Corrected Transposition Of The Great Arteries Research Articles

Diagnosis and treatment in a series of patients with cardiac corrected transposition of the great arteries (double discordance)

The atrio-ventricular and ventricle-arterial double discordance (DD) or corrected transposition of the great arteries is a rare heart disease, it occurs in 0.02-0.07 of every 1,000 live newborns. The objective of the study is to describe the diagnosis, treatment and evolution of a series of patients with DD. A retrospective and descriptive study was carried out, reviewing the records of patients diagnosed with DD in the last 22 years. Descriptive statistics were performed. Numerical variables were obtained using means and standard deviation and categorical variables using frequencies and percentages. Thirty patients were studied in 22 years with a ratio of 1.5:1 for men, with a mean age of 20 months. The situs was solitus in 24/29 patients (82.7%). Ventricular septal defect was the most frequent lesion in 25/29 (86.2%) Tricuspid insufficiency in 70%. Four patients diagnosed with pulmonary atrial hypertension. With atrio-ventricular block 20%. One with Wolff-Parkinson-White syndrome. Surgical treatment was carried out in 70% of patients. Eight with Glenn procedure (26.6%) and 4 with Fontan surgery (13.3%). Follow-up ranged from 1 month to 17 years. Five died (16.6%). Of the 25 patients in follow-up, 18 patients (72%) had normal ventricular function, 5 with Grade II Ross classification (20%) and 2 in Grade III (8%). The quality of life of these patients is improving and there is still controversy in the literature about the ideal time to perform the most appropriate surgical procedure.

Surgical Outcomes in Patients Undergoing a Double Switch Operation for Corrected Transposition

BackgroundCongenitally corrected transposition of the great arteries (CC-TGA) is a rare and complex form of congenital heart disease. Results of physiologic repair proved disappointing due to late right ventricular dysfunction and/or tricuspid regurgitation. The current study was performed to evaluate surgical outcomes in patients undergoing a double switch for CC-TGA. MethodsThis was a retrospective review of 121 patients who underwent a double switch over a 2-decade time frame (2002-2023). Patients were a median age of 32 months. Before the double switch, 49 of 121 patients (40%) had undergone left ventricular retraining. ResultsSixty-seven patients underwent an arterial switch, and 54 underwent a Rastelli procedure. There were 4 in-hospital deaths (3.3%), including 3 who had a Rastelli procedure (5.6%) and 1 who had an arterial switch (1.5%). At a median follow-up of 30 months, there were 4 late deaths (2 Rastelli and 2 arterial switch). Combined early and late mortality was 9.3% for the Rastelli and 4.5% for arterial switch. Combined mortality was 2.0% for patients who required left ventricular retraining vs 9.7% for those who did not. For the 117 patients discharged from the hospital, 93% have normal or low-normal left ventricular function, and 96% have mild or less neoaortic insufficiency. ConclusionsSurgical outcomes in patients undergoing a double switch procedure have been excellent both in the short- and midterm. However, the Rastelli procedure was associated with a more than 2-fold increase in mortality risk compared with the arterial switch.

Prenatal transposition of great arteries diagnosis and management: a Chinese single-center study.

