Atlas of Transesophageal Echocardiography, 2nd Edition (2007)

Chapter 8.1. Overview of Congenital Heart Disease

The excellent results obtained in infants and children using transthoracic echocardiography limit the need to use the transesophageal approach to elucidate lesions of congenital heart disease. In some patients, however, it is not possible to perform adequate transthoracic studies, particularly in adults with congenital heart disease. Furthermore, transesophageal echocardiography is the only practical method for assessing operative results during the procedure.

The septum primum covers the septum secundum and closes the fossa ovalis. In about 25% of patients this coverage is not complete, and a flow may occur across the septum. This is particularly likely in the setting of elevated right-sided pressures, which can open the patent foramen and result in paradoxic embolus. The secundum defect occurs at the level of the fossa ovalis and is the commonest of atrial septal defects. These lesions often present as a single large defect but may be fenestrated or multiple. Defects <2 cm in diameter can be closed by transcatheter patch closure, but larger defects must be closed surgically. The size of the defect is easily assessed by transesophageal echocardiography, especially if the multiplane probe is used.

The ostium primum type of atrial septal defect (partial atrioventricular septal defect or partial atrioventricular canal defect) occurs at the base of the septum. It is often associated with a cleft in the anterior mitral valve leaflet that makes it regurgitant. In contrast, sinus venosus atrial septal defects occur in the superior part of the septum. Drainage of the right upper pulmonary vein into the right atrium is a commonly associated anomaly. The superior vena cava may override the defect. In adults, this defect may be difficult to visualize by transthoracic echocardiography. When an atrial septal defect is suspected on the basis of other findings but is not found on transthoracic examination, transesophageal echocardiography is indicated.

Ventricular septal defects, especially small ones, are often visualized more effectively on transthoracic echocardiography than on transesophageal echocardiography because more planes are available from the transthoracic approach. Patent ductus arteriosus and other aortopulmonary communications, both congenital and surgically created, can be visualized by transesophageal echocardiography.

Stenosis of the pulmonary veins is usually diagnosed early. It can be seen as a narrowing of the vein, and Doppler examination demonstrates a high-velocity narrow jet with turbulence and spectral broadening. In adults, congenital stenosis must be differentiated from acquired narrowing produced by mediastinal lesions such as a tumor or sclerosing mediastinitis and pulmonary vein obstruction occurring after lung transplantation. In cor triatriatum, a membrane partitions the left atrium (or, rarely, the right atrium) into two chambers in such a way that the pulmonary veins are on one side of the membrane and the left atrial appendage is on the other side. Cor triatriatum is different from a supravalvar mitral membrane, which is located below and inferior to the left atrial appendage. These lesions may or may not result in obstruction to the flow. Congenital mitral stenosis is a rare lesion, similar in presentation to mitral stenosis. The valve is thickened and domes in diastole. Associated abnormalities of the papillary muscle and chordae may be present.

Subvalvular aortic stenosis results from a fibromuscular membrane that has a location that is variable relative to the aortic valve. These membranes are most commonly attached to the base of the anterior mitral leaflet and to the ventricular septum. Subvalvular membranes appear as linear echoes in the left ventricular outflow tract. Turbulent flow and a high gradient may be present, and there is often associated aortic regurgitation. Early, as opposed to mid- or late, systolic closure of the aortic valve is commonly seen. It may involve only one cusp.

In congenital aortic stenosis the valve domes in systole. The short-axis view shows a typical “circle within a circle” appearance. Careful planimetry yields a good estimate of the valve area. Care must be taken to move the probe up and down the esophagus to visualize even the smallest flow-limiting orifice at the top of the domed valve. Color Doppler allows visualization of the width of the jet. A jet that is 7 mm or smaller suggests severe aortic stenosis in an adult. The jet width should be measured at its origin from the aortic valve. Color Doppler should be turned off during planimetry of the orifice because it tends to overestimate the area. It is, however, useful in identifying the aortic orifice in patients with heavily calcified valves that show no discernible opening movement in systole (i.e., fixed orifice). In these cases, the first appearance of turbulent flow signals in early systole helps identify the stenotic orifice, which can then be studied through planimetry with the color Doppler turned off. After valvotomy the jet size is larger, and the planimetered area is also greater. Because the transesophageal study can be performed intraoperatively, it is useful in guiding surgery. Supravalvular aortic stenosis is a rare lesion that may present as a discrete membrane or may be tubular. Coarctation of the aorta is best seen on transthoracic echocardiography but may also be evaluated by the transesophageal approach. The best results are obtained with a multiplane probe.

Membranes occur in the right atrium. Examples include eustachian valves and Chiari networks. Obstruction is rare but may occur. Right ventricular muscle bundles may be prominent and are associated with right ventricular hypertrophy. Rarely, the hypertrophy may be sufficient to cause obstruction in the body of the ventricle. A small ventricular septal defect may be associated, and it is suspected that the jet from the defect may, at least in some cases, stimulate hypertrophy. Infundibular stenosis may occur alone or in association with tetralogy of Fallot. The right ventricular outflow tract is best viewed in the long axis using longitudinal plane examination.

In congenital pulmonary stenosis the valve domes in systole and may also be thickened. Stenosis in the proximal segments of the right or left pulmonary arteries can be visualized by transesophageal echocardiography; however, stenosis in the distal segments or in the peripheral branches cannot be visualized using echocardiographic techniques.

The pathophysiologic features of complete atrioventricular canal defect (atrioventricular septal defect), including absence of the atrioventricular septum, deficiency of the adjacent atrial and inlet ventricular septum, a common atrioventricular annulus, and shunting from the left atrium into the right atrium and from the left ventricle into the right ventricle, are well seen using the transesophageal approach. The severity of atrioventricular valve regurgitation can also be evaluated.

In tricuspid atresia, the tricuspid valve is absent and both atrial and ventricular septal defects are necessarily present to ensure patient survival. This lesion is often associated with a hypoplastic right ventricle. For the purpose of surgical planning, it is necessary to delineate the right ventricular outflow tract, the pulmonary valve, and the pulmonary artery. Surgical correction (i.e., the Fontan procedure) involves closure of the atrial septal defect, closure of any ventricular septal defect, and placement of a shunt from the right atrium to the pulmonary artery.

In transposition of the great vessels, the connection of the vessels to the ventricles can be seen and the relation of the pulmonary artery and the aorta can be delineated. Associated pulmonary valve stenosis can be seen, as can banding of the pulmonary artery. Coexisting atrial or ventricular septal defects and atrioventricular or semilunar valve regurgitation can also be assessed by transesophageal echocardiography. The adequacy of the Mustard procedure can be assessed immediately after the operation. Intra-atrial baffle leak or obstruction can be delineated. In congenitally corrected transposition of the great vessels, the discordant atrioventricular and ventriculoarterial connections, together with the presence and degree of Ebsteinization of the left-sided atrioventricular valve, can be assessed by transesophageal echocardiography.

Ebstein anomaly is characterized by the apparent displacement of one or more tricuspid valve leaflets into the right ventricle, resulting from a variable extent and degree of plastering of the leaflets to the contiguous ventricular septum or right ventricular wall. The degree of this displacement determines the size of the functional right ventricle. Transesophageal echocardiography is useful in assessing both these abnormalities and the severity of tricuspid regurgitation, which is often considerable. A patent foramen ovale or an atrial septal defect may be present, and may result in right-to-left shunting and cyanosis because of increased right atrial pressure. The anatomic features and the flow patterns of other complicated lesions, such as tetralogy of Fallot, double outlet right ventricle, and single ventricle can also be assessed through the transesophageal approach.

Abnormalities of the coronary arteries can be seen well. These include aneurysms, thrombi, and anomalous origins and course of the coronary arteries.

An important lesion is the sinus of Valsalva aneurysm. It may appear as a tube-like structure protruding into the right ventricle or the right atrium. Rupture may result in shunting into the right ventricle or the right atrium.

In the first part of this chapter the most common shunt lesions are illustrated, followed by the obstructive lesions (both left-sided and right-sided), and, finally, the more complex congenital pathologies. The second and third parts of this chapter supplement the material shown in the first part and provide an enhanced understanding of the role of transesophageal echocardiography in the assessment of congenital cardiac lesions.

FIGURE 8.1.1. Patent foramen ovale. A defect in the midportion of the atrial septum that measures 1 cm or less is most likely a patent foramen ovale (PFO). A. Five-chamber view shows a small defect in the interatrial septum, consistent with a PFO. B. After a peripheral venous bolus of normal saline, contrast echoes are noted moving from the right atrium (RA) into the left atrium (LA) through the defect. The mildly thickened edges of the defect are outlined by the arrows. This patient presented with refractory hypoxemia from right-to-left shunting across a PFO secondary to increased RA pressure following acute inferior myocardial infarction with right ventricle (RV) wall involvement. The RV inferior wall was akinetic on the transthoracic study. C. Another patient with a small defect (D), measuring about 1 cm, in the interatrial septum. D–G. Another patient with PFO. The black arrow in D shows bulging of the atrial septum (AS) into the LA, which occurred when the patient was asked to cough after receiving an intravenous injection of agitated normal saline. This resulted in the contrast echoes moving from the RA through the PFO into the LA (arrowheads in D,F, and G). Both cough and the Valsalva maneuver are useful in diagnosing a PFO because they transiently raise the RA pressure higher than that on the left, producing a right-to-left shunt. The lower arrowhead in F points to contrast echoes arriving in the aortic arch subsequent to their appearance in the LA. H,I. Right-to-left shunting (arrows) through a PFO demonstrated by color Doppler in another patient. Color Doppler examination is usually less sensitive than contrast echocardiography in detecting a PFO because the shunt flow velocity is often lower than the velocity threshold set for color Doppler. J. Left-to-right shunting (arrow) shown by color Doppler through a PFO in a patient with a large LA. Note the bulging of the atrial septum (AS) toward the RA. Contrast echocardiography was negative in this patient because coughing and the Valsalva maneuver failed to increase the RA pressure higher than the left, as evidenced by the absence of bulging of the AS into the LA. K. A PFO (arrow) is well visualized on the two-dimensional image. L,M. Although the atrial septum appears intact inL, color Doppler examination clearly shows shunting into the RA. An area of flow acceleration is seen on the LA aspect of the AS. N–P. Another patient with a PFO. Right-to-left shunting is shown by both color Doppler (arrowheads in N and O) and peripheral venous saline contrast injection (P). Q, R. A different patient in whom minimal right-to-left shunting (arrowheads) through a PFO could be demonstrated by color Doppler. In the last two patients, a recently introduced high-resolution color Doppler system was used. S–V. An elderly female with orthodeoxia in whom right-to-left shunting through a PFO was demonstrated by peripheral venous saline contrast injections (arrowheads in S and T).U. Shunting (arrow) was also demonstrated through the left upper pulmonary vein (LPV). Examination of the right lower pulmonary vein and right upper pulmonary vein (RUPV) did not show any contrast echoes. V. Contrast echoes (arrowheads) are seen in the superior vena cava (SVC) and pulmonary artery (PA) but not in the RUPV following intravenous normal saline injection. Left pulmonary artery angiogram did not show any evidence of a pulmonary arteriovenous malformation or fistula. We believe the shunting into the left upper pulmonary vein (LUPV) is based on pulmonary capillary dilatation as a result of mild pulmonary fibrosis, which was found on CT scan. AO, aorta; IVC, inferior vena cava; IAS, atrial septum; LAA, left atrial appendage; LPV, left pulmonary vein; LV, left ventricle. (A and B reproduced with permission from 

Cox D, Taylor J, Nanda NC. Refractory hypoxemia in right ventricular infarction from right-to-left shunting via a patent foramen ovale: efficacy of contrast transesophageal echocardiography. Am J Med 1991;91:653–655.

 S–V reproduced with permission from 

Thakur AC, Nanda NC, Malhotra S, et al. Combined interatrial and intrapulmonary shunting in orthodeoxia detected by transesophageal echocardiography. Echocardiography 1998;15:101–104.

)

FIGURE 8.1.2. Right-to-left shunting in hepatopulmonary syndrome. A,B. Contrast echoes (arrowheads) appear in the left upper pulmonary vein (LUPV) following IV injection of normal saline. Contrast appeared in the pulmonary vein before appearing in the left atrium (LA), which indicates that the shunt is at the level of the pulmonary vasculature. This patient has hepatopulmonary syndrome resulting from cirrhosis. In this syndrome, the pulmonary capillaries are dilated, allowing the passage of contrast from the right to the left circulation. AO, aorta; LV, left ventricle.

FIGURE 8.1.3. Contrast transesophageal echocardiographic detection of a pulmonary arteriovenous malformation draining into left lower pulmonary vein. A. Shows contrast echoes originating from the left lower pulmonary vein (LLPV) imaged in the longitudinal plane (L) examination. No contrast echoes are seen from the left upper pulmonary vein (LUPV), which is located further away from the probe as compared with LLPV. The left inset depicts pulsed Doppler spectral signals from LLPV. B. Computer tomography scan of the chest. The arrowhead points to the pulmonary arteriovenous malformation located in the lower lobe of the left lung. AA, ascending aorta; DA, descending thoracic aorta; L, longitudinal plane; LA, left atrium; PA, pulmonary artery; S, superior vena cava; V, vertebral body. (Reproduced with permission from 

Ahmed S, Navin C, Nekkanti R, et al. Contrast transesophageal echocardiographic detection of a pulmonary arteriovenous malformation draining into left lower pulmonary vein. Echocardiography 2003;20:391–394.

