Mimics of Ebstein's anomaly

The purpose of this study was to determine the most discriminating clinical and echocardiographic features that are most helpful in correctly identifying Ebstein's anomaly of the tricuspid valve from other causes of tricuspid regurgitation. Ebstein's anomaly is an uncommon malformation of the tricuspid valve with diagnostic echocardiographic features. Other cardiac disorders associated with tricuspid valve regurgitation and predominate right-sided heart chamber enlargement can be misdiagnosed as Ebstein's anomaly. All patients who were referred to our institution between 1982 and 1995 with the diagnosis of Ebstein's anomaly but were found to have other abnormalities of the tricuspid value or right ventricle were identified. Their clinical, echocardiographic, and surgical records were reviewed retrospectively. Twenty-two patients (12 males and 10 females), aged 7 to 68 years (mean 33 years), were referred to our institution with the diagnosis of Ebstein's anomaly but were found to have another abnormality that mimicked clinical and diagnostic features of Ebstein's anomaly. The most common initial symptom was exercise intolerance (13 [59%] patients) followed by atrial arrhythmia (seven [32%] patients). Two patients had cyanosis. Three patients had paroxysmal and six had chronic atrial fibrillation/flutter. Cardiomegaly on chest x-ray film was noted in 18 (82%) patients. Referral diagnosis of Ebstein's anomaly had been made by echocardiography (12 patients), cardiac catheterization (four patients), both techniques (five patients), and echocardiography and magnetic resonance imaging (one patient). All 22 patients had predominate right atrial and right ventricular enlargement, and 18 (82%) of 22 patients also had right ventricular dysfunction. However, Ebstein's anomaly was confidently ruled out with repeat comprehensive echocardiography at our institution by establishing (1) absence of significant apical displacement of the septal tricuspid valve leaflet (> or = 8 mm/m2) and (2) lack of a redundant, elongated, anterior tricuspid valve leaflet in all 22 patients (100%). All had significant tricuspid regurgitation caused by tricuspid valve dysplasia (nine patients), tricuspid valve prolapse (four patients), trauma (four patients), right ventricular dysplasia (three patients), endocarditis (one patient), and annular dilation caused by free pulmonary regurgitation (one patient). In all 15 patients who subsequently underwent surgery (tricuspid valve repair [seven patients] or replacement [eight patients]), the absence of Ebstein's anomaly was confirmed. Echocardiographic absence of the characteristic degree of displacement of the septal leaflet of the tricuspid valve (> or = 8 mm/m2) and the presence of a nonelongated, nonredundant anterior tricuspid valve leaflet consistently excluded the diagnosis of Ebstein's anomaly. Under such circumstances, other anomalies of the tricuspid valve or right ventricle were consistently identified. Recognition of the mimics of Ebstein's anomaly had important surgical implications.     

A modified repair technique for tricuspid incompetence in Ebstein's anomaly

OBJECTIVE: A modified technique for tricuspid valve repair in Ebstein's anomaly restructures the valve mechanism at the level of the true tricuspid anulus by using the most mobile leaflet for valve closure without plication of the atrialized chamber. Midterm results of this therapeutic approach for patients with Ebstein's anomaly and tricuspid valve incompetence are reported. METHODS: Between October 1988 and April 1997, the incompetent tricuspid valve was repaired with our technique in 19 patients (12 female, 7 male; 2 to 54 years, mean 21 years). The indication for operation was congestive heart failure of various degrees in all patients. Tricuspid incompetence was grade II in two patients, grade III in 14, and grade IV in three. Associated congenital malformations were simultaneously repaired (interatrial communication in 18, ventricular septal defect in two, pulmonary stenosis in two, mitral valve prolapse in one). Follow-up ranged between 10 and 103 months (median 28 months) and was complete for all patients. RESULTS: There were no operative deaths. One patient with active endocarditis and pulmonary abscess died 2 months after the operation of recurrent sepsis; there were no late deaths. During follow-up, New York Heart Association functional class improved from 2.8 before the operation to 1.9 without recurrent cyanosis, and tricuspid incompetence decreased from a mean grade of 3.1 to one of 0.9, without any echocardiographic deterioration of the tricuspid valve function or right ventricular dilation. CONCLUSIONS: Our technique allows tricuspid valve repair in patients with Ebstein's anomaly, even in cases usually reserved for primary valve replacement, without late functional deterioration.     

