Cardiovascular Ultrasound

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1 Cardiovascular Ultrasound BioMed Central Research Ebstein's Anomaly: Anatomo-echocardiographic correlation Luis Muñoz-Castellanos 1, Nilda Espinola-Zavaleta* 2, Magdalena Kuri- Nivón 3 and Candace Keirns 2 Open Access Address: 1 Embryology Department, Instituto Nacional de Cardiología "Ignacio Chávez", Juan Badiano N 1, Colonia Sección XVI, Tlalpan, Mexico City, Mexico, 2 Echocardiography in Out Patient Clinic, Intituto Nacional de Cardiología "Ignacio Chávez", Juan Badiano N 1, Colonia Sección XVI, Tlalpan, Mexico City, Mexico and 3 Morphology Department, Escuela Superior de Medicina-IPN, Diaz-Mirón y Plan de San Luis, Colonia Casco de Santo Tomás, Tacuba, Mexico City, Mexico Luis Muñoz-Castellanos - lmunoz@hotmail.com; Nilda Espinola-Zavaleta* - niesza2001@hotmail.com; Magdalena Kuri- Nivón - magerep@yahoo.com; Candace Keirns - mieshe@comcast.net * Corresponding author Published: 23 November 2007 Cardiovascular Ultrasound 2007, :43 doi: / Received: 31 October 2007 Accepted: 23 November 2007 This article is available from: 2007 Muñoz-Castellanos et al; licensee BioMed Central Ltd. This is an Open Access article distributed under terms of Creative Commons Attribution License ( which permits unrestricted use, distribution, and reproduction in any medium, provided original work is properly cited. Abstract Objective: The aim of this investigation is to demonstrate that in Ebstein's Anomaly (EA) (RV) is affected in its three portions and to establish an anatomoechocardiographic correlation between anatomic features and equivalent echocardiographic images. Methods: Thirty hearts with EA were studied. The alterations of each portions of RV were described. Fifty adult patients with this anomaly were studied by echocardiography. Results: Anatomy: All hearts had atrial situs solitus, 27 had concordant atrioventricular connection and 3 discordant, of se 2 had transposition of great arteries (TGA) and one double outlet (DORV). The degree of valve (TV) displacement showed a spectrum from I to III. The inlet of RV was markedly thin in 27. The trabecular portion had multiples muscular bands in all. The outlet portion was dilated in 20 and stenotic in. In 2 atrial septal defects were found. Echocardiography: All patients had atrial situs solitus, 42 with concordant atrioventricular connection and 8 with discordant, of se last patients had TGA and 3 DORV. The degree of TV displacement varied from I to III. The inlet of RV was markedly thin in 42. The trabecular portion had muscular bands in 4. The outlet portion was dilated in 31 and stenotic in 11. In 30 atrial septal defects were found. Conclusion: The EA affects whole RV and anatomoechocardiographic correlation provides an appropriate understanding of echocardiographic images in terms of a precise diagnosis, rapeutic decisions and prognosis. Background The characteristics of Ebstein's anomaly of valve include tering of leaflets to ventricular wall and redundancy and dysplasia of leaflets. The variable degrees of redundancy and dysplasia determine displacement of functional opening toward trabecular portion and outflow tract of [1-6]. These alterations of valve have traditionally constituted most conspicuous aspects of this congenital cardiac malformation [1,4,]. The histopathology of ventricular wall reveals a decrease or total absence of myocardial fibers in inlet portion [- 7]. The apical trabecular portion of ventricular free wall a characteristic pattern of anomalous muscular bands that connect ventricular septum to free wall [6]. Moreover, in infundibular portion of ventricular outflow tract, myocardial fibers are diminished in number, making it very thin. Subpulmonary and pulmo- Page 1 of 10

