ObjectiveTo evaluate clinical results of left ventricular retraining followed by double switch operation (DSO) for patients with congenitally corrected transposition of the great arteries (CCTGA) and a deconditioned morphologically left ventricle (mLV). MethodsClinical data of 14 patients with CCTGA and a deconditioned mLV who underwent surgical therapy in Fu Wai Hospital from May 2005 to May 2011 were retrospectively analyzed. There were 8 male and 6 female patients with their age of 2.5-72.0 (34.4±24.0) months and body weight of 5.1-23.0 (12.7±4.9) kg. Preoperative diagnosis was confirmed by echocardiography, angiography or cardiac catheterization. Major concomitant anomalies included tricuspid regurgitation (TR) in 13 patients, restrictive ventricular septal defect in 10 patients, atrial septal defect or patent foramen ovale in 7 patients, mild pulmonary valve stenosis in 4 patients, patent ductus arteriosus in 4 patients, and third-degree atrioventricular block in 1 patient. All the patients underwent first-stage morphologic left ventricular retraining under general anesthesia followed by second-stage atrial switch and arterial switch operations (DSO) under cardiopulmonary bypass with the interval of 0.67-34.0(10.23±9.47)months. ResultsAfter the first-stage morphologic left ventricular retraining, there was no postoperative complication or death. During follow-up, mLV end-diastolic diameter (mLVEDd) and posterior wall thickness of mLV were significantly larger than preoperative parameters (P < 0.05). The interventricular septum moved partially towards morphologically right ventricle (mRV). TR degree was significantly decreased, the pressure gradient across the pulmonary artery band was significantly increased (P < 0.05), and left ventricular ejection fraction (LVEF) was not statistically different from preoperative LVEF. And mLV/mRV pressure ratio was significantly increased (P < 0.05). After the second-stage DSO, 2 patients died with the in-hospital mortality of 14.3% (2/14). The causes of death included serious arrhythmia, circulatory collapse and sudden death. Early postoperative complications included pulmonary infection in 6 patients, atrial arrhythmias in 2 patients, pleural effusion in 2 patients, pneumothorax in 1 patient, diaphragmatic paralysis cured by diaphragm placation in 1 patient, respiratory tract hemorrhage in 1 patient, mild aortic insufficiency in 1 patient, peritoneal dialysis for 1 patient, extracorporeal membrane oxygenation for 1 patient, and tracheal intubation for a second time for 1 patient. All the 12 patients who were discharged alive were followed up for 2 to 8 years. One patient died during follow-up with the late mortality of 8.33% (1/12), and the cause of death was serious arrhythmia and circulatory collapse. Eight patients were in New York Heart Association (NYHA) classⅠ, and 3 patients were in NYHA class Ⅱ. Major late complications included left ventricular dysfunction in 3 patients, moderate aortic valve regurgitation in 3 patients, and moderate mitral valve regurgitation in 1 patient. ConclusionShort-term clinical results of left ventricular retraining followed by DSO for patients with CCTGA and a deconditioned mLV are satisfactory, and its middleand long-term results need further follow-up. But postoperative left ventricular dysfunction and new-onset aortic valve regurgitation deserve more attention.
ObjectiveTo evaluate the effects of anatomic correction for congenitally corrected transposition of the great arteries (ccTGA) and 10-year follow-up.MethodsFrom January 2008 to December 2018, 48 patients with ccTGA who underwent anatomic correction were reviewed. There were 29 males and 19 females with age of 39.2 (3-91) months. The cohort was divided into two groups: a biventricular anatomic correction group (39 patients) and a 1.5 ventricular anatomic correction group (9 patients). They were followed for in-hospital mortality, late mortality, long-term survival, freedom from reoperation, and heart function.ResultsThere were 3 early deaths and 2 early re-intervention in the biventricular anatomic correction group, but no death and only one re-intervention in the 1.5 ventricular anatomic correction group. Compared with the biventricular anatomic correction group, the operation time, tracheal intubation and ICU time were significantly reduced or shortened in the 1.5 ventricular anatomic correction group (P<0.05). The patients were followed up for 0.5-10.4 years. Four patients were lost. Two patients died in the biventricular anatomic correction group, and two patients received re-intervention. The 1-year, 5-year and 10-year survival rate was 88.2%, 84.0%, and 84.0%, respectively. There was no death or intervention in the 1.5 ventricular anatomic correction group. The quality of life of the other patients in the medium-term follow-up was satisfactory. Only 2 patients were classified as grade Ⅲ in cardiac function, and the other patients were classified as grade Ⅰ-Ⅱ.ConclusionAccording to the different anatomic characteristics of ccTGA, the individualized strategy of anatomic correction can achieve satisfactory surgical results, and the medium-term quality of life was good. Especially, 1.5 ventricular anatomic correction may obtain better therapeutic effects because of its lower operative mortality and less postoperative complications.
Objective To compare the early and mid-term results between Fontan operation and anatomic correction for congenitally corrected transposition of the great arteries (ccTGA). Methods The clinical data of 53 patients with ccTGA who underwent anatomic correction and Fontan operation from January 2009 to September 2021 in our hospital were reviewed, including 41 males and 12 females with a mean age of 55.02 (3-168) months. They were divided into an anatomic correction group (16 patients) and a Fontan operation group (37 patients) according to the operation. The hospitalization mortality, survival rate, postoperative complications, and free rate from re-intervention between the two groups were compared. Another 180 healthy children were recruited as a control group, and 14 children were matched with the propensity score matching method as a Fontan control group. The results of cardiopulmonary exercise testing (CPET) between the Fontan operation group and the Fontan control group were compared. Results There were 2 (12.5%) early deaths and 3 (18.8%) early re-intervention in the anatomic correction group, while 1 death and 2 re-intervention in the Fontan operation group. In addition, there were 9 patients (56.3%) in the anatomic correction group and 6 (16.2%) patients in the Fontan operation group suffering from arrhythmia after operation, respectively. Compared with the anatomic correction group, cardiopulmonary bypass time, aortic cross-clamping time, intubation time and ICU stay were significantly shortened in the Fontan operation group (P<0.05). CPET results showed that, percent predicted max VO2 in the Fontan operation group was lower than that in the Fontan control group (0.84±0.11 vs. 0.99±0.12, P＜0.05). The patients were followed up for 0.5-126.0 months. Two patients were lost in the Fontan operation group. There was no death and 1 re-intervention in the anatomic correction group, while no death or re-intervention in the Fontan operation group. The 1-year, 5-year and 10-year transplant-free survival rate of the anatomic correction group and the Fontan operation group was 87.5%, 87.5%, 87.5% and 97.3%, 97.3%, 97.3%, respectively (P＞0.05). The 48 patients were classified as grade Ⅰ-Ⅱ in cardiac function in the last follow-up. Conclusion There is no statistical difference in the transplant-free survival rate between the anatomic correction and the Fontan operation group. The postoperative complications in the Fontan operation group are decreased than those in the anatomic correction group. The Fontan operation is also a good choice, even though the patients with ccTGA meet the condition of the procedure of anatomic correction.