ebsteins anomaly - Medical Dictionary

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ebsteins anomaly		Congenital downward displacement of the tricuspid valve with the septal and posterior leaflets being attached to the wall of the right ventricle. [ Articles \| Books \| Images \| Discussion groups ]

Additional comments : Free Full Text Thrombolytic treatment as first option in recurrent tricuspid prosthetic valve thrombosis and Ebstein's anomaly. J Pharm Pharm Sci. 2005 Aug 12;8(2):332-4. Ebstein's anomaly is the most frequent cause of congenital tricuspid regurgitation. The coexistence of a mechanical heart prosthesis in a low-pressure circuit and poor compliance in the anticoagulant therapy contributed decisively to the appearance of recurrent mechanical heart valve thrombosis in these patients. A 49 years old female patient is reported where thrombolytic therapy with recombinant Streptokinase (TT-rSK) was the first treatment choice in seven recurrent episodes of prosthetic valve thrombosis. Ebstein's anomaly - review of a multifaceted congenital cardiac condition Swiss Med Wkly. 2005 May 14;135(19-20):269-81.Click here to read Ebstein's anomaly (EA) is a rare but fascinating congenital heart disorder accounting for <1% of all congenital heart defects. Since its description in 1866, dramatic advances in diagnosis and therapy have been made. In this review, we describe current diagnostic criteria and classification, natural history, clinical features, and prognosis, typical echocardiographic features and pathologic findings, and the spectrum of associated cardiac malformations including left heart anomalies associated with EA. Differences between Ebstein-like changes associated with congenitally corrected transposition and EA are described. The spectrum of typical ECG and conduction system changes, arrhythmias including accessory pathways and ectopic atrial tachycardias related to EA are also reviewed. Differential diagnosis of EA is discussed including tricuspid valve dysplasia and prolapse as well as arrhythmogenic right ventricular cardiomyopathy. The review describes management options in EA including catheter interventions, indication for operation and surgical options including tricuspid valve repair and replacement. Overall, EA is a complex congenital anomaly with a broad pathologic-anatomical and clinical spectrum and no two patients are alike. Therefore, precise knowledge of the different anatomic and hemodynamic variables, associated malformations and management options are essential. Management of EA patients is complex. Thus it is important that these patients are regularly seen by a cardiologist with expertise in congenital heart disease. Ebstein's malformation with atrial septal defect, right cor triatriatum, and right overt accessory atrioventricular pathway Heart. 2005 Apr;91(4):e25. A 31 year old man was admitted to hospital for abrupt syncope. Two dimensional echocardiogram in the apical four chamber view showed Ebstein's malformation. This is the first documented case of Ebstein's malformation with atrial septal defect, right cor triatriatum, and a right overt accessory atrioventricular pathway. Atrioventricular nodal re-entrant tachycardia in a patient with Ebstein anomaly Heart. 2004 Sep;90(9):e58. 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. Ebstein anomaly associated with unusual conduction delay and sick sinus syndrome Circ J. 2004 Jul;68(7):719-21. 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. Images in cardiovascular medicine. Fetal progression of Ebstein's anomaly. Circulation. 2003 Sep 23;108(12):e86-7. Canine tricuspid valve malformation, a model of human Ebstein anomaly, maps to dog chromosome 9 J Med Genet. 2003 May;40(5):320-4. BACKGROUND: Ebstein anomaly of the tricuspid valve is a congenital cardiac malformation characterised by downward displacement of the attachment of the septal and posterior leaflets of the tricuspid valve. Canine tricuspid valve malformation (CTVM) is morphologically similar to Ebstein anomaly; familial occurrence of CTVM has been described. Several observations suggest a genetic cause but most cases appear to be sporadic. METHODS: Three purebred Labrador Retriever kindreds enriched for CTVM underwent clinical examination and echocardiography. DNA was extracted from whole blood. Genotyping was carried out using polymorphic repeat markers with an average spacing of 15 cM and polymorphic information content of 0.74. RESULTS: Pedigree analysis identified CTVM segregating as an autosomal dominant trait with reduced penetrance. Genome wide linkage analysis in one kindred identified a CTVM susceptibility locus on dog chromosome 9 (CFA9) with a maximum multipoint lod score of 3.33. The two additional kindreds showed a conserved disease haplotype. CONCLUSIONS: This study identifies a CTVM susceptibility locus on CFA9 and a founder effect in apparently unrelated Labrador Retriever kindreds. These results provide the basis for a positional candidate cloning effort to identify