| Abstract: | We describe the cases of two neonates affected by the syndrome of pulmonary valve agenesis, in whom non-invasive diagnosis was possible by two dimensional echocardiography. The echocardiographic features we describe are the following: enlargement of the right ventricle in association with ventricular septal defect, malalignment type, dilatation of the right ventricular outflow tract, massive dilation of the main and branch pulmonary arteries, annular pulmonary stenosis. At the expected site of the pulmonary valve two ridges were seen, which did not have the typical motion of a fully developed valve. The ventricular septal motion was paradoxical in both cases. The diagnosis was confirmed at cardiac catheterization and the two neonates underwent operation unsuccessfully. The anatomic post-mortem examination confirmed the echocardiographic and angiographic findings. As successful treatment of this syndrome is difficult, we believe that a non-invasive echocardiographic diagnosis is very useful in order to avoid or, at least, delay a high risk cardiac catheterization. |
