Case description
The patient was diagnosed with a single right ventricle and mitral atresia and received pulmonary artery banding (PAB) during the infantile period for reduction of pulmonary blood flow and ventricle preload. Moderate AVV regurgitation was already observed at that time. Almost one month after PAB, replacement of the AVV with a mechanical valve (diameter 18 mm) was required for uncontrollable AVV regurgitation. After Glenn anastomosis as second-stage palliation followed by pulmonary artery augmentation with the e-PTFE patch, Fontan circulation was completed (age 2 years 5 months). Two years and 5 months after Fontan operation (age 4 years 10 months), repeat valve replacement was performed using a mechanical valve with a 21-mm diameter because of a stuck valve leaflet. One month after this repeat valve replacement, a stuck valve was again observed, another valve replacement was needed. During the same hospitalization, the stuck valve was observed yet again, and yet another valve replacement was performed using a mechanical valve with a 21-mm diameter. Because the patient’s ventricular structure possibly caused the disturbance of the valve leaflet, the bottom of the mechanical valve was raised with an artificial graft. Although repeated valve leaflet stuck was observed, laboratory examination did not reveal any coagulopathy. Repeated valve replacement resulted in severe ventricular dysfunction, which might result in congestion of blood flow through the valve leaflet. The patient was placed on much medication, including diuretics and β-blockers for severe heart failure. When the patient was 6 years 5 months old, a stuck valve was again observed, and rather than valve replacement, fibrous tissue around the stuck valve leaflet was removed, which resulted in recovery of motion of the valve leaflet. Because of the patient’s severe ventricular dysfunction, it was believed that it would be hard for the patient to be removed from cardiopulmonary bypass after AVV replacement (AVVR) and AVVR was not indicated then. However, one month after the operation, re-re-re-re valve replacement was needed because of a stuck valve during the same hospitalization. During the operation, an e-PTFE graft of the Fontan route was transected to obtain the surgical field for valve implantation, and then a bottom-raised mechanical valve with a 19-mm diameter implanted. Because of the excessive fibrous tissue observed on the AVV annulus, which could have hindered the valve leaflet motion, the same-size valve could hardly be implanted. The left atrial (LA) space was too small for the bottom-raised valve, and thus the LA wall was augmented with an e-PTFE patch. To increase cardiac output, right atrium to LA communication was created. On the 45th postoperative day, the patient was discharged from the hospital on warfarin potassium to maintain a prothrombin time–international normalized ratio between 2.5 and 3.0.