Progressive atrioventricular valvular regurgitation in single ventricle.

The hemodynamic status of the patient with single ventricle is not static. This study describes the rapid development of mechanical malfunction of the atrioventricular (AV) valve in a subset of patients with single ventricle. Eighty patients with single ventricle were reviewed. Eight patients with single ventricle and single AV valve were observed to have moderate to severe AV valvular regurgitation. Patient age when AV regurgitation was first detected was 22 months to 35 years (median 15 years). Clinically, all patients were symptomatic and had severe congestive heart failure. All patients underwent cardiac catheterization, which revealed moderate to severe AV regurgitation; 7 of 8 patients had well preserved ventricular function. Earlier catheterization revealed no significant AV regurgitation. Four patients underwent surgery: 2 had AV valve replacement with the Fontan procedure and 2 had Carpentier ring placement (1 with the Fontan procedure). The 3 survivors were in improved clinical condition. At surgery 1 patient had an abnormal AV valve with 4 leaflets, and the other 3 had myxomatous degeneration of the AV valve. Thus, AV regurgitation is a significant and specific complication that occurs in patients with single ventricle and single AV valve. If recognized before development of eventual myocardial dysfunction, surgical management should be considered.