Mustard thủ tục cho chuyển vị của các mạch máu lớn. Cine fluoroscopic ngực, postero trước (PA) quan điểm của một venogram caval cấp trên của tĩnh mạch. Còn lại: baffle tắc nghẽn ở ngã ba của vách ngăn tâm nhĩ phải và tĩnh mạch chủ cao cấp. | CHAPTER 19 Ebstein s anomaly Ebstein s anomaly which represents 1 of all congenital heart diseases is of clinical importance to pacemaker physicians because many of the patients who reach adulthood develop bradyarrhythmias Figure 189 . The disorder is characterized by downward displacement of the tricuspid valve orifice so that the cusps with the exception of the medial Figure Schematic of the apical displacement of the tricuspid valve seen in Ebstein s anomaly. This results in a superior atrialized portion of the right ventricle which is associated with atrial pressure yet ventricular intracardiac electrogram recordings broken circle . 81 82 Chapter 19 two-thirds of the anterior cusp originate from the right ventricular wall rather than from the tricuspid annulus 190 191 . This displacement may be as low as the junction of the inflow and the outflow portions of the right ventricle. The displaced tricuspid valve divides the right ventricle into two parts The atrialized portion which lies between the origin of the normal tricuspid annulus and the displaced tricuspid orifice. The remainder of the true right ventricle which lies beyond the tricuspid valve. The size of the atrialized portion of right ventricle varies greatly and its walls may be fibrous and paper thin or muscular and well formed. The tricuspid valve tissue is almost always redundant wrinkled and the chordae tendineae poorly developed or absent. The actual valve orifice is generally smaller than normal and is usually incompetent 190 191 . Cardiac arrhythmias and conduction disturbances occur in 20-25 of patients with Ebstein s anomaly 192 193 and commonly involve preexcitation syndromes. Pacemaker therapy is necessary in about 3-4 194 . The indications for pacemaker therapy include persistent atrial standstill 195 atrio-ventricular block which can be de novo 196 197 post surgery 198 or following radiofrequency ablation 199 . Due to the morphologic abnormalities in Ebstein s anomaly endocardial