This study aimed to assess the diagnostic value of prenatal echocardiography for identifying transposition of the great arteries (TGA) during pregnancy and evaluating the associated outcomes. We conducted a retrospective analysis of 121 prenatally diagnosed patients with TGA at our hospital between January 2012 and September 2022. This analysis included prenatal ultrasound, prenatal screening, clinical management and follow-up procedures. Among the 103 fetuses considered in the study, 90 (87.4%) were diagnosed with complete transposition of the great arteries (D-TGA), while 13 (12.6%) exhibited corrected transposition of the great arteries (CC-TGA). Diagnoses were distributed across the trimester, with 8 D-TGA and 2 CC-TGA patients identified in the first trimester, 68 D-TGA patients and 9 CC-TGA patients in the second trimester, and 14 D-TGA and 2 CC-TGA patients referred for diagnosis in the third trimester. Induction of labour was pursued for 76 D-TGA patients (84.4%) and 11 CC-TGA patients (84.6%), and 14 D-TGA patients (15.6%) and 2 CC-TGA patients (15.4%) continued pregnancy until delivery. Among the D-TGA patients, 9 fetuses (10.0%) underwent surgery, two of which were inadvertent fatality, while the remaining seven experienced positive outcomes. Additionally, seven TGA patients received palliative care, leading to four fatalities among D-TGA patients (5.2%), whereas 1 D-TGA patients and 2 CC-TGA patients survived. This study underscores the feasibility of achieving an accurate prenatal diagnosis of TGA during early pregnancy. The utility of prenatal ultrasound in the development of personalized perinatal plans and the application of multidisciplinary treatment during delivery are conducive.

Incidence and clinical predictors of early and late complications of implantable cardioverter-defibrillators in adults with congenital heart disease.

The Implantable cardioverter-defibrillator (ICD) has been proven to improve survival in adults with congenital heart disease (ACHD), however it is associated with a high rate of complications. We aimed to quantify the incidence of early (≤3 months) and late (>3 months) complications (EC, LC) in ACHD patients implanted with an ICD and identify their clinical predictors. We retrospectively reviewed 207 patients who had ICD follow-up at Toronto General Hospital between 1996 and 2019. Most common diagnoses were Tetralogy of Fallot (32.4%), dextro-transposition of the great arteries (17.9%), congenital corrected transposition of the great arteries (13%). No intraprocedural complications were observed. Median follow up was 3.4 years (0.1-23). 24 patients (12%) developed EC (4 hematomas, 20 lead dislodgments). A total of 56 LC occurred (46% lead failure, 21% infection, 11% prophylactic lead extraction, 9% neurologic pain, 9% erosion, 4% other) with an incidence rate of LC of 18% per 5 person-years. Anatomic complexity (odds ratio 2.9,p=0.02) and CRTD implant(OR 2.5,p=0.04) were associated with EC. Survival free from LC was 92%, 86%, 65% respectively after 1,5 and 10 years. Presence of legacy leads (hazard ratio 2.9,p=0.006) and subpulmonary ejection fraction (5% increase, HR 1.35,p=0.031) were associated with LC. ACHD patients at risk of SCD continue to benefit from newer device technology. However, these patients-particularly those with greater anatomic and device complexity-remain at increased risk of developing complications over their lifetime. Given the life expectancy of this population, careful consideration needs to be given when a device for primary prevention is being contemplated.

Clinical Case Reports 1976 - 79

In this paper five case reports were presented and include growth of hypoplastic right ventricle following pulmonary valvotomy, intracavitary electrocardiography in the diagnosis of Ebstein’s anomaly of the left atrioventricular valve in congenital corrected transposition of the great arteries, growth phenomenon as a cause of kinking of the right pulmonary artery in Waterston anastomosis, the usefulness of pulmonary vein wedge angiography in the visualization of an obstructed pulmonary artery, false aneurysm of the right pulmonary artery as a new complication of the Waterston anastomosis. For each case report, clinical, chest x-ray, electrocardiogram, angiogram, and other pertinent findings were presented. This was followed by discussion of etiology, diagnosis, and treatment options, as appropriate.

Anticoagulation management in adult patients with congenital heart disease: a narrative review

