color flow and spectral Doppler evaluation can be obtained. Mechanical valves generally have flow characteristics that differ markedly from native valves. For example, the typical bileaflet tilting disk valve (e.g. St. Jude), when open, has one major central orifice and two side orifices to allow forward flow [95, 107]. When closed, there are normally two small regurgitant (“washing”) jets at the pivot points of the valve, angled centrally; these are intended to minimize thrombus formation [109] (Fig. 16.21, Video 16.15). Abnormalities of valve occluder/leaflet motion (such as a frozen leaflet), vegetations, thrombus, pannus formation (tissue ingrowth at the site of the valve prosthesis), and paravalvar leaks/valve dehiscence, can all be seen well by TEE [94, 109]. For mechanical prosthetic valve endocarditis, TEE has been shown to be clearly superior to TTE: vegetations and annular abscesses can be visualized much more readily [41, 110–112]. With mechanical prostheses, vegetations can significantly interfere with occluder motion, causing stenosis, regurgitation, or both [109].

With mechanical AV valve evaluation, TEE can very useful. This is especially true with mechanical mitral valves, in which the superiority of TEE imaging compared to TTE is most striking. The esophageal position of the TEE probe enables imaging of the important atrial surface of the valve—an area where pathology tends to occur, often obscured by standard TTE. Excellent valve detail is obtainable from the mid esophageal window: rotation of the multiplane angle can be performed between 0° and 90° to provide optimal visualization of the prosthesis leaflets and leaflet motion from an edge-on view (particularly for bileaflet tilting valves). Restricted leaflet motion and pannus/vegetations above the valve can be very well seen (Figs. 16.22 and 16.23, Videos 16.16 and 16.17). One other important issue to note is that the native papillary muscles and chordal tissue can sometimes be left in place following AV valve replacement, and the chordae can remain quite mobile during the cardiac cycle. These structures should not be confused with intracardiac masses or thrombi.

Mechanical aortic valves can be more challenging to evaluate, as the mid esophageal plane of imaging tends to be parallel to the plane of the valve, and therefore more perpendicular to the direction of valve flow. Depending upon valve leaflet orientation, it might be difficult to obtain an edge-on profile of leaflet motion. Instead, often the leaflets can be viewed from an en face perspective. They can be examined for any asymmetry of motion, and any paravalvar leaks can be viewed from a number of different multiplane angles between 0°–120° (Fig. 16.3, Video 16.3). If transgastric and deep transgastric views are available, these can be particularly useful to evaluate the proximal (subaortic) portion of the valve, visualize valve motion, evaluate for valvar regurgitation, and also obtain a spectral Doppler tracing across the valve [109] (Fig. 16.24, Video 16.18). These latter transducer positions help to avoid the shadowing artifacts associated with imaging from the mid esophageal position.

Biologic prosthetic valves—both stented and stentless—generally have much less acoustic shadowing, and are therefore seen more readily both by TTE and TEE (Fig. 16.25a, b). The struts of a stented valve are echogenic and easily seen arching above the plane of the valve ring; however the thin valve leaflets should be seen contained within the valve (Fig. 16.25a). Stentless valves (homograft, xenograft, autograft) often have an echocardiographic appearance similar to that of native valve tissue (Fig. 16.25b). Biologic prosthetic valve motion and valve characteristics are generally seen very well by echocardiography. Unlike mechanical valves, these valves do not incorporate valve regurgitation as part of their design. However, very minor degrees of regurgitation are normally present in many patients [109]. Valve failure (stenosis and/or regurgitation) is readily seen and evaluated both by TTE and TEE, using imaging, color flow and spectral Doppler. Since TTE is adequate for most biologic valves, TEE is usually reserved for those patients in whom TTE imaging is suboptimal, in patients suspected of having endocarditis (see above), or in the intraoperative setting. One of the more useful aspects of the postoperative TEE study following prosthetic valve implantation (both biologic and mechanical) is to establish the flow profile across the new valve; this information (including velocities) can be used for subsequent comparisons.

