Cross-sectional echocardiographic features of Ebstein's anomaly of the tricuspid valve

Multicrystal real time-motion echocardiographic studies of 10 patients with Ebstein's anomaly of the tricuspid valve are reported. Eight patients also underwent hemodynamic and angiographic study. All but one of the patients had inferior and leftward displacement of the tricuspid valve. In seven patients multiple echoes were obtained from the tricuspid valve; all seven manifested elongated tricuspid valve leaflets, and in six the leaflets were thickened. Excessive excursion of the tricuspid valve was noted in five patients and a “whipping” motion seen in three. Nonspecific findings in nine patients included an increased right ventricular dimension and systolic anterior septal motion. Thus, cross-sectional echocardiography provides distinctive diagnostic findings that are specific for Ebstein's anomaly.     

Surgery for Ebstein's anomaly: the clinical and echocardiographic evaluation of a new technique

Ten consecutive patients (age range 4 to 44 years, mean 22) underwent surgical repair of Ebstein's anomaly by vertical plication of the right ventricle and reimplantation of the tricuspid valve leaflets. No patient died during or after operation. Intraoperative postbypass echocardiography documented a good result in nine patients but severe tricuspid regurgitation in one patient, who then underwent prosthetic valve replacement during a second period of cardiopulmonary bypass. Two of four patients who had had right ventricular papillary muscle dysfunction in the early postoperative period showed improved papillary muscle function with concomitant reduction of tricuspid regurgitation 6 months later. All patients were evaluated clinically and by echocardiography 2 to 23 months later. All patients showed clinical improvement, seven by one functional class and three by two classes. All were in sinus rhythm. The mean cardiothoracic ratio decreased by 6% (p less than 0.05). On bicycle ergometry performed in six patients, peak oxygen consumption exceeded 20 ml/kg per min in five. Tricuspid regurgitation diminished in eight patients (by three grades in two patients, by two grades in five and by one grade in one patient); it remained unchanged in two. Comparison of preoperative and postoperative pulsed Doppler flow velocities across the pulmonary valve showed an increase in the peak velocity of flow across the valve (mean 83 +/- 14 versus 97 +/- 11 cm/s, p less than 0.005) and a decrease in the time to peak velocity (mean 130 +/- 16 versus 91 +/- 23 ms, p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)     

Transesophageal three-dimensional echocardiographic demonstration of Ebstein's anomaly

We report three-dimensional transesophageal echocardiographic findings in an adult patient with Ebstein's anomaly. Using the anyplane technique and multiple views, especially the short-axis view of tricuspid valve, three-dimensional transesophageal echocardiography clearly demonstrated the intermittent tethering of all three leaflets of tricuspid valve to the right ventricular walls giving a "bubble-like" appearance. On the other hand, two-dimensional transesophageal echocardiography demonstrated well the tethering of the septal tricuspid leaflet, but tethering of the other two leaflets was not well seen. To our knowledge, these findings have not been demonstrated by three-dimensional transesophageal echocardiography before.     

Clinical and echocardiographic features of Ebstein's malformation in Sudanese patients

Ebstein's malformation is a rare congenital cardiac malformation, accounting for about 0.5% of all congenital cardiac lesions. We report our experience with the anomaly as encountered at the Sudan Heart Centre from July 2004 to April 2005. Diagnosis was based on the echocardiographic demonstration of displacement of the septal leaflet of the tricuspid valve towards the ventricular apex by greater than 8 millimetres per metre squared. RESULTS: In a period of 10 months, we identified 12 patients with the malformation, this number constituting 2% of all patients seen with congenital cardiac disease. The age ranged from 2 weeks to 35 years. Of the patients, half were asymptomatic, while the other half presented with congestive cardiac failure, 4 of these having cyanosis in addition to heart failure. Associated diseases included chronic renal failure, impaired hearing, stunted growth, and developmental delay. Electrocardiographic abnormalities included peaked and tall P waves, seen in four-fifths, an Rsr pattern, first degree atrioventricular block, atrial fibrillation, and Wolf-Parkinson-White syndrome, including the Mahaim pattern of pre-excitation. Associated abnormalities diagnosed echocardiographically included atrial septal defects, prolapse of the leaflets of the mitral valve, left ventricular dysfunction, atrial septal aneurysm, pulmonary valvar stenosis, and pericardial effusion. CONCLUSIONS: Ebstein's malformation was seen four times more frequently at the Sudan Heart Centre when compared to the average frequency reported in the Western literature. Many of the associated diseases encountered in Sudan had not previously been reported.     

