Pulmonary valve replacement for porcelain right ventricular outflow tract following repeated surgical intervention

Background Repeat surgery is common in adult congenital heart disease, and valve-related procedures are the most frequent indication for re-intervention. In these cases, problems such as advanced adhesion, deterioration and calcification of the prosthesis used, progression of cardiac dysfunction, and worsening of the general condition are often observed. Case presentation We herein report a 43-year-old patient with repaired pulmonary atresia and ventricular septal defect who experienced repeated right heart failure and protein-losing enteropathy after multiple bioprosthetic tricuspid and pulmonary valve replacements. The patient was successfully treated with a fourth pulmonary valve replacement and third tricuspid valve replacement using a mechanical valve. During surgery, peeling off and removing the right ventricular outflow conduit was risky due to dense adhesion to the ascending aorta with extremely severe calcification; thus, the mechanical pulmonary valve was implanted to a more proximal position of the right ventricular outflow tract after removing the leaflet only and leaving the stent of the bioprosthetic valve within the conduit. The right heart failure and protein-losing enteropathy were relieved with this surgery, and the patient has remained in remission for over 5 years. Conclusion Although severe adhesion and porcelain-like calcification caused by multiple surgical interventions were a major issue in this case, good surgical results were obtained. This method has a major advantage over conventional pulmonary valve replacement with right ventricle outflow tract reconstruction when the right ventricular outflow tract conduit shows severe adhesion and calcification.