2 Cardiovascular Ultrasound 2007, :43 nary obstructions can also be present [6,7]. In addition, 2% of patients with Ebstein's anomaly also present with a Wolf-Parkinson-White pre-excitation syndrome [8]. The hemodynamic effects are related to regurgitation, existence of an atrial septal defect and degree of dysfunction of atrialized portion of. Some cases have stenosis owing to reduction of functional valve opening secondary to fusion of leaflets. In such patients an atrial septal defect is obligatory for survival [8]. Echocardiography is non-invasive method of choice in diagnosis of this condition, inasmuch as it makes it possible to evaluate degree of leaflet tering, characteristics of leaflets and subvalvular apparatus, degree of regurgitation and/or stenosis, morphological and functional alterations in atrialized and associated anomalies, all of which aid in planning type of surgery to perform [9,10]. An understanding of morphological details of Ebstein's anomaly is basis of correct interpretation of diagnostic images and proper clinical judgment. The aims of this study are to a) demonstrate that all three portions of - inflow tract, trabecular portion and outflow tract-are affected. B) Correlate echocardiographic images of patients with Ebstein's anomaly of differing degrees of severity with anatomic specimen hearts with comparable malformations in order to validate high degree of echocardiographic diagnostic precision and enhance understanding of ultrasound images. Methods Thirty specimen hearts with Ebstein's anomaly from Embryology Department of Instituto Nacional de Cardiología "Ignacio Chávez" (INC) were examined. Diagnostic descriptions of congenital malformations were obtained by a sequential segmental method [11]. Atrial situs was established first, n types and modes of atrioventricular and ventriculoarterial connection and associated malformations. The three portions of, inflow tract, trabecular portion and outflow tract, were described with particular attention to morphology of valve. A modified version of Becker's dysplasia classification [1] was used. The degrees of leaflet tering to ventricular wall were calculated according to ir extension: grade I up to 2% of distance from atrioventricular junction to apex, grade II from 2 0%, grade III more than 0% of distance. The location of anatomic valve ring, functional opening and extension of atrialized portion of ventricular inlet were determined as well as characteristics of apical trabecular portion and morphology of ventricular infundibulum. The morphology of chambers of left heart and mitral and aortic valves were also examined. From June of 2004 to December of adult patients with Ebstein's anomaly were seen in out patient clinic of INC. Eight of se (16%) had left-sided Ebstein's anomaly. Twenty-nine were women and 21 men with a mean age of 31.7 ± 7.9 years. Complete clinical histories were taken and transthoracic and/or transesophageal echocardiograms performed on all patients. The echocardiogram was performed using a Philips Sonos 00 with an S3 electronic probe and a multiplane transesophageal probe. Images included parasternal long and short axis sections at level of great vessels to evaluate ventricular infundibulum and pulmonary valve. The 4-chamber image was used to examine atrialized portion of and functional according to Shiina's criteria [10], degree of tering of septal leaflet according to Becker [1], and characteristics of anterior mitral leaflet. The atrialized and functional portions of were examined in parasternal short axis images at level of two s and at level of great vessels, in a chamber long axis and in an apical 4 chamber. The severity of regurgitation was assessed according to previously established criteria using color-coded Doppler in apical four chamber s [10]. Associated anomalies were studied in apical fourchamber (mitral valve, left, atrial and ventricular septa), in parasternal short axis s (atrial septum, ventricular septum, and pulmonary valve) and in subcostal 4-chamber (atrial septum). In patients with left-sided Ebstein's anomaly segmental analysis described above was used. Atrial situs was evaluated in a subcostal, atrioventricular connection in apical four chamber, ventriculoarterial connection in a parasternal short axis plane at level of great vessels and an apical chamber with anterior angulation and in a subcostal. Characteristics of leaflets and degree of regurgitation were studied in a two-dimensional apical four chamber and with color Doppler, respectively. Associated anomalies were sought using apical and subcostal s. In patients with poor echocardiographic visibility or diagnostic doubts related to characteristics and types of septal defects transesophageal echocardiography was performed using planes at 0, 90 and 130. Page 2 of 10

