Case Report

A 21 - year- old female was admitted to our hospital with exertional dyspnea. Echocardiographic evaluation revealed Ebstein's anomaly with a severe tricuspid regurgitation(Figure 1), right atrial dilatation, a mild mitral regurgitation.No pericardial effusion was observed.

Figure 1

Figure 1

The localization of the tricuspid valve was identified as 38 mm superior to the mitral valve (Figure 2).

Figure 2

Figure 2

Mean pulmonary arterial pressure was measured as 40-45 mm Hg.

She was operated under endotracheal general anesthesia and in supine position.Following a median sternotomy,pericardium was opened longitudinally. After heparinization, extra-corporeal circulation was established between the venae cavae and the ascending aorta. A cross clamp was placed on aorta and by antegrade intermittent isothermic blood cardioplegia from aortic root,cardiac arrest was established. Standard right atriotomy was made. A tricuspid valvular structure consistent with the Ebstein anomaly and an atrialized ventricular segment was explored (Figure 3).

Figure 3

Figure 3

Atrialized segment was suspended with pledgeted U sutures (Figure 4)

Figure 4

Figure 4

Patent foramen ovale, which was revealed perioperatively, was closed primarily. After tricuspid valve repair, a 34 mm Kalangos Biodegradable Tricuspid Ring (Kalangos Biodegradable Tricuspid Ring, Bioring SA, Lonay, Switzerland,TRK 34A/173-015) was inserted for annuloplasty (Figure 5).

Figure 5

Figure 5

Incision of right atriotomy was closed. The post-operative course was uneventful with successful correction.. Postoperative echocardiographic data confirmed complete removal of the lesion and showed a significant reduction of the right atrial diameter(Figure 6).

Figure 6

Figure 6

Comments

Ebstein's anomaly is a rare congenital cardiac disease initially described by Wilhelm Ebstein in 1866(₁). Ebstein's anomaly 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(₂). Ebstein's malformation can be defined as an anomaly of the tricuspid valve existing in the setting of a right ventricular dysfunction(₃). Ebstein's anomaly occurs approximately once in 20,000 live births and only 5% of patients survive beyond the fifth decade(₁).

Echocardiography is a useful diagnostic tool in detecting Ebstein's anomaly(₄).But 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(₅). 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(₅).

In the study of Fang et al. echocardiographic features of 8 patients with Ebstein's anomaly with two-dimensional echocardiogram revealed: apical displacement of septal tricuspid leaflet (10-43 mm) in all cases (100%), elongated anterior tricuspid leaflet with whip-like motion in 7 patients (88%), tethering of septal tricuspid leaflet in 1 case (13%)(₄).

Operation includes tricuspid valve repair or replacement, closure of any interatrial communications, and appropriate antiarrhythmia procedures(₂). Surgical repair of Ebstein's anomaly improves functional class and exercise tolerance, eliminates right-to-left intracardiac shunting (if present), and reduces the incidence of supraventricular tachyarrhythmias(₆).

In the study of Szentkirályi et al., 16 patients with Ebstein anomaly were operated. In 15 cases atrial septal defect occurred as a concomitant anomaly, which was closed by direct suture in 9 cases and with patch in 6 cases(₇).

In the study of Brown et al., 539 patients with Ebstein anomaly underwent 604 cardiac operations at their institution. The mean age of the patients was 24 years and 53% were female patients. Survival at 5, 10, 15, and 20 years was 94%, 90%, 86%, and 76%, respectively. Survival free of late reoperation was 86%, 74%, 62%, and 46% at 5, 10, 15, and 20 years, respectively. Two hundred thirty-seven (83%) patients were in New York Heart Association functional class I or II, and 34% were taking no cardiac medication. The recurrence of congenital heart disease was reported in 9 of 232 (3.9%) liveborn children(₈).

Patients have good long-term survival and functional outcomes after undergoing surgery for Ebstein anomaly(₈). In the current era, overall early mortality after surgical repair in children and adults has fallen to less than 3% in experienced centers(6). Risk factors for poorer outcome included right, and/or left ventricular systolic dysfunction; increased hemoglobin/hematocrit values; male sex; right ventricular outflow tract obstruction; or hypoplastic pulmonary arteries(₉).

Moderate-to-severe functional tricuspid regurgitation (TR) should be corrected in patients undergoing surgery for left-sided valvular diseases, to improve long-term outcomes. Several techniques of surgical repair (suture annuloplasty or prosthetic annuloplasty) to correct this condition have been described. Multiple clinical studies have shown the superiority of prosthetic remodeling annuloplasty over the other surgical approaches. Despite this, suture-based annuloplasty remains the most commonly used technique for tricuspid valve repair(₁₀). The Kalangos Biodegradable Tricuspid Ring (Kalangos Biodegradable Tricuspid Ring, Bioring SA, Lonay, Switzerland) is a novel prosthesis for the treatment of tricuspid insufficiency(₁₁).In conclusion of this study; Kalangos Biodegradable Ring is a promising prosthesis in patients with functional tricuspid insufficiency, with encouraging initial results(₁₁).However, the concept of annulus remodeling using a biodegradable ring which preserves the growth potential of the native annulus opens new perspectives for valve repair procedures(₁₂).

Correspondence to

Doç. Dr. Ufuk YETKIN, 1379 Sok. No: 9,Burç Apt. D: 13 - 35220, Alsancak – IZMIR / TURKEY Tel: +90 505 3124906 , Fax: +90 232 2434848 e-mail: ufuk_yetkin@yahoo.fr