the CTVM disease gene. Identification of the CTVM gene will permit mutation screening of patients with Ebstein anomaly, which should provide additional insights into the genetic programmes of valve development. Ebstein's anomaly: one and a half ventricular repair Swiss Med Wkly. 2002 Aug 24;132(33-34):485-8. Patients with Ebstein's anomaly can present after childhood or adolescence with cyanosis, arrhythmias, severe right ventricular dysfunction and frequently with left ventricular dysfunction secondary to the prolonged cyanosis and to the right ventricular interference. At this point conventional repair is accompanied by elevated mortality and morbidity and poor functional results. We report our experience with three patients (8, 16 and 35 years of age) with Ebstein's anomaly, very dilated right atrium, severe tricuspid valve regurgitation (4/4), bi-directional shunt through an atrial septal defect and reduced left ventricular function (mean ejection fraction = 58%, mean shortening fraction = 25%). All underwent one and a half ventricular repair consisting of closure of the atrial septal defect, tricuspid repair with reduction of the atrialised portion of the right ventricle and end-to-side anastomosis of the superior vena cava to the right pulmonary artery. All patients survived, with a mean follow-up of 33 months. In all there was complete regression of the cyanosis and of the signs of heart failure. Postoperative echocardiography showed reduced degree of tricuspid regurgitation (2/4) and improvement of the left ventricular function (mean ejection fraction = 77%, mean shortening fraction = 40%). In patients with Ebstein's anomaly referred late for surgery with severely compromised right ventricular function or even with reduced biventricular function, the presence of a relatively hypoplastic and/or malfunctioning right ventricular chamber inadequate to sustain the entire systemic venous return but capable of managing part of the systemic venous return, permits a one and a half ventricular repair with good functional results. Ebstein's anomaly, atrial paralysis and atrio-ventricular block: an uncommon association Europace. 2002 Oct;4(4):451-4. We report here the case of a 22-year-old female patient with an incomplete Ebstein's anomaly, complete heart block and atrial standstill. Atrial paralysis associated with Ebstein's anomaly is the most important feature, since there is a report of familial Ebstein's anomaly associated with atrial standstill but isolated cases have not been described. The patient presented with atypical chest pain and a symptomatic bradycardia of 37 beats per minute. A VVIR pacemaker was implanted. She has subsequently been symptom free. Right atrial reduction for tachyarrhythmias in Ebstein's anomaly in infancy Tex Heart Inst J. 2001;28(4):297-300. A 20-month-old girl with Ebstein's anomaly developed supraventricular paroxysmal tachycardia, which seemed to be a result of the wall tension of the giant right atrium. Right atrial resection reduced the wall tension and overall dimensions of the right atrium and finally resolved the tachycardia and ectopic electrical conduction. Six-year follow-up electrocardiograms confirmed continuing normal sinus rhythm without occurrence of supraventricular paroxysmal tachycardia or other ectopic electrical activity The follow-up echocardiograms showed the size of the right atrium to be unchanged from the time of operation. There are few data in the available literature about performing right atriotomy to resolve a tachyarrhythmia associated with Ebstein's anomaly and none, to the best of our knowledge, about performing right atrial resection for this purpose. Ebstein's anomaly with imperforate tricuspid valve. Prenatal diagnosis Arq Bras Cardiol. 2000 Jul;75(1):59-64. Ebstein's anomaly is an uncommon congenital heart defect, with a prevalence of 0.3-0.5%. Its association with an imperforate tricuspid valve is an even more rare situation (less than 10% of cases). Prenatal diagnosis of this association by means of fetal echocardiography has not been reported. We describe here this association diagnosed before birth and confirmed after birth. The diagnostic potential and importance of fetal echocardiography during prenatal evaluation of cardiac malformations allows for adequate perinatal planning and management, with an obvious impact on morbidity and mortality. The atrioventricular junctions in Ebstein malformation Heart. 