3 Cardiovascular Ultrasound 2007, :43 Anatomo-echocardiographic correlation was established by selecting echocardiographic images that showed anatomic features comparable to equivalent hearts. In one case echocardiographic image and anatomic specimen belonged to same patient. Since Ebstein's anomaly affects all three portions of alterations of inflow tract with were shown first, with emphasis on leaflet tering to ventricular wall, dysplasia of atrioventricular valve and structure of posteroinferior portion of ventricular free wall. Alterations in ventricular apical trabecular portion and those of infundibulum were described as well as associated anomalies. Results Morphological aspects All thirty specimen hearts had atrial situs solitus. Twentyseven had concordant atrioventricular connections and three discordant atrioventricular connections. Two of latter had corrected transposition of great vessels and one ventricular double outlet. In all three atrioventricular connection was patent. The anatomic ring was normally situated at ventricular junction and functional opening was low. In fifteen it was located behind septomarginal trabeculum and was dilated. In remaining fifteen hearts valve had stenosis. In ten of se it was located at subinfundibular level below crista supraventricularis and in five it was located within ventricular infundibulum. Leaflet tering was Grade I in hearts, Grade II in 10 and Grade III in 1. Tering involved all three leaflets in one specimen, while in twenty-nine it involved septal and posterior leaflets. The anterior leaflet was redundant in twenty; in ten of se it took form of a fibromuscular curtain that joined ventricular septum to ventricular free wall, separating inflow and trabecular outflow portions. The leaflets presented all degrees of dysplasia: I (fibromyxomatous thickening), II (shortening and thickening of chordae tendineae) and III (leaflet tering, rudimentary or absent papillary muscles, fibromuscular histology of anterior leaflet). In twenty-seven hearts with concordant atrioventricular connection, posterior portion of inflow tract had formed a very thin fibrous aneurysmal sac between 0. and 2 mm thick. The three hearts with atrioventricular discordance did not present this abnormality. The trabecular portion was covered by tered leaflets that formed a cul-de-sac above apical portion of, which was occupied by multiple bands of interconnected myocardium. The outflow tract (infundibulum) was dilated in twenty specimens with thinning of anterior wall. Atrial septal defects were present in twenty hearts, in four interatrial septum was integral and in six foramen ovale was patent. Five specimens had ventricular septal defects, three perimembranous and two trabecular. In twentyfive hearts with integral ventricular septum septum bulged toward left. The atrium was dilated. The mitral septal leaflet was cleft in one heart. Anor presented an anomalous band of myocardium that joined septum with left ventricular free wall (Table 1). Echocardiographic findings The hearts of fifty patients studied by echocardiography were in normal position (atrial situs solitus). Fortytwo had concordant atrioventricular connections and eight discordant atrioventricular connections. Five of latter also had discordant ventriculoarterial connections and three ventricular double outlet. The echocardiographic characteristics of all of patients, based on division of into three portions are shown in Table 2. Anatomo-echocardiograhic correlation Grade I leaflet tering is shown in s 1, 2 and 3. The tered portion of septal leaflet (asterisk) can be seen in anatomic specimen. Dysplasia is apparent in thickening of free part of leaflet (arrow), short, thick or absent chordae tendineae and in atrialized and functional portions of (Figs. 1 and 2). A corresponding echocardiographic image tering of septal leaflet (asterisk). 3 anatomy of atrioventricular discordance with tering of septal leaflet (arrow), situated on left, which is also evident in corresponding echocardiographic image (arrow). Grade II tering is shown in 4 (asterisk). Dysplasia of free portion of septal leaflet takes form of nodular thickening. Grade II tering involved septal and posterior leaflets. In septal leaflet can be seen to be partially tered to interventricular septum with its free portion immediately behind trabecula septomarginalis. This finding also appears in equivalent echocardiographic image. s 6, 7 and 8 show an extreme degree of atrialization of with consequent reduction of functional portion of. Fusion of septal and posterior leaflets with formation of a cul-de-sac is also evident. Page 3 of 10

