SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
A case of Ebstein anomaly is reported, and the diagnostic potential of cine-CT in assessing the magnitude of the anatomic changes and the presence of right to left intracardiac shunting is evaluated.
Ebstein anomaly (EA) is a relatively uncommon congenital heart defect and it is very rarely associated with a chromosomal anomaly. We report two distinct rearrangements of the chromosomal region 11q arm in two unrelated patients with Ebstein anomaly, renal malformation, minor anomalies, and the Pierre Robin sequence. The first patient had an interstitial deletion of chromosome 11 [46,XY,del(11)(11q21q23), and the other had a tertiary trisomy of chromosome 11qter (47,XX,+der(22)t(11;22)(q23;q11.2) Its association with either a chromosome 11q deletion or a duplication in some individuals suggests that a rearrangement of the 11q region is likely to cause a shift of the individuals' underlying liability to develop EA above a certain threshold.
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.
Duplication of the tricuspid valve is a rare congenital cardiac anomaly. We experienced one infant case with Ebstein anomaly, who died at seven months of age. At autopsy, the clinical diagnosis was confirmed, and also the patient was found to have double tricuspid valve orifices, each of which were accompanied by subvalvular tension apparatuses. Tricuspid valve leaflets seen in Ebstein anomaly are known to have fenestrations as accessory orifices for some instances, but this case is unique and interesting in that each valve orifice has well-developed chordae tendinae and papillary muscles below it.
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.
We performed ECG-gated MR imaging in four patients (4 months-15 years old), all of whom had a previously diagnosed severe form of Ebstein anomaly of the tricuspid valve. The goal was to study the anatomy of this congenital heart defect with MR imaging. The axial and coronal scans provided the best details. All characteristic abnormalities were seen well: the maintained proximal and faulty distal attachment of the anterior tricuspid leaflet, the absence of both septal and posterior tricuspid leaflets, the size and delineation of the anatomically atrialized chamber of the right ventricle, and the small, compressed trabecular portion with the markedly dilated pumping outflow tract of the right ventricle proper. Our results suggest that MR may furnish valuable information in patients with Ebstein anomaly when echocardiography is either inconclusive or technically difficult to perform.
Assessment of ventricular systolic function has been based on the geometric models of ventricular shape. This study was designed to define normal values for a nongeometric myocardial performance index (MPI) in children and to evaluate the utility of MPI in congenital heart disease. The MPI measures the ratio of total time spent in isovolumic activity (isovolumic contraction time and isovolumic relaxation time) to the ejection time. The right ventricular (RV) and left ventricular (LV) MPI were measured in 152 normal children (ages 3 to 18 years) and 45 preoperative patients with Ebstein anomaly (age 1 week to 52 years). In normal children, the RV MPI was 0.32+/-0.03 and the LV MPI was 0.35+/-0.03. In the Ebstein group, both RV and LV MPI were abnormally increased compared with age-matched normal subjects (Ebstein group: RV MPI=0.49+/-0.12, LV MPI=0.42+/-0.09, P < .001). Increasing RV dysfunction was associated with progressively increasing (abnormal) values of RV MPI (P < .001). The myocardial performance index quantitatively reflects ventricular performance in patients with complex ventricular geometry (ie, Ebstein anomaly). In the absence of a geometric solution, this nongeometric index is particularly appealing for the assessment of RV or LV performance.
Ebstein's anomaly is a rare congenital cardiac anomaly showing significant clinical manifestations with a high mortality rate in the neonatal period. The prognosis of the patient is essentially determined by the severity in morphological changes, however, high pulmonary vascular resistance in the neonatal period may aggravate tricuspid regurgitation and lead to functional pulmonary atresia. We describe a critically ill neonate with morphologically mild Ebstein's anomaly who was successfully managed with intensive care including isoproterenol administration for functional pulmonary atresia. Isoproterenol is a potent pulmonary vasodilator with inotropic and chronotropic effects, and seemed to decrease the pulmonary vascular resistance allowing increased antegrade blood flow to the pulmonary artery and improved cardiac output. If tachycardia is not present, isoproterenol administration is recommended in critically ill neonates with anatomically mild Ebstein's anomaly and no associated cardiac defects.
Ebstein"s anomaly is the most common congenital heart disease associated with the Wolf-Parkinson-White syndrome. Between November 1973 and March 1993, we surgically treated 42 patients with Wolff-Parkinson-White syndrome and Ebstein's anomaly. The patient's ages ranged from 5 months to 59 years (mean 35.3 +/- 14.0 years). There were a total of 52 accessory pathways, 48 of which were located in the right (65%) or posteroseptal (29%) area. A left-sided accessory pathway was seen in only two patients (3.8%). Division of all right-sided accessory pathways was done during normothermic cardiopulmonary bypass with the heart beating; cryocoagulation was applied together with scalpel dissection of the atrioventricular groove. Division of the left-sided accessory pathways was done with the use cold potassium cardioplegic arrest. Thirty-five of these patients underwent tricuspid valve operation for Ebstein's anomaly and 11 of them underwent tricuspid valve replacement with a bioprosthesis. All 52 accessory pathways were successfully divided, although two patients required reoperation because of tachycardia caused by accessory pathways in different positions. Three hospital deaths (7.1%) occurred. There were no late deaths during the follow-up period (mean 94.3 +/- 52.4 months), but two patients required repeat tricuspid operation because of progression of the tricuspid regurgitation. Because no repeat operations were required during long-term follow-up patients who underwent valve repair or valve replacement, correction should be indicated in some patients.
