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.     

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.     

Ebstein's anomaly of the tricuspid valve: the cone repair

Ebstein's anomaly is a cardiac malformation affecting the tricuspid valve and right ventricle with a wide range of anatomic and pathophysiologic presentations. The cone repair technique is described in detail, as applied to several types of this complex congenital heart disease. The importance of extensive mobilization of displaced and tethered tricuspid leaflets to permit a good leaflet-to-leaflet coaptation after the cone construction is highlighted. This technique was performed in 100 patients with a hospital mortality rate of 3.0%, good clinical outcome, and no need for tricuspid valve replacement. Echocardiograph results showed good anatomic and functional tricuspid valves at immediate and long-term postoperative follow-up.     

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.     

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.     

Ebstein's anomaly appearing in the neonate. A new surgical approach

Ebstein's anomaly appearing during the neonatal period carries a high mortality rate. These infants exhibit cyanosis, acidosis, and congestive heart failure. The pathophysiologic characteristics consist of severe tricuspid regurgitation and functional pulmonary atresia. As a result of the inability of the right ventricle to generate forward flow through the pulmonary arteries, these infants remain dependent on ductal patency. Since May 1988, five newborn infants with severe Ebstein's anomaly have been admitted for treatment at our institution. At initial examination, they weighed 3.6 +/- 1.8 kg and had a mean oxygen tension of 29.6 +/- 2.3 mm Hg and a mean pH of 7.20 +/- 0.05. Chest roentgenography demonstrated a mean cardiothoracic ratio of 0.81 +/- 0.02. As determined by echocardiography, the right atria were massively enlarged, severe tricuspid regurgitation was present in all patients, and the pulmonary valves were not opening. All infants were dependent on prostaglandin E1 and attempts to wean them from this drug were unsuccessful. Palliative treatment consisted of tricuspid closure with autologous pericardium and an aortopulmonary shunt of 4 mm polytetrafluoroethylene tubing. There were no operative or late deaths. At discharge, mean oxygen tension was 42.2 +/- 0.85 mm Hg and mean systemic oxygen saturation was 83.2% +/- 1.94%. Infants have grown satisfactorily during the follow-up period. Three infants have since returned for further surgical intervention. One infant, at 11 months of age, underwent a Glenn anastomosis for progressive oxygen desaturation. Two infants have returned, at ages 23 and 22 months, for Fontan procedures, which represent their definitive operative management. We believe this new procedure offers excellent palliative treatment for Ebstein's anomaly in critically ill neonates. Feasibility of later definitive correction is demonstrated by the good results obtained with the Fontan procedure in two infants.     

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.     

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.     

Modified technique for the surgical treatment of severe tricuspid valve deformity in Ebstein's anomaly

Distortion of septal and atrial anatomy in Ebstein's anomaly places the atrioventricular node and His bundle at risk for injury at operation. We present a novel technique for creating a robust neo-annulus, remote from conduction tissue, adding to the armamentarium of techniques available for the reconstruction or replacement of the tricuspid valve. Three cases are described. All the patients were in sinus rhythm, with competent native or bioprosthetic valves, at short-term follow-up.     

Oblique plication for repair of the atrialized ventricle and tricuspid incompetence of Ebstein's anomaly.

An 8-year-old girl who weighed 42 kg presented Ebstein's anomaly with severe tricuspid incompetence, and mild systemic cyanosis during exercise. A new reconstructive procedure for this complex anomaly was used. Oblique transference of the displaced posterior leaflet was performed, which resulted in plication of the atrialized ventricle and reduction in the tricuspid annular diameter. This procedure requires neither detachment nor closure of the tricuspid valve.     

Biventricular repair of Ebstein's anomaly with pulmonary atresia in a low birth weight neonate

Cyanotic neonates with Ebstein's anomaly associated with pulmonary atresia are a subgroup with the high mortality rate in pediatric cardiac surgery. We report a case of a severely symptomatic low birth weight neonate with Ebstein's anomaly and pulmonary atresia. A biventricular repair with Danielson-type tricuspid valve repair was performed. The baby tolerated the procedure with adequate hemodynamics, but died of peritoneal hemorrhage on the second postoperative day. This report could be an addition to the recent literature regarding the feasibility of biventricular repair for symptomatic neonates with Ebstein's anomaly even in low birth weight neonates.     

Ebstein's anomaly of the tricuspid valve in combination of tetralogy of Fallot: total correction in infancy

Ebstein's anomaly of the tricuspid valve in combination with Tetralogy of Fallot is extremely rare. We report a patient with these combined entities in a baby presented with cyanosis and right heart failure soon after birth. Total correction toward biventricular repair was successfully conducted when he was 7 months old. For management of a patient with such rare complex anomalies, we recommend Carpentier's technique for Ebstein's anomaly to restore the morphology of the right ventricle, and "one and half ventricle repair" can be a salvage procedure if the right ventricle is not functionally tolerant.