2000 Apr;83(4):444-9. OBJECTIVE: To review the anatomical structure of the right atrioventricular junction, including the specialised atrioventricular conduction system, in hearts with Ebstein's malformation, to identify potential substrates for the abnormalities in conduction. METHODS: Five heart specimens representing the morphological spectrum of Ebstein malformation were examined grossly and histologically. RESULTS: On the endocardial surface, the atrioventricular junction was marked by a faint line in two hearts, and by a small ridge in the other three. Analysis of the right parietal junction in four hearts revealed only two accessory muscular atrioventricular connections. A plane of fibrofatty tissue separated atrial from ventricular myocardium in the right parietal junction in all hearts. The compact atrioventricular node was closer to the coronary sinus than usual. Accessory nodoventricular connections were present in four hearts, while accessory fasciculo-ventricular connections were found in one. The right bundle branch was hypoplastic or absent in four hearts. CONCLUSIONS: In this small series, the parietal atrioventricular junction was better developed than previously thought. Structural abnormalities of the atrioventricular conduction system, however, were present. These may account for some of the conduction abnormalities frequently observed with the Ebstein malformation. Images in cardiovascular medicine. Ebstein's anomaly associated with an anomalous coronary artery, 2 myocardial bridges, and a mahaim fiber Circulation. 1999 Oct 19;100(16):1757-9. Management of functional pulmonary atresia with isoproterenol in a neonate with Ebstein's anomaly Tohoku J Exp Med. 1997 Apr;181(4):459-65. 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--an autopsy study of 28 cases J Postgrad Med. 1997 Jan-Mar;43(1):8-11. Twenty eight autopsy specimens of Ebstein's anomaly were studied in order to evaluate the morphologic features of the abnormal tricuspid valve. All cases showed marked dilatation of the original tricuspid annulus, a normally positioned anterior leaflet and variable downward displacement of the posterior and septal leaflets. Sixteen cases showed a very large anterior leaflet. All three leaflets showed dysplastic features and a wide range of anatomic abnormalities in the valve and valve apparatus. A thin walled atrialised right ventricle was present in nine cases. Associated cardiac anomalies were seen in 21 cases, the commonest being an atrial septal defect (17 cases). Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebstein's anomaly Circulation. 1996 Aug 1;94(3):376-83. BACKGROUND: In patients with Ebstein's anomaly, localization of accessory pathways (APs) may be impeded by abnormal local electrograms recorded along the atrialized right ventricle and by the presence of multiple APs. The impact of these factors on radiofrequency (RF) current catheter ablation of APs has not been evaluated yet. METHODS AND RESULTS: Twenty-one patients with Ebstein's anomaly and reentrant atrioventricular tachycardias underwent electrophysiological evaluation and subsequent attempts at RF catheter ablation. Thirty-four right-sided APs were found, with 30 located along the atrialized ventricle. Local electrograms in this region were normal in 10 patients but fragmented in 11. Fragmented electrograms prevented the clear distinction between atrial and ventricular activation potentials as well as the identification of AP potentials. Right coronary artery mapping was performed in 7 patients. Abolition of all 26 APs was achieved in the 10 patients with normal local electrograms and in 6 of 11 patients with abnormal electrograms. Right coronary artery mapping allowed AP localization and ablation in 5 patients. In the 5 patients with abnormal electrograms and a total of 8 APs, 6 APs could not be ablated. Unsuccessfully treated patients received antiarrhythmic drugs. During 22 +/- 12 months of follow-up, 5 patients had clinical recurrences, including 4 who had undergone a successful RF procedure. CONCLUSIONS: In patients with Ebstein's anomaly and reentrant atrioventricular tachycardias, factors likely to account for failure of RF catheter ablation include an AP located along the atrialized right ventricle and the abnormal morphology of endocardial activation potentials generated in this region. An infant case of Ebstein's anomaly of the tricuspid valve with abnormal diastolic filling pattern of the left ventricle. Tohoku J Exp Med. 1996 Jul;179(3):205-11. We report herein an infant case of Ebstein's anomaly with abnormal diastolic filling pattern of the left ventricle. We also compare the case with a comparable case without such abnormal filling pattern. The patient, who had pulmonary valve stenosis, patent ductus arteriosus and mild mitral regurgitation, underwent transventricular valvotomy at 30 days of age. Postoperative course was uneventful. Serial Doppler echocardiographic studies were performed at 2 hr, 40 days, and 52 days of age. Peak velocity and flow velocity-time integral of early diastole, and ratios of the peak velocities and velocity time integrals of the early diastole and atrial contraction were below the 95% confidence limits of the normal controls. Our case suggests that left ventricular diastolic dysfunction may exist in the Ebstein's anomaly when it is associated with mitral valve abnormality such as mitral regurgitation. Surgical repair of Ebstein's anomaly Ann Surg. 1982 Oct;196(4):499-504. 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.

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