Systemic Atrioventricular Valve Replacement in Patients With Functional Single Ventricle.

To review the outcome after atrioventricular valve replacement in single ventricle patients. The medical records of 37 consecutive patients who underwent initial valve replacement between 2001 and 2016 were reviewed. Actuarial survival rates were 73%, 65%, and 59% at 1, 5, and 10 years, respectively. Multivariate analysis indicated body surface area <0.28 m2 (P = 0.007; hazard ratio, 31.1), preoperative inotropic support (P < 0.001; hazard ratio, 24.5), primary valve replacement (P = 0.044; hazard ratio, 6.1), oversized prosthesis (P = 0.001; hazard ratio, 14.5), and intra- or postoperative extracorporeal membrane oxygenation support (P < 0.001; hazard ratio, 53.2) were the risk factors for mortality. Cumulative incidences of redo replacement were 11%, 17%, and 17% at 1, 5, and 10 years, respectively. There was no risk factor for redo replacement. Among 11 patients undergoing valve replacement before or at the time of bidirectional cavopulmonary shunt, all 4 patients who reached Fontan completion survived. Among 13 patients undergoing valve replacement after bidirectional cavopulmonary shunt, only 3 patients reached Fontan completion. Among 11 patients undergoing valve replacement after Fontan completion, there were 3 operative mortalities and 2 late mortalities. For 7 of 8 hospital survivors, cardiac index improved from 2.2 L/min/m2 (interquartile range, 2.0-2.4) to 3.4 (2.8-3.7) (P = 0.002). Atrioventricular valve replacement was a reasonable choice of treatment for single ventricle patients. For small patients with impaired ventricular function who had no choice other than valve replacement, commercially available valves were oversized and outcomes remained poor.