Gender differences in human sepsis

Severe tricuspid valve regurgitation and decreased pulmonary blood flow in neonatal Ebstein's anomaly with pulmonary atresia, may result in cardiac respiratory failure and hypoxemia. The poor natural course and lack of standard surgical treatment make treatment for neonatal Ebstein's anomaly very difficult. The Blalock-Taussig operation was performed for hypoxemia in Ebstein's anomaly associated with pulmonary atresia and severe tricuspid regurgitation on 36th day after birth. On the 63rd day after birth, tricuspid valve orifice closure and right atrium plication (Starnes procedure) were accomplished under cardiopulmonary bypass. Heart failure became controllable and the patient condition was satisfactory at one year after surgery.     

Neutrophil (granulocyte) transfusions in the new millennium

BACKGROUND: Ebstein anomaly is a rare tricuspid valve anomaly. Some fetuses with Ebstein's anomaly have concurrent severe cardiac function impairment, which results in hydrops fetalis. Most of these fetuses are inevitably terminally ill. No reports have demonstrated the potential prenatal therapy for fetuses under such conditions. CASE: Ebstein's anomaly and hydrops fetalis were detected at 28 weeks' gestation. Tricuspid regurgitation with congestive heart failure was observed. From 28 to 34 weeks' gestation, intrauterine therapy with digoxin, 0.75 mg/d, was administered. The fetal hydrops status improved gradually, while the tricuspid valve regurgitation persisted. At 36 weeks' gestation the fetus was delivered normally. During the neonatal phase, digoxin was continued and gradually tapered off. The tricuspid valve regurgitation and cardiomegaly gradually improved. CONCLUSION: The favorable outcome in this case supports the positive effect of prenatal digoxin therapy for Ebstein's anomaly with hydrops fetalis. In such conditions, upon the appearance of hydrops and congestive cardiac failure, immediate digoxin therapy may be useful. This successful trial encouraged us to manage such fetuses more aggressively.     

Pericardial patch augmentation of anterior tricuspid leaflet in Ebstein's anomaly

Although refinement of tricuspid valvuloplasty and plication techniques has opened the way to a satisfactory outlook for the majority of patients with Ebstein's anomaly, paucity of tissue in the anterior tricuspid leaflet may preclude successful valve repair. In this particular circumstance, a modified Carpentier repair of the tricuspid valve with additional pericardial patch augmentation of the anterior leaflet may result in a well-functioning monocusp tricuspid valve.     

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.     

Morphologic spectrum of Ebstein's malformation: revisitation relative to surgical repair

OBJECTIVE: Our aim was to elucidate the morphologic spectrum of Ebstein's malformation of the tricuspid valve with regard to diagnosis and the feasibility of surgical repair, in the light of the currently favored reconstructive techniques. METHODS: We examined 23 autopsied hearts. Taking the displacement of the septal and mural leaflets of the abnormal tricuspid valve as our diagnostic criterion, we focused subsequently on the location of the distorted valvular orifice and the attachment and formation of the anterosuperior leaflet. We also assessed the dimensions of the components of the right ventricle relative to the plane of the displaced valvular orifice. RESULTS: In all hearts, the septal and mural leaflets were hinged at various points within the inlet of the right ventricle. In many cases, however, these leaflets were virtually absent. The plane of the effective tricuspid valvular orifice was displaced anterosuperiorly to varying degrees. In the most severe forms, the valvular mechanism took the form of a 1-leaflet valve. The length of the functional right ventricle when compared with the left ventricle ranged proportionally from 0.6 to 1. 1 (mean, 0.9). CONCLUSIONS: Ebstein's malformation is much more than simple "downward displacement" of the leaflets. In essence, the valvular orifice is formed within the ventricular cavity at the junction of the atrialized inlet and functional ventricular components. When surgical intervention becomes necessary, it is essential to make a detailed assessment of both valvular and ventricular abnormalities.     