Doppler Evaluation of Prosthetic Valves

For Doppler evaluation of prosthetic valves, the principles and techniques of valve interrogation and recording of flow velocity are similar to those used for evaluating native valve stenosis or regurgitation (see Chaps. 8 and 11) [113, 114]. However a few general comments are worth noting. Prosthetic valve regurgitation is primarily assessed with Doppler evaluation, mainly using color flow techniques, though spectral Doppler evaluation is helpful as well. It is important to differentiate between “normal” and pathologic prosthetic valve regurgitation. A mild degree of regurgitation is normally seen in virtually all mechanical valves (Fig. 16.20); as noted above, this can be seen in the form of the “washing” jets seen with bileaflet valves (Fig. 16.21, Video 16.15). Minor regurgitant jets are also seen with biologic valves, including THVs. Pathologic regurgitation—characterized as one or more prominent areas of color flow Doppler regurgitation—can be either central or paravalvular (outside the valve sewing ring). Most pathologic central regurgitation is seen in biologic valves, but paravalvular regurgitation can occur with both biologic and mechanical valves. Paravalvular regurgitation is seen as jets outside the sewing ring of the prosthesis (Fig. 16.26, Video 16.19). The degree of regurgitation can be estimated using the methods for quantification of native valvular regurgitation [113], although these can be more challenging with the shadowing and reverberations caused by the prosthetic valves (particularly mechanical valves). Commonly used parameters for semilunar valves include color flow Doppler jet width, vena contracta, pressure half-time, and diastolic flow reversal in the distal great artery; for AV valves, parameters include vena contracta, color flow Doppler jet area, as well as reversal of flow in the pulmonary or systemic veins. A discussion of this evaluation is given in Chaps. 8 and 11. Regardless of valve type and position, when pathologic regurgitation is suspected, a careful evaluation must be made as to possible etiology. This includes location of the regurgitation (central vs. paravalvular), and possible mechanism of regurgitation (leaflet dysfunction, improper valve size/geometric mismatch, valve dehiscence, etc.).

When evaluating antegrade flow across prosthetic valves, it is important to remember that the flow characteristics and velocities across prosthetic valves (particularly mechanical valves) will often differ from comparably sized native valves. In general, the spectral Doppler velocities across these valves tend to be higher due to the phenomenon of pressure recovery [107]. As mentioned above, several studies have presented expected Doppler velocities, gradients and effective orifice area for a wide range of biologic and mechanical aortic and mitral valves [95, 107, 108]. Tables 16.7 and 16.8 show representative data abstracted from one of these studies [108] for several common prosthetic aortic and mitral valves. It should be noted that the labeled valve “size” (e.g. 21, 23 mm) represents the outer valve diameter in millimeters as given by the manufacturer. However this parameter should not be considered alone, because the flow characteristics and cross-sectional area between two identically sized valves might be completely different. Hence the effective orifice area (EOA) presented in these tables represents a better parameter for valve comparisons and overall prosthetic valve evaluation; it is an important parameter utilized in adult patients for clinical prosthetic valve assessment. The EOA is analogous to valve orifice area for a native valve, and is calculated in the same manner by the continuity equation (Chap. 1):

$$ \mathrm{EOA}=\mathrm{Stroke}\mathrm{volume/VT{I}}_{PrV}$$
VTI_PrV = Velocity time integral (VTI) through the prosthesis, measured by continuous wave Doppler
Stroke volume = VTI of the left ventricular outflow tract (by pulsed wave Doppler) multiplied by the left ventricular outflow tract cross sectional area (with prosthetic mitral valves, this calculated stroke volume is valid assuming no significant aortic regurgitation exists).

Table 16.7 Doppler parameters across prosthetic aortic valves

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Table 16.8 Doppler parameters across prosthetic mitral valves

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The EOA is generally a better index of valve function than gradient alone, because it is will not vary with different flow states. An important concept for prosthetic valves is that the EOA must be appropriate for the flow requirements of the individual, otherwise patient-prosthetic mismatch (PPM) occurs. PPM is a term used to describe the clinical situation when the EOA of a prosthetic valve is too small in relation to a patient’s body size, resulting in abnormally high postoperative gradients [95, 115]. Studies in adults have shown that aortic PPM is associated with worsening symptoms and impaired exercise capacity, as well as adverse cardiac events and long-term mortality [116–119]; mitral PPM is associated with persisting pulmonary hypertension and increased congestive heart failure as well as reduced survival [120]. When indexed to body surface area, the EOA is the only parameter found to be consistently related to postoperative gradients and/or adverse clinical outcomes [121–123]. Table 16.9 shows threshold values for indexed EOA generally used to identify and quantify the severity of PPM in adults [95].

Table 16.9 Threshold values of indexed prosthetic valve effective orifice area (EOA) for the identification and quantification of prosthesis-patient mismatch

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As noted above, the information from Tables 16.7 and 16.8 are derived from studies in which data were compiled from a number of adult studies. The tables are voluminous and comprehensive, and the reader is referred to these for further reference regarding other prosthetic valves. Nonetheless, from these data, some simplified general guidelines can be formulated to assist in the assessment of possible prosthetic aortic and mitral valve stenosis, and these are summarized by Zoghbi et al [107] and presented in Table 16.10. These guidelines also utilize parameters such as Doppler velocity index (DVI) for prosthetic aortic valves, which is the ratio of velocities across the left ventricular outflow tract compared to the velocity across the prosthetic aortic valve, and the inverse relationship for mitral valves, the ratio of the prosthetic mitral valve VTI compared to the VTI across the left ventricular outflow tract. These dimensionless ratios—derived from the continuity equation—are much less dependent upon varying flow states. It should be noted that comparable data for prosthetic pulmonary and tricuspid valves is lacking, particularly regarding normal and abnormal EOAs and DVI/VTI. Therefore more general guidelines, also presented by Zoghbi et al, have been presented for these latter two valves as follows [107]:

Table 16.10 Doppler parameters across prosthetic aortic and mitral valves

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Findings suspicious for prosthetic pulmonary stenosis:
- Cusp or leaflet thickening or immobility.
- Narrowing of forward color map.
- Peak velocity through the prosthesis >3 or >2 m/s through a homograft (suspicious but not diagnostic of stenosis).
- Increase in peak velocity on serial studies (more reliable parameter).
- Impaired right ventricular function or elevated right ventricular systolic pressure.
Findings suspicious for tricuspid valve stenosis
- Peak velocity >1.7 m/s (because of respiratory variation, average ≥5 cycles).
- Mean gradient ≥6 mmHg (may be increased if there is valvular regurgitation).
- Pressure half-time ≥230 ms.
- Narrow inflow color map.
- Nonspecific signs such as enlarged right atrium and engorged inferior vena cava.

In general, an integrated approach, using a combination of the criteria discussed above, works best when evaluating forward flow across any prosthetic valve.

For pediatric patients, there is a notable paucity of available published information regarding normal velocities and EOAs across prosthetic valves, particularly the smaller mitral and aortic valves. Much of the information used in the pediatric population originates from adult data. Fortunately many of the same principles can still be applied, though comparable values to those obtained in adults are still lacking, and it is unclear whether certain parameters are equivalent in this group of patients. For example, one study evaluating St. Jude and Carbomedics mitral prostheses in children found that peak early Doppler velocity—not EOA—correlated best with the manufacturer’s geometric valve orifice area, and also pulmonary artery wedge pressure [124]. The use of the DVI and VTI ratios has not been established in the pediatric population. Also, PPM has not been evaluated closely in children, though it would seem evident that this particular parameter has direct relevance in the pediatric population because of growth considerations. While the goal for valve replacement in children is to implant the largest possible prosthesis, patient growth will inevitably lead to some degree of PPM, even with a normally functioning prosthesis [107]. As noted above, the most widely accepted and validated parameter for identifying PPM in adult patients is the indexed EOA, and Table 16.9 shows threshold values for indexed EOA generally used to identify and quantify the severity of PPM in adults [95]. This table might also serve as useful guide in children, although the applicability of these values in pediatrics has yet to be fully determined.

TEE Evaluation of Transcatheter Heart Valve Implantation

For catheter-based implantable heart valves, the role of TEE will vary based upon the type of THV and its position. With TAVR/TAVI (both SAPIEN and CoreValve), TEE plays an integral role in all three phases of the procedure: pre-procedural assessment of morphology and annular measurements, intraprocedural monitoring of all phases of the valve implantation (including guide wire and device positioning and valve deployment), and post-deployment assessment of possible paravalvular device leaks as well as ventricular function, mitral valve assessment, pericardial effusion, and aortic dissection [125, 126]. It is not uncommon to see some paravalvular regurgitation following valve implantation, particularly after TAVR/TAVI. An example of TEE monitoring during TAVR/TAVI (SAPIEN valve) is shown in Fig. 16.19, Video 16.20 (also in Chap. 11). For the Melody valve, TEE is generally not performed during valve implantation, though transthoracic imaging is routinely obtained after the procedure to assess valve function and competence. For valve-in-valve implantation, the role of TEE will vary depending upon the fluoroscopic visibility of the biologic valve. In most cases, when a stented bioprosthesis is readily visible on fluoroscopy, TEE might not be necessary. However in those cases in which the bioprostheses is not readily visible, or assessment of valve function is required, TEE can be very helpful [102].

Evaluation of Heart Transplantation/Ventricular Assist Devices

Management of heart transplant recipients requires ongoing cardiac assessment for rejection and cardiac function, and this necessitates both noninvasive and invasive surveillance using echocardiography and cardiac catheterization/myocardial biopsy. The bulk of noninvasive surveillance is provided primarily by TTE; the use of TEE for heart transplantation is generally confined to the immediate pre-transplant and post-transplant period. The specific applications for TEE in the setting of heart transplantation include the following:

Screening of cardiac transplant donors. Echocardiography is an essential part of the evaluation of any prospective cardiac transplant donor. Cardiac function and segmental wall motion must be assessed, and any potential intracardiac abnormalities (congenital heart disease, valvar abnormalities) must be identified [127]. Most prospective donors are mechanically ventilated, which can lead to poor TTE imaging, particularly in older patients. Indeed, one study in adults showed technically inadequate TTE images in up to 29 % of mechanically ventilated brain-dead potential donors [128]. Thus when TTE assessment is inadequate, TEE evaluation must be performed.
Evaluation of ventricular assist device implantation. The term ventricular assist device (VAD) refers to a broad range of mechanical circulatory devices designed to unload the failing heart, while at the same time provide adequate systemic (and in some cases pulmonary) perfusion [129]. These VADs are increasingly being utilized for patients with myocardial dysfunction, generally as a “bridge” to cardiac transplantation. Assist devices can be univentricular or biventricular, and there are two major types: pulsatile and continuous pumps. A number of VADs are currently available, and a full discussion of all these devices is beyond the scope of this chapter. Most of these devices are designed for the adult population; only a small number are available specifically for use in the pediatric population [130, 131], the most well-known being the Berlin Heart (EXCOR^®), which will be the example used for the following discussion. The Berlin Heart is a paracorporeal pulsatile VAD that is connected to a pneumatically driven external pump to provide pulsatile flow into the aorta. For left sided assist, an inlet cannula is placed in the apex of the left ventricle (or rarely, the left atrium) (Fig. 16.27). Blood flows through this cannula to an extracorporeal pump. A pneumatic force from a separate driving unit is used to compress a membrane in the pump, expelling the blood into the systemic circulation through a separate arterial cannula connected to the ascending aorta, representing the device outflow [132]. The extracorporeal blood pump is available in a variety of volume sizes between 10–80 ml (Fig. 16.27), enabling use for the entire pediatric age range, including neonates [129] For right ventricular assist, a similar procedure is performed with the receiving cannula in the right atrium, and the arterial cannula in the pulmonary artery. A recent prospective multicenter study showed a clear survival advantage of the Berlin Heart (left or biventricular VAD) vs. extracorporeal membrane oxygenation as a bridge to heart transplantation [133].
Fig. 16.27
Pictures of Berlin Heart. Figure a shows a pump for an older child (60 ml) on the left, next to an infant pump (10 ml) on the right. Figure b shows the intake cannula being implanted in the left ventricular apex. Figure c shows a pediatric pump that has been implanted. The intake and output cannulas enter the patient’s thorax, as shown by the two tubes. The other tube is connected to the external pneumatic driving unit
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For left sided VAD placement, intraoperative TEE serves several important roles. It is utilized to exclude important cardiac defects that could cause an intracardiac right to left shunt (e.g. patent foramen ovale) or compromise physiology after device placement (e.g. significant aortic regurgitation or mitral stenosis) [134]. In addition, TEE is used to evaluate the sites of device implantation within the heart, specifically the inflow connection to the atrium or ventricle, and the outflow connection to the aorta. Right and left ventricular size and function can also be assessed before and after device placement [135, 136]. Color flow and spectral Doppler are used to evaluate the flow characteristics into and out from the device. Thrombus in the cardiac chambers or attached to the inflow/outflow cannulae (if present) can also be evaluated. Monitoring for intracardiac air and appropriate de-airing also represent important applications of TEE in this setting. The type of cardiac connections, as well as the expected flow characteristics of a device, will vary by manufacturer, and these should be determined prior to performing the TEE [134]. An example of TEE evaluation of a Berlin Heart left sided VAD implantation is shown in Fig. 16.28, Video 16.21.
Fig. 16.28
Berlin Heart placement in a patient with dilated cardiomyopathy. Mid esophageal four chamber view, multiplane angle 0° (a, b) and 100° (c). The cannula (arrow) in the left ventricular apex withdraws blood returning from the left atrium. (a, b) When the blood has sufficiently filled the chamber in the device, it is pumped into the ascending aorta (yellow arrowhead), as shown from mid esophageal aortic valve long axis view, multiplane angle 102° (c). Ao aorta, LA left atrium, LV left ventricle, RA right atrium, RV right ventricle
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Following VAD implantation, echocardiography should be used for ongoing evaluation and monitoring while the patient is on support. Transthoracic echocardiography generally suffices for these purposes, but TEE can also be used in selected patients in whom precordial echocardiographic windows are suboptimal or nonexistent. Ongoing evaluation should involve assessment of cannula position, and chamber size/function, as well as surveillance for possible valvular abnormalities, pericardial effusion/hematoma, intracardiac thrombus, endocarditis, and aortic dissection [137].
Note: The reader should be aware that an increasing number of new VAD designs are undergoing development for both adult and pediatric patients, so the variety of devices available for pediatric patients (pulsatile and continuous) is likely to widen considerably in the near future [138, 139]. The National Heart, Lung and Blood Institute (NHLBI) has been very active in this endeavor; in 2004 they initiated a 5 year program to develop a family of pediatric circulatory support devices [140]. Based on the results and progress made from this program, in 2010 the NHLBI created another support program—known as Pumps for Kids, Infants and Neonates (PumpKIN)—in which awards were granted to selected contractors (several of whom participated in the previous program) enabling them to begin the work necessary to receive Investigational Device Exemptions from the Food and Drug Administration for pediatric circulatory support devices. These devices will soon be ready for clinical trials [140, 141].
Intraoperative monitoring/assessment of transplantation. A preoperative TEE assessment during heart transplantation, if performed, is generally of limited utility. Since the recipient heart will be removed and replaced, most intracardiac anatomic and functional information regarding the recipient’s native heart is irrelevant. However there could be some elements that have bearing upon the operative and postoperative course, particularly in patients with CHD. Specifically, abnormalities of cardiac position, systemic and pulmonary venous connections, and aortic/pulmonary artery anomalies (e.g. pulmonary atresia) need to be verified, as they could affect how an anatomically “normal” donor heart is implanted. Extra systemic venous or great artery length might need to be harvested for congenitally abnormal hearts. Additional useful information includes estimation of pulmonary arterial pressure (by tricuspid regurgitant jet velocity, if available).
Postoperative assessment of heart transplantation requires an understanding of the current surgical technique, which enables the examiner to assess and recognize possible complications. Typically, the heart transplant procedure involves a systemic venous bicaval anastomosis (donor SVC/IVC to the recipient SVC/IVC), anastomosis of a recipient left atrial cuff to a cuff fashioned from the donor left atrium, and main pulmonary artery/aortic anastomoses between donor and recipient great arteries. The composite left atrial anastomosis gives the post-transplant left atrium a dilated and “hourglass” appearance with a prominent ridge that could be confused for thrombus [142] (Fig. 16.29, Video 16.22). The postoperative TEE assessment of heart transplantation is comprehensive, evaluating a number of different factors: any residual congenital cardiac defect (e.g. patent foramen ovale), possible retained intracardiac air, intracardiac thrombus, right and left ventricular systolic function, AV and semilunar valve function, estimation of pulmonary artery pressure, and the patency of the donor-recipient anastomoses—aortic, pulmonary arterial, systemic and pulmonary veins [142, 143]. These assessments are particularly important when cardiac transplantation is performed in a patient with a complex form of CHD, e.g. dextrocardia or heterotaxy, and the possibility exists of kinking or torsion of these anastomoses [144]. Immediately following heart transplantation, overall biventricular systolic function is usually normal, although there can be paradoxical ventricular septal motion that can be expected to resolve with time. Also, right ventricular function can be affected if pulmonary vascular resistance is elevated [145].
Fig. 16.29
Following heart transplantation, imaging in the mid esophageal four chamber view, multiplane angle 0°. The anastomosis of the donor left atrium to the cuff of the recipient left atrium creates an area of echogenicity (arrow) that can be mistaken for thrombus. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle
Full size image
Assessment of post-transplant cardiac allograft function in the intensive care unit. Assessment of the transplanted heart essentially mirrors that of the postoperative TEE study discussed above. Other potential indications for TEE evaluation include surveillance for sources of systemic emboli, and also evaluation of possible pericardial effusion. Again, since TTE is usually sufficient, TEE is reserved for those patients with poor transthoracic acoustic images and/or when an open sternum prevents effective transthoracic imaging.

