Two cases of congenital trabecular hypoplasia of the right ventricle are reported. In the first, the neonatal diagnosis was missed and the child did well until the 13th month of life when a modified Blalock-Taussig shunt was done because of increasing cyanosis. Outcome was good until the 4th year of life when symptomatic atrioventricular block was detected in an emergency situation. A bidirectional Glenn anastomosis and pacemaker implantation were successfully carried out after clinical establization and the child is doing well up to now.

The second case presents the disease with its worst features:

severe cyanosis and acidosis in the first day of life. A modified Blalock-Taussig shunt was performed and death occurred soon after the operation.