Posterior annular plication: tricuspid valve repair in Ebstein's anomaly

BACKGROUND: Nonneonatal Ebstein's anomaly is a rare congenital anomaly for which numerous operative techniques have been described to repair the aberrant tricuspid valve, exclude the atrialized right ventricle, and address right heart dysfunction. We reviewed short-term outcomes in surgical patients treated by a simplified operative technique. METHODS: Our operative approach to Ebstein's anomaly is approximation of the anteroposterior commissure to the remnant septal leaflet with closure of the cul-de-sac longitudinally. Plication of the atrialized right ventricle (1 patient) and a bidirectional cavopulmonary connection (2 patients) were performed only if necessary. All patients were followed postoperatively by their cardiac surgeon and cardiologist. RESULTS: Seven patients with a mean age of 39 years (range, 3.6 to 63.8 years) underwent repair. Preoperatively all patients had 4+ tricuspid valve regurgitation and were New York Heart Association class III. Mean postoperative hospital stay was 7 +/- 2 days (range, 4 to 11 days). Mean follow-up is 42 +/- 18 months (range, 7 to 58 months). At last follow-up 5 patients are New York Heart Association class I and average tricuspid valve regurgitation is mild. CONCLUSIONS: Plication of the posterior annulus without plication of the atrialized right ventricle, resiting the tricuspid valve, or performing prophylactic cavopulmonary connection appears to be a reasonable operative approach to nonneonatal Ebstein's anomaly. Long-term follow-up of this cohort is necessary to determine the durability of such a surgical approach.     

Surgical management of Ebstein's anomaly in the adult

Ebstein's anomaly is a rare cardiac malformation that affects the tricuspid valve, right ventricle, and right atrioventricular junction. These anatomical and functional abnormalities cause important tricuspid regurgitation that results in right atrial and right ventricular dilatation and atrial and ventricular arrhythmias. Diagnosis is made by echocardiography. Operation includes tricuspid valve repair or replacement, closure of any interatrial communications, and appropriate antiarrhythmia procedures. Repair of Ebstein's anomaly eliminates right-to-left intracardiac shunting, improves exercise tolerance and functional class, and reduces supraventricular arrhythmias. In addition, quality of life and longevity are improved.     

A successful case of tricuspid valvulectomy for Ebstein's anomaly with severe tricuspid stenosis]

A 44-year-old male was admitted to our hospital complaining of general fatigue and dyspnea on exertion with congestive heart failure (NYHA IV). Cardiac catheterization and angiogram revealed Ebstein's anomaly with severe tricuspid stenosis and functioning small right ventricle. Because of tiny right ventricular chamber, the usual surgical treatment for Ebstein's anomaly i.e. tricuspid valve replacement or valvuloplasty were deemed to be inappropriate. Tricuspid valvulectomy was performed to relieve the inflow stenosis of the right ventricle and excellent symptomatic relief was achieved. It is very rare to seen an adult case of Ebstein's anomaly with severe tricuspid stenosis and functioning small right ventricle. Tricuspid valvulectomy may be justified to use in this rare condition.     

Repair of Ebstein's anomaly in an elderly patient; report of a case

The operative repair of Ebstein's anomaly is performed usually during the younger age. On the other hand, the operative indication of asymptomatic Ebstein's anomaly in adult patients has not been clearly defined. We encountered a 71-year-old female patient with asymptomatic Ebstein's anomaly. Because of severe tricuspid regurgitation (TR) and right ventricular dilatation, we repaired the tricuspid valve configuration. The operation was successful and medium term result was excellent. We believe that severe TR with moderate right ventricular dysfunction can be the operative indication in adult patients with asymptomatic Ebstein's anomaly especially when tricuspid valve repair is possible.     