Evaluation of Lung Transplantation

In lung transplantation, cadaveric or living donor lungs are transplanted into a recipient with end-stage pulmonary or pulmonary vascular disease. For each lung (or lobe) transplanted, the donor mainstem (or lobar) bronchus, branch pulmonary artery, and pulmonary venous cuff are anastomosed to the corresponding structures in the recipient [146]. There are specific applications of TEE for lung transplant patients [147]. The preoperative TEE study is used to evaluate cardiac anatomy and exclude any potential intracardiac defects, e.g. small atrial septal defect, which should be addressed at the time of transplantation. Right and left heart function should also be assessed, because impaired cardiac function can have significant impact on the post-transplant outcome. The postoperative TEE study is used to assess the pulmonary arterial and venous anastomoses, as well as cardiac function and AV and semilunar valve function [148]. If tricuspid or pulmonary regurgitant jets are present, they can be used to estimate pulmonary arterial pressure. The evaluation of pulmonary venous return is one of the most important applications of TEE for post-lung transplant assessment. Because of its proximity to the left atrium and pulmonary veins, TEE provides superior imaging for pulmonary venous imaging and color flow/spectral Doppler evaluation (Chap. 4). Stenosis, kinking, or thrombosis of the pulmonary venous anastomosis—a very serious complication of lung transplantation—is readily detectable by TEE (Fig. 16.30, Video 16.23). In contrast, TTE imaging and color flow Doppler display of the pulmonary venous anastomoses is often inadequate, particularly in the immediate post-transplant period. Hence TEE evaluation should be strongly considered whenever there is a possibility of pulmonary venous obstruction in the lung transplant patient [149, 150].

