Congenitally corrected transposition of the great arteries (ccTGA) is a complex cardiac malformation with inconsistent atrial-ventricular connections and inconsistent ventricular-arterial connections [1], which is classified into a variety of different types according to anatomical and clinical manifestations, ranging from asymptomatic to cyanotic manifestations, from hemodynamic equilibrium to the presence of heart failure. Asymptomatic to cyanotic manifestations, from hemodynamic equilibrium to the appearance of heart failure, surgical methods are numerous ranging from ccTGA with an intact ventricular septum without intervention, physiological correction, anatomical or even partial anatomical correction, to functional correction, with a survival rate of only 50% after 40 years after physiological correction, and anatomical correction firstly started to be reported in the mid-1990s, with good results in the near- to mid-term follow-ups, but with inconsistent reports on the long-term prognosis [2], and other surgical methods are to be explored. Data and Methods A total of 203 children with corrected aortic malposition were hospitalized from June 1999 to December 2014, and the age of admission was from 2,5 days to 17 years and 2 months (mean 39,8±29,7 months). There were 118 males (58%) and 85 females (42%) with 175 orthotopic (S,L,L) and 28 right-sided (I,D,D) hearts, 109 cases of combined left ventricular outflow tract obstruction (LVOTO) and pulmonary stenosis (PS), 13 cases of simple restrictive ventricular septal defect (VSD), and 13 cases of combined Ebstein’s deformity in 31 cases, tricuspid valve riding in 25 cases, mitral valve riding in 11 cases, 3 cases combined with complete ectopic pulmonary venous drainage (TAPVC), and 2 cases combined with coarctation of the aorta (COA), the surgical approach was divided into four groups. In the palliative surgery group (13 cases), the age at surgery ranged from 2,5 days to 4 years and 8 months (mean 21,2±57,6 months); weight ranged from 3,1 to 15 kg (mean 5,3±13,1 kg). Seven of the children in this group underwent pulmonary artery circumflexion (two to control pulmonary blood flow and reduce tricuspid regurgitation, and five to exercise the functional left ventricle); and six underwent body pulmonary artery shunt. In the group of traditional physiologic corrective surgery (39 cases): age at surgery 3 months to 16 years and 11 months (mean 53, 7 ± 41, 2 months); weight 6 to 58 kg (mean 16, 5 ± 12, 1 kg). There were 23 cases of combined pulmonary stenosis, of which 14 underwent Rastelli procedure and 9 pulmonary valve leaflet junction dissection and repair of ventricular septal defect (VSD). There were 16 cases of uncomplicated pulmonary stenosis, 9 cases of simple repair of VSD, 3 cases of tricuspid valvuloplasty, and 5 cases of tricuspid valve replacement. In the anatomical corrective surgery group (88 cases), the age at surgery ranged from 2 months and 25 days to 16,6 years (mean 38,7±47,4 months); weight ranged from 4,5 to 56 kg (flat 12,1±11,6 kg). Thirty-seven of them underwent the Senning+Swich procedure, in which 5 children underwent a one-stage pulmonary artery annuloplasty, and 51 underwent the Senning+Rastelli procedure, in which a bovine jugular vein with a valved conduit or a Gortex artificial conduit was used to connect the common pulmonary artery and the dissected right ventricle, and in 3 of them an anatomical one-and-a-half ventricle repair was performed. In the group of other corrective surgeries (63 cases), age at surgery ranged from 2 years to 17 years and 10 months (mean 58, 0±35, 1 month); weight ranged from 12 to 51 kg (mean 16, 5±7, 7 kg). Among them, 14 cases of superior vena cava-pulmonary artery bi-directional shunt were performed, and 4 cases of physiologic one and a half ventricle repair were performed. There were 25 cases of one-stage Fontan operation, 19 cases of two-stage Fontan operation and 1 case of three-stage Fontna operation. Surgical results Palliative surgery group: 13 children underwent palliative surgery, there was no surgical death, 2 cases of postoperative hypoxemia occurred after surgery, which was improved to 80%~85% after treatment. Conventional surgery group: 39 children underwent conventional surgery, with 2 deaths (5.1%) and 6 cases of postoperative complete atrioventricular block, 3 of which returned to sinus rhythm during hospitalization, and 3 of which had permanent pacemakers installed during hospitalization. Anatomical corrective surgery group: 88 children underwent Double Switch surgery, 9 cases died (8,0%), including 41 cases of Senning+Swich surgery, 5 cases died; 47 cases of Senning+Rastelli surgery, 4 cases died.The causes of postoperative death in the Senning+Switch group: 2 cases of VAD-assisted circulation for 3-6 days with severe hypoventilation and polycyclic heart failure, 2 cases of VAD-assisted circulation for 3-6 days with severe hypoventilation and polycythemia. days with severe low cardiac output and multiple organ failure; 1 case of pulmonary hypertension crisis, 1 case of postoperative coagulation dysfunction and multiple organ failure, 1 case of intraoperative finding of tricuspid valve riding, huge VSD and severe low cardiac output.Postoperative causes of death in the Senning+Rastelli group: 1 case of postoperative severe low cardiac output; 2 cases of intraoperative enlargement of the VSD and postoperative