Management of Ebstein's anomaly and pure tricuspid insufficiency in the neonate

Because the pulmonary vascular resistance is very elevated at birth, severe tricuspid regurgitation is poorly tolerated and even life-threatening in the newborn. The etiology may be tricuspid valve papillary muscle rupture or the more ominous Ebstein's anomaly, with its associated dysfunctional right ventricle. After the diagnosis is established and the patient is supported with prostaglandin infusion and nitric oxide, definitive surgical management is undertaken with the expectation of excellent outcomes for isolated tricuspid valve regurgitation. For neonates with Ebstein's anomaly, therapy is tailored to the severity of the malformation and the degree of right ventricular outflow tract obstruction, assessed in the context of declining pulmonary vascular resistance. The surgical approach may involve ligation of a patent ductus arteriosus, placement of a systemic to pulmonary shunt, establishment of functional tricuspid atresia, or tricuspid valve repair. With the application of these various approaches, the outlook for neonatal Ebstein's anomaly has improved remarkably.     

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.     

A case report of Ebstein's anomaly treated with Carpentier's procedure

A 42-year-old male patient was admitted with congestive heart failure. Echocardiogram and cardiac angiogram revealed Ebstein's anomaly and severe tricuspid valve incompetence. He underwent Carpentier's procedure for the treatment of Ebstein's anomaly. Carpentier's procedure consists of the following two new points; (1) the right atrium and atrialized ventricle is plicated longitudinally; (2) the anterior leaflet and the posterior leaflet of tricuspid valve are transposed with a clockwise rotation to the level of the normal tricuspid anulus. Compared with Hardy's procedure (transverse plication), longitudinal plication preserves the cavity and the function of the right ventricle and excludes atrialized chamber. In our case, there is a trivial residual regurgitation of tricuspid valve postoperatively, but the patient's clinical status has improved remarkably. We conclude that Carpentier's procedure is an effective operation for Ebstein's anomaly.     

Evidence-based case report for analgesic and anesthetic management of a parturient with Ebstein's Anomaly and Wolff-Parkinson-White syndrome

The rare congenital heart defect of Ebstein's anomaly is characterized by downward displacement and elongation of the septal cusp of tricuspid valve. As a result, it is often associated with a thin-walled and poorly contractile right ventricle, an enlarged atrium, tricuspid regurgitation, atrial septal defect with intracardiac shunt, pulmonary hypertension and tachyarrhythmia. Published reports or studies on analgesic and/or anesthetic management of laboring parturients with Ebstein's anomaly are limited. We present an evidence-based case report of the successful management of an obese laboring parturient with Ebstein's anomaly and Wolff-Parkinson-White syndrome under epidural analgesia. We reviewed the literature and present the best evidence available or its lack of, and the reasoning in supporting the choice of the analgesic and anesthetic management.     

Repair of septal and posterior tricuspid leaflets in Ebstein's anomaly

BACKGROUND AND AIM: In Ebstein's anomaly, the septal and posterior tricuspid leaflets are plastered to the endocardium. We postulated that tricuspid valve function could be corrected by restoring mobility of these leaflets. (Feasibility of such repair was explored by anatomical and clinical studies.) METHODS: Ten heart specimens with Ebstein's anomaly were examined to investigate the size of the tricuspid leaflets. We operated on four patients with Ebstein's anomaly: the plastered septal and posterior leaflets were mobilized from the endocardium, the atrialized right ventricle was longitudinally plicated, and the basal attachment of the mobilized leaflets was sutured (reattached) to the valve annulus. RESULTS: In heart specimens, approximately 40% of the total surface of the tricuspid leaflets was comprised of the septal and posterior leaflets. Clinically, all patients operated on returned to normal functional status after surgery. The mean cardiothoracic ratio on chest X-rays decreased from 0.70 to 0.55 (after surgery). Echocardiographic tricuspid regurgitation, graded from 0 to 4, decreased from 3.5 to 1.0, and tricuspid annular diameter ratio to the normal value reduced from 1.88 to 0.66. Angiographic right ventricular ejection fraction increased from 0.36 to 0.50, and end-diastolic volume ratio to the normal value decreased from 3.65 to 1.19. CONCLUSIONS: Repair of the septal and posterior tricuspid leaflets was found to be feasible and effective as tricuspid valvuloplasty for Ebstein's anomaly.     

