A Bicuspid Pulmonary Valve Associated with Tetralogy of Fallot

Congenitally bicuspid pulmonary valves are uncommon. When they occur, it is usually in association with other congenital cardiac lesions, most often a tetralogy of Fallot. We present a rare case of a patient with a congenitally bicuspid pulmonary valve who had pulmonary valve and RVOT reconstruction. The patient did well for 17 years, but needed further reconstruction when the pulmonary valve started getting stenosed and RV pressures went up significantly. The pulmonary valve showed fibroses, thickening, and focal calcification.