severe low cardiac output, and the other case of ductus venosus. residual obstruction reoperation affecting cardiac function. Echocardiograms at 6 months to 3 years after operation showed that after Senning+Switch operation, 9 children had mild to moderate neoaortic regurgitation of different degrees, 7 children had increased aortic anastomotic flow velocity, anastomotic flow velocity of 2,4~3,3m/s; 8 children had increased pulmonary anastomotic flow velocity, anastomotic flow velocity of 1,9~3,8m/s; after Senning+Rastelli operation, the external conduit flow velocity increased, anastomotic flow velocity of 1,9~3,8m/s; and after Senning+Rastelli operation, the external conduit flow velocity increased, the anastomotic flow velocity of 1,9~3,8m/s. Rastelli procedure, the external conduit and anatomic right ventricular anastomosis had increased flow velocity in 11 cases, with flow velocities of 2,1 to 4,1 m/s. Anatomical corrective surgery was performed with reoperation interventions in 7 cases, including reflux obstruction in the pulmonary vein in 2 cases, obstruction of the right ventricular outflow tract of the external conduit in 4 cases, and obstruction of the left ventricular outflow tract in 1 case. Other corrective surgery group: 45 of 63 children underwent functional single ventricle corrective surgery and 2 died during postoperative hospitalization.Fontan postoperative SpO2: 73-96%, mean 87,7±5,6%, no operative deaths were observed in the rest of the 18 cases who underwent cavopulmonary anastomosis and physiologic one and a half ventricle repair. Discussion I. Problems related to surgical anatomy The basic anatomical abnormality of ccTGA is the inconsistency of atrial-ventricular and ventricular-arterial connections, which can be hemodynamically normal in the absence of VSD, resulting in neither serious physiological dysfunction nor clinical cyanosis. The main problem arises in the right ventricle and the tricuspid valve in the somatic circulation, which are not inherently designed to have a blood flow supply, tension regulation, myofibers and conduction system. systems, etc., are not inherently designed to take on a high-pressure circulation for long periods of time. Due to the anatomical situation, the surgical options available for ccTGA are categorized according to the postoperative circulating ventricle (right, left, or biventricular): neonatal pulmonary artery annuloplasty, classic repair (physiologic repair), tricuspid valvuloplasty-replacement (alone), Senning C ASO, Senning C Rastelli, modified Nikaidoh + atrial rotation, 1+1/2 ventricles physiologic repair, 1+1/2 ventricles physiologic repair, and 1+1/2 ventricles physiologic repair. repair, 1+1/2 ventricular anatomic repair, Fonton procedure, and no surgical treatment [3]. The problems of performing physiologic repair surgery for ccTGA have been widely understood. Essentially, physiologic repair results in the same status as in patients with ccTGA combined with an intact ventricular septum (IVS) and without left ventricular outflow tract obstruction (LVOTO), but the procedure increases the potential risk of myocardial and conduction system injury. After physiologic repair, dilatation of the TV valve annulus and misalignment of the papillary muscles due to pressure imbalance septal excursion both have an aggravating effect on the progressive evolution of TR and both increase the risk of conduction block in the near and distant future of the heart [4]. Considering the early and late morbidity and mortality, anatomical corrective surgery has been more frequently used (see Table I). Nevertheless, physiologic repair surgery is still useful in certain specific cases in the early stages, including: 1. Physiologic repair surgery is feasible in patients with good right heart function, good TV function, and balanced ventricular development, especially in patients with relative contraindications to anatomical corrective surgery; 2. In some cases, such as mitral valve dysfunction, coronary artery malformations, small atrial development, and a right-situated heart, which can make anatomical correction more difficult In some cases, such as mitral valve dysfunction, coronary artery malformation, small atrial development, right-side heart, it will increase the difficulty of anatomical correction, then physiological repair surgery is the best choice [5], the group of 39 ccTGA children postoperative hospitalization survival rate of 95%, the postoperative near-term results are ideal. Third, anatomical corrective surgery Anatomical repair surgery is a generic term for double-tuning surgery (Senning/Mustard + ASO, S, A,) or (Senning/Mustard + Rastelli, S, R,). (1) In children with ccTGA with pulmonary stenosis, if hypoxia is severe, a body-pulmonary artery shunt may be performed first in infancy, followed by elective Double Switch surgery at a later date. In some children, due to the presence of pulmonary artery stenosis, which protects the function of the anatomical left ventricle, the pulmonary blood can also be basically normal, and this group of children, if the body-circulation oxygen saturation is >0,80 and relatively asymptomatic clinically, can be followed up regularly until the age of 4-5 years old and then undergo the Senning-Rastelli procedure in order to reduce the risk of replacing the valved tubes in the future and installing a pacemaker due to heart block [6], otherwise, the feasibility of a 1 half or single ventricle surgery. (2) In ccTGA without pulmonary stenosis, the size of the ventricular septal defect determines the timing of surgery. In restrictive VSD, almost all children have left ventricular degeneration when tricuspid regurgitation develops, and PAB should be performed to exercise left ventricular function followed by Double Switch surgery. However, when the child is ≥15 years of age, it is difficult to restore LV function by PAB and cannot adequately mitigate tricuspid regurgitation, so tricuspid valve replacement or heart transplantation should be considered [7]. Patients with pulmonary annular contraction can often present with a very favorable septal excursion, which reduces tricuspid regurgitation without the need for other surgical procedures. Although anatomical correction achieves similar near-term results to physiologic repair, the long-term risks and benefits of anatomical correction surgery are not yet fully understood. Early mortality rates of 5 or 6% after Double Switch have been reported in the literature, and Gaies et al. (Ann Arbor) analyzed the long-term results of the Senning + ASO and Senning + Rastelli procedures [8-9], showing 1-, 5-, and 10-year survival rates of 72%, 55%, and 55%, respectively. The postoperative mortality rate during hospitalization was 8 ((10,2%) in our group of 88 children who underwent Double Switch, which is still relatively high, and 7 (8%) post-Double Switch patients in our group have undergone re-surgical intervention. Other corrective surgeries (including segregation) 1. 1,5 ventricular repair of ccTGA This method is between anatomical correction and physiological repair, and was proposed by Mavroudis et al. It is suitable for ccTGA combined with VSD and LVOTO. Due to the anatomical morphology of the outflow tract and the location of the conduction bundle, the external conduit is usually not used for anatomical correction to fully relieve the LVOTO of ccTGA. LVOTO in ccTGA is usually more difficult. With this in mind, valved conduits are avoided in 1 and 5 ventricular repair, a procedure that consists of pulmonary valvotomy as well as cavopulmonary anastomosis. But after all, there is still the potential for long-term TR. Another type of surgical protocol for ccTGA with combined VSD and LVOTO was reported by DiBardino et al. (Houston) in 2004 [23]. In the original RS group, where the most common re-intervention was tube replacement due to tube failure to grow, the procedure was changed to a 1 and 5 ventricle anatomical repair using a Hemi-Mustard intra-atrial transfer procedure + Rastelli procedure consisting of a 1 and 5 ventricle anatomical repair, i.e., a 1 + 1/2 repair with a cavopulmonary shunt allowing for a reduction in antegrade flow to prolong the tube’s lifespan was used [10]. This type of procedure is useful in patients with Rastelli instability, combined right ventricular dysplasia or insufficiency, and TR, especially in patients with a small right ventricle in the septum. 2. Single ventricle (Fontan) surgery for ccTGA The Fontan procedure is another solution to the incongruity of the AV and VA links. indications for the Fontan procedure: small left ventricular outflow tract, insufficient morphology or function of the atrioventricular valves, imbalanced atrioventricular septal defects, double right ventricular outlets with ventricular defects away from the great vessels or multiple ventricular defects or combination of large VSDs, inability to (or a high risk of) biventricular repair, atrioventricular valve riding or in other cases where the risks of performing biventricular repair outweigh the benefits. The modified Fontan procedure has excellent physiologic corrective efficacy in complex congenital heart disease, with significantly higher survival rates reported in patients after Fontan than in patients with anatomic correction, with a 10-year survival rate of 91%, 87% without reoperation [6], and a 20-year postoperative survival rate of 79,3% [8]. After more than 20 years of follow-up, the long-term mortality rate and the proportion of patients who do not require reoperation are at least as high in patients with anatomical correction as in those who approach anatomical correction. Conclusions The choice of surgical approach for corrective aortic malalignment is dependent on anatomical conditions and physiological parameters.The Double Switch procedure, although it achieves anatomical correction ccTGA, is associated with complexity of surgical maneuvering, long operative and myocardial ischemia times, and a slightly higher postoperative mortality rate and long-term prognosis to be assessed when compared with the functional single-ventricle corrective procedure.The majority of patients who are suitable for the Senning + Rastelli repair are in fact candidates for Fontan surgery, but the timing and technique of Fontan affects ccTGA patients differently than other single ventricle patients, and tricuspid regurgitation can limit post-Fontan outcomes in the long term (the literature has documented that ccTGA survival with Fontan surgery declines further down the road), so it is not possible to require a fully Fontan procedure in patients suitable for anatomic biventricular correction. Alternatively, physiologic and anatomic repair of one and a half ventricles is an effective approach that may also yield better outcomes.