FIG 37.16 Echocardiographic images obtained during an atrial septostomy The catheter crosses the oval foramen (arrow) The balloon is inflated within the left atrium (LA), and its relationship to the mitral valve (MV) can be seen (A) After the balloon is pulled across the atrial septum, its location in the right atrium (RA) can be seen (B) LV, Left ventricle A septostomy can result in a rapid and dramatic improvement in systemic oxygenation This is important because preoperative brain injury has been associated with both the degree of hypoxemia and its duration.45 Another benefit is the ability, with the assurance of adequate interatrial mixing, to stop the prostaglandin infusion prior to surgery Doing so mitigates risk of apnea, hypotension, and fever associated with the infusion, as well as adverse hemodynamics from “ductal steal.” With the duct closed (or small), enteral feeds may be advanced without concern for significant systemic runoff compromising intestinal perfusion For these reasons, some institutions feel that septostomy promotes stability in the preoperative patient and use it liberally, performing the procedure in any patient in whom the anatomic substrate is not obviously adequate for mixing and before any concerning clinical change can occur Institutional practice should be guided by outcome data and complication rates for the individual institution Complications associated with septostomy in the setting of transposition are rare but include injury to the surrounding structures (atrioventricular valves, pulmonary, hepatic, or inferior caval veins), air embolism, and pericardial effusion The operator must take care not to inadvertently injure the atrial appendage, particularly in patients with left juxtaposition of the right atrial appendage (see Video 37.2) In this arrangement, the orifice of the right atrial appendage can be mistaken for the atrial septal defect, with the potential for severe complications The routine use of septostomy came under question following reports of an association between septostomy and brain injury.46,47 Other publications have not found this association.45,48 Furthermore, one study demonstrated no relationship between septostomy and preoperative brain injury or 12-month neurodevelopmental outcome in a cohort of patients with transposition.49 Surgical Management Historical Perspective The surgical treatment of transposition can be viewed as one of the great successes in the field of pediatric cardiac care Survival is currently the expectation in a disease once associated with a very high mortality rate Early attempts at surgical anatomic correction were uniformly fatal Poor outcomes were attributable to inadequate management of coronary flow, limited preoperative assessment of anatomy and function, limited microvascular surgical techniques, and primitive cardiopulmonary bypass circuits In addition, many babies tended to be extremely unwell at the time of surgery As a result, atrial septectomy became the only palliative option.50 By the 1960s, palliations involving atrial redirection were beginning to be adopted—first the Senning and then the Mustard procedure.51 Further improvement in survival can be ascribed to the advent of the balloon atrial septostomy This relatively simple procedure provided rapid circulatory stabilization and afforded a mostly uncomplicated period of growth and nutrition prior to atrial redirection, which could then occur at 6 to 12 months of age Anatomic correction, by means of the arterial switch operation, was first successfully performed by Dr Adib Jatene in 1975 Despite an initial substantial increase in mortality in many centers, as survival improved, the arterial switch would ultimately replace atrial redirection procedures and become standard of care for this condition by the 1990s.52 Atrial Redirection Procedures The ability to achieve stabilization of the patient by means of the atrial septostomy before surgery set the scene for important surgical advances in the early 1970s Atrial redirection surgery provided physiologic correction, in contrast to the anatomic correction more recently offered by the arterial switch operation Nowadays, therefore, the Mustard and Senning procedures are rarely, if ever, performed as primary procedures and will be only briefly discussed here.53–56 Their greatest relevance in current practice relates to the significant population of adults who are developing late complications of the procedure, such as baffle obstruction, arrhythmias, ventricular dysfunction, and end-stage heart failure, along with their use in patients with congenitally corrected ... The surgical treatment of transposition can be viewed as one of the great successes in the field of pediatric cardiac care Survival is currently the expectation in a disease once associated with a very high mortality rate