Cardiorespiratory response to exercise in Ebstein's anomaly

Fourteen patients with unrepaired Ebstein's anomaly underwent maximal exercise testing between October 1982 and April 1984. Compared with control subjects, these patients had significantly lower values for total work performed, exercise time, maximal oxygen uptake, blood oxygen saturation at rest and during exercise, and heart rate and systolic blood pressure during maximal exercise. They had a significant increase in heart rate at rest. The ventilatory equivalent for oxygen was increased significantly both at rest and during exercise. Thus, patients with Ebstein's anomaly have decreased exercise tolerance owing to both cardiac and respiratory limitations.     

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.     

Morphological aspects of Ebstein's anomaly in adults

The essence of the Ebstein's malformation is that the tricuspid valve leaflets do not attach normally to the valve annulus, and the effective orifice is displaced downward into the right ventricular cavity at the junction of the inlet and trabecular components of the right ventricle. Only the septal and posterior leaflets are displaced and divide the right ventricle into two portions. The inlet portion is usually integrated functionally with the right atrium ("atrialized portion"), while the other, including the trabecular and outlet portions, constitutes the functional right ventricle. The proximal atrialized right ventricle often has a wall thinner than the distal functional right ventricle, due to partial congenital absence of myocardium. An atrial septal defect is present in more than one-third of hearts, and the majority of the remainder has a patent foramen ovale resulting in a right-to-left shunt. The downward displacement of the septal tricuspid valve leaflet is associated with discontinuity of the central fibrous body and septal atrioventricular ring, thus creating a potential substrate for accessory atrioventricular connections and ventricular pre-excitation making the patient at risk of sudden death. Angiography has demonstrated that a significant number of patients with Ebstein's anomaly also have morphofunctional abnormalities of the left ventricle, which may be explained by increased fibrosis in the left ventricular wall and ventricular septum as demonstrated by histological studies. Regarding embryology, the leaflets and tensile apparatus of the tricuspid valve are believed to be formed mostly by a process of delamination of the inner layers of the inlet zone of the right ventricle. The downward displacement of the leaflets in Ebstein's anomaly suggests that delamination from the inlet portion failed to occur.     

Ebstein's anomaly in adults. Arrhythmias: diagnosis and therapeutic approach

Patients with Ebstein's anomaly (M.E.) are known to have a high potential for developing arrhythmia, in the vast majority, of the tachycardia type. Most of these tachycardias are based on accessory pathways (AP) located along the anomalous atrioventricular valve, found in up to 30% of this patient cohort. Next to this main representative for congenital arrhythmogenic substrates, various types of acquired tachycardia were found in patients with M.E., such as atrial ectopic tachycardia (AET), atrial flutter (A. Flutt.), atrial reentry tachycardia (ART), atrial fibrillation and ventricular tachyarrhythmia. Refractoriness to medical treatment and a higher potential for side effects in these patients resulted in an increasing referral to interventional electrophysiology recent years. A total of 37 patients with M. E. and tachycardia underwent an attempt for radiofrequency current (RFC) treatment at our institution, 30 of whom were adults aged 18 to 61 yrs (33 +/- 12.2. yrs). Just 4 patients under went surgery for reconstruction of the anomalous valve and closure of an ASD (2 patients), in whom a total of 6 cases of atrial tachycardia were found, 3 common A. Flutt. and 3 atriotomy-scar related ART. Of the 26 unoperated patients, one presented with typical AV-nodal reentry tachycardia and the remaining patients with atrioventricular reentry tachycardia, based on a total of 39 APs. 27/39 Aps had bidirectional conduction properties, causing the WPW syndrome in 16 patients, 8 APs were concealed and the remaining 4 were Mahaim fibers. Within 39 sessions, 26 of the 30 patients were successfully treated by RFC ablation of the arrhythmogenic substrates. Mean session duration lasted for 313 +/- 151 min (range 95 to 660 min), and a fluoroscopy time of 56.0 +/- 38.4 min (2.2 to 146.8 min) was required. There were no severe acute complications. Conclusions: In patients with M. E. not only a high potential for the development of tachycardia, but also a tendency towards multiple arrhythmogenic substrates in the single patient can be ascertained. RFC ablation can be used safely and effectively for treatment for various types of tachycardias in patients with M. E.. Such therapy carries the potential for a definitive treatment and should be taken early into consideration as it meets the requirements this patient cohort.     

