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Ebstein Anomaly (Ebsteins Anomaly)

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A congenital heart defect characterized by downward or apical displacement of the TRICUSPID VALVE, usually with the septal and posterior leaflets being attached to the wall of the RIGHT VENTRICLE. It is characterized by a huge RIGHT ATRIUM and a small and less effective right ventricle.

Also Known As:

Ebsteins Anomaly; Ebstein's Anomaly; Anomaly, Ebstein; Anomaly, Ebstein's

Networked: 113 relevant articles (5 outcomes, 5 trials/studies) for this Disease, Comments

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Atrial Heart Septal Defects (Atrial Septal Defect)
2.	Tachycardia (Tachyarrhythmias)
3.	Pulmonary Atresia (Pulmonary Valve Atresia)
4.	Tricuspid Atresia
5.	Pulmonary Valve Stenosis (Pulmonary Stenosis)

Experts

1.	Watanabe, Taiju: 1 article (02/2010)
2.	Shirai, Toshizumi: 1 article (02/2010)
3.	Oshima, Nagahisa: 1 article (02/2010)
4.	Makita, Satoru: 1 article (02/2010)
5.	Wottke, Michael: 1 article (01/2010)
6.	Schreiber, Christian: 1 article (01/2010)
7.	Ruzicka, Daniel Junichiro: 1 article (01/2010)
8.	Lange, Rüdiger: 1 article (01/2010)
9.	Krane, Markus: 1 article (01/2010)
10.	Hörer, Jürgen: 1 article (01/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Ebstein Anomaly:

1.	PlasticsIBA 08/01/1975 - "Three patients with Ebstein anomaly were severely disabled but have improved after plastic repairs, although mild tricuspid insufficiency persists. " 10/01/2000 - "METHODS: From January 1990 to August 1999 48 patients underwent surgical repair of Ebstein's anomaly by various plastic techniques. " 12/01/1990 - "[Reconstructive plastic correction of Ebstein's anomaly]" 10/01/1982 - "Tricuspid valve replacement and various types of plastic reconstruction are the two methods of treating Ebstein's anomaly. " 10/01/2000 - "Surgical treatment of Wolf-Parkinson-White syndrome during plastic operations in patients with Ebstein's anomaly." Order ALL the reference details at left...
2.	OxygenIBA 04/01/1988 - "Exercise tolerance, as defined by maximal oxygen uptake, was significantly greater in patients after operation for Ebstein's anomaly than in the preoperative patients. " 04/01/1988 - "Definitive operation (tricuspid valvuloplasty or replacement and atrial septal defect closure) for Ebstein's anomaly results in significant improvement of exercise tolerance, normalization of systemic arterial oxygen saturation and reduction of excess ventilation at rest and during exercise." 01/01/1995 - "For chronic fetal hypoxia due to maternal Ebstein's anomaly, oxygen was administered daily to the mother by mask for 105 days. " 01/01/1995 - "Pregnancy complicated by Ebstein's anomaly: oxygen administration to mother for chronic fetal hypoxemia. " 08/01/1989 - "The cardiac defects that best responded to PGE1 were pulmonary atresia with or without ventricular septal defect, Ebstein's anomaly, tricuspid atresia, pulmonic stenosis, double outlet right ventricle, and those that the arterial oxygen saturation increase was less than 10 vol. " Order ALL the reference details at left...
3.	One-Step dentin bonding system (One-Step)IBA 10/01/2005 - "OBJECTIVE: To evaluate efficacy of one-step and two-step surgery approach in patients with arrhythmias combined with Ebstein's anomaly. " Order ALL the reference details at left...
4.	LithiumIBA 01/01/2005 - "A profound ultrasound screening of the organs should be performed in case of lithium exposition, whereas newer studies showed no increase in risk for Ebstein anomaly. " 01/01/1989 - "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. " 03/01/2009 - "The risk of Ebstein's anomaly associated with lithium use is increased relative to the general population. " 01/01/2008 - "In the retrospective studies there were, in the Lithium Baby Registry, 225 registered cases with 25 anomalies, 18 of them being cardiac, of which six had Ebstein's anomaly. " 01/01/2007 - "Although first trimester lithium use is associated with Ebstein's anomaly, the risk was overestimated in the past. " Order ALL the reference details at left...
5.	tranilast (N 5')IBA 11/01/1989 - "To determine which morphologic features are associated with early death, the complete echocardiograms and medical records of 16 consecutive patients with Ebstein's anomaly and concordant atrioventricular connections who presented in the fetal (n = 5) or neonatal (n = 11) period were reviewed. " 09/15/1993 - "Since 1990, radiofrequency ablation of accessory atrioventricular (AV) pathways has been attempted in 10 patients (age range 3.5 months to 30 years) with congenital heart disease: Ebstein's anomaly (n = 5), heterotaxy with AV discordance (n = 3), tetralogy of Fallot (n = 1) and total anomalous pulmonary venous return (n = 1). " 07/01/2009 - "Thirty-six patients (78%) had associated cardiac lesions, including atrial septal defect (n = 16 [35%]), ventricular septal defect (n = 17 [37%]), patent ductus arteriosus (n = 14 [30%]), and Ebstein's anomaly (n = 5 [11%]). " 04/01/1999 - "Diagnoses included pulmonary atresia with intact ventricular septum (n = 15), Ebstein anomaly (n = 5), levotransposition of the great arteries (n = 3), pulmonary stenosis with right ventricular hypoplasia (n = 2), tetralogy of Fallot (n = 3), dextrotransposition of the great arteries (n = l), and Uhl anomaly (n = l). " 07/01/1996 - "1994 50 neonates underwent cardiac surgery using CPB (n = 23 transposition of the great arteries; n = 4 pulmonary atresia; n = 6 critical pulmonary stenosis; n = 5 hypoplastic left heart syndrome; n = 3 Ebstein's anomaly; n = 2 interrupted arch with hypoplastic left ventricle; n = 2 single ventricle; n = 1 each: double outlet right ventricle, tricuspid atresia, critical aortic stenosis, rhabdo-myosarkoma, corrected transposition of the great arteries.) Thirty-one patients entered the study. " Order ALL the reference details at left...
6.	Phenobarbital (Luminal)FDA Link 12/01/1975 - "Electrophysiologic characteristics of five patients with Ebstein's anomaly of the tricuspid valve were defined with studies using luminal intracardiac electrode catheters. " Order ALL the reference details at left...
7.	Nitric Oxide (Nitrogen Monoxide)FDA Link 11/01/1998 - "The successful use of nitric oxide (NO) inhalation in a critically ill neonate with Ebstein's anomaly of the tricuspid valve is reported for the first time. " 11/01/1998 - "Inhaled nitric oxide therapy in a critically ill neonate with Ebstein's anomaly." 04/01/2003 - "Diagnostic and therapeutic uses of inhaled nitric oxide in neonatal Ebstein's anomaly." Order ALL the reference details at left...
8.	Alprostadil (Muse)FDA LinkGeneric 03/01/1995 - "A diagnosis of Ebstein anomaly was made, and intravenous PGE1 was started to keep patent the ductus arteriosus. " 08/01/1989 - "The cardiac defects that best responded to PGE1 were pulmonary atresia with or without ventricular septal defect, Ebstein's anomaly, tricuspid atresia, pulmonic stenosis, double outlet right ventricle, and those that the arterial oxygen saturation increase was less than 10 vol. " Order ALL the reference details at left...
9.	sildenafil (Viagra)FDA Link 02/01/2008 - "Sildenafil use in a symptomatic neonate with severe Ebstein's anomaly of the tricuspid valve." 09/01/2006 - "A novel approach incorporating sildenafil in the management of symptomatic neonates with Ebstein's anomaly." Order ALL the reference details at left...
10.	Therapeutic UsesIBA 04/01/2003 - "Diagnostic and therapeutic uses of inhaled nitric oxide in neonatal Ebstein's anomaly." Order ALL the reference details at left...

