Tetralogy of fallot with absent pulmonary valve - Prenatal diagnosis and management in the second trimester

Tetralogy of Fallot with absent pulmonary valve is a rare congenital cardiac malformation detected in 3% to 6% of tetralogy of Fallot patients. The prognosis depends on respiratory complications. In the present case the diagnosis was made at 18 week's gestation with two-dimensional and Doppler echocardiography. A ventricular septal defect and overriding aorta, absent valve echo, pulmonary regurgitation, and cystic pulsatile dilatation of the left pulmonary artery were the main diagnostic criteria. Severe respiratory complications were assumed in combination with a hyperechogenic left lung and termination of pregnancy was performed at 20 weeks' of gestation.