4 Cardiovascular Ultrasound 2007, :43 Table 1: Morphological Findings in Ebstein's Anomaly Right ventricular region Morphological finding N = 30 Inflow Tract Degree of tering I II III 10 1 Tricuspid dysplasia 30 Dilatation of anatomic atrioventricular ring Functional opening Mild Moderate Severe Low Dilated stenotic Ventricular wall Aneurysmal sac Trabecular Portion Uncovered 100% Covered by leaflet tering 2% 10 0% 100% 10 Outflow tract (infundibulum) Anomalous apical muscular bands 30 Stenotic Dilated 20 Pulmonary Valve Stenosis Atresia Associated Abnormalities ASD (Ostium secundum) 20 Patent foramen ovale ASD + VSD Cleft mitral valve 1 Moderator band in left 1 Transposition of great vessels* 2 Right ventricular double outlet* 1 *Atrioventricular discordance ASD: Atrial septal defect; VSD: Ventricular septal defect * 20 The anterior leaflet was always redundant, forming a curtain that separated atrialized and functional portions of (Figs 9 and 10). 11 grade I tering of septal leaflet and severe dysplasia characterized by large nodules on three leaflets with short, thick, and/or absent chordae tendineae. The anatomic and echocardiographic images are of same patient. The nodular thickening of valve in echocardiogram and huge atrial dilatation in anatomic specimen are noteworthy. 12 reveals paper thin aneurysmal sac in posterior portion of both in anatomic specimen and echocardiographic image (asterisk). Anomalies of apical portion of can be seen in s 13 and 14. Multiple bands of myocardium join ventricular free wall to septum. These structures are also visible in echocardiographic equivalent images. s 10 and 1 show thinning and dilatation of walls of ventricular infundibulum in anatomic specimen and echocardiogram, respectively. Associated defects included foramen secundum type atrial septal defect (Figs 1, 2, 7, 8, 11), perimembranous ventricular septal defect (Fig 6), moderator band in left (Fig 16) and prolapse of anterior mitral leaflet (Figs 2,4,16). Discussion Ebstein's anomaly is seen as part of a generalized disturbance in development of and, in some cases, of left [12]. In normal development primordial valve derives from ventricular myocardial wall. It peels off as a sheet with 3 flaps that later differentiate into 3 leaflets [13]. It has been posited that this process does not occur in Ebstein's anomaly, and leaflets, particularly septal and posterior leaflets, remain tered to ventricular wall [14]. Page 4 of 10

5 Cardiovascular Ultrasound 2007, :43 Table 2: Echocardiographic findings in Ebstein's Anomaly Right ventricular region Echocardiographic finding N = 0 Inflow Tract Tering I II III Tricuspid Valve dysplasia 0 Dilatation of anatomic atrioventricular ring Functional opening Tricuspid regurgitation Ventricular wall Mild Moderate Severe Low Dilated Stenotic Mild Moderate Severe Aneurysmal sac Trabecular portion Uncovered 100% 10 Covered by leaflet tering 2% 0% 100% Multiple muscular bands in RV apex 4 Outflow tract (infundibulum) Stenotic Dilated Pulmonary Valve Stenosis Atresia Associated abnormalities ASD (Ostium secundum) 24 Patent foramen ovale 11 ASD + VSD 6 VSD 2 ISA 1 Mitral valve prolapse 4 Unicuspid mitral valve 1 Supravalvular mitral membrane 1 Noncompacted left 1 Left with moderator band 1 Right Cor triatrium 1 Transposition of great vessels* Left ventricular double outlet * 3 * Discordant ventriculoarterial connection. RV: Right ventricular; ASD: Atrial septal defect; VSD: Ventricular septal defect; ISA: Interatrial septal aneurysm * During 1960's and 1970's this congenital heart disease was believed to be extremely rare [1]. Diagnosis was established on basis of