Objective To summarize the clinical experiences of using the remaining coronary buttons to reconstruct the neoaortic root in the arterial switch operation (ASO) and discuss the clinical significance of preserving the morphology of aortic sinus in improving postoperative coronary artery perfusion. Methods From January 2003 to June 2009, 110 patients with transposition of great arteries (TGA) combined with ventricular septal defect (VSD ) or the Taussig Bing anomaly treated in our hospital were enrolled in this study. The patients were at the age between 2 days and 2 years averaged 91.1 days, and their body weight varied between 1.79 and 9.50 kg with an average weight of 4.70 kg. The patients were divided into two groups based on different surgical strategies. For group A (n=78), we reconstructed the neoaortic root with the remaining coronary buttons by shortening the diameter of the proximal neoaorta. While for group B (n=32), we excised the remaining coronary buttons with the aortopulmonary diameter mismatched. The risk factors of hospital mortality were analyzed with stepwise logistic regression. Results Twelve patients died early after operation with a death rate of 10.9% (12/110). Mortality in group A was significantly lower than that in group B ［6.4%(5/78) vs. 21.9%(7/32)，P= 0.019］. Clinical followup was completed in 72 survivors with the followup time of 3 months to 5 years after operation. Late death occurred in 3 patients including 1 in group A and 2 in group B. Five patients underwent reoperations. Univariate logistic regression analysis showed that risk factors for early postoperative death were TaussigBing anomaly (χ2=4.011,P=0.046), aortic arch anomaly (χ2=4.437,P=0.036), single coronary artery pattern (χ2=5.071,P=0.025) and patients in group B (χ2=5.584, P= 0.019). Multivariate analysis confirmed that the aortic arch anomaly (χ2= 5.681, P=0.010 ) and patients in group B (χ2=3.987, P=0.047 ) were two independent risk factors for early mortality after operation. Conclusion The modified technique which uses the remaining coronary buttons to reconstruct the neoaortic root can preserve the morphology of neoaortic root better. The special anatomical morphology of aortic sinus has close relation to the perfusion of coronary arteries. The lowering hospital mortality may be due to the better perfusion of the coronary arteries.
ObjectiveTo summarize the surgical experience of infants with transposition of the great arteries (TGA) and intramural coronary artery (IMCA) in our center, and analyze the early and mid-term outcomes.MethodsWe retrospectively analyzed the clinical data of 384 infants with TGA undergoing arterial switch operation (ASO) from June 2010 to December 2018 at Fuwai Hospital. According to operative records, 21 (5.5%) infants had IMCA, among whom 20 were males, with a median age of 33 (9-319) d. Coronary transfer using double coronary buttons with unroofed intramural course was performed in all 21 infants.ResultsThere was no statistical difference in the early mortality after ASO between infants with IMCA and infants with normal coronary anatomy (9.5% vs. 3.0%, P=0.15). In the IMCA group, 2 dead patients presented inadequate coronary artery perfusion after first aortic unclamping. In addition, 1 patient underwent extracorporeal membrane pulmonary support for myocardial dysfunction. The follow-up was available for all 19 survivors, with an average follow-up time of 29.0-120.0 (74.8±27.3) months. During the follow-up, all patients had no obvious symptoms, death, reoperation, or coronary complications. One patient developed moderate pulmonary valve regurgitation and another patient developed distal stenosis of the right pulmonary artery.ConclusionFor infants with TGA and IMCA, coronary transfer using double coronary buttons with unroofed intramural course is a safe and reliable technique, with satisfactory early and mid-term outcomes.
