Biventricular remodeling after cone repair in Ebstein anomaly: magnetic resonance imaging data analysis

Various surgical techniques have been reported for repairing Ebstein anomaly. Cone repair provides nearly anatomical tricuspid valve (TV) reconstruction with promising outcomes. We reviewed our experience with cone repair to evaluate biventricular remodeling and the outcomes of the annular support procedure.

Between January 2008 and December 2021, cone repair was performed in 33 consecutive patients with Ebstein anomaly. Mean age was 32.0±16.8 years (range, 1.1-66.8 years). Previous TV repair had been performed in two patients with the Hetzer and the Carpentier techniques (6%). Severe tricuspid regurgitation (TR) was observed in 32 patients (97%). Twenty patients had preoperative magnetic resonance imaging (MRI) data; 15 patients had postoperative MRI data.

Modifications included the addition of an annuloplasty band (21 patients with pericardial strips, two patients with prosthetic rings) in 23 patients (69.7%) and papillary muscle repositioning in one patient (3%). Bidirectional cavopulmonary anastomosis was performed in two patients (6%), with one of them undergoing a Fontan operation in the third postoperative years. No mortality was observed. The mean follow-up duration was 7.5±4.6 years. Two patients (6%) required late TV re-repair in the first and sixth postoperative years. At follow-up, five patients (16.1%) reported no or trivial TR, 16 (51.6%) had mild TR, and 5 (16.1%) had mild to moderate TR. Freedom from late TV reoperation was 78.8%±13.4% at 5 years. The TV reoperation rate was significantly low in the patients who underwent tricuspid annuloplasty with a band (P=0.02). Preoperative and postoperative MRI data demonstrated a significant right ventricular (RV) volume decrease after cone repair [RV end-diastolic volume index (mL/m2): preoperative/postoperative =207.4±40.2/105.5±41.3, P=0.001]. Left ventricular ejection fraction (LVEF) remained unchanged after cone repair, while left ventricular stroke volume (LVSV) significantly increased [LVEF (%): preoperative/postoperative =60.8±5.3/61.2±5.4, P=0.10; LVSV (mL): preoperative/postoperative =64.0±1.8/71.4±12.7, P=0.041].

Cone repair for Ebstein anomaly has low mortality and morbidity rates. The addition of an annuloplasty band was associated with a low incidence of the TV reoperation. Moreover, cone repair might impact left ventricular function due to ventricular interdependency. Longer follow-up is essential to determine the late durability of cone repair and both ventricular functional changes.