clinical, electrocardiographic and cardioangiographic characteristics, and it was believed to represent 0.3% of all congenital heart conditions [16]. Cross-sectional echocardiography facilitated diagnosis, especially during prenatal period [17]. Today this cardiac malformation is recognized as more common than had been realized and number of cases diagnosed has increased significantly [18]. Although many studies have contributed to our understanding of Ebstein's anomaly, it has remained incomplete. Ebstein's anomaly has a highly variable natural history that depends on degree of abnormality of valve, with a spectrum from mild to severe forms [1,4,,18]. Cases with severe deformity can lead to congestive heart failure during neonatal period or even intrauterine death. At or extreme patients with mild degrees of leaflet tering can remain asymptomatic until adulthood [19]. Our study provides significant information based on an analysis of three portions of and anatomo-echocardiographic correlation in Ebstein's anomaly. This comparison of anatomic specimens with corresponding echocardiographic images of different degrees of septal valve displacement has potential of Page of 10

6 Cardiovascular Ultrasound 2007, :43 (asterisk)1 anomaly with of mild (Grade chambers I) tering of aofheart septal with Ebstein's leaflet of chambers of a heart with Ebstein's anomaly with mild (Grade I) tering of septal leaflet (asterisk). Thickening of free portion of leaflet (arrow) is evident. The dotted line represents atrioventricular junction where fibrous ring is located. The atrialized portion of is small. Note patent foramen ovale type atrial septal defect. The 4 chamber echocardiographic image same type of findings seen in anatomic specimen. The dotted line indicates plane of atrioventricular junction. The majority of is functional. Abbreviations: RA: Right atrium; ARV: Atrialized ; FRV: Functional ; AL: Anterior leaflet; PL: Posterior leaflet; SL: Septal leaflet; ASD: Atrial septal defect; LA: Left atrium; LV: Left. enhancing echocardiographer's understanding of anomaly. This should contribute to more precise diagnoses, leading to early and appropriate treatment of patients with a wide spectrum of manifestations of condition. While Ebstein's anomaly involves all portions of, alterations of inflow tract best typify 2 tering of of chambers septal leaflet (asterisk) mild (Grade I) of chambers mild (Grade I) tering of septal leaflet (asterisk). Note nodular dysplasia of free portion of leaflet. Koch's triangle over atrioventriculaar septum and atrial septal defect are visible. The 4 chamber echocardiographic image grade I tering of septal leaflet. The atrialized portion of is smaller than functional portion. The double arrow points to atrial septal defect. K: Koch's triangle. Or abbreviations as before. onposition normal uated 3 left of aand heart Ebstein's with atrioventricular anomaly of a discordance valve in sit of a heart with atrioventricular discordance in normal position and Ebstein's anomaly of a valve situated on left. There is grade I tering of septal leaflet (arrow) and dysplasia of free portion of leaflet. The 4 chamber echocardiographic image features similar to those of specimen heart. Abbreviations as before. malformation and have served to define it. Its three fundamental pathological features are leaflet tering to ventricular wall, dysplasia and redundancy of leaflet tissue and thinning of ventricular myocardial wall or its replacement with connective tissue. All of se characteristics were observed in our series. Leaflet tering and dysplasia, toger with dilatation of valve ring, constitute anatomic cause of regurgitation observed with this condition. In addition, presence of shortened chordae tendineae leads to diagnosis in those cases in which tering is mild and dysplasia predominant, as occurred in only case in our series in which anatomic specimen and echocardiogram were of same patient (figure 11). 4 septal of leaflet (asterisk) grade II tering of of grade II tering of septal leaflet (asterisk). The 4 chamber echocardiographic image discontinuous leaflet tering similar to anatomic specimen. Abbreviations as before. Page 6 of 10