Successful radiofrequency current catheter ablation of accessory atrioventricular pathway after tricuspid replacement in Ebstein's anomaly

A 15-year-old female with Ebstein's anomaly was referred to hospital for radiofrequency (RF) current catheter ablation of her refractory paroxysmal supraventricular tachycardia (PSVT) after tricuspid valve replacement. A surface ECG showed ventricular preexcitation of type B Wolff-Parkinson-White (WPW) syndrome. In a baseline electrophysiological study, two types of PSVT with left and right bundle branch block (LBBB and RBBB) configurations were induced. The LBBB type was antidromic and the RBBB type was orthodromic atrioventricular reciprocating tachycardia (AVRT) with a right posterolateral accessory pathway. RF current was successfully delivered at the posterolateral site above the prosthetic valve (V-delta interval = -30 msec). The patient has been free from arrhythmias during a follow-up period of 9 months. RF current ablation seems to be useful for AVRT patients with corrected Ebstein's anomaly.     

Evolution of mammalian HNRPG and its relationship with the putative azoospermia factor RBM

Inadvertent disruption of tricuspid valve apposition by a pacing catheter in a neonate is described. Severe cyanosis due to atrial level shunting resulted, similar to the pathophysiologic state seen in Ebstein's anomaly.     

Ruptured chordae tendineae of the tricuspid valve due to nonpenetrating trauma. Echocardiographic findings

A case of traumatic tricuspid insufficiency is presented. In the diagnosis of ruptured chordae tendineae of the tricuspid valve, two-dimensional echocardiography was an essential diagnostic procedure, while M-mode echocardiography showed no specific findings. Ruptured chordae tendineae of the tricuspid valve were confirmed at operation.     

Conversion of complex neonatal Ebstein's anomaly into functional tricuspid or pulmonary atresia

BACKGROUND: Ebstein's anomaly, due to failure of delamination of one or more leaflets of the tricuspid valve (TV), is associated with varying degrees of tricuspid regurgitation (TR) and dysplasia of the right ventricle (RV). Although refinement of tricuspid valvuloplasty and plication techniques have opened the way to a satisfactory outlook for the majority of older children and adults, Ebstein's anomaly presenting at neonatal age, secondary to ineffective forward flow into the pulmonary and systemic circulation, has a reported mortality rate of as high as 75%. In order to improve the dismal outcome in neonatal Ebstein's anomaly, we have strived for early univentricular palliation. PATIENTS AND METHODS: Univentricular repair was performed in five neonates (median age 5 days; range 2-14 days) with Ebstein's anomaly, ductal dependent pulmonary blood flow, severe TR, absence of forward flow across the pulmonary valve, and small left ventricular (LV) area due to right-to-left bowing of the ventricular septum and ineffective LV loading (median indexed LV area 10.5 cm2/m2). In addition, two neonates had moderate pulmonary regurgitation (PR), one with additional pulmonary stenosis. In all patients, the indexed area of the combined right atrium and atrialized RV was greater than that of the combined functional RV, left atrium, and left ventricle (median 22.0 and 20.8 cm2/m2, respectively). The median preoperative systemic oxygen tension was 35 mmHg and the median pH 7.28. Repair consisted of TV closure with a pericardial patch (with the coronary sinus draining into the RV) (n = 3) or, in the presence of PR, resection of the dysplastic TV and division and oversewing of the main pulmonary artery (n = 2), as well as excision of the atrial septum, resection of redundant right atrial wall, and construction of an aortopulmonary shunt (n = 5). RESULTS: The median indexed LV area increased from 10.5 to 18.8 cm2/m2 as a result of more effective loading of the left ventricle. There was no intraoperative or late mortality. The patients were extubated at a median of 7 days postoperatively. At discharge, the median systemic oxygen tension was 46 mmHg. In all five patients, at 6, 7, 10, 12 and 16 weeks of age, a bidirectional cavopulmonary anastomosis has been constructed. CONCLUSIONS: In neonates with Ebstein's anomaly and ductal dependent pulmonary blood flow, rational palliation consists of the surgical creation of tricuspid atresia or, in the additional presence of PR or pulmonary stenosis, the creation of pulmonary atresia. These procedures may result in effective LV decompression and more effective volume loading of the left ventricle with increase of systemic output and improved clinical outcome.     

