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The Ebstein anomaly is a rare congenital heart disease involving the position and structure of the tricuspid valve. Although most cases are sporadic, familial occurrence has been documented. We report on 2 sisters, born to consanguineous parents, who were diagnosed prenatally with severe Ebstein anomaly.
A case of Ebstein anomaly in a 2-day-old newborn is presented. The complementary roles of two-dimensional Doppler echocardiography and magnetic resonance imaging in managing this cardiac malformation are emphasized.
Ebstein's anomaly is a complex malformation that has been treated by various surgical techniques, with variable results. We have developed a repair-by plication of the free wall of the atrialized portion of the right ventricle, posterior tricuspid annuloplasty, and right atrial reduction--that has been used since 1972 in 16 patients. The repair is based on the construction of a monocusp valve by the use of the anterior leaflet of the tricuspid valve, which is usually enlarged in this anomaly. There were 14 operative survivors and no instances of complete heart block. Two patients with refractory dysrhythmias due to the Wolff-Parkinson-White syndrome underwent successful intraoperative epicardial and endocardial mapping and surgical division of accessory pathways. This repair has resulted in improvement in the operative mortality and in gratifying early and late results in most patients.
By the application of all methods of fetal echocardiography the prenatal diagnosis and a prognostically correct assessment was reached in a severe case of Ebstein's anomaly of tricuspid valve associated with pulmonary atresia, severe tricuspid regurgitation, and a hypoplastic main pulmonary artery. It is demonstrated that only the application of two-dimensional color-coded Doppler-echocardiography followed by a spectral-Doppler-echocardiography can result in an accurate evaluation of hemodynamical alterations and thus in the prognostic assessment in the reported case.
The Ebstein's malformation occurs in 0.5% of patients with congenital heart disease. The prenatal diagnosis of such a malformation in the 33rd week of pregnancy is reported. The fetal echocardiography was performed owing to a severe nonimmune hydrops fetalis. The typical distal displacement of the annular attachment of the tricuspid valve leaflets could be viewed in the apical four-chamber view. The application of the pulsed Doppler ultrasound enables the analysis of the cardial haemodynamics and thus the assessment of the severity and prognosis of the diagnosed malformation. In our case the prognosis was interdisciplinary estimated as being very poor (severe cardiac lesion with congestive heart failure already in utero); In the following days intrauterine death occurred. The autopsy confirmed all the prenatal findings.
The report relates experience with six patients with Kent bundles, five of whom had the Wolff-Parkinson-White syndrome, operated upon for tachyarrhythmias caused by the presence of the pathway. It was successfully divided in four patients, its antegrade conduction capacity interrupted in one, and a failure occurred in the sixth patient. In the three patients who had a Kent bundle with a short effective refractory period for antegrade conduction, the life-endangering potential of the Kent pathway was corrected. To emphasize the complexities of the tachyarrhythmias in Ebstein's anomaly, two patients have mild but controllable postoperative supraventricular tachyarrhythmias (SVT) due to a Mahaim pathway. The surgical problems encountered in these patients are reviewed.
Pulmonary atresia with intact ventricular septum is an uncommon congenital cardiac anomaly which very often present varying degrees of downward displacement and dysplasia of the tricuspid valve. We describe a case of pulmonary atresia and intact ventricular septum associated with Ebstein's malformation of the tricuspid valve first diagnosed with echocardiography and confirmed by angiocardiography and anatomic studies.
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BACKGROUND: Ebstein's anomaly is an uncommon congenital cardiac abnormality that may be associated with cyanosis and arrhythmias. For those female patients with the anomaly who survive to adult life there is little information available about pregnancy, maternal complications, and fetal outcome. This study was designed to address this issue so that these patients can receive appropriate advice and management. METHODS AND RESULTS: Forty two pregnancies in 12 women with Ebstein's anomaly were studied. The mothers' cardiac lesions were assessed on the basis of symptoms, the presence of cyanosis or arrhythmia, and by echocardiographic grading of severity. In the absence of important maternal cyanosis or arrhythmia, pregnancy was well tolerated. Neonatal outcome was good though there was an increased risk of prematurity and dysmaturity in the babies born to mothers with cyanosis. CONCLUSIONS: This study indicates that women with Ebstein's anomaly who reach child-bearing age can be advised that pregnancy is likely to be well tolerated with good fetal outcome. Maternal arrhythmia or cyanosis are indications for closer maternal and fetal observation.