7 Cardiovascular Ultrasound 2007, :43 tering of of chambers septal leaflet of a heart grade III of chambers of a heart grade III tering of septal leaflet. The arrows point to tering of posterior leaflet. The flap of free portion of septal leaflet can be seen (asterisk) as well as redundant anterior leaflet with 3 accessory openings. The 4 chamber echocardiographic image same features and greater size of atrialized portion of with significant reduction of functional portion. Abbreviations as before. Of three leaflets, anterior has been identified as least affected in terms of tering. However, it is excessively large with an anomalous fibrous border. On occasion it has a fibromuscular structure or is entirely made up of myocardial tissue. It inserts normally into valve ring at atrioventricular junction. However, its distal insertion can be focal into medial and anterior papillary muscles, and in florid cases insertion is anomalous in a linear and continuous way on border between of lar portion wall (short 7 of ofarrows) septal and andchambers posterior severe (longreduction arrow) leaflets extreme to of functional ventricutering of chambers extreme tering of septal and posterior leaflets to ventricular wall (short arrows) and severe reduction of functional portion of (long arrow). Note atrial septal defect. The echocardiographic image hammock-like aspect of tered posterior and septal leaflets that separate atrialized and functional portions of. The atrialized portion of predominates. Ao: Aorta. Or abbreviations as before. inflow and outflow tracts. The communication between inflow tract and trabecular portion occurs through anteroseptal and anterolateral commissures and/or fenestrations in redundant leaflet. The structure of is always abnormal in Ebstein's anomaly. The posterior wall of inflow tract has diminished myocardial tissue of variable degrees of severity including total replacement of myocardium with III) leaflet6 tering of cardiac to ventricular chamberswall show (arrows) extreme (grade of cardiac chambers show extreme (grade III) leaflet tering to ventricular wall (arrows). There is severe reduction of functional portion of, apical portion of which is tunnel-shaped chamber. The echocardiographic image great predominance of atrialized portion of and a perimembranous ventricular septal defect. VSD: Ventricular septal defect. Or abbreviations as before. 8 of chambers of chambers. There is anatomic continuity between septal and posterior leaflets tered to ventricular wall, forming a cul-de-sac (arrows) with reduction of apical portion of, which is occupied by multiple trabeculae. The echocardiogram similar features to those seen in anatomic specimen. TFO: Tricuspid functional opening. Or abbreviations as before. Page 7 of 10

8 Cardiovascular Ultrasound 2007, :43 sue from a 9 redundant opening septal of anterior leaflet functional that portion circumscribes leaflet of and afunctional flap of tis of functional portion of a redundant anterior leaflet and a flap of tissue from septal leaflet that circumscribes functional opening. The echocardiographic image in an intermediate plane (60 ) redundancy of anterior leaflet. I: Infundibulum. Or abbreviations as before. fibrous tissue. In our series this feature only occurred in hearts with atrioventricular concordance. When atrioventricular connection was discordant ventricular wall was normal [20]. The trabecular portion of presented multiple interconnected muscular bands that gave a cavernous aspect to this area with little development of apical portion of. As far as ventricular outflow tract was concerned, dilatation of infundibulum was noteworthy with thinning of anterior wall secondary to decreased myocardial fibers [7]. that septum separates a 10 redundant and of atrialized anterior functional ventricular and leaflet portion functional free that wall, ofinserts forming portions directly aof curtain into of functional portion of a redundant anterior leaflet that inserts directly into septum and ventricular free wall, forming a curtain that separates atrialized and functional portions of. Note functional opening (arrow) in a subinfundibular position. The echocardiographic long axis image of chambers separation between two portions of by a redundant