Tricuspid atresia is a type of cyanotic congenital heart disease in which the main pathological changes are tricuspid atresia or absence of the tricuspid orifice, absence of direct communication between the right atrium and the right ventricle, associated with patent foramen ovale or atrial septal defect, enlarged left ventricle, and hypoplastic right ventricle. The patient’s echocardiogram shows no normal tricuspid valve leaflet and its activity detected at the right atrioventricular orifice, but a band of thickened fibrous tissue with strong echogenic bands, sometimes with a gap in its center, and a pulmonary valve that may be normal, stenotic, or atretic. The right atrium is often enlarged separated from the right ventricle by a strongly echogenic band. Depending on the presence or absence and size of the ventricular septal defect, the right ventricle has varying degrees of dysplasia. The origin and interrelationship of the great vessels are important for the pathologic changes of tricuspid atresia malformations and form the basis for staging. The site and size of the atrial septal defect should be observed, and the presence of the ventricular septal defect as well as the size and morphology of the defect should be observed. Color Doppler is used to observe the presence or absence of blood flow signals through the tricuspid orifice, right-to-left shunting at the level of the atrial septum, left-to-right shunting at the level of the ventricular septum or at the level of the aorta, and obstruction of blood outflow from the aorta or pulmonary artery, showing abnormal signals from concomitant malformations, such as left superior vena cava and pulmonary vein malformation drainage. Echocardiography is accurate, reliable, and noninvasive for the diagnosis of tricuspid atresia, and is an ideal test that can provide a reliable basis for surgical treatment.