With improvements in treatment of congenital heart disease more paediatric patients are surviving with palliative or corrective interventions during childhood, thus becoming adults with congenital heart disease (ACHD). Overall, the ACHD population is at a higher risk of arrhythmias and stroke. The abnormal structure and function of their corrected hearts suggests that in addition to established stroke risk factors, such as prior stroke or older age, additional stroke risk factors need to be considered to determine the risk and establish the indication for oral anticoagulation (OAC) in ACHD patients. In structurally normal hearts non-vitamin-K oral anticoagulants (NOACs) offer at least equal stroke prevention with a better safety profile compared to vitamin K antagonists (VKA) in patients with atrial fibrillation (AF) or pulmonary embolism. Current guidelines recommend NOACs in ACHD patients with simple lesions and indication for OAC, while there is less certainty about their safety in ACHD patients with moderate or complex congenital heart disease such as patients with transposition of the great arteries (TGA) after atrial switch operation (Senning or Mustard operation), Fontan circulation or congenital corrected transposition of the great arteries (ccTGA). This review summarises the available evidence characterising stroke risk in patients with ACHD and the use of anticoagulants and interventional therapies to reduce that risk.

Impact of electrophysiological features acquired after anatomical repair of congenital corrected transposition of the great arteries on late mortality and ventricular dysfunction.

In patients with anatomically repaired congenitally corrected transposition of the great arteries, the impact of electrophysiological features on postoperative ventricular dysfunction remains less well known. Our goal was to investigate the role of fragmented QRS and QRS duration in mortality and systemic ventricular dysfunction after anatomical repair of corrected transposed great arteries. Consecutive patients who underwent anatomical repair in our institution from January 2005 to December 2017 were enrolled in this retrospective analysis. Fragmented QRS was defined as ≥1 discontinuous deflections in narrow QRS complexes, and ≥2 in wide QRS complexes, in 2 contiguous electrocardiogram leads. The primary end point was a composite of all-cause mortality and systemic ventricular dysfunction. A total of 74 patients were included. Among them, 30, 15 and 29 underwent the Senning arterial switch, the Senning Rastelli and the hemi-Mustard/bidirectional Glenn/Rastelli procedures, respectively. The primary end point occurred in 9 (12.2%) patients and included 7 late deaths and 2 cases of late-onset systemic ventricular dysfunction. Fragmented QRS and QRS prolongation were noted in 19 (25.7%) and 21 (28.4%) patients, respectively. In patients with the primary end point, QRS fragmentation (6/9 vs 10/65; P < 0.001) and QRS prolongation (6/9 vs 15/65; P = 0.013) were noted more frequently than in patients without the primary end point. No statistical differences in these electrocardiogram findings were found among patients treated with 3 surgical strategies. Appearance of QRS fragmentation or QRS prolongation is associated with death or ventricular dysfunction in anatomically repaired corrected transposition of the great arteries. Although there is a trend that QRS fragmentation and QRS prolongation appear more frequently in patients who had the Senning-arterial switch operation, there is no statistically significant difference associated with these electrocardiogram features among varied procedures.

Heart transplantation for end-stage heart failure combined with Q fever isolated to the heart: a case report.

Background Active infection is generally considered a contraindication for heart transplantation. The rare combination of a patient with an active Coxiella burnetii infection and a congenital corrected transposition of the great arteries requiring heart transplantation impose challenging treatment decisions. We would like to demonstrate that if Q fever is restricted to the heart only, heart transplantation is also beneficial from an infectious point of view, therefore treating two severe conditions simultaneously.Case summary A patient with end-stage heart failure due to congenital corrected transposition of the great arteries and requiring heart transplantation developed chronic Q fever and endocarditis. Different antibiotic regimes were tried due to severe adverse reactions. Antibiotic treatment was precisely monitored by measuring Q fever polymerase chain reaction (PCRs) and phase I IgG antibody titres. A positron emission tomography scan revealed that Q fever was confined to the heart only after which it was decided to perform heart transplantation. Based on the results of PCR and antibody testing, antibiotic treatment was stopped after 1 year. After 5 years of follow-up, patient is still in an optimal condition.Discussion In case of a patient with end-stage heart failure and chronic Q fever, a combined treatment with PCR-/antibody monitored antibiotics and heart transplantation can cure both conditions.

Prenatal Diagnosis and Outcome of Congenital Corrected Transposition of the Great Arteries - A Multicenter Report of 69 Cases.

