Abstract
Objective: To report a case of tricuspid atresia (TA), a rare cyanogenic congenital heart disease with high mortality, highlighting the importance of intrauterine diagnosis in planning prophylactic and therapeutic measures that allow for increased patient survival. Case details: This is a three years old patient with TA. The heart disease was suspected at intrauterine period by morphological ultrasound and two-dimensional fetal echocardiography color Doppler and confirmed on postnatal echocardiography. Neonates with this heart disease have early clinical manifestations and require immediate intervention at birth. Management included scheduled cesarean delivery, early intervention with prostaglandin E1 and scheduling of palliative and corrective surgeries. Currently, the patient is stable and in good clinical evolution. Final considerations: The diagnosis of TA during the fetal period makes it possible to reduce mortality and increase the survival of patients due to early intervention through scheduled delivery, therapeutic management immediately after birth and planning for staggered surgical correction.