The stenotic form of Ebstein syndrome is characterized by significant cyanosis, class II or higher cardiac function, mild to moderate cardiac enlargement, reduced pulmonary flow, pressure difference between the enlarged right atrium and the functional right ventricle, right-to-left intracardiac shunt, and a double sphere sign on cardiovascular imaging, requiring surgical treatment. Diagnosis of right-to-left intracardiac shunt: While clinical symptoms and signs may give important indications for the diagnosis of Ebstein syndrome, accurate diagnosis depends on the following ancillary tests, especially selective cardiovascular angiography. 1) electrocardiogram; 2) X-ray; 3) echocardiogram; 4) right heart catheterization. In particular, the simultaneous recording of pressure curves and intracardiac ECG often reveals a zone of migration between the right atrium and the functional right ventricle. The pressure recorded in this zone is the same as in the right atrium, while the intracavitary ECG pattern is the same as in the right ventricle. This shifted zone is the atrialized right ventricle. This finding is often helpful in the diagnosis of Ebstein’s syndrome. The main identification belongs to which congenital heart disease: 1, arteriovenous ductus arteriosus, ventricular septal defect, atrial septal defect: In the early stage of the disease, there is no cyanosis because the blood is shunted from the artery to the vein, which is a left-to-right shunt type; however, in the late stage, the pressure on the left side of the heart exceeds the right side, and cyanosis appears. 2, Tetralogy of Fallot, transposition of the great arteries, pulmonary atresia: because part or all of the venous blood shunts directly into the artery, it belongs to the right-to-left shunt type, so there is cyanosis after birth. 3.Pulmonary stenosis, aortic stenosis, aortic constriction: Despite the presence of cardiac malformation, there is no abnormal channel between the left and right sides, which is a non-shunt type, and therefore, cyanosis does not appear throughout life.