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FIGURE 8.1.4. Secundum atrial septal defect. A. Gross specimen shows a large atrial secundum defect (ASD) in the fossa ovalis region. B. Schematic shows a secundum ASD. C.Arrowheads demonstrate a secundum ASD. Note the enlarged right atrium (RA). CT, crista terminalis. D,E. Arrows show a large secundum ASD. Color-flow signals are visualized moving from the left atrium (LA) into the RA through the ASD (E). F. Secundum ASD in another patient. The defect measures 2.19 cm in the longitudinal plane (right panel) and 0.89 cm in the transverse plane (left panel), which illustrates that it is important to view the defect in multiple planes to assess its true size and extent. G–J. Large secundum defect in another patient. Color Doppler examination (G) shows a large defect (D) in the interatrial septum (IAS). Color M-mode (H) and pulsed Doppler (I,J) studies demonstrate the shunt to be left-to-right throughout the cardiac cycle except for a tiny right-to-left component during the isovolumic relaxation period (red signals in H and minimal flow signals above the baseline in I and J). Shunt volume in ASD can be estimated as follows: the maximal Doppler color-flow jet width (cm) at the defect site is taken as the diameter (D) of the defect, which is assumed to be circular. The area of the defect is then calculated using the formula π D2/4. The mean velocity of the shunt flow (V) in cm/sec across the defect and the flow duration (T) in seconds are obtained next by placing the pulsed Doppler sample volume (SV) at the defect site in the area of brightest or aliased color-flow signals and parallel to the flow direction, and then applying planimetry to the Doppler spectral trace so obtained over one cardiac cycle. The shunt volume (L/min) across the defect is calculated as a product of the area of the defect, mean velocity, flow duration, and heart rate (beats/min) divided by 1000. The diameter of the defect in the patient shown in G through J was 2 cm, mean shunt flow velocity was 60 cm/sec, flow duration was 0.5 second, and heart rate was 95 beats/min. The shunt volume was calculated as follows: π D2/4 × V × T × HR/1000 = 3.14 × (4/4) × 60 × 0.5 × 95/1000 = 8.95 L/min. K,L. A different patient with secundum atrial septal defect. An eccentric medially directed tricuspid regurgitation (TR) jet is seen moving into the LA through the defect (K). L. The patch used to close the defect. M,N. Another adult patient with two large secundum-type defects seen by two-dimensional echocardiography (M). Color Doppler study (N) demonstrates two large and one very small defect (numbered 1, 2, and 3). These findings were confirmed at surgery. AA, ascending aorta; AO, aorta; DA, descending thoracic aorta; HR, heart rate; LV, left ventricle; MV, mitral valve; PA, pulmonary artery; RV, right ventricle; SVC, superior vena cava; TV, tricuspid valve. (G and J reproduced with permission from 

Mehta RH, Helmcke F, Nanda NC, et al. Transesophageal Doppler color-flow mapping assessment of atrial septal defect. J Am Coll Cardiol 1990;16:1010–1016.

)

FIGURE 8.1.5. Atrial septal aneurysm. A. Biplane study shows the atrial septal aneurysm (AN) bulging into the right atrium (RA) during systole when examined in the longitudinal plane (right). In the transverse plane (left), the aneurysm appears as a circular structure in the short-axis view. B. Transverse plane imaging demonstrates the aneurysm in short axis as it bulges into the RA in systole, and its relation to the right atrial appendage (RAA). C,D. Longitudinal plane examination demonstrates the aneurysm bulging into the left atrium (LA) during diastole. E. In this view, the transverse plane skims the surface of the aneurysm, resulting in an erroneous appearance of a mass in the LA. F. M-mode examination shows the AN (arrow) bulging into the LA and into the RA during systole. The arrow in G and H shows another patient with an AN, which mimics an LA cyst in HAO, aorta; AV, aortic valve; LV, left ventricle; PE, pericardial effusion; RPA, right pulmonary artery; RVO, right ventricular outflow tract. (A through F reproduced with permission from 

Zboyovsky KL, Nanda NC, Jain H. Transesophageal echocardiographic identification of atrial septal aneurysm. Echocardiography 1991;8:435–437.

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FIGURE 8.1.6. Partial atrioventricular septal (canal) defect. A–C. A large defect (D) is shown in the basal (inferior) portion of the atrial septum (AS) with no intact septum separating it from the attachment of the atrioventricular valves. B. Color Doppler examination demonstrates flow signals moving from the left atrium (LA) through the defect into the right atrium (RA), then through the open tricuspid valve (TV) into the right ventricle (RV). C. Color M-mode shows left-to-right shunting throughout the cardiac cycle except for a very small right-to-left component (dark red signals) occurring during the isovolumic relaxation period. D–F. Another patient with a partial atrioventricular canal defect. D. Left-to-right shunt (arrow) in the basal portion of the AS. E. An associated PFO (arrow). F. Patch closure (arrow) of the defect. Note the absence of shunting. G.Schematic demonstrates a large atrioventricular canal defect, an aneurysm of the membranous ventricular septum (VS), and mitral regurgitant flow passing through the defect into the RA. H–J. Another patient with partial atrioventricular canal defect. In H, the arrow points to the defect and the arrowhead to prolapse of the anterior mitral leaflet.I,J. Color Doppler examination shows left-to-right shunting (arrowhead in I) through the defect. AO, aorta; IVS, ventricular septum; LV, left ventricle; MV, mitral valve. (Areproduced with permission from 

Mehta RH, Helmcke F, Nanda NC, et al. Transesophageal Doppler color-flow mapping assessment of atrial septal defect. J Am Coll Cardiol 1990;16:1010–1016.

)

FIGURE 8.1.7. Sinus venosus atrial septal defect. A. Gross specimen shows a large sinus venosus atrial septal defect (ASD). B–E. A large defect is seen in the superior portion of the atrial septum, with flow signals (arrows) moving from the LA through the defect to the right atrium (RA) in C and from the RA to the left atrium (LA) in D. E. The superior vena cava (SVC) is seen straddling the atrial septum. Flow signals (arrow) are seen moving from the anomalous right upper pulmonary vein (RUPV) into the RA and then through the defect into the LA. F,G. Postoperative studies show the patch (upper arrow in F, arrow in G) used to close the defect. IVC, inferior vena cava; RPA, right pulmonary artery.

FIGURE 8.1.8. Sinus venosus atrial septal defect. A,B. Examination at a plane angulation of 106° demonstrates the defect (D) in the superior portion of the atrial septum. The entrance of superior vena cava (SVC) into the right atrium (RA) is identified by the presence of the prominent crista terminalis (CT). C. Examination at a plane angulation of 76° fails to reveal the presence of the defect in the superior portion of the atrial septum. D. Color Doppler examination at a plane angulation of 126° shows the right superior pulmonary vein (RSPV) entering the SVC near its entrance into the RA. This was confirmed at surgery. E,F. An associated left superior vena cava (LSVC) is seen entering an enlarged coronary sinus (CS) in F. G. The patch (P) used to close the defect. AV, aortic valve; LA, left atrium; LV, left ventricle; RAA, right atrial appendage; RPA, right pulmonary artery; RV, right ventricle. (Reproduced with permission from 

Maxted W, Finch A, Nanda NC, et al. Multiplane transesophageal echocardiographic detection of sinus venosus atrial septal defect. Echocardiography 1995;12:139–143.

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FIGURE 8.1.9. Sinus venosus atrial septal defect. A,B. Examination at a plane angulation of 97° demonstrates the defect (D) in the superior portion of the atrial septum. C.Color Doppler examination at a plane angulation of 17° shows the opening of the right superior pulmonary vein (RSPV) into the right atrium (RA). CT, crista terminalis; LA, left atrium; RPA, right pulmonary artery; SVC, superior vena cava. (Reproduced with permission from 

Maxted W, Finch A, Nanda NC, et al. Multiplane transesophgeal echocardiographic detection of sinus venosus atrial septal defect. Echocardiography 1995;12:139–143.

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FIGURE 8.1.10. Sinus venosus atrial septal defect (ASD). A. A large defect (D) is seen in the transverse and longitudinal planes together with a left-to-right shunt. B. A large sinus venosus defect (D) with left-to-right shunting (blue signals) is shown in another patient. Flow signals (red) from an anomalous right upper pulmonary vein (RUPV) are seen moving into the right atrium (RA) and then through the defect into the left atrium (LA). C–F. Another patient with a large sinus venosus defect (D) with left-to-right shunting (D). E. Flow signals (red) are seen moving from an anomalous RUPV into RA and then through the defect into LA. F. Postoperative study shows patch closure of the defect. RUPV flow signals are now seen entering the LA. AO, aorta; IAS, atrial septum; RAA, right atrial appendage; RPA, right pulmonary artery; SVC, superior vena cava.

FIGURE 8.1.11. Isolated anomalous left upper pulmonary vein (LUPV) drainage. A. The left upper pulmonary vein (LPV) is imaged in its usual position. B. Instead of draining into the left atrium (LA), the LPV opens into the right atrium (RA) underneath the atrial septum (arrow). C. Estimation of shunt flow volume in isolated partial anomalous pulmonary venous connection in another patient. The Doppler spectral trace (right) was obtained by placing the pulsed Doppler sample volume cursor parallel to the flow direction in a pulmonary vein (left; APV), located on the left side but not connected to the LA. The shunt flow volume (L/min) was calculated as a product of the cross-sectional area of the anomalous pulmonary vein (APV) (obtained by the formula π D2/4, with D equal to 0.98 cm, representing the lumen width at the site of pulsed Doppler interrogation), the mean flow velocity (38 cm/sec), the flow duration (T, 0.79 sec), and the heart rate (84 beats/min), and dividing it by 1000. The shunt flow volume was calculated to be 1.90 L/min in this patient. RV, right ventricle. (C reproduced with permission from 

Mehta RH, Jain SP, Nanda NC, et al. Isolated partial anomalous pulmonary venous connection: echocardiographic diagnosis and a new color Doppler method to assess shunt volume. Am Heart J 1991;122:870–873.

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FIGURE 8.1.12. Isolated right-sided anomalous pulmonary venous return into the right atrium (RA). A. Simultaneous biplane (T, transverse plane; L, longitudinal plane) study shows red flow signals (V1) in the area normally occupied by the right superior pulmonary vein. Note that these flow signals do not extend into the left atrium (LA) as they normally should. B–D. Longitudinal plane examination. Prominent blue signals are noted in the RA in the vicinity of the right pulmonary artery (RPA) and originating just anterior to the interatrial septum (arrow), just across from where the right superior pulmonary vein signals should normally be seen entering the LA. These represent the RA entrance and the course of the flow signals from the anomalous pulmonary vein (V1). D. Spatial waveform obtained by pulsed Doppler interrogation of V1AO, aorta; SV, sample volume;SVC, superior vena cava. (Reproduced with permission from 

Sanyal RS, Nanda NC, Snell D, et al. Transesophageal echocardiographic findings of complete unilateral anomalous pulmonary venous connection of right lung to right atrium. Echocardiography 1994;11:93–100.

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FIGURE 8.1.13. Isolated right-sided anomalous pulmonary venous return into the right atrium (RA). Same patient as in Figure 8.1.11. A–D. Longitudinal planes. A,B. The extracardiac course and the entrance of the second anomalous pulmonary vein (V2) into the RA are seen. C,D. The inferior vena cava (IVC) is separately visualized and enters the RA more posteriorly. E. The left superior pulmonary vein (LSPV) enters the left atrium (LA) normally. CT, crista terminalis; RAA, right atrial appendage; RPA, right pulmonary artery; SVC, superior vena cava. (Reproduced with permission from 

Sanyal RS, Nanda NC, Snell D, et al. Transesophageal echocardiographic findings of complete unilateral anomalous pulmonary venous connection of right lung to right atrium. Echocardiography 1994;11:93–100.

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FIGURE 8.1.14. Isolated right-sided anomalous venous return into right atrium (RA). Same patient as in Figures 8.1.11 and 8.1.12. Transverse plane. Following surgery, pulmonary vein flow signals from the right side (RPV) are seen entering the left atrium (LA) posterior to the surgically inserted patch (P). LV, left ventricle; MV, mitral valve;RVO, right ventricular outflow tract. (Reproduced with permission from 

Sanyal RS, Nanda NC, Snell D, et al. Transesophageal echocardiographic findings of complete unilateral anomalous pulmonary venous connection of right lung to right atrium. Echocardiography 1994;11:93–100.

)

FIGURE 8.1.15. Left-sided superior vena cava (SVC). A–F. The large rounded echo-free space lateral to the MV in A is the left-sided superior vena cava (LSVC). Longitudinal plane examination (B,C) demonstrates the LSVC imaged anterior to the left atrium (LA). (B). C. Following intravenous left arm injection of normal saline, contrast signals are seen in the LSVC. D–G. The LSVC imaged behind the aortic arch (ACH) in the same patient. E–G. Contrast signals (arrowheads) in the LSVC following intravenous left arm injection of normal saline. In suspected cases, contrast echocardiography is useful in confirming the diagnosis. AO, aorta; AV, aortic valve; LV, left ventricle; MV, mitral valve;RV, right ventricle.

FIGURE 8.1.16. Left-sided superior vena cava (SVC). Diagrammatic representation of left-sided superior vena cava (LSVC) draining into the coronary sinus (CS). Left: the more common course of the LSVC, anterior to the left pulmonary artery (LPA). Right: the much less common course of LSVC, posterior to the LPA. AO, aorta; IVC, inferior vena cava;LAZ, left azygos vein; LPV, left-sided pulmonary veins; RAZ, right azygos vein; RSVC, right-sided superior vena cava. (Reproduced with permission from 

Nanda NC, Pinheiro L, Sanyal R, et al. Transesophageal echocardiographic examination of left-sided superior vena cava and azygos and hemiazygos veins. Echocardiography 1991;8:731–740.