and irregular anterior leaflet. PA: Pulmonary artery. Or abbreviations as before. Right anomaly chambers 11 show valve grade of anianatomic teringspecimen and severe with dysplasia Ebstein's of Right chambers of an anatomic specimen with Ebstein's anomaly show grade I tering and severe dysplasia of valve. Note huge dilatation of atrium, numerous fibromyxomatous nodules on 3 leaflets and short, thickened and absent chordae tendineae. The echocardiographic images show nodules on leaflets. Abbreviations as before. The anatomo-echocardiographic correlation spectrum of leaflet tering from mild to extreme. In some cases with mild leaflet tering, regurgitation is caused by severe dysplasia affecting tensor apparatus, with short, thickened or absent chordae tendineae. When chordae are absent free leaflets insert directly into ventricular wall. Patent foramen ovale is atrial septal defect most frequently associated with Ebstein's anomaly (67%). It can occur as an isolated entity or with extension to a foramen secundum or superior sinus venosus. Ventricular septal defects are not common. The association of Ebstein's anomaly with obstruction of ventricular outflow tract is often observed. In our series 33% of cases had pulmonary stenosis and/or atresia. It bears mentioning that in this anomaly left chambers are not always nor- junction Ebstein's tricular ventricular 12 anomaly posterior of and an wall chambers aneurysmal significant (asterisk) of adilatation fibrous specimen sacaart on atriovenwith of chambers of a specimen heart with Ebstein's anomaly significant dilatation at atrioventricular junction and an aneurysmal fibrous sac on ventricular posterior wall (asterisk). The echocardiographic image same finding (asterisk). Abbreviations as before. Page 8 of 10

9 Cardiovascular Ultrasound 2007, :43 tion wall to occupied 13 underdevelopment ventricular of by multiple functional septum myocardial of portion (black apical bands arrow) of trabecular that join porfree of functional portion of underdevelopment of apical trabecular portion occupied by multiple myocardial bands that join free wall to ventricular septum (black arrow). The 4 chamber echocardiographic image a trabecular pattern of apical portion (white arrow) very similar to that of anatomic specimen. Abbreviations as before. mal; in our series we found such alterations of mitral valve as cleft of medial leaflet, unicuspid mitral valve, prolapse of anterior leaflet and mitral supravalvular membrane. The last finding was found in association with noncompaction of left and anomalous myocardial band. In 1972 areas of fibrosis in ventricular septum and left ventricular wall with cytolysis and collapsed sarcolemmas were described [12]. ning of significant its 1 wallof(asterisk) dilatation functional of portion infundibulum of (I) with thin of functional portion of significant dilatation of infundibulum (I) with thinning of its wall (Asterisk). The echocardiographic image at level of great vessels dilatation of infundibulum (I). Abbreviations as before. lously. In inflow tract valve is tered to ventricular wall in variable degrees, while ventricular wall presents thinning, fibrosis and absence of myocardium. The trabecular portion is poorly developed, and infundibular walls are thin. In summary, Ebstein's anomaly is a disease of entire, in some cases with alterations of left as well. Conclusion This study leads us to conclude that in Ebstein's anomaly entire ventricular must be examined meticu- 14 of functional portion of multiple myocardial bands that joint free wall to ventricular septum (black arrow). The 4 chamber image a similar trabecular pattern (white arrow) to that of anantomic specimen. Abbreviations as before. cular tum (stylus) band 16 that inofajoins heart left with freeebstein's wall with anomaly an ventricular anomalous sepmus of left an anomalous muscular band that joins free wall with ventricular septum (stylus) in a heart with Ebstein's anomaly. The echocardiographic image an anomalous band in left similar to that of anatomic specimen. Note tering of septal leaflet (asterisk) to ventricular septum. LAA: Left atrial appendage; AMB: Anomalous muscular band. Or abbreviations as before. Page 9 of 10

