[Pulmonary atresia with intact interventricular septum. Report of a case diagnosed by fetal echocardiography].

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare cardiac congenital malformation involving the right ventricle (RV) in which the communication through pulmonary valve, is absent. A case of a congenital heart disease (CHD) consisting of pulmonary atresia with intact ventricular septum (PA/IVS) and small right ventricle (RV) or type I of Greenwold, coming from a twin pregnancy in which the other was an inutero dead fetus, is reported. Although the case was referred after the death of one of the fetuses, the prenatal diagnosis was made by the use of echocardiographic studies and confirmed by anatomopathology of the still-born fetus with the CHD. The very useful echocardiographic prenatal diagnosis and surgical therapeutical options are emphasized and discussed.