Congenital corrected transposition of the great arteries (ccTGA) is a rare congenital cardiac anomaly which remains difficult to diagnose prenatally. We aim to investigate the natural history, associated anomalies and the outcome of patients in prenatally diagnosed ccTGA. This was an international multicenter retrospective analysis of fetuses with a diagnosis of ccTGA from 2002 to 2017. We reviewed clinical and echocardiographic databases of seven centers. Anatomic survey and fetal echocardiography were performed according to international guidelines of ISUOG. We considered 69 fetuses with prenatally suspected ccTGA. There was an overall survival rate of 91 % among 54patients with a confirmed diagnosis. Survival to live birth was 96 % (52/54) and survival on an intention-to-treat basis was 94 % (49/52). The mean gestational age at the time of diagnosis was 25.6 ± 5.9 weeks of gestation. In 7 out of 54fetuses (13 %), ccTGA was an isolated finding. Dextro/mesocardia was present in 15 cases (27.8 %). Intracardiac anomalies were present in 46/54 cases (85.2 %) with the most frequent anomaly being a ventricular septal defect present in 41 fetuses (75.9 %). Complete heart block was diagnosed in 10 cases (18.5 %). Extracardiac anomalies were observed in 9 out of 54 cases (16.7 %). Prenatal karyotyping of the fetus was available in 30/54 (55.6 %) cases with chromosomal anomalies in 4/30 (13.3 %). ccTGA is a rare cardiac anomaly often accompanied by a variable spectrum of further intracardiac abnormalities. Accurate diagnosis of ccTGA, which can be integrated into parental counselling, is feasible with a favorable short-term outcome for affected neonates.

P1334 Multimodal imaging assessment of a very rare cause of heart failure in adults

Abstract Introduction Congenitally Corrected Transposition of the Great Arteries (CCTGA) is a rare defect consisting in the abnormal twisting of the heart during fetal development. As a result, the two ventricles and their valves are reversed. CCTGA is frequently associated with other cardiac abnormalities. 25% of patients are developing heart failure, related to perfusion mismatch (the morphological left ventricle is supplied by a single coronary artery), and to the progressive deterioration of the structural right ventricle situated on the systemic side of the circulation. Case report A 45-year-old male was referred to our hospital for fatigue and dyspnea, occuring in the last five months. Physical examination revealed tachypnea, a slightly intense systolic murmur at the apex, and pulmonary congestion, in the absence of cyanosis, peripheral edema or jugular venous distension. Heart rate and blood pressure were normal. Usual laboratory work-up indicated increased levels of NT-proBNP, without any other abnormalities. ECG presented signs of pressure overload of the systemic ventricle (Figure Ia). Transthoracic echocardiography (TTE) highly suggested the diagnosis of CCTGA, due to atrioventricular valve displacement, with the morphological tricuspid valve closer to the apex in 4-chamber view (Figure Ib). TTE showed also dilated and dysfunctional left ventricle, mild left atrioventricular regurgitation, and normally functional right ventricle. Cardiac computed tomography emphasized a specific feature of CCTGA: the parallel emergence of aorta and pulmonary trunk, with the aortic arch crossing over the left pulmonary artery (Figure Ic). Cardiac magnetic resonance imaging confirmed dilatation and low ejection fraction of the systemic ventricle (20%), and displayed presence of trabeculations and the moderator band in the systemic ventricle (Figure Id). None of these evaluations found additional cardiac structural anomalies. Thus, patient was diagnosed with heart failure due to isolated CCTGA. Discussions and relevance of case report. This case emphasizes a very rare cause of heart failure in adults. CCTGA is reported in 0.5-1% of all congenital diseases, especially in males. Isolated CCTGA accounts for less than 10% of all cases, and represents the phenotype that is usually diagnosed in adulthood. In the absence of associated anomalies, the prognosis of these patients is particularly affected by the occurrence of heart failure in the 4th or 5th decade of life. Meanwhile, this case highlights the importance of a multimodal approach in CCTGA, and the specific contribution of each imaging method in the process of an accurate diagnosis. Abstract P1334 Figure I

