First trimester diagnosis of Absent Pulmonary Valve (APV): a case report of prenatal treatment with a review of literature and data from The Polish National Registry of Fetal Cardiac Pathology

Abstract Absent of pulmonary valve syndrome is a rare congenital heart defect, which is diagnosed prenataly in 0,8% of fetuses with congenital heart defect based on the data from National Polish Registry Of Fetal Cardiac Anomalies. We present a case of pregnat woman and fetus with that heart defect, which was detected in the 1st trimester and treated prenatally with digoxin, amnioreduction, tocolysis and steroids following by the cardiac sugery in the neonatal period. Despite an intensive therapy, the infant died on the 3rd month of age. We belive that the main reason of poor outcome was premature delivery at the 35th week of gestation. We present unique cardiac images proving the changing characterists of this type anomay since 1 st trimester