The clinical features and natural history of Ebstein's anomaly are analyzed on the basis of experience with 67 consecutive patients who have had a mean follow-up of 12 years. Of the total group, 26 (39%) of the patients remained in functional Class I or II and 41 (61%) progressed at some time into Class III or IV. Death occurred in 14 (21%) of the patients, and these were characterized by one or more of the following features: (1) they were in functional Class III or IV, (2) they had moderate to severe cardiomegaly with a cardiothoracic ratio greater than 0.65, (3) they had cyanosis or an arterial oxygen saturation of less than 90%, and (4) they were infants when the diagnosis was made (10 patients, of whom 5 died). In patients in Class III or IV, a comparison was made between patients treated medically (10) and those treated surgically--either with tricuspid valve replacement (15) or with a modified tricuspid annuloplasty (16). It appears that modified tricuspid annuloplasty is the procedure of choice, because despite an operative mortality of 25% (4 patients), 12 of the 16 patients so treated improved clinically or had a reduction in heart size.
Ebstein's anomaly of the tricuspid valve occurs as an isolated defect with other forms of congenital heart disease such as transposition of the great arteries or tetralogy of Fallot or, rarely, in association with extracardiac malformations. Because so little is known about this latter group, we studied four cases clinically, at autopsy, and by means of a retrospective chart review. Major extracardiac changes most often involved the craniofacial region, central nervous system, and limbs. Karyotypes were normal, and no distinctive syndromes or anatomic patterns were identified. Since the timing of Ebstein's anomaly is quite precise, ascertainment of such cases on the basis of cardiac morphology may enhance the understanding of etiology and pathogenesis. Both causes and mechanisms may well be diverse, for the clinical histories and anatomic findings in present and previously reported cases vary considerably. Isolated Ebstein's anomaly may develop from topographically and temporally localized damage. Ebstein's anomaly with extracardiac defects may involve damage during a longer, and perhaps earlier, period.
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.
Nine patients with Ebstein's anomaly of the tricuspid valve were studied by two-dimensional echocardiography, using the standard long and short axis views as well as the apex four chamber view. With this latter view, the displacement of the tricuspid valve into the right ventricle was clearly seen in all nine cases of Ebstein's anomaly and was not noted in a control population. The severity of the tricuspid displacement was assessed by comparing the position of the mitral and tricuspid valves relative to the cardiac apex. The apex four chamber view allowed visualization of the atrioventricular (AV) ring simultaneously with the displaced tricuspid valve, and therefore the size of the "atrialized" right ventricle, true right ventricle and right atrium could be determined. These dimensions compared favorably with angiography.
Ebstein's anomaly of the tricuspid valve is associated with right ventricular dilatation in approximately two-thirds of cases. Dilatation may be massive. It involves not only the right ventricular wall proximal to the tricuspid valve (atrialized ventricle) but also the right ventricle distal to the valve (funtional right ventricle), including the right ventricular infundibulum. For further definition of the pathogenesis of dilatation of the functional right ventricle (distal chamber), a morphometric histopathologic study was performed on 10 hearts with Ebstein's anomaly and 10 normal age-matched control hearts. In the group with Ebstein's anomaly, five hearts exhibited dilated ventricles and five did not. The study demonstrated that dilatation of the right ventricle in Ebstein's anomaly was associated not only with thinning of the wall but also with an absolute decrease in the number of myocardial fibers counted through the thickness of the wall from endocardium to epidcardium.
Eight patients with Ebstein's anomaly, corrected by the Carpentier technique between 1984 and 1987, were evaluated by echocardiography and by conventional Doppler and color-flow Doppler mapping. Because this technique aims at restoring not only tricuspid valve function but also right ventricular size and geometry, the following parameters were studied: 1) right ventricular size, by measuring the apex-annulus distance compared with that of a group of 30 normal volunteers; and 2) tricuspid valve motion and function. The apex-annulus distance ranged from 5.8 to 8 cm (mean, 6.4 cm) in the Ebstein group and 3.8 to 5.1 cm (mean, 4.3 cm) in the control group. There was no tricuspid stenosis. Right ventricular filling was normal as documented by color-flow mapping. Tricuspid regurgitation was trivial in three patients, mild in four, and severe in only one, in whom a significantly restricted motion of the leaflets was demonstrated.
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.