)

FIGURE 8.1.17. Left-sided superior vena cava (SVC) draining into the coronary sinus. A 47-year-old man with nephrotic syndrome and no other congenital cardiac abnormality. A. Transverse (T) imaging planes. The five-chamber (left) view shows enlargement of the coronary sinus (CS), resulting from drainage of the left-sided superior vena cava (LSVC), which is imaged in the aortic short-axis plane (right). B. Longitudinal (L) imaging planes. The two-chamber plane (left) views both the LSVC and the CS, but fails to show their continuity. Counterclockwise rotation of the transducer from this position shifts the plane to the left, resulting in a long-axis delineation of the LSVC and its entrance into the CS (right). C. The relationship of enlarged CS to left circumflex coronary artery (LCX) viewed in transverse and longitudinal planes. D,E. Composite illustrations shows the connection of the LSVC to the CS and their relationship to adjacent cardiac structures. The images used to generate each of these composites were taken from three different but adjacent transverse planes obtained by rotation with slight withdrawal of the probe when imaging the LSVC. E, inset. A spectral trace obtained from color Doppler–guided pulsed Doppler examination of the LSVC. F,G. Another set of composite illustrations, each acquired by combining two adjacent transverse plane images. These demonstrate the LSVC and left azygos vein (LAZ) and their relation to other cardiac structures. H. Longitudinal plane examination shows the relationship of the right SVC to the right pulmonary artery (RPA, left) and the relationship of the LSVC to the left pulmonary artery (LPA, right). The right-sided superior vena cava (RSVC) was imaged by clockwise rotation of the transducer so that the plane was shifted to the right (1 in the inset), whereas the LSVC was imaged by counterclockwise rotation, which moved the plane to the left (2 in the inset). I. Transverse plane examination demonstrates the LSVC located anterior to the LPA (left). The image on the right, obtained by slightly withdrawing the transducer, shows the entrance of the LAZ into the LSVC. Because the LAZ enters the LSVC in almost a perpendicular manner, the axes of the vessels are practically at right angles to each other. In this instance, the LAZ is imaged in long axis, whereas the LSVC is viewed in short axis. These images were obtained by withdrawing the transducer to acquire a standard transesophageal plane, which views the distal ascending aorta (DA) in short axis together with the main pulmonary artery bifurcation and then rotating the transducer counterclockwise to move the plane to the left. J. Transverse and longitudinal plane examination demonstrates the relation of the LAZ to the DA. These planes were obtained by advancing the transducer and rotating it counterclockwise (plane moves to the left) from the position used to obtain images shown in IAO, aorta; LA, left atrium; LAA, left atrial appendage; LV, left ventricle; PE, pericardial effusion; RA, right atrium; RV, right ventricle. (Reproduced with permission from 

Nanda NC, Pinheiro L, Sanyal R, et al. Transesophageal echocardiographic examination of left-sided superior vena cava and azygos and hemiazygos veins. Echocardiography 1991;8:731–740.

)

FIGURE 8.1.18. Left-sided superior vena cava (LSVC) draining into the coronary sinus. A 40-year-old woman, status postrepair of an atrial septal defect, presented with systemic hypertension, left ventricular hypertrophy, and mitral regurgitation (MR). A. Four-chamber view shows a dilated coronary sinus (CS) resulting from the drainage of the LSVC, noted at surgery for repair of atrial septal defect. Contrast injection into a left arm vein using normal saline demonstrates contrast echoes in the CS, right atrium (RA), and right ventricle (RV). B. Transverse plane examination (left) at the level of the aortic root (AO) demonstrates the relationship of the LSVC to the left atrial appendage (LAA) and left upper pulmonary vein (LUPV). Longitudinal plane examination (right) demonstrates the coexistence of a right-sided superior vena cava (RSVC). C,D. Composite illustrations prepared by combining two transverse plane images obtained by rotation of the transesophageal probe demonstrate an enlarged CS entering the RA. The inset in Drepresents a spectral trace obtained by color Doppler—guided pulsed Doppler interrogation of the CS. E. Longitudinal plane examination. The two-chamber view (left) shows marked enlargement of the CS. Note also the presence of MR in this systolic frame. Withdrawing the probe and rotating it counterclockwise (to shift the plane to the left) brings into view the entrance of the LSVC into the CS (right). F. The probe is withdrawn further to the level where the distal ascending aorta (DA) and the main pulmonary artery bifurcation are visualized. When the probe is rotated counterclockwise (to move the plane to the left), it demonstrates the LSVC (imaged in short axis) to be located posterior to the left pulmonary artery (LPA) and in the vicinity of the aorta (AO) in this transverse plane examination (left), in contrast to Figure 8.1.11, where the LSVC was located anterior to the LPA. In the longitudinal plane examination (right), the LSVC is imaged in long axis. G. Withdrawing the probe and rotating it counterclockwise to image the proximal DA brings into view the LAZ, which is seen to enter LSVC in the transverse plane examination (left). Longitudinal plane examination (right, top) shows a posterior intercostal vein (ICV) joining the LAZ posterior to the DA, viewed in long axis. The spectral trace (right, bottom) was obtained by color Doppler—guided pulsed Doppler interrogation of the ICV. LA, left atrium; LAZ, left azygos vein; LV, left ventricle; MV, mitral valve; RPA, right pulmonary artery; TV, tricuspid valve; VS, ventricular septum. (Reproduced with permission from 

Nanda NC, Pinheiro L, Sanyal R, et al. Transesophageal echocardiographic examination of left-sided superior vena cava and azygos and hemiazygos veins. Echocardiography 1991;8:731–740.

)

FIGURE 8.1.19. Left-sided superior vena cava (LSVC) entering the coronary sinus. A 20-year-old man with tetralogy of Fallot and pulmonary atresia. In this patient, the inferior vena cava (IVC) also drained into the coronary sinus (CS). The right superior vena cava (RSVC) drained normally into the right atrium (RA). A. Transverse plane examination shows enlargement of the CS due to LSVC drainage (left). Longitudinal plane examination (right) shows the left azygos vein (LAZ) joining the LSVC. B,C. Composite illustrations, each prepared by placing together contiguous images obtained during transverse (B) and longitudinal (C) plane examinations. The IVC is clearly seen entering the CS. This finding and the presence of LSVC were confirmed angiographically. DA, descending thoracic aorta; HV, hepatic vein; LA, left atrium; LV, left ventricle; MV, mitral valve; RV, right ventricle; TV, tricuspid valve; VS, ventricular septum. (Reproduced with permission from 

Nanda NC, Pinheiro L, Sanyal R, et al. Transesophageal echocardiographic examination of left-sided superior vena cava and azygos and hemiazygos veins. Echocardiography 1991;8:731–740.

)

FIGURE 8.1.20. Left-sided superior vena cava (LSVC). A,B. Enlargement of the coronary sinus (CS) resulting from anomalous drainage of the left-sided superior vena cava (SVC). C. Pulsed Doppler interrogation of the enlarged coronary sinus shows prominent flow signals throughout the cardiac cycle. LA, left atrium; LPV, left upper pulmonary vein; LV, left ventricle; RV, right ventricle.

FIGURE 8.1.21. Left-sided superior vena cava (LSVC). A,B. Relationship of the LSVC to the left upper pulmonary vein (LUPV) and left atrium (LA). C. Contrast signals (arrowheads) moving from the markedly enlarged coronary sinus (CS) into the right atrium (RA) following a left arm vein saline contrast injection. The coronary sinus is enlarged as a result of the increased (anomalous) flow. D. Pulsed Doppler interrogation of the LSVC imaged behind the aorta in the same patient shows continuous flow throughout the cardiac cycle (arrowheads). LV, left ventricle; MV, mitral valve.

FIGURE 8.1.22. Ventricular septal aneurysm. A,B. An aneurysm (arrows) of the membranous septum bulging into the right ventricle (RV). C,D. Another patient with a trabecular ventricular septal aneurysm (arrows) associated with a defect. Flow signals are seen moving from left ventricle (LV) to the RV through the ventricular septal defect.E. Associated severe TR. IVS, ventricular septum; LA, left atrium; RA, right atrium; TV, tricuspid valve.

FIGURE 8.1.23. Transesophageal echocardiographic delineation of ventricular septal aneurysm producing right ventricular outflow obstruction in an adult. A. The arrow points to the ventricular septal aneurysm bulging into right ventricular outflow tract. The ventricular septal defect is delineated by the arrowhead. B. Color Doppler examination shows a narrow turbulent jet (black arrowheads) indicative of significant obstruction produced by the aneurysm (arrow). AV, aortic valve; LA, left atrium; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle; TV, tricuspid valve. (Reproduced with permission from 

Baweja G, Nanda NC, Nekkanti R, et al. Three-dimensional transesophageal echocardiographic delineation of ventricular septal aneurysm producing right ventricular outflow obstruction in an adult. Echocardiography 2004;21:95–97.

)

FIGURE 8.1.24. Perimembranous ventricular septal defect. The defect (D in A and the arrow in B) is demonstrated together with an aneurysm (AN) of the ventricular septum.AO, aorta; AV, aortic valve; LA, left atrium; LV, left ventricle; RV, right ventricle.

FIGURE 8.1.25. Trabecular ventricular septal defect. A large defect (D) is seen in the trabecular (muscular) portion of the ventricular septum (VS). LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

FIGURE 8.1.26. Perimembranous ventricular septal defect. A–E. A. A large defect (D) is seen just below the tricuspid valve (TV) and aortic valve with flow signals (arrow in B) moving through it into the right ventricle (RV). C. A large zone of flow acceleration (arrow) on the left ventricle (LV) side of the defect. Color Doppler–guided continuous wave Doppler shows a high velocity of 5 m/sec. Using the Bernoulli equation, this translates into a pressure gradient of 100 mm Hg across the defect. Because this patient's systolic blood pressure was 125 mm Hg, the PA systolic pressure is estimated to be about 25 mm Hg. Thus, pulmonary hypertension is absent. E. A smaller second ventricular septal defect (VSD) (D2) is noted in addition to the large defect (D1) seen earlier. F,G. A perimembranous ventricular septal defect (arrowheads) with left-to-right shunting in another patient. H,I. Schematics show a perimembranous VSD. AO, aorta; LA, left atrium; LVOT, left ventricular outflow tract; MV, mitral valve; RA, right atrium; VS, ventricular septum.

FIGURE 8.1.27. Ventricular septal (VS) defect associated with atrial septal (IAS) defect. A. A large ventricular septal defect (open arrow) and an associated secundum atrial septal defect (closed arrow). B,C. Color Doppler examination. B. Flow signals moving from the left atrium (LA) to the right atrium (RA) (upper arrow) and from the left ventricle (LV) to the right ventricle (RV) (lower arrow). C. The ventricular septal defect is seen just underneath the tricuspid valve (TV), demonstrating its perimembranous location.

FIGURE 8.1.28. Ventricular septal defect mimic. A side-lobe artifact (arrows), which simulates the presence of a ventricular septal defect in all three patients shown here. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle; VS, ventricular septum.

FIGURE 8.1.29. Patent ductus arteriosus. A,B. Turbulent flow (arrow) in the main pulmonary artery (MPA) produced by a patent ductus arteriosus (PDA) in this adult patient.C. Continuous wave Doppler examination shows flow throughout the cardiac cycle. D. The connection (arrow) between the PDA and the MPA. E–G. Color Doppler examination also delineates the PDA and its connection with the MPA. H. The relationship of the PDA with the MPA and the left pulmonary artery (LPA) is shown. I. Schematic shows a PDA.J,K. These views, obtained after surgery, show absence of turbulence in the MPA. AO, aorta; LA, left atrium; PV, pulmonary valve; RPA, right pulmonary artery; SVC, superior vena cava.

FIGURE 8.1.30. Patent ductus arteriosus (PDA). A. Color Doppler examination shows turbulent flow signals moving from the aorta (AO) into the pulmonary artery (PA) through the PDA. Color M-mode in B and pulsed Doppler in C show continuous flow throughout the cardiac cycle through the PDA. DA, descending aorta; SV, Doppler sample volume. D.Gross specimen shows a PDA.

FIGURE 8.1.31. Left pulmonary vein (LPV) obstruction. A. An extensive, thick linear echo indicative of a membrane (M) is seen interposed between dilated left-sided pulmonary veins (PV) and the left atrium (LA) in this 9-year-old boy. B. Color Doppler examination. A narrow jet (J) is visualized originating from a small area of discontinuity in the membrane and moving into the LA. The jet measured 2 mm at its origin, indicative of severe obstruction. C. High-pulse-repetition-frequency Doppler interrogation of the jet demonstrates a high peak velocity of 1.8 m/sec and continuous flow throughout the cardiac cycle with little phasic variation. Note also the presence of spectral broadening, indicative of turbulent flow. D. Postoperative study. After surgical repair there is marked increase in proximal jet width to 7 mm; significant reduction in peak velocity to 1.3 m/sec; and development of prominent phasic variations. Spectral broadening is still noted and indicates persistence of turbulent flow. E. Postoperative study shows pulmonary veins communicating with the LA through two relatively wide channels, one located posteriorly (upper closed arrows) in the usual location of the pulmonary vein—the LA junction, and the other more anteriorly (lower closed and open arrows), representing direct anastomosis of the left common pulmonary vein with the left atrial appendage (LAA). AO, aorta. (Reproduced with permission from 

Samdarshi TE, Morrow R, Helmcke FR, et al. Assessment of pulmonary vein stenosis by transesophageal echocardiography. Am Heart J 1991;122:1495–1498.