Atresia of the right atrioventricular orifice

The results are reported of a study of 83 necropsied hearts with atresia of the right atrioventricular orifice. It is emphasised that right atrial or atrioventricular orificial atresia is a better term to describe this anomaly than "tricuspid atresia". Use of the latter term can be confusing when the morphologically tricuspid valve is located beneath the left atrium. It is accepted that the definition employed may include cases in which the mitral valve may be atretic, blocking normal exit from the right atrium, but it is argued that such cases would present clinically as "tricuspid atresia" and therefore are correctly designated as right atrial orificial atresia. The results show that the majority of hearts with right atrial orificial atresia have the ventricular morphology of primitive ventricle, most with, but a few without an outlet chamber. However, in a minority of hearts an imperforate membrane interposes between the right atrium and a formed but hypoplastic right ventricle. In two of the hearts, the imperforate membrane showed features of Ebstein's malformation. The hearts could be further subdivided according to the ventriculoarterial connection. Most had normally connected arteries (66 of 83), and all but 2 also had normal relations between the arteries; in these 2 hearts there was "anatomically corrected malposition". Twelve hearts showed transposition, one had double-outlet outlet chamber, and another persistent truncus arteriosus. The remaining 3 hearts, all without outlet chamber, had by definition a double outlet connection. A segmental approach provides the best way of classifying this anomaly, and an embryological explanation is offered for the variations in anatomy observed.     

Development of animal models for the chronobiotics of melatonin analogs

We reviewed the records of patients admitted to our centre with the diagnosis of isolated tricuspid valve infective endocarditis and analysed the clinical presentation, etiopathogenic agent, echocardiographic features and therapeutic approach, namely the indication for cardiac surgery. Between 1988 and 1996, 11 cases of confirmed tricuspid valve endocarditis were identified, corresponding to 5% of the cases of endocarditis admitted to our centre in the same period. A predisposing factor was found in ten of the patients, half of them intravenous drug addicts and Staphylococcus aureus was the most frequent agent isolated. Fever and pleuro-pulmonary manifestations were predominant clinical features. Transthoracic echocardiography had a crucial role in the diagnosis and transesophageal echocardiography was important to characterize vegetations. Four patients underwent cardiac surgery, for persistent infection. In two cases, excision of the vegetations and ring annuloplasty was performed. In two patients not addicted to drugs, the tricuspid valve was replaced with a bioprosthesis, since the extension of the damage to the valve did not allow repair. One patient, with early endocarditis of a tricuspid bioprosthesis died before surgery was attempted.     

Insertion of prosthetic tricuspid valve two years after the tricuspid valvulectomy

A case of a 14-year-old boy who underwent a prosthetic tricuspid valve insertion two years after the tricuspid valvulectomy due to intractable right-sided active endocarditis is presented. At the initial operation, the tricuspid valve was thoroughly resected because of marked destruction and attachment of vegetations, and a concomitant ventricular septal defect was directly closed. When a prosthetic valve was placed in the tricuspid portion, a semicircular Dacron patch was attached to the right side of the interventricular septum in a fashion of up-chord and down-arc to cover the bundle of His. The straight edge of the patch was located near the AV node and was not sutured to the tricuspid valve annulus. A 31 mm of CarboMedics prosthetic valve was sutured to the tricuspid valve annulus and to the free edge of the patch at the position near the AV node to prevent AV block. Postoperative course was uneventful and ECG showed regular sinus rhythm.     

Case of MPO-ANCA positive interstitial pneumonitis and necrotizing, crescentic glomerulonephritis

A 56-year-old female who was diagnosed incomplete endocardial cushion defect, underwent closure of ostium primum defect and repair of mitral cleft 2 years ago. Mitral and tricuspid ring annuloplasty was done 9 months after the first operation because of an increment of mitral and tricuspid valve regurgitation. The Third operation, mitral valve replacement by mechanical valve and tricuspid valve replacement by bioprosthetic valve, was performed because of the gradually increased mitral stenosis and tricuspid regurgitation. Post-operative course was uneventful. Resected anterior cusp of mitral and tricuspid valve revealed hypertrophy and shortness macroscopically, and revealed fibrosis and calcification histopathologically. It was considered that remarkable regurgitation was due to secondary change of both valves. We concluded that valve replacement should be performed for case of secondary change of atrioventricular valve in adult patient.     