Objective To evaluate the impact of an integrated management mode of prenatal diagnosis-postnatal treatment for congenital heart disease (CHD) on perioperative and long-term outcomes of the arterial switch operation (ASO), and to analyze the efficacy of ASO over six years in a single center. Methods This retrospective study analyzed the clinical data of 183 children who underwent ASO at Guangdong Provincial People's Hospital from January 2018 to December 2024. The cohort included 106 patients (57.9%) of transposition of the great arteries with intact ventricular septum (TGA/IVS), 61 patients (33.3%) of transposition of the great arteries with ventricular septal defect (TGA/VSD), and 16 patients (8.7%) of taussig-bing anomaly (TBA). Perioperative indicators were compared between 91 patients in the prenatal-postnatal integrated management group (an integrated group) and 92 patients in the traditional management group (a non-integrated group). Long-term survival and reoperation rates were analyzed using Kaplan-Meier curves. Results The overall perioperative mortality rate was 4.9% (9/183), showing a downward trend year by year. The primary cause of perioperative mortality was low cardiac output syndrome (LCOS), which occurred in 12 patients (6.6% incidence) with a mortality rate of 75%. The integrated group had a higher proportion of males (89% vs. 72.8%, P<0.05) and lower body weight [3.13 (2.75, 3.35) vs. 3.30 (3.00, 3.67), P<0.05] compared to the non-integrated group. The age at surgery was significantly earlier in the integrated group [7 (3, 10) vs. 14 (9, 48), P<0.05], and all children in the Integrated Group underwent ASO within the optimal surgical window (100% vs. 82.6%, P<0.05). Intraoperatively, cardiopulmonary bypass (CPB) time [173 (150, 207) vs. 186 (159, 237), P<0.05] and aortic cross-clamp (ACC) time [100 (90, 117) vs. 116 (97, 142), P<0.05] were significantly shorter in the integrated group. although the integrated group had longer postoperative mechanical ventilation time [145 (98, 214) vs. 116 (77, 147), P<0.05] and higher 48-hour maximum vasoactive inotropic score (VISmax) [15 (10, 21) vs. 12 (8, 16), P<0.05], there was no statistically significant difference in the incidence of severe complications (LCOS, NEC, ECMO) or mortality rate (3.3% vs. 6.5%, P=0.51) between the two groups, despite earlier surgical intervention and a higher proportion of critically ill cases in the integrated group. The length of hospital stay in the emergency surgery group was significantly shorter than that in the elective surgery group [20 (15, 28) vs. 25 (21, 30), P<0.05], suggesting that early surgery may be of potential benefit. A total of 163 patients were successfully followed up for a median of 4.7 years, with a 5-year survival rate of 95.1% and a freedom from reintervention survival rate of 95.1%. There were no late deaths, and the most common postoperative complication was pulmonary artery stenosis. Conclusion The integrated management model allowed critically ill children with lower body weights to safely undergo surgery, significantly optimizing the timing of surgery and shortening intraoperative times. The long-term risk of reoperation after ASO is primarily concentrated on pulmonary artery stenosis, necessitating long-term follow-up and monitoring.
目的：采用Rastelli术治疗合并室间隔缺损、肺动脉狭窄、完全性大动脉转位病人，探讨围手术期麻醉管理特点。方法：选用大剂量芬太尼静脉复合麻醉方案，在深低温、低流量体外循环下实施Rastelli手术并进行多指标监测和临床观察。结果：麻醉经过平稳，围手术期患者生命体征稳定。结论：选择恰当的麻醉方案，有效的心脏保护,保持酸碱平衡和水、电解质稳定是成功进行麻醉管理的必要条件。
Objective To introduce a modified REV procedure of complicated transposition of the great arteries （TGA） or double outlet right ventricle （DORV） which was combined with ventrieular septal defect （VSD） and pulmonary valve stenosis（PS）. Methods From Sep. 2005 to Feb. 2006, 3 children with complicated transposition of the great arteries underwent a modified REV operation. This modified REV operation was designed on the basis of classical REV procedure to preserve the native pulmonary artery valve and its function. Results Two patients recovered uneventfully but one died after extraeorporeal membrane oxygenator （ECMO） treatment. After 4 and 1 months follow-up respectively, the discharged 2 patients were asymptomatie and the eehoeardiography revealed that the pressure gradient between left ventrieular-main pulmonary were estimated to be 15 and 5mmHg. Conclusion This modified REV operation for preservation of pulmonary artery valve is an ideal procedure to complicated transposition of the great arteries. Advantages and disadvantages of this modified REV procedure were discussed.
ObjectiveTo evaluate the long-term clinical effect and risk factors of tricuspid valve replacement (TVR) as a relief treatment for adult patients with congenitally corrected transposition of the great artery (CCTGA).Method We retrospectively analyzed the clinical data of 47 adult patients with CCTGA who underwent tricuspid valve replacement in Fuwai Hospital between 2000 and 2017 year. There were 27 males and 20 females with operation age of 14–62 (38.8±13.5) years. Preoperative echocardiography showed moderate or more tricuspid regurgitation in all patients. The basic data of patients before and during operation were recorded. Survival was followed up by telephone and ultrasound report.ResultsThe average follow-up time was 6.5±3.7 years. The 1-year, 5-year and 10-year survival rate or the incidence of heart transplant-free was 94.6%, 90.5% and 61.7%, respectively. During the follow-up period, the long-term right ventricular ejection fraction of most patients (>90%) was still greater than or equal to 40%. Increased preoperative right ventricular end diastolic diameter (RVEDD) was a risk factor for death or heart transplantation (risk ratio 1∶11, P=0.04). The survival rate of patients with RVEDD (>60 mm) before operation was significantly reduced (P=0.032).ConclusionTVP is a feasible treatment for adult patients with CCTGA. The increase of preoperative RVEDD is a risk factor for long-term mortality.