)

FIGURE 8.1.32. Cor triatriatum. A. The schematic shows a membrane separating the left atrium (LA) into two chambers, with pulmonary veins on one side and the left atrial appendage (LAA) on the other. Notice the small orifice in the membrane causing obstruction to blood flow. B–I. An adult patient with a cor triatriatum membrane (arrowheads). In F through H, the membrane appears to be an extension of the “Q-tip” separating the LAA from the left upper pulmonary vein (LUPV). In E, the membrane (arrowheads) appears to have a double attachment to the interatrial septum (IAS). G,I. Color Doppler examination shows laminar flow signals moving through the membrane, indicating the absence of obstruction. J,K. Another adult patient with cor triatriatum. J. A wide opening (1.4 cm; arrow) in the membrane is seen separating the LUPV from the LAA. K. Color Doppler study demonstrates some turbulence, but the peak velocity measured less than 1 m/sec, indicating the absence of any significant obstruction to flow. AO, aorta; AV, aortic valve; IVS, ventricular septum; LV, left ventricle; MPA, main pulmonary artery; MV, mitral valve; RA, right atrium; RV, right ventricle.

FIGURE 8.1.33. Congenital mitral stenosis. Diastolic frames demonstrate thickened mitral leaflets with a very small orifice and a narrow flow jet (arrow in B), indicative of severe stenosis. Note the unobstructed opening of the tricuspid valve (TV) in A. This patient was known to have mitral stenosis since childhood. LA, left atrium; LV, left ventricle; MV, mitral valve; RA, right atrium; RV, right ventricle.

FIGURE 8.1.34. Abnormal papillary muscle insertion. The papillary muscle (PM) inserts directly into the anterior mitral leaflet (AML). Note the absence of the chordae tendinae. AO, aorta; LA, left atrium; LV, left ventricle; PML, posterior mitral leaflet; RV, right ventricle.

FIGURE 8.1.35. Transesophageal echocardiogram in a stenotic double orifice parachute mitral valve (MV) with a single papillary muscle. A. Arrowheads point to diastolic doming of the double orifice MV producing a typical “seagull” appearance. LA, left atrium; LV, left ventricle. B. Arrowheads point to two separate jets moving from the LA into the LV through the two orifices. C. Transgastic view. Arrowheads demonstrate the two orifices that have a typical “figure-of-eight” configuration. D. Transgastric view showing thickened chordae (arrowheads) converging from both orifices of the mitral valve into a single large papillary muscle (PM). (Reproduced with permission from 

Yesilbursa D, Miller A, Nanda NC, et al. Echocardiographic diagnosis of a steotic Double orifice parachute mitral valve with a single papillary muscle. Echocardiography 2000;17:349–352.

)

FIGURE 8.1.36. Discrete subaortic membranous stenosis. The arrows in A,C, and D and MB in B point to a prominent subaortic membrane. E. Two jets (arrowheads) of moderate aortic regurgitation (AR). F. Postoperative study shows the absence of the membrane in the left ventricular outflow tract (LVOT). AO, aorta; AV, aortic valve; LA, left atrium; LV, left ventricle; MV, mitral valve; RV, right ventricle; RVO, right ventricular outflow tract; VS, ventricular septum.

FIGURE 8.1.37. Discrete subaortic membranous stenosis. A,B. A discrete membrane (arrows) is shown attached to the base of an aortic cusp in A and to the anterior mitral leaflet in B. C. Flow acceleration and turbulence in the left ventricular outflow tract (LVOT) (arrowhead) produced by the membrane. D. Postoperative study shows widening of the LVOT and absence of the membrane. AO, aorta; AV, aortic valve; LA, left atrium; LV, left ventricle; MV, mitral valve; RA, right atrium; RV, right ventricle; VS, ventricular septum.

FIGURE 8.1.38. Discrete subaortic membranous stenosis. A,B. The membrane (M) is clearly separated from the aortic valve (AV) cusps. C. Prominent early systolic preclosure of the AV (arrow) with coarse fluttering typical of this entity. D. The attachment of the membrane (M) to the base of the anterior mitral leaflet is well seen. LA, left atrium; LV, left ventricle; MV, mitral valve; RA, right atrium; TV, tricuspid valve.

FIGURE 8.1.39. Discrete subaortic membranous stenosis. A. A narrow jet (arrow) in the left ventricular outflow tract (LVOT) results from obstruction produced by a subaortic membrane. B. Gross specimen of the subaortic membrane resected from the patient shown in A. AO, aorta; LA, left atrium; LV, left ventricle; RVOT, right ventricular outflow tract.

FIGURE 8.1.40. Discrete subaortic membranous stenosis. Modified Konno's operation. In this patient the left ventricular outflow tract (LVOT) was widened by surgically creating a ventricular septal defect and then closing it with a patch. A. During systole the patch bulges into the right ventricle (RV), widening the LVOT. B. Color Doppler examination shows minimal turbulence in the LVOT. Note the presence of associated mild mitral regurgitation (MR) in this patient. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

FIGURE 8.1.41. Bicuspid aortic valve (AV). A. A vertically oriented, redundant bicuspid AV in diastole. B. Another patient with a vertically oriented bicuspid valve with restricted opening, consistent with stenosis. C. Schematic corresponds to the echo image in B. D, E. Two other patients show an obliquely oriented bicuspid aortic valve (AOV inE) with a raphe (R in E) that extends from the anterior cusp to the aortic wall in both patients. LA, left atrium; RA, right atrium; RVO, RVOT, right ventricular outflow tract.

FIGURE 8.1.42. Quadricuspid aortic valve (AV). A. Diastolic frame in a 69-year-old woman clearly shows four aortic cusps with a large eccentrically located area of leaflet noncoaptation. B. Systolic frame shows the four cusps in the open position. C. Color Doppler examination in the five-chamber view (plane angle 0°) shows mosaic colored signals filling the proximal left ventricular outflow tract (LVOT) completely in diastole, indicative of severe aortic regurgitation (AR). D. Color Doppler examination shows flow signals from the AR filling the area of cusp noncoaptation. A,B, and D were imaged at a plane angle of 44°. 1, accessory cusp; 2, left cusp; 3, right cusp; 4, noncoronary cusp. AO, aorta; LA, left atrium; LCA, left coronary artery; RA, right atrium; RCA, right coronary artery; RVO, right ventricular outflow tract. (Reproduced with permission from 

Patel JN, Osman K, Nanda NC, et al. Quadricuspid aortic valve diagnosed by multiplane transesophageal echocardiography. Echocardiography 1994;11:201–205.

)

FIGURE 8.1.43. Quadricuspid aortic valve (AV) mimic. A redundant tricuspid AV (systolic frame) that mimics a quadricuspid valve in diastole is shown. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle. (Reproduced with permission from 

Patel JN, Osman K, Nanda NC, et al. Quadricuspid aortic valve diagnosed by multiplane transesophageal echocardiography. Echocardiography 1994;11:201–205.

).

FIGURE 8.1.44. Redundant aortic valve (AV). A myxomatous AV appears to have multiple cusps in diastole (1–6), but in systole only three cusps are seen. Figures 8.1.43 and8.1.44 underscore the importance of carefully examining the AV throughout the cardiac cycle for assessment of its morphology. LA, left atrium; RA, right atrium; RVO, right ventricular outflow tract. (Reproduced with permission from 

Patel JN, Osman K, Nanda NC, et al. Quadricuspid aortic valve diagnosed by multiplane transesophageal echocardiography. Echocardiography 1994;11:201–205.

)

FIGURE 8.1.45. Infected quadricuspid aortic valve (AV). A,B. Transverse plane examination demonstrates four aortic cusps (1, 2, 3, and 4) and a saccular aneurysm (AN) of the noncoronary sinus of Valsalva communicating with the aortic root. C,D. The aneurysm also is visualized in the longitudinal plane views. E. Color Doppler examination shows mosaic signals completely filling the proximal portion of the left ventricular outflow tract (LVOT) in diastole, indicating the presence of severe aortic regurgitation (AR). This was a 33-year-old man with past drug abuse who presented with a 1-month history of fever and increasing dyspnea on exertion. AO, aorta; LA, left atrium; LV, left ventricle;MV, mitral valve; RA, right atrium; RPA, right pulmonary artery; RV, right ventricle; RVO, right ventricular outflow; SVC, superior vena cava. (Reproduced with permission from

Finch A, Osman K, Kim KS, et al. Transesophageal echocardiographic findings of an infected quadricuspid aortic valve with an anomalous coronary artery. Echocardiography 1994;11:369–375.

)

FIGURE 8.1.46. Infected quadricuspid aortic valve (AV): anomalous origin of right coronary artery from left main coronary artery. Same patient as in Fig. 8.1.45. A. Transverse plane aortic short-axis view demonstrates the left main coronary (LM) artery trifurcating into circumflex (CX), ramus (R), and left anterior descending (LAD) branches. B. A segment of the right coronary artery (RC) is seen between the aorta (AO) and the right ventricular outflow tract (RVOT)/main pulmonary artery (PA). Coronary arteriography demonstrated an anomalous right coronary artery arising from the left main coronary artery. LA, left atrium; SVC, superior vena cava; AN, anomalous. (Reproduced with permission from 

Finch A, Osman K, Kim KS, Transesophageal echocardiographic findings of an infected quadricuspid aortic valve with an anomalous coronary artery. Echocardiography 1994;11:369–375.

)

FIGURE 8.1.47. Unicommissural unicuspid aortic valve (AV). In the long-axis view, the unicuspid valve appears to have two separate leaflets, one large (posterior) and the other smaller (anterior). This appearance mimics a bicuspid aortic valve. AO, aorta; LA, left atrium; LVOT, left ventricular outflow tract. (Reproduced with permission from

Osman K, Nanda NC, Kim KS, et al. Transesophageal echocardiographic features of unicuspid aortic valve. Echo-cardiography 1994;11:469–473.

).

FIGURE 8.1.48. Unicommissural unicuspid aortic valve (AV). Same patient as in Figure 8.1.44. Short-axis view at the level of the AV demonstrates only one commissural attachment to the aortic wall. The inset shows the smallest orifice area. This measures 0.4 cm2, indicative of severe stenosis, which was confirmed subsequently by the surgeon.LA, left atrium; RA, right atrium; RVO, right ventricular outflow tract. (Reproduced with permission from 

Osman K, Nanda NC, Kim KS, et al. Transesophageal echocardiographic features of unicuspid aortic valve. Echocardiography 1994;11:469–473.

)

FIGURE 8.1.49. Bicuspid aortic valve (AV) mimicking unicommissural or acommissural AV. A. Short-axis view clearly shows two commissural attachments in this patient with a stenotic bicuspid AV (left). In some views it mimicked a unicommissural AV, however, because only one commissural attachment was noted because of improper transducer angulation (right). B. Short-axis view in another patient with a stenotic bicuspid aortic valve does not show any distinct and convincing commissural attachment, raising the possibility of an acommissural aortic valve (left). This was seen from imaging the valve at the tip rather than the base (right), where the commissural attachments are typically seen. (Reproduced with permission from 

Osman K, Nanda NC, Kim KS, et al. Transesophageal echocardiographic features of unicuspid aortic valve. Echocardiography 1994;11:469–473.

)

FIGURE 8.1.50. A–M. Aortic valve (AV) stenosis. A–M. B demonstrates systolic doming of the AV consistent with stenosis. C. A narrow jet (arrowheads) originates from the AV in systole. D. Poststenotic dilatation of the ascending aorta (AO). E. The numbers 1, 2, and 3 show the location of short-axis sections in subsequent frames. These sections are taken at the base (3), at the middle of the valve (2), and at the flow-limiting orifice at top of the domed valve (1). The corresponding short-axis views are shown in F and G(base); H and I (midlevel); and J and K (at the top of the domed valve). Note that the minimal valve area (0.83 cm2) is obtained at the flow-limiting orifice at the top of the domed valve and that it corresponds to the catheterization derived AV area. L. Composite demonstrates cross-sectional areas at the different locations on the domed valve. M.Color Doppler examination shows turbulent flow signals appearing in the AV orifice. To obtain the correct area (flow-limiting orifice) the minimal area must be determined. To do this, the probe must be moved up and down the esophagus until the smallest short-axis area at the top of the domed AV is obtained. Color Doppler is useful in identifying the location of the aortic orifice in patients with calcified valves that do not move in systole. However, planimetry of the color jet overestimates valve area and should not be used.LA, left atrium; LV, left ventricle; LVO, left ventricular outflow tract; PA, pulmonary artery; RA, right atrium; RV, right ventricle; RVO, right ventricular outflow tract. (Lreproduced with permission from 

Kim KS, Maxted W, Nanda NC, et al. Comparison of multiplane and biplane transesophageal echocardiography in the assessment of aortic stenosis. Am J Cardiol 1997;79:436–441.

)

FIGURE 8.1.51. Redundant bicuspid aortic valve (AV). The arrow in A points to mild systolic doming of the AV, but the valve appears to open well when viewed in short axis(B), consistent with minimal stenosis. C. A late systolic frame shows mild preclosure, resulting in decreased cross-sectional area, which should not be confused with aortic stenosis. The arrow in D shows a redundant valve prolapsing into the left ventricular outflow tract (LVOT), producing severe aortic regurgitation (AR) (arrowhead in E). F,G.Short-axis views show AV redundancy and noncoaptation with resultant AR (black arrows). AO, aorta; LA, left atrium; LV, left ventricle; MV, mitral valve; RA, right atrium; RV,right ventricle; RVO, right ventricular outflow tract.

FIGURE 8.1.52. Supravalvular aortic stenosis. A. Narrowing of the aorta (AO; arrows) beyond the aortic valve (AV), producing turbulent flow. B. Supravalvular narrowing (arrow) in another patient. C. A narrowed, turbulent jet (arrows) in the area of stenosis in the same patient shown in BAA, ascending aorta; LA, left atrium; LVO, left ventricular outflow tract; RPA, right pulmonary artery.

