Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Ebstein Anomaly”

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.

At least 37 records · Page 2Linked to original sources
PubMed2001

[Methods of myocardium defense in radical correction of Ebstein anomaly].

Experience of myocardium defence while correcting Ebstein anomaly in 75 patients was analyzed. In 25 patients antegrade pharmacorefrigerating crystalloid cardioplegia based on potassium chloride was applied, in 10--method of Kirsch using miganesiun-aspartate-procaine solution. In 40 patients antegrade pumping of normothermic potassium chloride solution with subsequent antegrade infusion of cooled magnesium-aspartate-procaine solution applied for primary heart arrest. Application of combined defence of myocardium using normothermal crystalloid cardioplegia and refrigerating, of antegrade infusion of magnesium-asparate-procaine cardioplegic solution has advantages in comparison with another methods of myocardium defence, reduces the threat of an acute cardiac insufficiency occurrence in early postoperative period.

Child↗
PubMed2004

Atrioventricular nodal re-entrant tachycardia in a patient with Ebstein anomaly.

The first case of successful slow pathway modulation is reported in a patient with Ebstein anomaly and recurrent atrioventricular nodal re-entrant tachycardia. Typical signals were recorded during electrophysiological study at the slow pathway region between the His bundle and the coronary sinus where ablation was performed successfully. Thus, slow pathway modulation seems to be a safe procedure even in selected patients with Ebstein anomaly.

Adult↗
PubMed2004

Early and medium-term results for repair of Ebstein anomaly.

OBJECTIVES: We evaluated the early and medium-term single-center results for primary repair of Ebstein anomaly in both adults and children. METHODS: The records were reviewed of patients undergoing repair of Ebstein anomaly at the Children's Hospital of New York from September 1990 to September 2002. Functional, demographic, and echocardiographic parameters were studied both preoperatively and postoperatively, along with functional status and adverse events. The repair technique involved vertical plication of the atrialized ventricle and valve leaflet reimplantation after clockwise rotation. RESULTS: A total of 25 patients (19 children and 6 adults) underwent repair. The average age was 14.2 +/- 15.9 years, and the average follow-up was 4.1 +/- 3.4 years. Three patients required reoperation for right ventricular overload (1 child) and progressive, severe tricuspid regurgitation (2 adults); both adults received tricuspid valve replacements, one at 4 years and the other at 8 years post-repair. Three patients had radiofrequency ablation procedures performed intraoperatively. Ten patients (40%) had moderate-to-severe tricuspid regurgitation perioperatively. However, 18 children (95%) and 5 adults (83%) demonstrated significant improvement in exercise capacity late postoperatively. Two children died suddenly 11 months and 4 years after repair. DISCUSSION: Ebstein repair has good functional outcomes in children despite residual tricuspid regurgitation, likely because of reduction in right ventricular volume loading and relative annular and ventricular plasticity. Adult patients did not demonstrate the same durability of valve repair and frequently required tricuspid valve replacement. Intraoperative radiofrequency ablation represents an important adjunctive treatment for intractable arrhythmias, which may now represent relative indications for operative intervention.

Adolescent↗
PubMed2006

Results of surgery for Ebstein anomaly: a multicenter study from the European Congenital Heart Surgeons Association.

OBJECTIVE: Since most centers' experience with Ebstein anomaly is limited, we sought to analyze the collective experience of participating institutions of the European Congenital Heart Surgeons Association with surgery for this rare malformation. METHODS: The records of all 150 patients (median age 6.4 years) who underwent surgery for Ebstein anomaly in the 13 participating Association centers between January 1992 and January 2005 were reviewed retrospectively. Patients with congenitally corrected transposition were excluded. RESULTS: Most patients (81%) had Ebstein disease type B or C and significant functional impairment (61% in New York Heart Association class III or IV) and 16% had prior operations. Surgical procedures (n = 179) included valve replacement (n = 60, 33.5%), valve repair (n = 49, 27.3%), 1(1/2) ventricle repair (n = 46, 25.6%), palliative shunt (n = 13, 7.26%), and other complex procedures (n = 11, 6.14%). There were 20 hospital deaths (operative mortality 13.3%) after valve replacement in 5 patients, valve repair in 3, 1(1/2) ventricle repair in 7, palliative procedures in 3, and miscellaneous procedures in 2. Younger age and palliative procedures were univariate risk factors for operative death, but only age was an independent predictor on multivariable analysis. CONCLUSIONS: Most patients coming to surgery presented in childhood and were significantly symptomatic. More than half underwent valve replacement or repair, but a considerable proportion had severe disease necessitating 1(1/2) ventricle repair or palliative procedures. Operative mortality did not differ significantly among repair, replacement, and 1(1/2) ventricle repair but was associated with palliative procedures for severe disease early in life, young age being the only independent predictor of operative death.