Left ventricular geometry and function in adults with Ebstein's anomaly of the tricuspid valve

We postulated that the abnormal shape, size, and function of the right heart and adjoining ventricular septum in adults with Ebstein's anomaly of the tricuspid valve might in turn alter the shape and function of the left ventricle. Seven adult patients with uncomplicated Ebstein's anomaly were studied. Left ventricular geometry was determined by two-dimensional echocardiography. Left ventricular function was assessed by treadmill exercise and radionuclide angiography at rest and with exercise. Paradoxic ventricular septal motion was consistently present. Left ventricular eccentricity (ratio of two minor axes in the short-axis view) was uniformly abnormal, averaging 1.35 +/- 0.23 (normal = 1.02 +/- 0.05). The ratio of right to left ventricular cavity size averaged 1.70 +/- 0.44 (normal 0.65 +/- 0.30), and tricuspid valve displacement into the right ventricular cavity averaged 52% (normal 8%). Functional right atrial size averaged 27.6 +/- 5.2 cm2 (normal right atrial area = 13.1 +/- 2.2 cm2). Resting left ventricular ejection fractions were below 50% in all but two patients. In response to Bruce protocol exercise stress, there were consistently appropriate increments in heart rate, blood pressure, and peak double product and, with one exception, radionuclide left ventricular ejection fraction. There were significant correlations between tricuspid valve displacement and functional right atrial size versus resting left ventricular ejection fraction and left ventricular eccentricity. These data support the hypothesis that derangements in right heart morphology and function in Ebstein's anomaly contribute to significant alterations in left ventricular geometry, but the geometric alterations are associated with tangible but less significant changes in left ventricular systolic function.     

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 refractory atrial tachycardia

The incidence of accessory pathways in patients with Ebstein's anomaly is high, ranging from 20% to 30%, with right and multiple pathways being more commonly encountered. Radiofrequency ablation can eliminate the accessory pathway, but success rates of ablation treatment and the chances of recurrence are generally less satisfactory as compared to those observed when the procedure is performed on structurally normal hearts. We report the case of a 56-year-old man with Ebstein's anomaly and refractory atrial tachycardia. The special abnormality of the tricuspid valve led to the failure of preoperative electrophysiological mapping and radiofrequency ablation treatment. Finally, the anomalies observed in this patient were corrected with simultaneous surgery and pathway resection.     

Outcome in neonates with Ebstein's anomaly.

The presentation and outcome of 50 patients with neonatal Ebstein's anomaly seen from 1961 to 1990 were reviewed. The majority (88%) presented in the 1st 3 days of life; cyanosis (80%) was the most common presenting feature. Associated defects, present in 27 infants (54%), included pulmonary stenosis in 11 and atresia in 7. Nine patients (18%) died in the neonatal period; there were 15 late deaths (due to hemodynamic deterioration in 9, sudden death in 5 and a noncardiac cause in 1) at a mean age of 4.5 years (range 4 months to 19 years). Actuarial survival at 10 years was 61%. A new echocardiographic grade (1 to 4 in order of increasing severity of the defect) was devised with use of the ratio of the area of the right atrium and atrialized right ventricle to the area of the functional right ventricle and left heart chambers. Cardiac death occurred in 0 of 4 infants with grade 1, 1 (10%) of 10 with grade 2, 4 (44%) of 9 with grade 3 and 5 (100%) of 5 with grade 4. In a multivariate analysis of clinical and investigational features at presentation, echocardiographic grade of severity was the best independent predictor of death. Neonates with Ebstein's anomaly have a high early mortality rate and those surviving the 1st month of life remain at high risk of late hemodynamic deterioration or sudden death. Echocardiographic grading of severity of the defect permits prognostic stratification.     

