Absent pulmonary valve, imperforate tricuspid valve, and dysplastic right ventricle.

We report a very rare case involving an absent pulmonary valve, an imperforate tricuspid valve, a ventricular septal defect (VSD), and a dysplastic right ventricle in a patient who underwent a Fontan operation successfully. Upon performing a bidirectional cavopulmonary anastomosis, we closed the VSD and coronary artery-to-right ventricle fistulae, and plicated the right ventricular free wall in order to obliterate the right ventricular cavity.