Evaluation of Aortic Dissection

Aortic dissection is characterized by a separation in the ascending aortic wall between intima and media, resulting in a large intimal flap that separates a false from a true lumen in the ascending or descending aorta (or both). It is seen much more commonly in the adult age group (higher incidence of males than females, with a ratio between 2:1 to 5:1), with the most frequently associated condition being hypertension in approximately 80–90 % of patients [151, 152]. However, congenital abnormalities of aortic wall—notably Marfan syndrome, Loeys-Dietz syndrome, vascular Ehlers-Danlos, and other connective tissue abnormalities—are also associated with aortic dissection [153]. Recently, patients with Turner syndrome have also been reported to have an increased risk of aortic dilatation and aortic dissection [154]. Other congenital abnormalities that might predispose to an “aortopathy” and aortic dissection include bicuspid aortic valve, aortic coarctation, and iatrogenic causes such as cannula insertion or balloon dilation of coarctation [155]. The etiology is thought to be due to rupture of vasa vasorum but increased shear forces, and abnormalities of aortic media, can also result in intimal tears that ultimately lead to dissection. Left undiagnosed, aortic dissection can be a dangerous and potentially catastrophic condition, with complications including rupture, pericardial tamponade, aortic regurgitation, and coronary artery ischemia [152, 156].

Aortic dissection is commonly classified using either the Stanford or DeBakey classifications, both of which are based upon the location of the dissection. Stanford A involves the ascending aorta; Stanford B involves the descending aorta [157]. DeBakey I involves ascending and descending aorta, as well as the aortic arch; DeBakey II involves only the ascending aorta; DeBakey III involves only the descending aorta [158]. Typically, ascending aortic dissections (Stanford A, DeBakey II) carry much higher risk for complications compared to descending aortic dissections [152].

While other imaging modalities such as cardiac magnetic resonance imaging and computed tomography have assumed a primary role in the diagnosis of this condition, TEE provides an excellent imaging alternative, offering great sensitivity and specificity because of its superior spatial and temporal resolution [153, 159, 160]. Moreover, because of its portability and availability, TEE (when performed jointly with TTE imaging) is expeditious and can serve as rapid means to secure a diagnosis, particularly in an unstable patient. The advantage of TEE over static imaging modalities is that it readily detects and evaluates aortic regurgitation and pericardial effusion, and also assists in intraoperative assessment, although it cannot assess the abdominal aorta [161].

The aortic arch can be seen and evaluated by TEE, but the position of the esophagus in relation to the aortic arch presents some challenges. Unlike TTE, the entire aortic arch and descending aorta cannot be visualized in one plane, and instead must be evaluated in sections with a combination of multiplane angles, and rotation of the TEE probe. At the same time, the probe must be alternately advanced and withdrawn to visualize the more superior and inferior portions of the entire aorta. Because of the possibility of artifacts mimicking a dissection, an important practice in two-dimensional echocardiography is to visualize the abnormality in more than one plane. Therefore, several multiplane angles (preferably orthogonal) should be utilized to confirm the intra-luminal presence of the flap, and color flow Doppler can be used to show the flow patterns in and out of the false lumen. For ascending aortic evaluation, the most important views include the mid to upper esophageal views—the mid esophageal ascending aortic short and long axis views (ME Asc Ao SAX, ME Asc Ao LAX), as well as the upper esophageal ascending aortic arc long and short axis views (UE Ao Arch LAX, UE Ao Arch SAX). With the upper esophageal views, slow probe rotation from right to left will display the ascending aorta, then aortic arch, and finally descending aorta. The probe can be alternately advanced and withdrawn to visualize various portions of these structures. The descending aorta is best visualized by rotation of the probe so that faces posteriorly and away from the heart, using the descending aorta short and long axis views (Desc Ao SAX, Desc Ao LAX) to achieve orthogonal tomographic images, while withdrawing/advancing the probe to evaluate the different portions of the descending thoracic aorta [152].

Whether the dissection affects the ascending or descending aorta (or both), the characteristic echocardiographic feature of the dissection remains the same: intimal disruption that manifests as a thin, mobile intimal flap, separating the aorta into true and false lumens. In many instances, the false lumen is larger than the true lumen. There can be one or more re-entry sites between true and false lumens. Color flow Doppler will often show a difference in flow patterns between the true and false lumens; there can be much lower Doppler flow velocity in the false lumen, and spontaneous echo contrast or thrombus formation will sometimes be present [152]. In some cases, flow in the two lumens occurs in opposite directions, e.g. when aortic regurgitation is present (Fig. 16.31, Video 16.24). Because disruption of the aortic valve, aortic root, sinuses and coronary artery ostia may be associated with an aortic dissection, it is important to assess valve competence and global as well as segmental ventricular systolic function during the TEE evaluation.

Summary

This chapter reviewed a number of additional areas in which TEE has applications for the pediatric and young adult population. Some of these applications pertain equally to patients with and without congenital heart disease, and some have significant overlap with TEE as it is performed in adult patients with cardiac disease. These additional applications illustrate the wide applicability and important additive value that TEE can provide in cardiac patients of all ages.

