Echocardiographic images of pulmonary atresia with intact ventricular septum (PA/IVS)

(A) Echocardiogram in a fetus at 32 weeks gestation with PA/IVS demonstrates the normally formed aortic arch with a vertical ductus arteriosus (*). In the normal fetus, the ductus arteriosus lies in a more horizontal plane as it carries blood from the main pulmonary artery to the descending aorta. In the fetus with PA/IVS, the ductus arteriosus provides retrograde pulmonary blood flow and it lies in a more vertical position.
(B) Four-chamber view in a fetus at 32 weeks gestation with PA/IVS demonstrating a severely hypoplastic RV and shunting of blood from the RA through the foramen ovale (*) to the LA and LV.
(C) Four-chamber view in a neonate with PA/IVS demonstrating a small tricuspid valve and RV compared with the normally sized mitral valve and LV.
(D) Four-chamber view in a neonate with PA/IVS with measurements of the tricuspid and mitral valves. Note the small tricuspid valve (measurement 1) and RV compared with the normally sized mitral valve (measurement 2) and LV; the tricuspid valve measures 0.3 centimeters, which correlates to a Z-score of approximately -4.