FIGURE 8.1.53. Transesophageal echocardiography in the right-sided aortic arch (AA) without dissection. A. Color Doppler examination shows the proximal AA oriented in a left-to-right and anteroposterior direction. The superior vena cava (SVC) is imaged anterior to AA. Imaging of a large vascular structure such as the SVC next to the AA may mimic aortic dissection. B. Slight advancement and rotation of the probe from the position used to image the proximal AA brings into view the distal portion of the AA, which is identified by the posteroanterior direction of blood flow. Both A and B were imaged at a plane angulation of 0 degree. AZ, azygos vein. C. The right innominate vein (RIV) is seen joining the SVC. Saline contrast injections through a catheter placed in the right external jugular vein demonstrated contrast signals entering the SVC from RIV (arrowhead). (Reproduced with permission from 

Nanda NC, Samal AK, Bakir S. Transesophageal echocardiographic diagnosis of right-sided aortic arch. Echocardiography 1998;15:409–417.

)

FIGURE 8.1.54. Transesophageal echocardiography in right-sided aortic arch (AA) with traumatic aortic injury (dissection). A. The dissection flap as well as the communication (arrow) from the AA into the pseudoaneurysm (PSA) are clearly shown. The superior vena cava (SVC) is imaged anterior to AA. B. Shows the origin of the right subclavian artery (RSA) from the AA. The small arrow points to the dissection flap. C. Azygos vein (AZ) viewed in long axis next to the descending aorta (DA). V represents a venous valve at the junction of a tributary. D. Pulsed Doppler interrogation of the AZ vein shows typical venous type of flow. PLE, pleural effusion. (Reproduced with permission from 

Nanda NC, Samal AK, Bakir S. Transesophageal echocardiographic diagnosis of right-sided aortic arch. Echocardiography 1998;15:409–417.

)

FIGURE 8.1.55. Coarctation of the aorta. A. Longitudinal plane examination in a 58-year-old man previously diagnosed with aortic valve (AV) stenosis who presented with a 4-month history of progressive dyspnea and dizziness exacerbated by exertion. The coarcted segment (arrows) and poststenotic dilatation of the descending thoracic aorta (DA) distal (D) to the coarctation are shown. Inset: the coarctation site (arrows) on the aortogram. B. Color Doppler–guided high-pulse-repetition-frequency Doppler examination demonstrates a high velocity of 3.5 m/sec (equivalent to a peak pressure gradient of 49 mm Hg) across the coarctation (arrow). C. Collaterals (arrows) are present in the vicinity of the coarctation. Inset: a spectral tracing from a collateral vessel, which typically shows flow signals in systole and diastole. D. A large posterior intercostal artery (C) carries blood into the descending thoracic aorta beyond the coarctation site. This is demonstrated by color and pulsed Doppler (inset) examinations. E. Schematic shows longitudinal plane examination. The arrow points to the coarctation site. DA(P), descending thoracic aorta proximal to the coarctation site; FA, flow acceleration. (Reproduced with permission from 

Ryan K, Sanyal RS, Pinheiro L, et al. Assessment of aortic coarctation and collateral circulation by biplane transesophageal echocardiography. Echocardiography 1992;9:277–285.

)

FIGURE 8.1.56. Coarctation of the aorta (AO) associated with bicuspid aortic valve (AV) and subaortic membranous stenosis. A,B. Longitudinal plane examination in a 23-year-old woman with a 1-month history of shortness of breath and absence of left radial and lower extremity pulses. The coarcted segment (arrows) and poststenotic dilatation of the descending aorta (DA) distal (D) to the coarctation are well seen. FA represents the flow acceleration or convergence noted immediately proximal to the coarctation. C.Longitudinal plane examination demonstrates aliased signals in LVOT consistent with high flow velocity of approximately 2.5 m/sec (inset). D. Longitudinal plane examination demonstrates the subaortic membrane (horizontal arrows) and the bicuspid AV. Tethering of the left cusp of the AV to the membrane is also shown (right, oblique arrow). This finding was confirmed at surgery. E. Transverse plane examination demonstrates a thickened bicuspid AV with a raphe (R, arrow) in the open (left) and closed (right) positions.F. Transverse plane examination demonstrates eccentric closure of the bicuspid aortic valve as well as tethering (arrow) of the left aortic cusp to the subaortic membrane (M).LA, left atrium; LV, left ventricle; MV, mitral valve; PA, pulmonary artery; RV, right ventricle; RVO, right ventricular outflow tract; VS, ventricular septum. (Reproduced with permission from 

Ryan K, Sanyal RS, Pinheiro L, et al. Assessment of aortic coarctation and collateral circulation by biplane transesophageal echocardiography. Echocardiography 1992;9:277–285.

)

FIGURE 8.1.57. Coarctation of the aorta. A. Longitudinal plane examination in a 17-year-old-female with a 1-year history of dyspnea on exertion. The narrowed segment (arrows) as well as the poststenotic dilation of the descending thoracic aorta (DA) distal (D) to the coarctation site are well visualized. B. Transverse plane examination demonstrates a large collateral (C) vessel (dilated posterior intercostal artery) bringing flow to the descending thoracic aorta (DA) beyond the coarctation site. C,D. Color Doppler–guided pulsed Doppler interrogation of the collateral vessels shows prominent flow signals in systole and diastole. The collateral vessel in D probably represents a dilated bronchial artery. DA(P), descending thoracic aorta proximal to coarctation. (Reproduced with permission from 

Ryan K, Sanyal RS, Pinheiro L, et al. Assessment of aortic coarctation and collateral circulation by biplane transesophageal echocardiography. Echocardiography 1992;9:277–285.

)

FIGURE 8.1.58. Coarctation of the aorta. Gross specimen shows coarctation of the aorta.

FIGURE 8.1.59. Right ventricular trabeculation. Prominent nonobstructing trabeculations (TB) are seen in the right ventricular outflow tract (RVOT) adjacent to the pulmonary vein (PV)

FIGURE 8.1.60. Double-chambered right ventricle (RV). A. A small ventricular septal defect (arrow). A prominent hypertrophied muscle band divides the RV into two chambers, C1 and C2 (A,B). C. Turbulent flow signals (arrow) consistent with obstruction are produced by the hypertrophied muscle bands running transversely through the RV body. AO, aorta; LA, left atrium; LV, left ventricle; RA, right atrium; VS, ventricular septum.

FIGURE 8.1.61. Infundibular stenosis. The arrows in A and B show narrowing of the right ventricular outflow tract (RVO) below the pulmonary valve (PV), which is structurally normal. P in A points to a patch that had been used to close a ventricular septal defect. C. Turbulent flow signals (arrow) in the RVOT and pulmonary artery (PA) produced by infundibular stenosis. AO, aorta; LA, left atrium; LV, left ventricle; MV, mitral valve; RA, right atrium.

FIGURE 8.1.62. Infundibular stenosis. A. Marked narrowing of the right ventricular outflow tract (RVO) (arrow) consistent with infundibular stenosis. B,C. Turbulent flow signals (arrows) produced by infundibular stenosis. D. Infundibular stenosis (arrows) in another patient. The pulmonary valve (PV) is only mildly thickened. AO, aorta; LA, left atrium; PA, pulmonary artery; RV, right ventricle; SVC, superior vena cava.

FIGURE 8.1.63. Bicuspid pulmonary valve. Wide separation of the PV leaflets (arrowheads) in systole, indicative of absence of stenosis. The PV was bicuspid in this patient. LV, left ventricle; RA, right atrium; RVOT, right ventricular outflow tract.

FIGURE 8.1.64. Pulmonary valve (PV) stenosis. A. The PV shows restricted opening in systole, consistent with stenosis. The valve leaflets appear only mildly thickened. B.Color Doppler examination shows a narrow turbulent jet at the PV level, confirming the presence of severe stenosis. AO, aorta; AV, aortic valve; LA, left atrium; PA, pulmonary artery; RA, right atrium; RVOT, right ventricular outflow tract.

FIGURE 8.1.65. Bicuspid pulmonary valve (PV) stenosis associated with levotransposition or corrected transposition of the great arteries. Transverse (T) plane at the level of the great vessels in a 14-year-old boy. A. Diastolic frame shows the PV in the closed position. Note the transversely oriented commissure and the abnormal location of the valve posterior to and to the right of the AV. B. Systolic frame shows markedly restricted motion of the PV leaflets and a very small orifice, indicative of severe stenosis. AV, aortic valve; LA, left atrium; RA, right atrium. (Reproduced with permission from 

Finch AD, Snell DR, Sanyal RS, et al. Transesophageal echocardiographic identification of a bicuspid pulmonary valve associated with congenitally corrected transposition of the great arteries. Echocardiography 1993;10:359–362.

)

FIGURE 8.1.66. Idiopathic dilatation of the pulmonary artery (PA). A,B. The PA is markedly dilated. The pulmonary valve (PV) is not thickened and opens normally. No other abnormality was detected in this adult, either clinically or on echocardiographic examination. LA, left atrium; LVO, left ventricular outflow tract; RVO, right ventricular outflow tract.

FIGURE 8.1.67. Complete atrioventricular septal (canal) defect. A. The four-chamber view in this elderly woman demonstrates both the atrial (open arrow; ASD) and ventricular (closed arrow) components of the atrioventricular septal (canal) defect. Shunting occurred only at the atrial level because the ventricular component was found closed by redundant septal tricuspid valve (TV) leaflet tissue. Both atrial (D) and ventricular (open arrow) defects are well seen. B–E. Another patient with complete atrioventricular septal defect. The right ventricle (RV) is markedly hypertrophied because of the presence of pulmonary hypertension. C. The open leaflets (arrows) of the common atrioventricular valve. D. Atrioventricular valve regurgitation jet (open arrows) straddling the interatrial septum (IAS). E. Two jets of regurgitation (R, R) are clearly seen originating from the common atrioventricular valve (closed arrow), one extending into the right atrium (RA) and the other into the left atrium (LA). The open arrow points to the ventricular defect, through which left-to-right shunting is shown (blue signals). F. Another patient with a common atrioventricular septal defect demonstrating left ventricle (LV) to RA shunt (arrow) as well as mitral regurgitation (MR). Note the huge atrial defect and a much smaller ventricular defect. VS, ventricular septum.

FIGURE 8.1.68. Ebstein anomaly. A,B. Transverse plane examination in a 24-year-old man shows the free (A) component of the anterior tricuspid leaflet as well as the part tethered (arrowheads) to the right ventricle (RV) free wall. B. Marked displacement (arrows) of the septal (S) tricuspid leaflet. C. Longitudinal plane examination shows both the free (P) and tethered (arrowheads) components of the posterior (P) tricuspid leaflet. A, anterior TV leaflet. AV, aortic valve; LA, left atrium; LV, left ventricle; RA, right atrium; VS, ventricular septum. (Reproduced with permission from 

Maxted W, Nanda NC, Kim KS, et al. Transesophageal echocardiographic identification and validation of individual tricuspid valve leaflets. Echocardiography 1994;11:585–596.

)

FIGURE 8.1.69. Ebstein anomaly. A,B. The four-chamber view (at multiplane angulations of 180° and 0°) in a 72-year-old woman shows ventricular deviation (arrowheads) of a diminutive septal leaflet (S) of the tricuspid valve (TV). The anterior tricuspid leaflet has two portions—one freely mobile (A) and the other tethered (arrow in B) to the anterior wall of the right ventricle (RV). C–E. Multiplane angulations of 111° (C), 93° (D), and 90° (E) show free (P, A) and tethered (arrow in C and arrowheads in D) components of both posterior (P) and anterior (A) tricuspid leaflets. F. Color Doppler examination done at a plane angulation of 180° shows predominantly nonmosaic red flow signals filling a large portion of the huge right atrium (RA) in systole, indicative of very severe TR. Absence of mosaic signals reflects an insignificant pressure gradient across the noncoapting and, hence, very severely incompetent TV, with the RV and RA acting practically as one chamber. AO, aorta; LA, left atrium; LV, left ventricle; MV, mitral valve; VS, ventricular septum; EV, eustachian valve; TR, tricuspid regurgitation. (Reproduced with permission from 

Maxted W, Nanda NC, Kim KS, et al. Transesophageal echocardiographic identification and validation of individual tricuspid valve leaflets. Echocardiography 1994;11:585–596.

)

FIGURE 8.1.70. Ebstein anomaly with secundum atrial septal defect. Transverse (A) plane examination in a 32-year-old female demonstrates marked ventricular deviation (arrowheads) of the septal (S) leaflet of the tricuspid valve (TV). The anterior (A) leaflet is elongated. Longitudinal plane (B,C) examination shows both free (P) and tethered (arrowheads) portions of the posterior (P) tricuspid leaflet. D in C denotes an associated secundum atrial septal defect. AO, aorta; LA, left atrium; LV, left ventricle; MV, mitral valve; RA, right atrium; RV, right ventricle. (Reproduced with permission from 

Maxted W, Nanda NC, Kim KS, et al. Transesophageal echocardiographic identification and validation of individual tricuspid valve leaflets. Echocardiography 1994;11:585–596.

