Lateral tunnel Fontan operation patients with atrioventricular valve (AVV) regurgitation have an increased incidence of Fontan failure (death, take-down or transplant). The outcomes of patients undergoing AVV repair during Fontan palliation were reviewed to determine the optimal technique and timing of repair.
Hospital records for all patients with AVV regurgitation at the time of their Fontan procedure were reviewed retrospectively. Patients with staged single-ventricle palliation culminating in a lateral tunnel Fontan operation who had their first AVV repair at the Children's Hospital, Boston, were included. AVV regurgitation was graded by semi-quantitative color Doppler echocardiography on a scale of 1 to 4, as was ventricular dysfunction.
Among 859 lateral tunnel Fontan patients, 27 (3%) had a total of 30 AVV repairs (18 tricuspid, six mitral, six common AVV). Of the 27 first-time AVV repairs (16 TV, six MV, five CAVV), six were performed pre-Fontan, and 21 at or after Fontan. The median age at the first AVV repair was 3 years (range: 0.6-9.4 years). Preoperatively, the median echocardiographic severity of AVV regurgitation was grade 3 (range: 2-4). At median follow up of 1.2 years (range: 0-9.5 years) the severity of AVV regurgitation was reduced significantly to median grade 2 (range: 0-4; p <0.001). No patient with initial AVV repair at the time of Fontan underwent reoperation for AVV regurgitation. In all cases, ventricular function was maintained or improved, with preoperative median systemic ventricular function grade 1.5 (range: 1 to 4) versus postoperative grade 1 (range: 1-4; p = NS). There were no Fontan failures in the intermediate term.
AVV regurgitation and ventricular systolic function can be maintained or improved in the intermediate term following AVV repair in single-ventricle patients.