Live/real time three-dimensional transthoracic echocardiographic assessment of Ebstein's anomaly

We describe our experience in using live/real time three-dimensional transthoracic echocardiography (3D TTE) in the assessment of five adult patients with Ebstein's anomaly. The technique was found useful in assessing the distribution and extent of tethering of each of the three leaflets of the tricuspid valve (TV) to the underlying right ventricular walls and the ventricular septum. The characteristic bubble-like appearance resulting from bulging of the non-tethered areas of the TV leaflets was also well visualized in three dimensions and their size measured. Thus, an estimate of the nontethered or free segments of all three leaflets of the TV could be obtained using this technique. This has important implications when considering these patients for surgical repair of the TV. Visualization of all three leaflets of the TV and their extent of tethering by 3D TTE also made it easier to identify the boundaries of the functioning right ventricular chamber potentially providing a more reliable assessment of its volumes and ejection fraction. Cropping of color Doppler 3D TTE data sets provided en face viewing of the TV regurgitation vena contracta permitting accurate assessment of its shape and size. This has the potential to provide a more accurate quantitative estimation of TV regurgitation severity as compared to two-dimensional color Doppler. In conclusion, live/real time 3D TTE appears useful in supplementing two-dimensional echocardiography in more comprehensively assessing the morphologic features of Ebstein's anomaly.     

Left ventricular noncompaction and mid-caviter narrowing associated with Ebstein's anomaly: three-dimensional transthoracic echocardiographic image

An 50 year-old asymptomatic female was admitted to preoperative cardiovascular evaluation for noncardiac surgery. Two-dimensional transthoracic echocardiography demonstrated that left ventricular dimensions and ejection fraction were normal. The mitral valve was normal both in morphology and function. The attached margin of septal and anterior leaflet of the tricuspid valve was apically displaced. But right ventricle and right atrium dimensions were normal, and Doppler interrogation showed a slight tricuspid regurgitation. Two and three-dimensional transthoracic echocardiography showed prominent numerous trabeculations in the left ventricular apex. Colour flow Doppler studies confirmed the presence of blood flow within the trabeculations. Multi-plane three-dimensional transthoracic echocardiography showed a muscular band leading to left ventricular mid-caviter narrowing. Pulse wave Doppler echocardiography demonstrated that no intraventricular gradient existed. Ventricular noncompaction associated with Ebstein's malformation has been reported. But left ventricular noncompaction and mid-caviter narrowing associated with Ebstein's anomaly have not been reported so far.     

Lithium and Ebstein's anomaly

The article deals with Ebstein's anomaly, lithium and their relationship. Some studies suggest that lithium might be involved as a teratogen increasing the incidence of Ebstein's anomaly in the offspring of female patients with manio-depressive psychosis and lithium-administered during pregnancy. The second part of the article contains data on the incidence of Ebstein's anomaly in the Czech Socialist Republic between 1960 and 1985. The results indicate a steady rise in the incidence of this congenital malformation over the above period of time.     

Ebstein's anomaly in adult

Ebstein's anomaly is a rare congenital cardiac disease initially described by Wilhelm Ebstein in 1866. It is defined as the significant apical displacement of the part of the tricuspid valve causing significant tricuspid regurgitation and reduction of the functional right ventricle, right atrial and right ventricular dilatation and atrial and ventricular arrhythmias. Ebstein's anomaly occurs approximately once in 20,000 live births and only 5% of patients survive beyond the fifth decade. We present a case of Ebstein's anomaly in a 69-year-old Italian woman. Also this case is illustrative of Ebstein's anomaly in adult.     

Ebstein's anomaly in infants

Ebstein's disease is a congenital malformation of the tricuspid valve that causes various hemodynamic alterations depending on the anatomic changes in the valve, presence or absence of atrial septal defect and reduction in the ventricular function. We present 19 patients with Ebstein's malformation of the tricuspid valve younger than 2 years of age. The long-term follow-up was from 1 week to 16 years with a mean of 40 months. Eight children died, 6 were lost in the follow-up and 5 survived. Early cyanosis with associated lesions give a bad prognosis. If the child survives the first year of life he has a better long term prognosis.     