Valve replacement for Ebstein's anomaly of the tricuspid valve.

The role of valve replacement in the treatment of Ebstein's anomaly of the tricuspid valve remains controversial. Between 1965 and 1977, five patients with Ebstein's anomaly ranging in age from 11 to 57 years (mean 29) underwent tricuspid valve replacement in our institution. All of the patients were cyanotic but one, three were in Functional Class III, and two were in Class IV (New York Heart Association classification). The valve was placed above the coronary sinus. Plication of the atrialized portion of right ventricle was required in only one patient. Four patients had additional closure of an atrial septal defect. There were no deaths. On follow-up (range 1 to 13 years), four patients are in Functional Class I and one is in Class II. None is cyanotic. Four patients are in sinus rhythm and one has a pacemaker because of postoperative complete heart block. We conclude that valve replacement and, when necessary, plication of the atrialized right ventricle produce excellent clinical improvement.     

A new reconstructive operation for Ebstein's anomaly of the tricuspid valve

Ebstein's anomaly is a complex malformation involving the tricuspid valve and the right ventricle. Various surgical techniques, either repair or replacement of the abnormal tricuspid valve, have been used with variable results. In an attempt to enlarge the indications of conservative procedures, we developed a technique of repair that comprises the reconstruction of a normally shaped right ventricle and the repositioning of the tricuspid valve at the normal level. In a series of 14 patients operated on between January 1980 and December 1986 in our institution, only one patient with an associated atrioventricular septal defect required a tricuspid valve replacement. All of the other patients, regardless of the complexity of the tricuspid malformation, were able to benefit from this conservative technique. There were two hospital deaths and no late deaths in this series. All surviving patients displayed a marked improvement over their preoperative status with regard to functional class (92% are in class I or II) and rhythm disturbances. Echocardiographic and Doppler studies demonstrated a normal shape of the right ventricle and good tricuspid valve function in all the patients but one.     

De Vega's tricuspid annuloplasty for Ebstein's anomaly

A case of Ebstein's anomaly as demonstrated by echocardiography and electrophysiological studies is discussed. In view of deterioration in tricuspid regurgitation and right ventricular dysfunction, successful surgical repair employing De Vega's tricuspid annuloplasty along with plication of the atrialised portion of the right ventricle is described. Intraoperative transesophageal echocardiography following the procedure revealed satisfactory repair and a significant decrease in tricuspid regurgitation. This simple technique appears to be effective in patients having anterior leaflet sufficiently large area and motion. Adequate long-term follow-up in a large series of patients is essential to confirm that it is also beneficial and durable.     

Tricuspid valve replacement in the true annulus of Ebstein's anomaly without involvement of atrioventricular conduction disturbance

Two female patients with Ebstein's anomaly, aged 12 and 45 years, underwent new procedures of tricuspid valve replacement in the true annulus without involvement of atrioventricular conduction disturbance. The first patient had a relatively small atrialized right ventricle. A Dacron patch was sutured on the atrialized portion from the right ventricular side. Buttress stitches were placed on the whole of the atrialized ventricle except at the part of the His' bundle. A porcine valve was sutured in the true annulus of the tricuspid valve and on the top of the patch. The second patient had a fairly large atrialized ventricle, and plication was made only in the inferior part of the atrialized ventricle form the coronary sinus. A porcine valve was sutured in the true tricuspid annulus except at the part of Koch's triangle, where the porcine valve was placed on the false annulus.     

Surgical treatment of Ebstein's anomaly: valve replacement and reconstruction

Because of the relative infrequency of Ebstein's anomaly the type of effective surgical treatment remains controversial. Various authors have reported different results depending upon the type of surgical treatment involved. In TYIH between 1974-1987, 30 cases with Ebstein's anomaly have been operated upon; 12 cases underwent surgical repair and in the remaining 18 cases a tricuspid valve replacement was performed. Four out of the 12 reconstruction cases remained on cardiopulmonary by-pass (CPB) because of the failure of the right ventricle and artificial valves were implanted in these cases. One of these patients died. In all, 3 patients died as a result of annuloplasty and the postoperative courses of these patients were eventful. Among the remaining 18 patients with artificial valve replacement only one patient died and the remainder of the tricuspid valve replacement group had an uneventful postoperative period.     