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Division of Cardiology, Children’s Hospital Los Angeles, Department of Pediatrics, Keck School of Medicine, University of Southern California, 4650 Sunset Blvd, Mailstop #34, Los Angeles, CA, 90027, USA
Pierre C. Wong MD

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Childrens Hospital Los Angeles Department of Cardiology, University of Southern California Keck School of Medicine, Los Angeles, California, USA
Pierre C. Wong
Pediatric Cardiovascular Anesthesiology, Texas Children's Hospital, Houston, USA
Wanda C. Miller-Hance

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Large vegetation on the anterior leaflet of mitral valve, resulting in chordal destruction and severe mitral regurgitation (MPG 4146 kb)

Aortic valve endocarditis, seen from a mid esophageal aortic valve long axis view (multiplane angle 85°–116°) (MPG 8328 kb)

Endocarditis in a patient with a prosthetic aortic valve (St. Jude). The mid esophageal four chamber view demonstrates a perivalvar abscess that extends into the noncoronary cusp, causing a fistulous tract communicating with the right atrium. A large vegetation has developed in this area and shunting is seen into the right atrium. There is marked aortic regurgitation seen through an area of valve dehiscence (MPG 48550 kb)

Infected sinus of Valsalva aneurysm from aortic valve endocarditis. The preoperative study, obtained from the mid esophageal aortic valve short axis and long axis views, shows a large vegetation of the aortic valve and erosion of the right sinus of Valsalva, with blood filling the aneurysm during diastole. Following aortic valve and aortic root surgery, no residual vegetation is seen and the aortic valve manifests normal function, with no insufficiency (MPG 23246 kb)

Infected pseudoaneurysm off ascending aorta. This TEE was performed to evaluate the aortic valve in a patient with a previous aortic valve surgery and persistent fungemia. A large pseudoaneurysm (arrow) was discovered using the upper esophageal window, multiplane angle 60°. Note that the superior portion of aorta and innominate vein can be seen well in this patient by TEE (MPG 7110 kb)

Thrombus in the left ventricular apex of a patient with Duchenne’s muscular dystrophy and dilated cardiomyopathy. Mid esophageal four chamber view, multiplane angle 0° (MPG 7026 kb)

Thrombus in the superior vena cava, probably associated with a catheter, as seen from mid esophageal bicaval view, (multiplane angle 99°) (MPG 6592 kb)

Thrombus in the left atrial appendage, as viewed from a modified mid esophageal aortic valve short axis view with leftward rotation. There are mobile filamentous strands arising from the thrombus. AoV aortic valve, LA left atrium. Video courtesy of Siemens Medical Systems USA, Inc. © 2012–13 Siemens Medical Solutions USA, Inc. All Rights Reserved (MPG 5100 kb)

Example of spontaneous echo contrast in a patient after repair of D-transposition of the great arteries, with a pseudoaneurysm arising from a previous cannulation site in the aorta. Video obtained from the upper esophageal ascending aorta long axis view. Note the visible swirling of flow due to red cell aggregation. The left pulmonary artery is being compressed by the pseudoaneurysm (MPG 6956 kb)

Wilm’s tumor (arrow) invading the right atrium by direct extension from the inferior vena cava, as seen from the mid esophageal bicaval view, multiplane angle 113°. The multiplane angle is rotated to 55° and all four chambers are visualized. The tumor obstructs IVC inflow. At the end of the video, the extracted tumor is shown (MPG 10052 kb)

Multiple rhabdomyomas in a patient with tuberous sclerosis, including one that caused near complete obstruction of the left ventricular outflow tract. Mid esophageal four chamber view, multiplane angle 0° shows a large tumor in the outflow tract. Turbulent color flow Doppler is seen (MPG 22644 kb)

Fibroma attached to the left ventricular free wall, visualized from mid esophageal aortic valve long axis view, multiplane angle 90°. The fibroma is very large, circumscribed, and has a heterogeneous appearance, studded with echolucent areas most likely representing cystic degeneration or necrosis (MPG 19366 kb)

Left atrial myxoma, seen from mid esophageal four chamber view, multiplane angle 0°. A large, lobulated myxoma is attached to the left atrial wall, just posterior to the aortic root. Multiple fimbriations of the tumor are freely mobile (MPG 17046 kb)

Right atrial hemangioma as seen from the mid esophageal four chamber and mid esophageal right ventricular inflow-outflow view. Note the heterogeneous nature of the large mass in the right atrium. As can be seen by the rapid atrial rate, the patient had an atrial arrhythmia, probably chaotic atrial tachycardia, during the study (MPG 21928 kb)

Prosthetic mitral valve (bileaflet tilting disk). Mid esophageal mitral commissural view, multiplane angle 69°. The multiplane angle is rotated until both leaflets are profiled and open symmetrically in diastole. There is the usual color flow Doppler profile across the valve (MPG 2942 kb)

Prosthetic mitral with a frozen leaflet (arrow), causing stenosis of the valve. Mid esophageal four chamber view, multiplane angle 0° (MPG 11380 kb)

Concentric pannus formation (arrows) above the mitral valve prosthesis, causing significant supravalvar narrowing, seen during diastole. Mid esophageal mitral commissural view, multiplane angle 58° (MPG 12516 kb)