Ebstein's anomaly with pulmonary hypoplasia. Diagnosis with Doppler color echocardiography in the fetus]

A case of Ebstein's anomaly with pulmonary hypoplasia diagnosed in utero by color Doppler echocardiography in a 26 weeks fetus is presented. We emphasize the importance of ultrasound in the early detection of this congenital abnormality that allowed our medical team to offer correct assistance to a severe ill child at birth.     

Ebstein's anomaly in adults: Doppler-echocardiographic evaluation

A systematic approach and segmental analysis are required for comprehensive assessment including both morphologic and functional abnormalities associated with Ebstein's anomaly. The essence of the disease is an apical displacement of both the septal and the posterior tricuspid leaflets, exceeding 20 mm or 8 mm/m2 in adults. As a consequence, the right heart consists of three components including the true right atrium, the functional right ventricle (RV) and an intervening zone that is anatomically ventricular but functionally right atrial (atrialized RV). The thin wall of the atrialized RV may result in an aneurysm between the anatomic tricuspid annulus and the apically displaced posterior leaflet. The annular attachment of the anterior leaflet is normal, which may be dysplastic and adherent to the RV wall. Tricuspid regurgitation is usually moderate to severe. Size, shape and function of the functional RV must be described. The paradoxical motion of the interventricular septum causes alterations in left ventricular geometry and function. An interatrial communication is frequently present. Echocardiography is the method of choice to diagnose Ebstein's anomaly on its own or in association with other heart defects.     

Signal-averaged electrocardiogram in Ebstein's anomaly

We sought to establish pathogenetic links between electrophysiology, histopathology, and ventricular tachyarrhythmias in patients with Ebstein's anomaly. The atrialized right ventricle (ARV) is the site of mechanically inducible ventricular tachyarrhythmias, but relations between the arrhythmogenic substrate, the type of tachyarrhythmias, and the trigger(s) have not been established. This study comprised 23 patients (10 men and 13 women; aged 18 to 58 years; mean 32 +/- 3) who did not undergo surgery and 6 pre- and postoperative patients with Ebstein's anomaly, diagnosed by transthoracic and transesophageal echocardiography. Twenty-one patients had classic Ebstein's anomaly and 2 had mild forms. Signal-averaged electrocardiograms (SAECGs) identified slow conduction by using 3 time-domain variables calculated by an automated algorithm and inspected visually. Two variables were required to establish the presence of late potentials. SAECGs were repeated in 6 patients after surgical exclusion of the ARV. Five surgical specimens of the ARV and the true right atrium were examined histologically. Mathematic simulations were used to illustrate anchored and unanchored spiral/scroll waves. SAECGs were positive in 21 patients with classic Ebstein's anomaly and were negative postoperatively in the 6 so studied. The ARV was characterized histologically by clusters of cardiomyocytes isolated within a fibrous matrix. We hypothesize that SAECGs identify slow conduction residing in the ARV, and that excitation of this arrhythmogenic substrate provokes spiral/scroll waves that cannot anchor because clusters of cardiomyocytes are isolated within a fibrous matrix. The waves meander erratically as polymorphic ventricular tachycardia or break up into ventricular fibrillation.     

Echocardiographic assessment of the right ventricle in Ebstein's anomaly: relation to clinical outcome

Two-dimensional echocardiography was performed in 16 patients with Ebstein's anomaly to assess right ventricular anatomy and function in relation to clinical features and prognosis. Measurements of right ventricular anatomy and function were established in 10 normal subjects for comparison. Ten patients were in New York Heart Association functional class I, four in class II and one each in classes III and IV. Right ventricular morphology and the three tricuspid valve leaflets were assessed from right ventricular inflow tract and apical four chamber views. The anterior tricuspid leaflet was abnormal but not displaced in all patients; the septal and posterior leaflets were displaced in 14 (88%) and 11 (69%) patients respectively. The posterior leaflet was best visualized from the right ventricular inflow tract, and in two patients this view was required for the echocardiographic diagnosis of Ebstein's anomaly, based on displacement of the septal tricuspid valve leaflet. An index of right ventricular function, the fractional area contraction, was defined as the difference between the end-diastolic and the end-systolic area, normalized to the end-diastolic area. This index was calculated for both the proximal (atrialized) right ventricle and the total right ventricle. Total right ventricular end-diastolic area and fractional area contraction exceeded 95% confidence limits when compared with values in the normal group. During a median follow-up period of 4 years three patients died. They had severe right heart morphologic or functional abnormalities; two were in functional class III or IV and one was asymptomatic. None of the survivors had severe symptoms.(ABSTRACT TRUNCATED AT 250 WORDS)