10 Cardiovascular Ultrasound 2007, :43 This anatomo-echocardiographic correlation of Ebstein's anomaly has potential of enriching understanding of images this malformation presents. Such a comprehension can contribute to a precise diagnosis that will lead to early and appropriate treatment of patients with this type of congenital heart disease. Authors' contributions LMC and NEZ participated to written manuscript, MKN made photographs of heart specimens and helped to draft manuscript and CK helped to draft manuscript and made translation from spanish into english. All authors read and approved final manuscript. References 1. Becker AE, Becker MJ, Edwards JE: Pathologic spectrum and dysplasia of valve: features in common with Ebstein's malformation. Arch Pathol 1971, 91: Lev M, Liberthson RR, Joseph RH, Seten CE, Kunshe RD, Echner FAO, Millar RA: The pathologic anatomy of Ebstein's disease. Arch Pathol 1970, 90: Anderson KR, Lie JT: Pathologic anatomy of Ebstein's anomaly of heart revisited. Am J Cardiol 1978, 49: Anderson KR, Zuberbuhler JR, Anderson RH, Becker AE, Lie JT: Morphologic spectrum of Ebstein's anomaly of Herat. A re. Mayo Clin Proc 1979, 4: Muñoz-Castellanos L, Barros W, García F, Salinas HC: Estudio patológico de la displasia y el adosamiento valvulares en la enfermedad de Ebstein. Arch Inst Cardiol Mex 1993, 63: Kuri Nivón M, Muñoz Castellanos L, Salinas Sánchez C: Estudio morfológico de 23 corazones con anomalía de Ebstein. Arch Inst Cardiol Mex 199, 6: Anderson K: The ventricular myocardium in Ebstein's anomaly. A morphometric histopathologic study. Mayo Clin Proc 1979, 4: Attie F, Rosas M, Rijlaarsdam M, Buendía A, Zabal C, Kuri J, Granados N: The adult patient with Ebstein's anomaly: Outcome in 72 unoperated patients. Medicine 2000, 79: Farooki ZQ, Henry JG, Green EW: Echocardiographic spectrum of Ebstein's anomaly of valve. Circulation 1976, 3: Shiina A, Seward JA, Edwards WD, Hagler DJ, Tajik AJ: Two-dimensional echocardiographic spectrum of Ebstein's anomaly: detailed anatomic assessment. J Am Coll Cardiol 1984, 3: Tynan MJ, Becker AE, Macartney FJ, Jiménez M, Shinebourne EA, Anderson RH: Nomenclatura and classification of congenital heart disease. Br Heart J 1979, 41: Bialostozky D, Medrano G, Muñoz-Castellanos L, Contreras R: Vectorcardiographic study and anatomic observations in 21 cases of Ebstein's malformation of valve. Am J Cardiol 1972, 30(4): VanMierop LHS, Alley RD, Kausel HW, Stranahan S: The anatomy and embryology of endocardial cushion defects. J Thorac cardiovascular Surg 1992, 43: Celermajer DS, Deanfield JE: Diseases of valve. In Paediatric Cardiology Second edition. Edited by: Anderson RH, Baker EJ, MaCartney FJ, Rigby ML, Shinebourne EA, Tynan M. Churchill Livingstone, London; 2002: Keith JD: Ebstein's disease. In Heart disease in infancy and chilhood 3rd edition. Edited by: Keith JD, Rowe RD, Vlod P. MacMillan New York; 1978: Rowe RD, Freedom RM, Mehrizi A, Bloom RK: The neonate with congenital heart disease. 2nd edition. Saunders Philadelphia; 1981: Shardland GK, Chita SK, Allan LD: Tricuspid valve dysplasia or displacement in intrauterine life. J Am Coll Cardiol 1991, 17: Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, Allan L, Nihoyannopoulos P, Somerville J, Deanfield : Ebstein's anomaly: Presentation and outcome from fetus to adult. J Am Coll Cardiol 1994, 23: Zuberbuhler JR, Allwork SP, Anderson RH: The spectrum of Ebstein's anomaly of valve. J Thorac Cardiovasc Surg 1979, 77: Freedom RM: Discordant atrioventricular connection and congenitally corrected transposition. In Paediatric Cardiology 2nd edition. Edited by: Anderson RH, Baker EJ, Macartney F, Rigby ML, Shinebourne EA, Tynan M. Churchill Livingstone; 2002: Publish with BioMed Central and every scientist can read your work free of charge "BioMed Central will be most significant development for disseminating results of biomedical research in our lifetime." Sir Paul Nurse, Cancer Research UK Your research papers will be: available free of charge to entire biomedical community peer reed and published immediately upon acceptance cited in PubMed and archived on PubMed Central yours you keep copy BioMedcentral Submit your manuscript here: Page 10 of 10