Adolescent↗
PubMed2005

Pseudo-Meigs' syndrome caused by retroperitoneal tumor in a patient with Ebstein anomaly.

We report an extremely rare case of pseudo-Meigs' syndrome caused by a retroperitoneal tumor in a patient with Ebstein anomaly. A 60-year-old woman was admitted because of marked hydrothorax and ascites. She also had pleural effusion. Because echocardiography revealed Ebstein anomaly, medical treatment was performed, under the diagnosis of heart failure. However, her pleural effusion and ascites did not completely disappear. Computed tomography (CT) scan showed a round solid retroperitoneal pelvic mass, with a diameter of approximately 5 cm in the vesicouterine fossa. After resection of the tumor, which was histologically found to be fibroma, her pleural effusion and ascites disappeared.

Ebstein Anomaly↗
PubMed1988

MR imaging of Ebstein anomaly: results in four cases.

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.

Adolescent↗
PubMed2004

Ebstein anomaly associated with unusual conduction delay and sick sinus syndrome.

A 64-year-old man was admitted to our hospital because of palpitation, dyspnea on effort, and facial edema. The echocardiographic diagnosis was Ebstein anomaly. Although the 12-lead electrocardiogram showed an atrial rate of 150 beats/min and no typical flutter wave, the electrophysiological study showed counterclockwise rotation of excitation along the tricuspid annulus. Because of sinus arrest and syncope, a permanent pacemaker was implanted, but the right atrium was not captured by electrical stimulation at 5 V/0.4 ms, except for the orifice of coronary sinus, and the intracardiac P wave was only 0.2 mV or less. This is a rare case of Ebstein anomaly characterized by unusually prolonged conduction in the atrium, the basis of which was global myocardial damage, including the ventricles.

Ebstein Anomaly↗
PubMedDate not supplied

Prenatal diagnosis of Down's syndrome in the presence of isolated Ebstein's anomaly.

Ebstein's anomaly is a rare congenital cardiac defect, characterized by the displacement of the tricuspid valve into the right ventricle, that occurs approximately once in 20,000 live births. The association of Ebstein's anomaly and chromosomal abnormalities, such as Down's syndrome, is extremely unusual. Prenatal diagnosis of trisomy 21 in a fetus with isolated Ebstein's anomaly has not been previously reported.

Abortion, Eugenic↗
PubMed1998

Nongeometric quantitative assessment of right and left ventricular function: myocardial performance index in normal children and patients with Ebstein anomaly.

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.

Adolescent↗
PubMed1997

Management of functional pulmonary atresia with isoproterenol in a neonate with Ebstein's anomaly.

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 Anomaly↗
PubMed1995

Surgical treatment of patients with Wolff-Parkinson-White syndrome and associated Ebstein's anomaly.

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.

Adult↗
PubMed1995

Epidural analgesia for labour in a patient with Ebstein's anomaly.

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.

Adult↗
PubMed1982

Surgical repair of Ebstein's anomaly.

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.

Adolescent↗
PubMed2004

The use of the Doppler pulmonary artery velocity time integral to optimize placement of a ventricular pacing lead in a patient with Ebstein's anomaly.

Ebstein's anomaly is a rare congenital heart defect. Patients with severe symptomatic tricuspid regurgitation requiring surgical correction often have conduction system disease. We present a case of a 14 year-old girl with Ebstein's malformation and bioprosthetic tricuspid valve who required permanent pacing for symptomatic bradycardia. The placement of the right ventricular pacing lead was facilitated by the use of the Doppler pulmonary artery velocity time integral as a surrogate for stroke volume. This case demonstrates the importance of site-specific pacing and the utility of Doppler echocardiography to optimize lead placement and cardiac performance in patients with Ebstein's anomaly and advanced conduction system disease.

Adolescent↗
PubMed1984

Caesarean section under extradural analgesia in a patient with 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.

Adult↗