)

FIGURE 8.1.71. Ebstein anomaly. A–C. Transverse plane four-chamber views in a 17-year-old male show the freely moving portion of the anterior tricuspid leaflet as well as the component tethered (arrowheads) to the right ventricle (RV) anterior free wall. Two parts of the septal tricuspid leaflet (S) are also demonstrated—a small portion has a bubble-like appearance near the annulus, and the other, larger component shows marked inferior displacement (arrows) into the RV. The bubble-like appearance of the tethered portions of the anterior and septal leaflets results from nonuniform tethering of the leaflets to RV wall. D. Longitudinal plane examination shows two portions of the posterior leaflet—one freely moving (P) and the other tethered to the RV posterior (inferior) wall (arrowheads). E,F. Transgastric examination also shows both the freely moving (P) and the tethered portions (arrowheads) of the posterior tricuspid leaflet. G. Color Doppler examination in the four-chamber view demonstrates mosaic signals filling a large portion of the right atrium (RA) in systole, indicative of severe tricuspid valve (TR). CH, chordae tendinae; LA, left atrium; LV, left ventricle; MV, mitral valve; PA, pulmonary artery;VS, ventricular septum. (Reproduced with permission from 

Maxted W, Nanda NC, Kim KS, et al. Transesophageal echocardiographic identification and validation of individual tricuspid valve leaflets. Echocardiography 1994;11:585–596.

)

FIGURE 8.1.72. Corrected transposition of the great vessels with Ebstein malformation of the left-sided atrioventricular valve. Transverse (A) and longitudinal (B,C) plane examinations in a 13-year-old boy show ventricular deviation (arrowheads) of both the septal (S) and posterior (P) leaflets of the left-sided tricuspid valve (TV). A, anterior TV leaflet; CH, chordae tendinae. LA, left atrium; LV, left ventricle; MV, mitral valve; RA, right atrium; RV, right ventricle. (Reproduced with permission from 

Maxted W, Nanda NC, Kim KS, et al. Transesophageal echocardiographic identification and validation of individual tricuspid valve leaflets. Echocardiography 1994;11:585–596.

)

FIGURE 8.1.73. Ebstein anomaly. A. Schematic shows Ebstein anomaly with a secundum atrial septal defect. The anterior tricuspid valve (TV) leaflet is elongated and the septal leaflet is plastered against the ventricular septum. The opening of the TV is displaced into the right ventricle (RV). B. Pathology specimen shows the septal leaflet of the TV plastered over the ventricular septum and the RV inferior wall.

FIGURE 8.1.74. Tricuspid atresia. A. Schematic of tricuspid atresia. A thick, atretic tricuspid valve (TV) is seen with a hypoplastic right ventricle (RV) and both atrial and ventricular septal defects. B. Tricuspid atresia. Atretic tricuspid valve (ATV) and large atrial secundum (D) and ventricular septal defects (VSD) are shown. C. Color Doppler examination in the same patient shows shunt flow (open arrow) from the right atrium (RA) to the left atrium (LA). The closed arrow points to the VSD. LV, left ventricle; MV, mitral valve.

FIGURE 8.1.75. Tricuspid atresia. A. An atretic tricuspid valve (ATV) and a large ventricular septal defect (VSD) are seen in this adult patient. B. Shunt flow from right atrium (RA)/superior vena cava (SVC) to right pulmonary artery (RPA) through a direct surgical anastomosis is shown. The atrial septal defect, but not the VSD, has been closed. C.Pulsed Doppler examination shows shunt flow into RPA throughout the cardiac cycle. LA, left atrium; LV, left ventricle; MV, mitral valve; RV, right ventricle; VS, ventricular septum.

FIGURE 8.1.76. Tetralogy of Fallot. A,B. The aorta (AO) overriding the ventricular septum (VS). The arrow points to the ventricular septal defect. C,D. Color Doppler examination shows shunt flow from the left to the right ventricle (RV) through the ventricular septal defect. E,F. Another patient with tetralogy of Fallot. A large ventricular septal defect and infundibular stenosis (arrow) are imaged using longitudinal plane examination. G. A different patient with tetralogy of Fallot. Longitudinal plane study demonstrates a large ventricular septal defect (arrow) and severe infundibular stenosis (IS). The pulmonary valve (PV) is only mildly thickened. H. Postoperative study in another patient shows the patch (arrow) used to close the ventricular septal defect. I. Gross specimen of tetralogy of Fallot shows the aorta partly arising from the RV. The ventricular septal defect is seen just below the aortic valve (AV). LA, left atrium; LV, left ventricle; LVOT, left ventricular outflow tract; MV, mitral valve; RA, right atrium;RVOT, right ventricular outflow tract.

FIGURE 8.1.77. Transposition of the great vessels. A. The pulmonary artery (PA) arises from the left ventricle (LV) and the aorta (AO) from the right ventricle (RV). B–D. Gross specimens showing the aorta arising from the RV (B,D) and the main and left pulmonary arteries arising from the LV (C,D). E. The PA is located posterior and to the right of the aortic root (AO), indicative of transposition. The left main coronary artery (LMCA) can be seen arising from the aortic root. F. Another patient with transposition of the great vessels. The PA is located posterior and to the left of the aorta (AO). The LMCA arises from the aortic root at approximately the 12 o'clock position and then courses to the left posterior to the PA. G,H. A different patient with transposition of the great vessels, status post–Mustard procedure. G. The intraatrial baffle separating the pulmonary venous return from the systemic venous flow is well visualized. H. An M-mode study shows the motion pattern of the baffle. I. Obstruction (arrowhead) in the pulmonary venous portion (PVP) of the atrium in another patient with transposition of the great vessels who had undergone a Mustard procedure. SVP, systemic venous portion of the atrium. LAA, left atrial appendage; TV, tricuspid valve.

FIGURE 8.1.78. Transposition of the great vessels. A. The pulmonary artery (PA) is located posteriorly as compared to the aortic root and valve (AOV), typical of the transposition of the great vessels. The arrow points to associated subpulmonic membranous obstruction, and D indicates a large ventricular septal defect. B–H. A 13-year-old boy with transposition of the great vessels demonstrating the posterior location of the PA and pulmonary valve (PV) as compared to the aorta and the aortic valve (AV). In B, the PV appears redundant and prolapses into the left ventricular outflow tract (LVOT). D,E, and H show systolic doming of the PV, with turbulent flow signals in the PA consistent with stenosis. PV thickening is well seen in F. A large associated ventricular septal defect (arrowheads in D and H and D in G) also is visualized. G. Both the mitral valve (MV) and tricuspid valve (TV) are shown opening into the left ventricle (LV), typical of a double inlet LV. H. A secundum atrial septal defect (arrow) is present. IAS, atrial septum; LA, left atrium; RA, right atrium; SVC, superior vena cava.

FIGURE 8.1.79. Double outlet right ventricle: juxtaposition of the atrial appendages. Both left atrial appendage (LAA) and right atrial appendage (RAA) are imaged on the left side and appear to have similar morphology. C, catheter in the left atrium (LA). AO, aorta; SVC, superior vena cava.

FIGURE 8.1.80. Double outlet right ventricle. A–E. Both the aorta (AO) and the pulmonary artery (PA) are shown arising from the right ventricle (RV) in this 10-year-old boy. The tricuspid valve (TV) straddles the ventricular system (VS). C. Large ventricular septal defect (VSD) and secundum atrial (D) septal defect are also noted. Pulsed Doppler examination of the VSD (noncommitted) shows flow signals moving into the RV from the left ventricle (LV) throughout the cardiac cycle, except during atrial systole, when the shunt reverses. E. Short-axis view shows posterior location of the PA as compared to the AO, indicative of transposition of the great vessels. F. Color Doppler examination in another patient with double outlet RV and transposition of the great vessels (PA is located posterior to AO), demonstrating flow signals (blue) moving into both outflow vessels during systole. AS, Atrial septum; AV, aortic valve; LA, left atrium; MV, mitral valve; PV, pulmonary valve; RA, right atrium.

FIGURE 8.1.81. Tricuspid valve (TV) straddling the ventricular septum (VS). The TV is shown straddling the VS. Note the presence of a large ventricular septal defect. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

FIGURE 8.1.82. Single ventricle. A. A systolic frame shows the atrioventricular valves in the closed position. B,C. Both atrioventricular valves open into a large single ventricle. No ventricular septum is present. LA, left atrium; RA, right atrium.

FIGURE 8.1.83. Single ventricle: transposition of the great vessels. A–D. This patient has essentially a large single ventricle located posteriorly (PO VEN). Both atrioventricular valves communicate with this ventricle. A small, rudimentary and thick-walled second ventricle also is seen anteriorly (AN VEN). There is prolapse of the anterior mitral valve (MV) leaflet and septal and anterior tricuspid valve (TV) leaflets (arrowheads in B). E–J. The pulmonary artery (PA) is located posteriorly as compared to the aorta (AO), typical of transposition of the great vessels. In F, both great vessels are seen arising from the posterior ventricle. In G and H, the pulmonary valve (PV) is shown to be bicuspid (BPV), whereas the aortic valve (AV) is tricuspid (TAV). I,J. An associated secundum atrial septal defect (ASD) with flow signals moving into left atrium (LA) from right atrium (RA) (arrowhead in J). IAS, atrial septum; RAA, right atrial appendage; VSD, ventricular septal defect.

FIGURE 8.1.84. Dilatation of the sinuses of Valsalva. Three different patients with mildly dilated sinuses of Valsalva are shown. A. The sinuses (arrows) viewed in long axis. B.Mild dilatation of all three sinuses imaged in short axis in another patient. C. Localized dilatation (arrowhead) of the noncoronary sinus seen in the aortic short-axis view in a different patient. AO, aorta; AV, aortic valve; LA, left atrium; LAA, left atrial appendage; RV, right ventricle; RVO, right ventricular outflow tract.

FIGURE 8.1.85. Sinus of Valsalva aneurysm. A large aneurysm (arrowheads) involving the left coronary sinus. LA, left atrium; RA, right atrium; RVOT, right ventricular outflow tract.

FIGURE 8.1.86. Sinus of Valsalva aneurysm with rupture into the right ventricle (RV). A. The site of rupture (arrowhead) of the aneurysm (AN) into the RV just beneath the anterior tricuspid valve (ATV) and septal tricuspid valve (STV) leaflets of the tricuspid valve (TV). B–D. A huge aneurysm (arrowheads) involving the noncoronary sinus. E,F. The rupture is confirmed by color Doppler examination, which shows turbulent flow signals (arrowheads) moving from the aneurysm into the RV. AV, aortic valve; LA, left atrium;PA, pulmonary artery; RA, right atrium.

FIGURE 8.1.87. Sinus of Valsalva aneurysm with rupture into the right ventricle (RV). The arrowheads in A and the arrow in B demonstrate a tube-like protrusion of the right sinus of Valsalva aneurysm into the RV. C. Short-axis view shows aneurysmal dilatation of the right coronary sinus. D. Color Doppler examination shows mosaic colored turbulent flow signals moving from the aneurysm into the RV indicative of rupture. Note the presence of prominent flow acceleration on the aortic side at the site of rupture. AO, aorta;AV, aortic valve; LA, left atrium; LV, left ventricle; RA, right atrium; RVOT, right ventricular outflow tract.

FIGURE 8.1.88. Anomalous separate origin of left circumflex coronary artery (LCX) from a separate ostium in the left coronary sinus. A 62-year-old man evaluated before prosthetic replacement of a severely regurgitant aortic valve (AV). A. Aortic short-axis view. The LCX and left anterior descending (LAD) coronary arteries arise from adjacent but separate orifices. B. Color M-mode echocardiography shows flow signals in both vessels. C. Coronary angiogram shows separate orifices of the two coronary arteries. AO, aorta; RVOT, right ventricular outflow tract. (Reproduced with permission from 

Boogaerts J, Samdarshi TE, Nanda NC, et al. Anomalous separate origin of left circumflex coronary artery from a separate ostium in the left coronary sinus: identification by transesophageal color Doppler echocardiography. Echocardiography 1990;7:165–167.

)

FIGURE 8.1.89. Anomalous origin of the left circumflex coronary artery from the right sinus of Valsalva. A 33-year-old woman with congenital bicuspid aortic valve (AV) stenosis. Oblique aortic short-axis view demonstrates the right-sided origin of the anomalous vessel (CX), which then angles sharply posteriorly, and subsequently takes a leftward course between the aorta (AO) and the left atrium (LA). Note the presence of color-flow signals within the vessel lumen. B. This view shows adjacent but separate origins of the circumflex (CX) and right coronary (RCA) arteries from the right sinus of Valsalva. C. The coronary artery arising from the left sinus of Valsalva courses anteriorly—like a left anterior descending artery (LAD) and does not show any evidence of bifurcation. It is important to document this congenital anomaly, because it may contribute to myocardial infarction and sudden death. LMCA, left main coronary artery; RA, right atrium; SVC, superior vena cava. (Reproduced with permission from 

Samdarshi TE, Hill DL, Nanda NC. Transesophageal color Doppler diagnosis of anomalous origin of left circumflex coronary artery. Am Heart J 1991;122:571–573.

)

FIGURE 8.1.90. Anomalous origin of the right coronary artery (RCA) from the left anterior descending artery. Aortic short-axis view demonstrates the RCA originating from the left anterior descending coronary vessel (LAD). The circumflex coronary artery (LCX) is imaged immediately anterior to the RCA. LA, left atrium.

FIGURE 8.1.91. Coronary artery aneurysms. A. Left main coronary artery aneurysm. A large aneurysm (AN) involves the distal left main coronary artery in this patient. Its origin from the aorta (AO) and the proximal portion are uninvolved. B,C. Left anterior descending coronary artery (LAD) aneurysm. A large aneurysm containing a thrombus (TH) is seen in the distal LAD. The left main (LM) coronary artery and the proximal LAD are not dilated (insets in B). D–F. Right coronary artery aneurysm. The arrow in D points to the origin of the right coronary artery (RCA), which is aneurysmally dilated. E,F. Another patient with a proximal RCA aneurysm containing a thrombus (TH). LA, left atrium; LV, left ventricle; PA, pulmonary artery; RA, right atrium.