Surgical treatment of Wolf-Parkinson-White syndrome during plastic operations in patients with Ebstein's anomaly.

OBJECTIVE: Ebstein's anomaly is the most common pathology associated with the accessory conduction pathways. METHODS: From January 1990 to August 1999 48 patients underwent surgical repair of Ebstein's anomaly by various plastic techniques. The pathways were identified and characterized at preoperative electrophysiologic mapping in 17 (34.5%) patients. There were seven males and ten females. The patients age ranged 6-35 years (mean 12.7+/-2.1 years). Five patients were in NYHA class II and were in 12 in NYHA class III. The cardiothoracic ratio ranged from 0.59 to 0.69 (mean 0.65+/-0.08). Tachycardia was present in 15 patients with Wolf-Parkinson-White (WPW) syndrome. Atrial septal defect was present in 12 patients (70. 6%). Accessory conduction pathways were in the right posterior septal area in seven patients, in right posterior septal area and free wall in seven patients and in right free wall in three. The pathways were successfully ablated in all patients during cardiopulmonary bypass. RESULTS: There were no deaths, no relapses. Follow-up ranged from 4 months to 7 years (mean 4.7+/-1.1 years). There were no late deaths, but one patient required successful tricuspid valve replacement because of severe tricuspid insufficiency. At follow-up 57.8% of patients were in NYHA class I. CONCLUSIONS: Preoperative electrophysiologic study allows to identify the accessory conduction pathways in patients with Ebstein's anomaly. The combined approach of tricuspid valve repair and surgical ablation of accessory atrioventricular connections has been proved safe and effective. This operative procedure allows to improve functional results in patients with Ebstein's malformation and concomitant accessory conduction pathways.     

A case report of Ebstein's anomaly treated with Hetzer's procedure

A 27-year-old male who had been diagnosed with Ebstein's anomaly was admitted with uncontrollable congestive heart failure. The echocardiogram revealed severe tricuspid valve incompetence and the electrocardiogram showed atrial fibrillation. He underwent Hetzer's repair procedure for tricuspid valve incompetence and Minzioni's right atrial isolation technique to restore sinus rhythm. His congestive heart failure quickly disappeared and sinus rhythm was restored after operation. He was discharged 3 weeks postoperatively and remains well 22 months after his operation. Hetzer's 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. We conclude that Hetzer's procedure is an effective operation for Ebstein's anomaly.     

Tricuspid valve incompetence caused by an intracardiac needle-like foreign body

We report a rare case of tricuspid valve incompetence caused by a needle-like foreign body with an unknown route of invasion. A 55-year-old woman who had suffered from chronic heart failure for more than 25 years was diagnosed as having Ebstein's anomaly after a transthoracic echocardiogram with Doppler imaging showed severe tricuspid regurgitation and displacement of the septal leaflet toward the right ventricle inlet. Surgery revealed a foreign body attached to the tricuspid valve, causing regurgitation. Valve replacement was performed and the patient's condition improved remarkably thereafter.     

An unusual first presentation of Ebstein's anomaly in a 72-year-old patient

We report the unusual case of a 72-year-old woman with a primary presentation of right heart failure in the setting of recently diagnosed Ebstein's anomaly with concomitant atrial fibrillation (AF). The patient had New York Heart Association (NYHA) class III dyspnea for 12 months, with refractory right heart failure prior to undergoing surgical management in the form of a tricuspid annuloplasty ring and plication of the atrialized ventricle. The patient had an uneventful postoperative recovery and enjoyed an improvement in her exercise tolerance (NYHA class I) with minimal echocardiographic evidence of tricuspid regurgitation (TR) at the latest follow-up.