Prosthetic aortic valve (bileaflet tilting disk) viewed from deep transgastric position. At a multiplane angle of 25° the valve is seen from the side, and the usual peri-valvar washing jets can be seen by color flow Doppler. The multiplane angle is then rotated until both leaflets are profiled and symmetric leaflet motion is noted in diastole and systole (about 95°). This view affords a good edge-on view of leaflet motion and flow across the valve, and also provides an excellent angle for spectral Doppler evaluation (MPG 13948 kb)

Paravalvar regurgitation in a child who underwent mitral valve replacement with a mechanical bileaflet prosthesis (the patient previously underwent repair of an atrioventricular septal defect). This video was obtained from a mid esophageal four chamber view. The prosthesis was too large for the annulus and required insertion at an angle, which resulted both in a large area of paravalvular regurgitation (seen to the left of the prosthesis) as well as a very small effective orifice (MPG 13054 kb)

Transcatheter aortic valve replacement/implantation (TAVR/TAVI). This video, obtained from a mid esophageal aortic valve long axis view at 120°–130° first shows the abnormal aortic, which is thickened and has restricted motion. A catheter and then the balloon-mounted valve are seen, with the balloon shown as it is expanded and the valve implanted in the aortic position. During balloon dilation, rapid ventricular pacing is performed to reduce ventricular ejection, thereby stabilizing the valve for placement. Following valve implantation, leaflet motion is seen and there rare two jets of regurgitation seen—one central (transvalvular), one peripheral (paravalvular). LM left main coronary artery (Echocardiographic images were obtained from a Siemens SC 2000 platform and are courtesy of Siemens Medical Systems USA, Inc. © 2012–13 Siemens Medical Solutions USA, Inc. All Rights Reserved) (MPG 39428 kb)

Berlin Heart placement in a patient with dilated cardiomyopathy. Mid esophageal four chamber view. The cannula in the left ventricular apex withdraws blood returning from the left atrium. When the blood has sufficiently filled the chamber in the device, it is pumped into the ascending aorta, as shown from mid esophageal view, multiplane angle 102° (MPG 36604 kb)

Following heart transplantation, imaging in the mid esophageal four chamber view, multiplane angle 0°. The anastomosis of the donor left atrium to the cuff of the recipient left atrium creates an area of echogenicity (arrow) that can be mistaken for thrombus (MPG 29690 kb)

Post-lung transplant, with thrombosis of the right pulmonary veins due to a large lymph node. Mid esophageal view, multiplane angle 0°. There is extensive thrombus in the right pulmonary veins. No flow was seen in the vessel by color flow Doppler. Normal flow is seen in the contralateral left pulmonary veins (MPG 17500 kb)

Dissection of the ascending and descending aorta (DeBakey Type I) in a patient with Marfan’s syndrome. The patient also had a dilated aortic root and significant aortic valve regurgitation. The intimal flap is clearly seen, as well as the true and false lumens. Upper esophageal aortic arch long axis view, multiplane angle 0°, shows the false lumen to be much larger than the true lumen in the ascending aorta and aortic arch. Retrograde diastolic flow reversal is seen only in the true lumen by color flow mapping. Mid esophageal descending aortic long axis view, multiplane angle 90° (probe rotated leftward) shows the dissection extending into descending aorta (MPG 56434 kb)

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Wong, P.C. (2014). Additional Applications of Transesophageal Echocardiography. In: Wong, P., Miller-Hance, W. (eds) Transesophageal Echocardiography for Congenital Heart Disease. Springer, London. https://doi.org/10.1007/978-1-84800-064-3_16

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DOI: https://doi.org/10.1007/978-1-84800-064-3_16
Published: 26 August 2013
Publisher Name: Springer, London
Print ISBN: 978-1-84800-061-2
Online ISBN: 978-1-84800-064-3
eBook Packages: MedicineMedicine (R0)

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Additional Applications of Transesophageal Echocardiography

Abstract

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Other Applications, Including the Critical Care Setting

Indications and Guidelines in Pediatric and Congenital Heart Disease

Echocardiography in Patients with Adult Congenital Heart Disease

Keywords

Introduction

Infective Endocarditis

Echocardiographic Manifestations of IE

Vegetations

Valvar Dysfunction

Intracardiac Abscesses

Aneurysm Formation/Fistulous Tracts

Congestive Heart Failure/Pericardial Effusion

Use of TEE for Evaluation of Infective Endocarditis

Cardiac Masses

Cardiac Thrombi

Cardiac Tumors

Rhabdomyoma

Fibroma

Myxoma

Other Cardiac Tumors

Role of TEE in the Evaluation of Cardiac Tumors

Evaluation of Prosthetic Valves

TEE Evaluation of Prosthetic Valves

Doppler Evaluation of Prosthetic Valves

TEE Evaluation of Transcatheter Heart Valve Implantation

Evaluation of Heart Transplantation/Ventricular Assist Devices

Evaluation of Lung Transplantation

Evaluation of Aortic Dissection

Summary

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