FIGURE 8.1.92. Fistula of right coronary artery to coronary sinus. This 46-year-old man presented with episodes of atrial flutter. A. The aortic short-axis view demonstrates a markedly enlarged right coronary artery (RCA; lumen width = 20 mm) with mosaic colored flow signals within it and in the aortic root (AO), indicative of turbulent blood flow. B.The RV inflow view demonstrates several large, bounded echo-free spaces containing mosaic colored signals indicating turbulent blood flow. These structures represent segments of the enlarged and tortuous right coronary artery viewed in the long-axis, short-axis, and oblique-axis planes. Mosaic flow signals from one of these structures are seen moving through an area of discontinuity into the grossly enlarged adjacent coronary sinus (CS), near its entrance into the right atrium (RA). C. After surgical obliteration of the fistulous connection, mosaic colored flow signals in the CS are replaced by blue signals indicative of laminar flow. J, jet from coronary sinus entering RA; LA, left atrium;LV, left ventricle; RVOT, right ventricular outflow tract; TV, tricuspid valve. (Reproduced with permission from 

Samdarshi TE, Mahan EF III, Nanda NC, et al. Transesophageal echocardiographic assessment of congenital coronary artery to coronary sinus fistulas in adults. Am J Cardiol 1991;68:263–266.

)

FIGURE 8.1.93. Left circumflex coronary artery to coronary sinus fistula. A 35-year-old woman with frequent ventricular ectopics. A. The aortic (AO) short-axis view demonstrates enlargement of the left main (LMC) and circumflex (LCX; lumen width = 16 mm) coronary arteries. B. Mosaic colored signals indicative of turbulent blood flow are seen moving from the left circumflex coronary artery into the coronary sinus (CS), near its entrance into the right atrium (RA). Large circular and linear bounded spaces were also noted in this patient adjacent to the coronary sinus and represented the enlarged and tortuous LCX segments viewed in long and short axis. C. Color Doppler–guided pulsed Doppler interrogation of the communication (C) site demonstrates aliased high-velocity signals throughout the cardiac cycle. LA, left atrium; RV, right ventricle; RVOT, right ventricular outflow tract; SV, Doppler sample volume. (Reproduced with permission from 

Samdarshi TE, Mahan EF III, Nanda NC, et al. Transesophageal echocardiographic assessment of congenital coronary artery to coronary sinus fistulas in adults. Am J Cardiol 1991;68:263–266.

)

FIGURE 8.1.94. Left circumflex coronary artery to coronary sinus fistula. This 53-year-old woman presented with nonanginal chest pain. A,B. The aortic (AO) short-axis view demonstrates marked enlargement of the left circumflex (LCX; lumen width = 13 mm) coronary artery (CA). There is also enlargement of the left main CA (LMCA; lumen width = 12 mm), with a prominent atherosclerotic plaque (P) noted within its lumen. C. The LCX CA is shown communicating with another structure, located in the usual position of the coronary sinus (CS) in this plane and adjacent to a left-sided pulmonary vein (LPV). D,E. After surgical obliteration of the fistula, mosaic colored turbulent flow signals within the coronary sinus are replaced by laminar flow signals (blue), except at its entrance into the right atrium (RA), where the fenestrated (F) thebesian valve results in persistence of turbulent blood flow. LA, left atrium; LAD, left anterior descending coronary artery; LV, left ventricle; RV, right ventricle; RVOT, right ventricular outflow tract. (Reproduced with permission from 

Samdarshi TE, Mahan EF III, Nanda NC, et al. Transesophageal echocardiographic assessment of congenital coronary artery to coronary sinus fistulas in adults. Am J Cardiol 1991;68:263–266.

)

FIGURE 8.1.95. Aortopulmonary communication. A 7-month-old infant with truncus arteriosus. A. A high basal view demonstrates a short main pulmonary artery (MPA) arising from the truncal vessel (TRV) and dividing into right (RPA) and left (LPA) branches, which appear to be normal in size. B. Color-flow signals are noted arising from the truncal vessel and entering the pulmonary artery. C. The five-chamber view demonstrates the truncal vessel overriding the interventricular septum. The arrow points to the ventricular septal defect. D. Color-flow signals are seen in the right ventricle (RV) and left ventricle (LV) and in the ventricular septal defect. E,F. Postoperative study demonstrates the valved bovine pericardial extracardiac conduit (C) connecting the RV to the pulmonary arteries. Note the absence of communication between the truncal vessel (now the neoaorta, AO) and the main pulmonary artery. With two-dimensional imaging only (F), a false impression of restriction at the distal conduit anastomosis is created as a result of the plane of scan through the oversewn stump of the main pulmonary artery. The arrow points to the detached and oversewn stump of the main pulmonary artery. HV, homograft valve in the conduit; SV, semilunar valve. LA, left atrium; RA, right atrium. (Reproduced with permission from 

Samdarshi TE, Morrow WR, Nanda NC, et al. Transesophageal echocardiography in aortopulmonary communications. Echocardiography 1991;8:383–395.

)

FIGURE 8.1.96. Aortopulmonary communication. A 7-month-old infant with an aortopulmonary window. A. A large (13-mm) defect is seen between the ascending aorta (AO) and the main pulmonary artery (MPA). B. Prominent color-flow signals are seen moving from the AO into the MPA and the proximal right (RPA) and left (LPA) branches through the defect. The pulmonary valve (PV) is clearly seen. C. The postoperative study demonstrates the patch (P) used to close the defect. RVOT, right ventricular outflow tract. (Reproduced with permission from 

Samdarshi TE, Morrow WR, Nanda NC, et al. Transesophageal echocardiography in aortopulmonary communications. Echocardiography 1991;8:383–395.

)

FIGURE 8.1.97. Aortopulmonary communication. A 4-year-old patient with transposition of the great arteries and pulmonary atresia. A. The high basal short-axis view demonstrates color-flow signals representing flow from the aorta (AO) into the right pulmonary artery (RPA) through the Waterston shunt (D). B. High-pulse-repetition-frequency Doppler interrogation of the shunt reveals a peak velocity of 3.77 m/sec across the shunt. (Reproduced with permission from 

Samdarshi TE, Morrow WR, Nanda NC, et al. Transesophageal echocardiography in aortopulmonary communications. Echocardiography 1991;8:383–395.

)

FIGURE 8.1.98. Aortopulmonary communication. A 1-year-old child with tetralogy of Fallot and complete atrioventricular septal defect. A. Left: Prominent color-flow signals are noted representing flow from the aorta (AO) into the main pulmonary artery (MPA) through a surgically created central shunt (S). Flow is encoded as mosaic because of turbulence and frequency aliasing. Right: High-pulse-repetition-frequency Doppler interrogation of the shunt demonstrates continuous flow from the aorta to pulmonary artery with a high velocity throughout the cardiac cycle. B. High-pulse-repetition-frequency Doppler interrogation of the left-sided Blalock-Taussig shunt (BT) also shows continuous flow. LPA, left pulmonary artery; RPA, right pulmonary artery. (Reproduced with permission from 

Samdarshi TE, Morrow WR, Nanda NC, et al. Transesophageal echocardiography in aortopulmonary communications. Echocardiography 1991;8:383–395.

)

FIGURE 8.1.99. Aortopulmonary communication. A 10-year-old patient with tetralogy of Fallot, pulmonary atresia, and nonconfluent pulmonary arteries. A. Color signals are seen depicting flow from the lateral wall of the aorta (AO) into the distal right pulmonary artery (RPA) through the Waterston shunt (S). B. Pulsed Doppler interrogation of the shunt reveals flow throughout the cardiac cycle. C. Postoperative study demonstrates the proximal anastomosis of the Gore-Tex conduit (C), which carries blood from the right ventricle (RV) to the right and left pulmonary arteries. D. Pulsed Doppler interrogation of the conduit demonstrates predominantly laminar systolic flow. CF, conduit flow; LV, left ventricle. (Reproduced with permission from 

Samdarshi TE, Morrow WR, Nanda NC, et al. Transesophageal echocardiography in aortopulmonary communications. Echocardiography 1991;8:383–395.

)

FIGURE 8.1.100. Aortopulmonary communication. A 10-year-old patient with double outlet right ventricle and pulmonary artery (PA) banding. A,B. Prominent flow signals are seen moving from the aorta (A) into the right pulmonary artery (RPA) and the main pulmonary artery (MPA) through the Waterston shunt (D, arrow). The shunt measures 4 mm in diameter. C. Left: Longitudinal plane imaging demonstrates discrete narrowing of the MPA produced by banding. Right: High-pulse-repetition-frequency Doppler interrogation reveals a high velocity (V) of 3.04 m/sec across the narrowed segment. D. Postoperative study demonstrates closure of the Waterston shunt and unrestricted PA bifurcation. E.Postoperative study. Left: The anastomosis (size by color Doppler = 1 cm) between the superior vena cava (SVC) and the RPA is seen. Right: Pulsed Doppler interrogation of the RPA reveals continuous flow throughout the cardiac cycle. LA, left atrium; LPA, left pulmonary artery. (Reproduced with permission from 

Samdarshi TE, Morrow WR, Nanda NC, et al. Transesophageal echocardiography in aortopulmonary communications. Echocardiography 1991;8:383–395.

)

FIGURE 8.1.101. Left ventricular (LV) diverticulum. A–C. A large diverticulum (arrowheads) involves the LV inferior wall. This was confirmed at surgery. MV, mitral valve; RV,right ventricle.

FIGURE 8.1.102. Multiple left ventricle (LV) papillary muscles. Three papillary muscles (arrowheads) visualized in the LV short-axis view. This is not a specific finding because normal papillary muscles may have more than one head, which can result in a similar picture.

 

 

 

 

 

Suggested Readings

Ahmed S, Nanda NC, Nekkanti R, et al. Contrast transesophageal echocardiographic detection of a pulmonary arteriovenous malformation draining into left lower pulmonary vein. Echo-cardiography 2003;20:391–394.

Alboliras ET, Gotteiner NL, Berdusis K, et al. Transesophageal echocardiographic imaging for congenital lesions of the left ventricular outflow and the aorta. Echocardiography 1996;13:439–446.

Andrade A, Vargas-Baron J, Rijlaarsdam M, et al. Utility of transesophageal echocardiography in the examination of adult patients with patent ductus arteriosus. Am Heart J 1995;130:543–546.

Ascione L, Caso P, De Leva F, et al. Transesophageal color flow echocardiographic evaluation of supra valve mitral ring in an adult period. Echocardiography 1994;11:231–235.

Baweja G, Nanda NC, Nekkanti R, et al. Three-dimensional transesophageal echocardiographic delineation of ventricular septal aneurysm producing right ventricular outflow obstruction in an adult. Echocardiography 2004;21:95–97.

Benheim A, Karr SS, Sell JE, et al. Routine use of transesophageal echocardiography and color flow imaging in the evaluation and treatment of children with congenital heart disease. Echocardiography 1993;10:583–593.

Benson MJ, Cahalan MK. Cost-benefit analysis of transesophageal echocardiography in cardiac surgery. Echocardiography 1995;12:171–183.

Bettex DA, Schmidlin D, Bernath MA, et al. Intraoperative transesophageal echocardiography in pediatric congenital cardiac surgery: a two-center observational study. Anesth Analg 2003;97:1275–1282.

Blackshear JL, Safford RE, Lane GE, et al. Unruptured noncoronary sinus of Valsalva aneurysm: preoperative characterization by transesophageal echocardiography. J Am Soc Echocardiogr 1991;4:485–490.

Boogaerts J, Samdarshi TE, Nanda NC, et al. Anomalous separate origin of left circumflex coronary artery from a separate ostium in the left coronary sinus: identification by transesophageal color Doppler echocardiography. Echocardiography 1990;7:165–167.

Chang RY, Kuo CH, Rim RS, et al. Transesophageal echocardiographic image of double-chambered right ventricle. J Am Soc Echocardiogr 1996;9:347–352.

Child JS. Echocardiographic assessment of adults with tetralogy of Fallot. Echocardiography 1993;10:629–640.

Cox D, Taylor J, Nanda NC. Refractory hypoxemia in right ventricular infarction from right-to-left shunting via a patent foramen ovale: efficacy of contrast transesophageal echocardiography. Am J Med 1991;91:653–655.

Cyran S, Kimball TR, Meyer RA, et al. Efficacy of intraoperative transesophageal echocardiography in children with congenital heart disease. Am J Cardiol 1989;63:594–598.

Dhar PK, Fyfe DA, Sharma S. Multiplane transesophageal echocardiographic evaluation of defects of the atrioventricular septum: the crux of the matter. Echocardiography 1996;13:663–676.

Dobbertin A, Warnes CA, Seward JB. Cor triatriatum dexter in an adult diagnosed by transesophageal echocardiography: a case report. J Am Soc Echocardiogr 1995;8:952–957.

Duch PM, Chandrasekaran K, Mulhern CB, et al. Transesophageal echocardiographic diagnosis of pulmonary arteriovenous malformation. Role of contrast and pulsed Doppler echocardiography. Chest 1994;105:1604–1605.

Essop MR, Skudicky D, Sareli P. Diagnostic value of transesophageal versus transthoracic echocardiography in discrete subaortic stenosis. Am J Cardiol 1992;70:962–963.

Finch A, Osman K, Kim KS, et al. Transesophageal echocardiographic findings of an infected quadricuspid aortic valve with an anomalous coronary artery. Echocardiography 1994;11:369–375.

Finch AD, Snell DR, Sanyal RS, et al. Transesophageal echocardiographic identification of a bicuspid pulmonary valve associated with congenitally corrected transposition of the great arteries. Echocardiography 1993;10:359–362.