P254 Congenitally corrected transposition of the great arteries with an ebstenoid tricuspid valve, aortic atresia, hypoplastic aortic arch and a dysplastic pulmonary valve

Abstract Funding Acknowledgements No financial support received Introduction Congenitally corrected transposition of the great arteries (CCTGA) is a rare cardiac malformation accounting for around 0.5% of congenital heart disease. Common associations are ventricular septal defects (VSD), pulmonary stenosis and heart block. CCTGA associated with aortic atresia is very rarely described in the literature. Description We present a case of a term neonate weighing 2.5kg. Fetal echo had initially made the diagnosis at 21/40. The baby was born in good condition and the postnatal echo confirmed the diagnosis of CCTGA. There were 2 secundum atrial septal defects, the sub-systemic tricuspid valve was ebstenoid with remarkably trivial regurgitation, there were multiple muscular ventricular septal defects with predominantly left to right flow, although flow appeared bi-directional in some views. The atretic aortic valve was anterior and to the left of the dysplastic, bicuspid pulmonary valve. The pulmonary valve leaflet motion was restricted with peak velocity of 2.4m/s and mild incompetence. The hypoplastic aortic arch filled retrogradely via the arterial duct. There was discrete coarctation with peak velocity of 2.2m/s down the descending aorta. The persistent left SVC drained to the coronary sinus. Following several discussions there was agreement that the most appropriate management for him would be to receive palliative care. He was discharged home at a week of age and continued to have intensive support from his local hospice and the cardiac team, until he passed away peacefully at home at 16 days of age. Discussion The multidisciplinary team decided surgery was extremely high risk due to the anatomy described above particularly in the context of his dysplastic, bicuspid pulmonary valve, which would have provided systemic outflow if intervention had been undertaken. Figure 1: 4 chamber view showing reverse offsetting of the atrioventricular valves, arch view showing a hypoplastic aortic arch with restrograde flow from the arterial duct, short axis view showing the semilunar valves and their relative positions, long axis view showing aortic atresia with retrograde flow into the ascending aorta Abstract P254 Figure 1

Left ventricular retraining in corrected transposition: Relationship between pressure and mass

BackgroundA subset of patients with corrected transposition of the great arteries (CC-TGA) will require left ventricular (LV) retraining before undergoing a double-switch procedure. LV retraining results in an immediate increase in LV pressure but not in LV mass. The purpose of this study was to evaluate the relationship between LV pressure and mass during LV retraining. MethodsThis was a retrospective review of 36 patients with CC-TGA who were enrolled in LV retraining. The median age at enrollment was 12 months. The majority (82%) had an Ebstenoid tricuspid valve or moderate to severe tricuspid valve regurgitation before pulmonary artery banding. ResultsTwenty-seven of the 36 patients underwent LV retraining and a double switch, including 14 patients with a single pulmonary artery band (PAB), 12 patients with 2 PABs, and 1 patient with 3 PABs. There was no early or late mortality associated with these procedures. Ninety percent of the patients demonstrated a linear relationship between LV pressure and mass; however, 3 patients (10%) demonstrated a different LV retraining pattern characterized by excess LV mass compared to LV pressure. Two of these 3 patients are LV retraining failures, whereas the third patient did undergo a double switch. ConclusionsThe data show that the majority of patients enrolled in LV retraining have a predictable relationship between LV mass and pressure. A minority demonstrate an excess accrual of mass with respect to pressure load. These results suggest there may be an important distinction between normal and pathological hypertrophy during the LV retraining process.