Refractory head and neck pain. A difficult problem and a new alternative therapy

Mitral valve repair was performed in six patients by transferring the posterior tricuspid leaflet with its sub-valvular apparatus onto the mitral valve. This new technique considers the tricuspid valve as the patients own tissue bank where the posterior leaflet and eventually the adjacent part of the anterior leaflet is used as a "donor" valve, based on the knowledge that the right atrio-ventricular valve can be efficiently repaired with a very low risk of significant dysfunction. The mitral repair consists of incorporating the tricuspid autograft by securing the tricuspid papillary muscle to the mitral papillary muscle and by suturing the leaflet tissue where required. A mitral annuloplasty ring reinforces the repair. The tricuspid valve is subsequently repaired by annular plication and leaflet suture. A tricuspid ring is necessary to maintain efficient remodeling. The six patients ages ranged from 20 to 70 years. A etiology, was rheumatic in the first case and degenerative in the following. In three cases, sterilised endocarditis was responsible for ruptured chordae and leaflet destruction. The mitral insufficiency was located in a commissural area in 4 cases, and was due to a widespread posterior prolapse in 2. Post-operative control transesophageal echocardiography confirmed the excellent results of the repair and proved that, in selected cases, the tricuspid leaflet inserted onto the mitral apparatus is very efficient in correcting mitral insufficiency, without causing significant tricuspid impairment. With a 3 to 7 month follow-up, the results are stable.     

Ebstein's anomaly (morphologic and morphometric study

12 hearts with Ebstein's anomaly were studied. This disease is a combined anomaly of heart formation with dysplasia of the folds of the tricuspid valve (TV), its tendinous chordas and papillary muscles, dysplasia of the trabecular part of the right ventricle and dysplastic interrelationships between TV and the right ventricle: attachment by short chordas of posterior and septal folds of TV with the right ventricle walls, anomalous distal attachment of the anterior fold of TV. "Atrialisation" of the right ventricle is the reflection of the folds dysplasia the extreme form of which is the loss of communication between TV and trabecular part and formation of the direct communication between TV and the infundibular part of the right ventricle.     

Endocarditis of the tricuspid valve associated with congenital coronary arteriovenous fistula

A 17 year old girl with a congenital right coronary artery to coronary sinus fistula presented with recurrent septic pulmonary embolism secondary to tricuspid valve endocarditis. The diagnosis was made on the basis of echocardiography and cardiac angiography.     

The Pediatric Radiofrequency Ablation Registry's experience with Ebstein's anomaly. Pediatric Electrophysiology Society

INTRODUCTION: Abnormal anatomy and complex electrophysiology in patients with Ebstein's anomaly of the tricuspid valve may confound attempts at radiofrequency ablation (RFA). METHODS AND RESULTS: Data for 65 pediatric Ebstein's patients (9.8+/-5.4 years, 4 months to 20 years; 39+/-25 kg, 5.1 to 108 kg) were obtained from the Pediatric Radiofrequency Ablation Registry. The degree of tricuspid regurgitation (DOTR) and the degree of Ebstein's anomaly were assessed with echocardiography/Doppler. Leading indications were drug refractoriness (24 [37%] of 65 patients) and life-threatening arrhythmia (14 [22%] of 65 patients). For the 65 patients, 82 typical (nondecremental) accessory pathways (APs) (62% right free wall, 34% right septal, and 4% left sided), 17 other supraventricular tachycardias (1 ectopic atrial, 7 AV reentry, 5 Mahaim, and 4 intra-atrial reentry tachycardias), and 1 ventricular mechanism were mapped. Thirty-four (52%) of 65 patients had a single AP (21 right free wall, 10 septal, and 3 left); 19 (29%) of 65 patients multiple APs; 6 (9%) of 65 patients a single AP plus a non-AP mechanism; and 6 (9%) of 65 patients non-AP mechanism(s) only. RFA acute success rates and recurrence rates for right free wall, right septal, and other mechanisms were 79%/32%, 89%/29%, and 75%/27%. Mild DOTR and a body surface area (BSA) < or = 1.7 m2 independently predicted a better acute success rate. BSA < or = 1.7 m2 also predicted long-term success. CONCLUSION: In this patient subset, life-threatening arrhythmias and multiple electrophysiologic mechanisms are commonly encountered during RFA. Mild DOTR and a BSA < or = 1.7 m2 predict a higher acute success rate. While acute success rates are relatively high, recurrence is frequent.