Folk TG, Kon ND, Nomeir AM, et al. Coincidental finding of cor triatriatum by intraoperative transesophageal echocardiography in a patient with severe mitral regurgitation from myxomatous degeneration. Echocardiography 1994;11:579–583.

Fyfe DA. Multiplane transesophageal echocardiography for congenital heart disease: leveling the playing field. Echocardiography 1996;13:651–652.

Fyfe DA, Kline CH. Transesophageal echocardiography for congenital heart disease. Echocardiography 1991;8:573–584.

Fyfe DA, Ritter SB, Snider AR, et al. Guidelines for transesophageal echocardiography in children. J Am Soc Echocardiogr 1992;5:640–644.

Georgeson S, Neibart RM. Quadricuspid aortic valve diagnosed by transesophageal echocardiography. Am Heart J 1996;132:1292–1293.

Goldberg N, Schifter D, Aron M, et al. Double orifice mitral and tricuspid valves. Echocardiography 1996;13:85–90.

Gonzalez-Juanatey C, Testa A, Vidan J, et al. Persistent left superior vena cava draining into the coronary sinus: report of 10 cases and literature review. Clin Cardiol 2004;27:515–518.

Hashimoto H. Double-orifice mitral valve with three papillary muscles. Chest 1993;104:1616–1617.

Hijazi Z, Wang Z, Cao Q, et al. Transcatheter closure of atrial septal defects and patent foramen ovale under intracardiac echocardiographic guidance: feasibility and comparison with transesophageal echocardiography. Catheter Cardiovasc Interv 2001;52:194–199.

Hoppe UC, Dederichs B, Deutsch HJ, et al. Congenital heart disease in adults and adolescents: comparative value of transthoracic and transesophageal echocardiography and MR imaging. Radiology 1996;199:669–677.

Justo RN, Nykanen DG, Boutin C, et al. Clinical impact of transcatheter closure of secundum atrial septal defects with the double umbrella device. Am J Cardiol 1996;77:889–892.

Kavanaugh-McHugh A, Tobias JD, Doyle T, et al. Transesophageal echocardiography in pediatric congenital heart disease. Cardiol Rev 2000;8:288–306.

Kichura GM, Castello R. Abnormalities of the interatrial septum as a potential cardiac source of embolism: patent foramen ovale and atrial septal aneurysm. Echocardiography 1994;10:441–449.

Kim KS, Maxted W, Nanda NC, et al. Comparison of multiplane and biplane transesophageal echocardiography in the assessment of aortic stenosis. Am J Cardiol 1997;79:436–441.

Krauss D, Weinert L, Lang RM. The role of multiplane transesophageal echocardiography in diagnosis PDA in an adult. Echocardiography 1996;13:95–97.

Kronzon I, Tunick PA, Freedberg RS, et al. Transesophageal echocardiography is superior to transthoracic echocardiography in the diagnosis of sinus venosus atrial septal defect. J Am Coll Cardiol 1991;17:537–542.

Krumsdorf U, Ostermayer S, Billinger K, et al. Incidence and clinical course of thrombus formation on atrial septal defect and patient foramen ovale closure devices in 1,000 consecutive patients. J Am Coll Cardiol 2004;43:302–309.

Lam J, Neirotti RA, Nijveld A, et al. Transesophageal echocardiography in pediatric patients: preliminary results. J Am Soc Echocardiogr 1991;4:43–50.

Lin FC, Chang HJ, Chern MS, et al. Multiplane transesophageal echocardiography in the diagnosis of congenital coronary artery fistula. Am Heart J 1995;130:1236–1244.

Lloyd TR, Vermilion RP, Zamora R, et al. Influence of echocardiographic guidance on positioning of the buttoned occluder for transcatheter closure of atrial septal defects. Echocardiography 1996;13:117–121.

Masani ND. Transoesophageal echocardiography in adult congenital heart disease. Heart 2001;86:II30–II40.

Maxted W, Finch A, Nanda NC, et al. Multiplane transesophgeal echocardiographic detection of sinus venosus atrial septal defect. Echocardiography 1995;12:139–143.

Maxted W, Nanda NC, Kim KS, et al. Transesophageal echocardiographic identification and validation of individual tricuspid valve leaflets. Echocardiography 1994;11:585–596.

Mehta RH, Helmcke F, Nanda NC, et al. Transesophageal Doppler color flow mapping assessment of atrial septal defect. J Am Coll Cardiol 1990;16:1010–1016.

Mehta RH, Jain SP, Nanda NC, et al. Isolated partial anomalous pulmonary venous connection: echocardiographic diagnosis and a new color Doppler method to assess shunt volume. Am Heart J 1991;122:870–873.

Miller DS, Schwartz SL, Geggel RL, et al. Detection of partial anomalous right pulmonary venous return with an intact atrial septum by transesophageal echocardiography. J Am Soc Echocardiogr 1995;8:924–927.

Miller-Hance WC, Silverman NH. Transesophageal echocardiography (TEE) in congenital heart disease with focus on the adult. Cardiol Clin 2000;18:861–892.

Mirza S, Nanda NC, Baweja G, et al. Multiple fistulae connecting the right coronary artery to the coronary sinus. Echocardiography 2004;21:199–202.

Monducci I, Tomasi C, Bacchi M, et al. Usefulness of biplanar transesophageal echocardiography in arrhythmogenic right ventricular dysplasia: clinical experience with seven cases. Echocardiography 1996;13:1–8.

Morimoto K, Matsuzaki M, Tohma Y, et al. Diagnosis and quantitative evaluation of secundum-type atrial septal defect by transesophageal Doppler echocardiography. Am J Cardiol 1990;66:85–91.

Mugge A, Daniel WG, Klopper JW, et al. Visualization of patent foramen ovale by transesophageal color-coded Doppler echocardiography. Am J Cardiol 1988;62:837–838.

Muhiudeen I, Silverman N. Intraoperative transesophageal echocardiography using high resolution imaging in infants and children with congenital heart disease. Echocardiography 1993;10:599–608.

Nanda NC, Pinheiro L, Sanyal R, et al. Transesophageal echocardiographic examination of left-sided superior vena cava and azygos and hemiazygos veins. Echocardiography 1991;8:731–740.

Nanda NC, Samal AK, Bakir S, et al. Transesophageal echocardiographic diagnosis of right sided aortic arch. Echocardiography 1998;15:409–417.

Obeid AI, Carlson RJ. Evaluation of pulmonary vein stenosis by transesophageal echocardiography. J Am Soc Echocardiogr 1995;8:888–896.

Ootaki Y, Yamaguchi M, Yoshimura N, et al. Unroofed coronary sinus syndrome: diagnosis, classification, and surgical treatment. J Thorac Cardiovasc Surg 2003;126:1655–1656.

Osman K, Nanda NC, Kim KS, et al. Transesophageal echocardiographic features of unicuspid aortic valve. Echocardiography 1994;11:469–473.

Pascoe RD, Oh JK, Warnes CA, et al. Diagnosis of sinus venosus atrial septal defect with transesophageal echocardiography. Circulation 1996;94:1049–1055.

Patel JN, Osman K, Nanda NC, et al. Quadricuspid aortic valve diagnosed by multiplane transesophageal echocardiography. Echocardiography 1994;11:201–205.

Pearson AC, Nagelhout D, Camp A, et al. Atrial septal aneurysm and stroke: a transesophageal echocardiographic study. J Am Coll Cardiol 1991;18:1223–1229.

Pedra CA, Pedra SR, Esteves CA, et al. Percutaneous closure of perimembranous ventricular septal defects with the Amplatzer device: technical and morphological considerations. Catheter Cardiovasc Interv 2004;61:403–410.

Podolsky LA, Jacobs LE, Schwartz M, et al. Transesophageal echocardiography in the diagnosis of the persistent left superior vena cava. J Am Soc Echocardiogr 1992;5:159–162.

Rainer RS, Wanat FE, Nanda NC, et al. Multiple secundum type atrial septal defects: identification by transthoracic color Doppler echocardiography. Echocardiography 1990;7:567–569.

Ravi BS, Wanat FE, Mariano D, et al. Transesophageal echocardiographic demonstration of atrial septal aneurysm prolapsing into right ventricular inflow. Echocardiography 2004;21:291–293.

Ritter SB. Transesophageal echocardiography in children: new peephole to the heart (editorial). J Am Coll Cardiol 1990;16:447–450.

Ritter SB. Transesophageal real-time echocardiography in infants and children with congenital heart disease. J Am Coll Cardiol 1991;18:569–580.

Ryan K, Sanyal RS, Pinheiro L, et al. Assessment of aortic coarctation and collateral circulation by biplane transesophageal echocardiography. Echocardiography 1992;9:277–285.

Samdarshi TE, Hill DL, Nanda NC. Transesophageal color Doppler diagnosis of anomalous origin of left circumflex coronary artery. Am Heart J 1991;122:571–573.

Samdarshi TE, Mahan EF III, Nanda NC, et al. Transesophageal echocardiographic assessment of congenital coronary artery to coronary sinus fistulas in adults. Am J Cardiol 1991;68:263–266.

Samdarshi TE, Morrow R, Helmcke FR, et al. Assessment of pulmonary vein stenosis by transesophageal echocardiography. Am Heart J 1991;122:1495–1498.

Samdarshi TE, Morrow WR, Nanda NC, et al. Transesophageal echocardiography in aortopulmonary communications. Echocardiography 1991;8:383–395.

Santini F, Bonato R, Pittarello D, et al. Intraoperative transesophageal echocardiography during surgery for congenital heart disease. Cardiovasc Imaging 1992;4:127–132.

Sanyal RS, Nanda NC, Snell D, et al. Transesophageal echocardiographic findings of complete unilateral anomalous pulmonary venous connection of right lung to right atrium. Echocardiography 1994;11:93–100.

Sarodia BD, Stoller JK. Persistent left superior vena cava: case report and literature review. Respir Care 2000;45:411–416.

Schneider B, Hanrath P, Vogel P, et al. Improved morphology characterization of atrial septal aneurysm by transesophageal echocardiography: relation to cerebrovascular events. J Am Coll Cardiol 1990;16:1000–1009.

Schurger D, Bartel T, Muller S, et al. Multiplane transesophageal echocardiography is the only definitive ultrasound approach in adult supra valvular aortic stenosis. Int J Cardiol 1996;53:305–309.

Scott PJ, Blackburn ME, Wharton GA, et al. Transesophageal echocardiography in neonates, infants and children: applicability and diagnostic value in everyday practice of a cardiothoracic unit. Br Heart J 1992;68:488–492.

Seward JB. Ebstein's anomaly: ultrasound imaging and hemodynamic evaluation. Echocardiography 1993;10:641–664.

Seward JB, Tajik AJ. Transesophageal echocardiography in congenital heart disease. Am J Card Imaging 1990;4:215–222.

Sharma S, Stamper T, Dhar P, et al. The usefulness of transesophageal echocardiography in the surgical management of older children with subaortic stenosis. Echocardiography 1996;13:653–661.

Shirani J, Woo D, Gotsi W, et al. Mitral stenosis, sinus venosus atrial septal defect, and partial anomalous pulmonary venous return: diagnosis by multiplane transesophageal echocardiography. Echocardiography 1996;13:635–637.

Sloth E, Hasenkam JM, Sorensen KE, et al. Pediatric multiplane transesophageal echocardiography in congenital heart disease: new possibilities with a miniaturized probe. J Am Soc Echocardiogr 1996;9:626–628.

Staffen RN, Davidson WR. Echocardiographic assessment of atrial septal defects. Echocardiography 1993;10:545–552.

Stern H, Erbel R, Schreiner G, et al. Coarctation of the aorta: quantitative analysis by transesophageal echocardiography. Echocardiography 1987;4:387–395.

Stumper OFW, Elzenga NJ, Hess J, et al. Transesophageal echocardiography in children with congenital heart disease: an initial experience. J Am Coll Cardiol 1990;16:433–441.

Stumper OFW, Fijlaarsdam M, Vargas-Barron J, et al. The assessment of juxtaposed atrial appendages by transesophageal echocardiography. Int J Cardiol 1990;29:365–371.

Stumper O, Sutherland GR, Geuskens R, et al. Transesophageal echocardiography in evaluation and management after a Fontan procedure. J Am Coll Cardiol 1991;17:1152–1160.

Subahi SA, Al-Damegh S, Akhtar MJ, et al. Acquired intercostal arteriovenous fistulas: transesophageal Doppler echocardiography diagnosis. Echocardiography 1996;13:639–641.

Wang KY, Hsieh K, Yang M, et al. The use of transesophageal echocardiography to evaluate the effectiveness of patent ductus arteriosus ligation. Echocardiography 1994;10:53–57.

Weintraub R, Shiota T, Elkadi T, et al. Transesophageal echocardiography in infants and children with congenital heart disease. Circulation 1992;86:711–722.

Willens HJ, Levy R, Perez A. Diagnosis of accessory mitral valve tissue by transesophageal echocardiography. Echocardiography 1994;11:39–45.

Williams RG. Echocardiography in the management of single ventricle: fetal through adult life. Echocardiography 1993;10:331–342.

Xu J, Shiota T, Ge S, et al. Intraoperative transesophageal echocardiography using high-resolution biplane 7.5 MHz probes with continuous-wave Doppler capability in infants and children with tetralogy of Fallot. Am J Cardiol 1996;77:539–542.

Yesilbursa D, Miller A, Nanda NC, et al. Echocardiographic diagnosis of a stenotic double orifice parachute mitral valve with a single papillary muscle. Echocardiography 2000;17:349–352.

Zboyovsky KL, Nanda NC, Jain H. Transesophageal echocardiographic identification of atrial septal aneurysm. Echocardiography 1991;8:435–437.