Therapies and Procedures

1.	Bioprosthesis 01/01/1999 - "Seven of the surviving patients are in functional class I, while one patient is in class II. This experience suggests that TVR with bioprosthesis is a good therapeutical option for children with Ebstein's anomaly since the operative risk is low, the functional status improved in all patients and the durability of bioprosthesis in tricuspid position has been good." 11/01/1998 - "CONCLUSIONS: Bioprosthesis durability in the tricuspid position in patients with Ebstein's anomaly compares very favorably with bioprosthesis durability in other cardiac valve positions, especially for pediatric patients, and also compares favorably with tricuspid bioprosthesis durability in patients with other diagnoses." 11/01/1998 - "However, our early experience with patients having Ebstein's anomaly suggests that tricuspid bioprostheses in this anomaly might have better durability. " 05/01/1998 - "Glisson's capsule was also used for tricuspid valve reconstruction and as a bioprosthesis in six patients with Ebstein's anomaly. " 12/01/1995 - "Thirty-five of these patients underwent tricuspid valve operation for Ebstein's anomaly and 11 of them underwent tricuspid valve replacement with a bioprosthesis. " Order ALL the reference details at left...
2.	Cryosurgery 11/01/1991 - "Bidirectional bundle branch reentry tachycardia associated with Ebstein's anomaly: cured by extensive cryoablation of the right bundle branch." 11/01/1993 - "In a patient with the Wolff-Parkinson-White syndrome, Ebstein's anomaly of the tricuspid valve, a right atrial Chiari net and a patent foramen ovale two unusual complications developed after surgical epicardial dissection combined with cryoablation of the anomalous pathway. " Order ALL the reference details at left...
3.	Fontan Procedure (Fontan Operation) 02/01/2001 - "There was no significant decline, however, in Ebstein's anomaly (p = 0.270), Fontan procedure (p = 0.182), and in the Mustard patients (p = 0.188). " 05/01/2000 - "CONCLUSION: Adjustable interatrial communication for the patients undergoing modified Fontan procedure or correction of Ebstein's anomaly may be advantageous." 10/01/1995 - "Modified Fontan procedure for Ebstein's anomaly of the tricuspid valve--an alternative surgical approach preserving Ebstein's anatomy." 01/01/1998 - "Study patients comprised three groups: eight patients with congestive heart failure (CHF) due to Ebstein's anomaly or single-ventricle heart after Fontan operation; six patients with complete transposition of the great arteries (TGA) after an anatomic surgical correction who may be candidates for ischemic heart disease; and five age-matched normal subjects. " 02/01/2001 - "Patients from 6 major diagnostic groups were identified, including patients with atrial septal defect (ASD, n = 93), transposition of the great arteries corrected with the Mustard procedure (n = 84), congenitally corrected transposition of the great arteries (CCTGA, n = 41), Tetralogy of Fallot (n = 168), Ebstein's anomaly (n = 37), and Modified Fontan procedure (n = 52). " Order ALL the reference details at left...
4.	Electrodes (Electrode) 10/01/1970 - "The use of an electrode catheter in the diagnosis of Ebstein's anomaly of the heart." 03/01/1966 - "Electrode catheters and the diagnosis of Ebstein's anomaly of the tricuspid valve." 12/01/1975 - "Electrophysiologic characteristics of five patients with Ebstein's anomaly of the tricuspid valve were defined with studies using luminal intracardiac electrode catheters. " Order ALL the reference details at left...
5.	Prostheses and Implants (Prosthesis) 11/01/1988 - "Tricuspid valve replacement for Ebstein's anomaly in childhood with a Starr-Edwards caged-ball prosthesis. " 05/01/1985 - "[Late results of tricuspid valve replacement with biological prostheses in Ebstein's anomaly]" 11/01/1984 - "[Radical surgical correction of Ebstein's anomaly; experience with 54 tricuspid valve prostheses]" 09/01/1984 - "[Case of successful replacement of an earlier implanted xenoaortic prosthesis in Ebstein's anomaly]" 01/01/1973 - "Post superior vena cava-right pulmonary artery shunt; total surgical correction of Ebstein's anomaly with Starr-Edwards prosthesis." Order ALL the reference details at left...

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