Ebstein's anomaly is an uncommon congenital cardiac defect which is associated with cyanosis and arrhythmias. There have been very few previous reported cases of successful outcome in pregnancy in women with this disorder. We describe the successful analgesic management of an obstetric patient who had been known to have Ebstein's anomaly since childhood. Her first pregnancy was uneventful and analgesia during labour was provided by an epidural. During her second pregnancy she presented to our hospital as her condition had deteriorated. Symptomatic control was achieved with digoxin. Despite this, several episodes of hospitalization were needed pre-partum for rest and oxygen therapy. After the onset of spontaneous labour, analgesia was managed by an epidural using bupivacaine. Invasive monitoring was not deemed appropriate due to increased risk and questionable usefulness. Vaginal delivery was managed with elective lift-out forceps to minimize the stress of pushing. When reviewed two months post-partum she still required digoxin although her symptoms had improved considerably. The successful management of Ebstein's anomaly in pregnancy should include team management from early in pregnancy.
Explore the source record for details and available documents.
Ebstein's anomaly is a complex malformation that has been treated by various surgical techniques, with variable results, since 1958. In 1972, the authors developed a repair that consisted of plication of the free wall of the atrialized portion of the right ventricle, posterior tricuspid anuloplasty, and right atrial reduction. 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. Not all patients are candidates for this procedure, however, because of significant abnormalities of the anterior leaflet such as inadequate size or attachment of the free edge of the leaflet to the ventricular wall. This report describes a ten-year experience with surgical repair of Ebstein's anomaly.
Ebstein's anomaly is a rare congenital malformation of the tricuspid valve, often associated with an atrial septal defect. Death occurs usually from cardiac arrhythmias. The successful use of a two-catheter technique for elective Caesarean section with extradural analgesia is described and the hazards associated with Ebstein's anomaly in pregnancy and anaesthesia are discussed.
Ebstein's anomaly of the tricuspid valve has a known association with the Wolff-Parkinson-White syndrome. Radiofrequency ablation has become the treatment of choice for the latter, but there have been no reports of the feasibility of this procedure after tricuspid valve replacement. We present a patient who first exhibited evidence of intermittent preexcitation and associated symptomatic arrhythmia 9 years after tricuspid valve replacement for Ebstein's anomaly, and describe the challenges posed by this case.
Ebstein's anomaly is characterized by abnormalities both in the basal and in the free attachments of the leaflets of the tricuspid valve. Fairly frequently the developmental defect is associated with a WPW syndrome (Type B). The main obstetric problems arise from tachyarrhythmias. Since tachycardias in these patients are due to physical stress, correct planning of delivery calls for lightening of the hemodynamic burden of labor, careful analysis of the cardiac status, meticulous preparation for optimum antiarrhythmic therapy and correct timing for induction of labor. The case of a young primipara with Ebstein's anomaly is presented to typify the need for cooperation of subspecialities.
The newborn reported here presented with congestive heart failure and cyanosis on the first day of life. Echocardiographic examination revealed complete atrioventricular septal defect and Ebstein anomaly, a rare combination that has not been previously reported in the literature.
The surgical prognosis of Ebstein's anomaly depends on the quality of tricuspid valve repair and right ventricular function. In patients with right ventricular failure, a decrease in afterload was attained by a cavo-bipulmonary anastomosis associated with the intraventricular repair. Fifty-nine out of 111 patients operated for Ebstein's anomaly were considered to be at high risk and were selected for this study. The inclusion criteria were one or more of the following factors: massive tricuspid regurgitation, extensive atrialisation of the right ventricle, poor right ventricular function, chronic atrial fibrillation. This population was divided into two groups with the same preoperative features: Group I (45 operated patients: tricuspid valvuloplasty with longitudinal plicature of the right ventricule: Group II (14 operated patients): same intracardiac repair as Group I and associated cavo-bipulmonary anastomosis. The operative mortality was 24% (11/45) in Group I and 7% (1/14) in Group II (p < 0.05). The 5 year actuarial was 68.6% in Group I and 61.8% in Group II (NS). The reoperation rate was 11% (5/45) in Group I and 0% in Group II. In Group II, the persistence of significant tricuspid regurgitation was better tolerated and the frequence of reoperation was decreased with respect to Group I. The authors conclude that high risk patients with Ebstein's anomaly have a lower operative mortality and improved functional tolerance when there is persistent tricuspid regurgitation after cavo-bipulmonary anastomosis.
Pulmonary atresia with intact ventricular septum is a disorder that involves the whole right ventricle. An associated Ebstein deformity of the tricuspid valve is found in 10% of the cases, further complicating the anatomy and the function of the right ventricle. From January 1966 to December 1990, pulmonary atresia, intact ventricular septum, and Ebstein deformity of the tricuspid valve were observed in 11 cases in our institution; four of them were necropsy findings and the remaining seven were patients treated surgically. Of the latter, two were boys and five were girls (age range 1 day to 18 days). Two patients were managed by pulmonary valvotomy, three by systemic-pulmonary artery shunt, and two had a combination of the two. Operative mortality was 57%. Eight hearts were available for morphologic study. All had viscero-atrial situs solitus, D-loop ventricular structure, and normally related great arteries. A correlation between the degree of tricuspid valve dysplasia and right ventricular cavity size was observed in all. Furthermore, in five cases the anterior leaflet of the tricuspid valve obstructed the right ventricle at the ostium infundibuli level. In two of our surgical patients, a protruding anterior tricuspid valve leaflet was identified and excised and both patients survived. Ebstein anomaly of the tricuspid valve further complicates surgical management and outcome of pulmonary atresia and intact ventricular septum. Potential obstruction at the ostium infundibuli level should always be considered during repair. Various degrees of right ventricular inlet dysplasia, always present in this complex, may contraindicate an anatomic correction. In these cases a modified Fontan-type procedure should be considered as a valid surgical option.