P764Impact of conduction disturbance after anatomical repair for congenital corrected transposition of the great arteries

P764Impact of conduction disturbance after anatomical repair for congenital corrected transposition of the great arteries

P5480Arrhythmias and heart failure in adults with isolated corrected transposition of the great arteries

P5480Arrhythmias and heart failure in adults with isolated corrected transposition of the great arteries

Discordance is the Key

AbstractCongenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart disease characterised by atrioventricular as well as ventriculoarterial discordance. It is usually associated with a variety of severe intracardiac defects. Few patients with this abnormality survive past 50 years. A 54 Yr old male patient referred to our hospital for the management of post MI VSD. At the presentation Patient had mild central cyanosis, clubbing grade 2 and the vitals are stable. Cardiac examination revealed a 2/6 long ESM in a left parasternal area with pulsations in left 2nd and 3rd ICS. There is a lot of discrepancy in both clinical examination and workup. After detailed evaluation patient is diagnosed as having cyanotic congenital heart disease – L TGA + VSD with PS. Survival up to this age is very rare with this combination of associated defects in LTG. The patient is misdiagnosed as coronary artery disease with mechanical complication in another hospital. Unless there is suspicion and having knowledge about the asymptomatic presentation of congenital heart diseases up to this age group, patients will be misdiagnosed and treatment will be delayed.

Successful thrombolytic therapy in a patient with congenital corrected transposition of the great arteries

The aim of this report is to emphasize the importance of thrombolytic therapy in selected patients, such as those with congenital heart defects in whom a coronary artery anomaly can be observed. We present here a 63 year-old female patient who was admitted to our emergency department with ST segment elevation myocardial infarction and a history of a congenital heart defect. We treated the patient successfully with thrombolytic therapy instead of primary percutaneous intervention, because of the suspicion of a coronary artery anomaly. On the following day, we performed coronary angiography on the patient, which revealed the anomalous origin of the coronary arteries, with the left and right coronary arteries originating from the right sinus of Valsalva and the circumflex artery originating from the left sinus of Valsalva. This anomaly in this patient group is described for the first time. Coronary artery anomaly may be observed in patients with congenitally corrected transposition of the great arteries, and in the case of requiring emergency reperfusion, thrombolytic treatment can be an alternative strategy in this patient group.

Identification of fetal cardiac anatomy and hemodynamics: a novel enhanced screening protocol.

BackgroundPrenatal cardiac screening is of great importance as it contributes to appropriate neonatal management and helps parents to make a decision regarding their pregnancy. The aim of our study was to evaluate the efficiency of a newly proposed screening protocol in the detection of fetal congenital heart disease (CHD).MethodsThis was a prospective study. A total of 52 cases of confirmed CHD fetuses and 248 cases of randomly selected normal fetuses were included in the study. Two sonographers with similar experience performed the cardiac screenings under two different protocols independently. The conventional protocol (Protocol A) paid greater attention to the four-chamber view and the outflow tract views. A 6-month training program was provided to sonographers performing scans under the new protocol (Protocol B), which emphasized systematically evaluating fetal cardiac anatomy and hemodynamics. Color Doppler was mandatory and some ultrasonic signs for special cardiac anomalies were also introduced into this protocol.ResultsProtocol B detected more cardiac anomalies than did Protocol A (96.2 % vs. 61.5 %, P < 0.01). Specifically, Protocol B was superior to Protocol A in detecting cardiac malpositions, abnormal systemic and pulmonary venous connection, right aortic arch, transposition of the great arteries, and congenital corrected transposition of the great arteries. By visualizing flow disturbance and retrograde flow with color Doppler, Protocol B was better than Protocol A in screening valvular associated malformations, such as pulmonary atresia, pulmonary stenosis, tricuspid dysplasia, etc. For the normal fetuses, Protocol B was better than Protocol A in reducing the false-positive detection of septal defects.ConclusionsThe current study introduces an enhanced protocol for fetal cardiac screening, under which the obstetric screening sonographers systematically identify fetal cardiac anatomy and hemodynamics. A short-term training program makes it possible for the screening sonographers to become familiar with the new protocol, and its value has been confirmed due to improvements made in screening efficiency.Electronic supplementary materialThe online version of this article (doi:10.1186/s12884-016-0933-9) contains supplementary material, which is available to authorized users.