Ebstein's anomaly with the Wolff-Parkinson-White syndrome

Thirty-three patients were studied with Ebstein's anomaly, associated to Wolff-Parkinson-White syndrome with the purpose of analyze their electrophysiologic characteristics. In this patients the right preexcitation was before the activation of the right ventricle mass, overshadowing the manifestations of the right bundle branch block (usual in patients with Ebstein's anomaly without preexcitation). In conclusion the absence of manifestations of right bundle branch block in the presence of Ebstein's anomaly diagnosed by hemodynamic study or echocardiography let us think in the coexistence of the preexcitation and suggest the pertinent electrophysiologic study; as the association of supraventricular tachycardia in this group is very high (94%) most of them paroxysmal orthodromic tachycardia.     

Ebstein's anomaly in neonates.

Ebstein's anomaly is a rare congenital heart disease abnormality in which the tricuspid valve leaflets do not attach normally to the tricuspid valve annulus. The effective tricuspid valve orifice is displaced apically into the right ventricle (RV), near the junction of the inlet and the trabecular parts of the RV. The authors present a retrospective study of the patients with Ebstein's anomaly admitted to a neonatal intensive care unit, in the period between January 1993 and March 2000. There were ten patients, representing 0.24% of total neonates and 1.99% of total congenital heart disease admitted to the institution in the same period. Fifty per cent were male and only one case had prenatal diagnosis. Holosystolic murmur (100%) from tricuspid regurgitation and cyanosis (80%) were the most frequent clinical findings. Chest X-ray was abnormal in 90% of the neonates, with a "balloon-shaped" enlarged heart. The main electrocardiographic findings were right atrial enlargement (70%) and arrhythmias (40%). Apical displacement of the septal leaflet of the tricuspid valve, to a maximum of 20 mm, and leaflets tethering to underlying RV myocardium were found in all patients. Tricuspid valve regurgitation was found in 90% (severe form in four cases). An atrial intracardiac shunt, mostly right-to-left, was also found in 50%. Digoxin was used (40%) to restore sinus rhythm. Fifty per cent of the neonates received intravenous prostaglandins. Two patients required a surgical procedure. Two patients died in the neonatal period. During the follow-up period (range 0.3-74.6 months), only one episode of supraventricular tachycardia was recorded. At present seven patients are clinically stable, three of them on medication.     

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.     

Exercise testing in adults after repair of aortic coarctation: evaluation of cardiopulmonary exercise capacity and B-type natriuretic protein levels

The aim of the study was to investigate, in adult patients after successful repair of aortic coarctation, potential relationships between B-type natriuretic peptide levels and exercise capacity and the following factors: arterial hypertension, residual stenosis of the ascending aorta, and age at the time of surgery. The study group comprised 74 patients (45 men) aged 19 to 61 years (mean, 31.2 +/- 9.8 yr), who had undergone surgery at the age of 0.5 to 34 years (mean, 10.4 +/- 6.8 yr). The surgery was performed between 5 and 34 years earlier (mean, 21.4 +/- 6.2 yr). A subgroup with residual aortic stenosis (significant when > or =25 mmHg) comprised 32 patients; a subgroup without residual stenosis comprised 42 patients. Patients were also divided into subgroups without arterial hypertension (n=32), with exercise-induced arterial hypertension (n=10), and with persistent arterial hypertension (n=32). All patients were in New York Heart Association functional class I. The control group comprised 30 healthy subjects (18 men) aged 26 to 46 years (mean, 32.2 +/- 6.6 yr). After testing exercise capacity in accordance with a modified Bruce protocol, we concluded that the exercise capacity of adults is reduced after surgical repair of aortic coarctation. This reduction is more pronounced in patients who have arterial hypertension, but it is unaffected by residual stenosis of the descending aorta. Serum B-natriuretic peptide concentrations, as determined by immunoradiometric assay, are significantly elevated, which may result from pressure overload of the left ventricle or from residual myocardial lesions due to coarctation repair at an older age.     

Successful pregnancy in a patient with Ebstein's anomaly

A case is described of a successful pregnancy in a woman with Ebstein's anomaly. It is suggested that careful assessment should be made before advising against starting or terminating pregnancies, since successful outcomes are possible.     

Successful pregnancy in a patient with Ebstein's anomaly

A case is described of a successful pregnancy in a woman with Ebstein's anomaly. It is suggested that careful assessment should be made before advising against starting or terminating pregnancies, since successful outcomes are possible.