Ebstein’s Malformation With Imperforate Tricuspid Valve

A neonate was admitted at the age of 8 days with a history of increasing cyanosis and tachydyspnea after a normal pregnancy and delivery at 42 weeks’ gestation. A transthoracic echocardiography appeared to indicate pulmonary atresia with an intact ventricular septum. The child was intubated, intravenous prostaglandin E1 was started, and the patient was transferred to our hospital. The newborn presented with central cyanosis while being ventilated with 100% oxygen. No heart murmur was perceptible. Transthoracic echocardiography revealed marked apical displacement of the tricuspid valve leaflets (Figure 1 and Movie I). Linear attachments of the anterior and the mural leaflets resulted in complete separation of the inlet portion from the apical trabecular and outlet portions of the right ventricle (Figure 2 and Movie II). On color Doppler, we were unable to demonstrate either antegrade or regurgitant flow across the valve …