Utility of dual source CT with ECG-triggered high-pitch spiral acquisition (Flash Spiral Cardio mode) to evaluate morphological features of ventricles in children with complex congenital heart defects.

We evaluated the ability of dual source CT (DSCT) with ECG-triggered high-pitch spiral acquisition (Flash Spiral Cardio mode) to depict the morphological features of ventricles in pediatric patients with congenital heart defects (CHD). Between July 2013 and April 2015, 78 pediatric patients with CHD (median age 4months) were examined using DSCT with the Flash Spiral Cardio mode. The types of ventricular abnormalities were ventricular septal defect (VSD) in 42 (the malaligned type in 11, perimembranous type in 23, supracristal type in 2, atrioventricular type in 2, and muscular type in 4), single ventricle (SV) in 11, and congenital corrected transposition of the great arteries (ccTGA) in 4. We evaluated the accuracy of the diagnosis of the VSD type. In cases of SV and ccTGA, we assessed the detectability of the anatomical features of both ventricles for a diagnosis of ventricular situs. DSCT confirmed the diagnoses for all VSDs. The type of defect was precisely diagnosed for all patients. The anatomical features of both ventricles were also depicted and ventricular situs of SV and ccTGA was correctly diagnosed. The results suggest that DSCT has the ability to clearly depict the configuration of ventricles.

Long-term course of a patient with congenital corrected transposition of the great arteries and dextrocardia — The Fisher King

Long-term course of a patient with congenital corrected transposition of the great arteries and dextrocardia — The Fisher King

The results of a two-stage double switch operation for congenital corrected transposition of the great arteries with a deconditioned morphologically left ventricle.

The purpose of this retrospective study was to evaluate a two-stage double switch operation, morphological left ventricular (mLV) retraining followed by an atrial-arterial switch operation, in the management of patients with congenitally corrected transposition of the great arteries (CCTGA) and a deconditioned mLV. Between May 2005 and May 2011, 14 patients with CCTGA and a deconditioned mLV anomaly underwent the two-stage double switch operation. There were eight males and six females aged between 2.5 and 72 months (mean: 34.4 ± 24.0 months) old and weighing from 5 to 23 kg (mean: 12.7 ± 4.9 kg). The major associated malformations included: tricuspid regurgitation (TR, n = 13); restrictive ventricular septal defect (n = 10); atrial septal defect or patent foramen ovale (n = 7); mild pulmonary stenosis (n = 5) and patent ductus arteriosus (n = 4). These patients underwent morphological left ventricular retraining by means of pulmonary artery banding under general anaesthesia, which was then followed by a double switch operation under general anaesthesia and cardiopulmonary bypass. There were no deaths or complications during the hospital stay or follow-up for the mLV retraining. In comparison with preoperative conditions, the mLV end-diastolic diameter (mLVEDd), the posterior wall thickness of the mLV and the mLV/mRV pressure ratio were all increased; the interventricular septum had moved partially to the midline position and TR had decreased. After the atrial-arterial switch procedure, 2 patients died during the perioperative period. The causes of death included serious cardiac arrhythmia with circulatory collapse and sudden death. The others were followed up for 2-8 years: 1 patient died from serious cardiac arrhythmias with circulatory collapse in the follow-up period. With regard to the others, 8 were evaluated as New York Heart Association Functional Class I, and the other 3 as Class II. Moderate aortic valve regurgitation was noted in 3 patients and moderate mitral regurgitation in 1 patient. For CCTGA children with degraded mLV, the two-stage double switch procedure can be performed with low mortality and morbidity and may be an appealing alternative to conventional repair. mLV retraining should be performed as early as possible. The second-stage atrial-arterial switch procedure showed satisfactory early and mid-term results. More attention should be paid to the long-term function of the mLV and the aortic valve.