Abstract： Objective?To evaluate clinical experiences and long-term outcome of morphologic left ventricle （mLV） retraining for congenitally corrected transposition of the great arteries （cCTGA）. Methods From May 2005 to May 2011, 24 patients with cCTGA anomaly underwent left ventricle retraining by means of pulmonary artery banding in Fu Wai Hospital. There were 13 males and 11 females with their age of 0.17-22.00 （3.73±4.35） years and body weight of 5.10-61.00（15.71±10.95）kg. Major concomitant malformations included tricuspid valve insufficiency （TR）in 23 patients （mild in 11 patients, moderate in 7 patients, severe in 5 patients）, restrictive ventricular septal defect in 18 patients, atrial septal defect in 5 patients, patent foramen ovale in 5 patients, patent ductus arteriosus in 4 patients, mild pulmonary stenosis in 5 patients, and aortic coarctation in 1 patient. All the patients were preoperatively diagnosed by echocardiography, cardiovascular angiography or cardiac catheterization. The mLV end diastolic diameter （mLVEDD） was 8-32（21.56±6.60）mm, posterior wall thickness of mLV was 2-7 （4.29±1.52）mm , mLV to morphologic right ventricle （mRV） pressure ratio （mLV/mRV） was 0.12-0.65 （0.41±0.12）. Pulmonary artery banding operation was performed through upper partial sternotomy or median sternotomy without circulatory arrest. Results The mLV/mRV pressure ratio reached to 0.57-0.93 （0.76±0.10） under direct pressure monitoring after surgery. There was no in-hospital death in this group. Echocardiography before discharge showed that the structure and function of the two ventricles were good, the interventricular septum moved partially towards mRV, mLVEDD was increased slightly, and there was a tendency of reduced TR. Postoperative follows-up was from 1 to 35 months, and there was no late death during follow-up. All the patients were in good general condition with stable vital signs and New York Heart Association （NYHA） classⅠ-Ⅱ. The mLVEDD was 14-40 （26.17±7.11） mm, posterior wall thickness of mLV was 4-9 （4.95±1.44）mm, mLV/mRV pressure ratio was 0.52-0.98 （0.72±0.16） , and TR was significantly decreased. Fourteen patients successfully underwent staged complete double-switch procedure. Conclusion Left ventricle retraining is a safe and effective method to train mLV for cCTGA patients. Pressure load and posterior wall thickness of mLV are increased, mLV cavity is dilated, and TR is significantly reduced after the surgery.
Abstract： Objective To investigate the early and mid-term outcomes of morphologic tricuspid valve replacement by means of intravalvular implantation in corrected transposition of great arteries（cTGA）. Methods From January 2009 to January 2012，11 patients with cTGA were surgically treated in Fu Wai Hospital. There were 9 male patients and 2 female patients with their mean of age of（37.8±11.7）years and mean body weight of（73.0±11.3）kg. All the patients underwent morphologic tricuspid valve replacement with preservation of the entire valvular and subvalvular apparatus. Simultaneous surgical procedures included repair of ventricular septal defect in 2 patients，repair of atrial septal defect in 4 patients，pulmonary valvuloplasty in 1 patient，reconstruction of functional right ventricular outflow tract in 4 patients and repair of coronary-pulmonary artery fistula in 1 patient. Postoperative New York Heart Association （NYHA） classification, cardiothoracic ratio, morphological right ventricle ejection fraction, end-diastolic dimension of morphological right ventricle and left atrium were evaluated during follow-up. Results All the 11 patients were successfully surgically treated and followed up for an average duration of（13.0±10.6）months. There was no statistical difference between postoperative and preoperative average cardiothoracic ratio （0.54±0.06 vs. 0.57±0.09，t=1.581，P＞0.05），morphologic right ventricle ejection fraction （52.8%±9.0% vs. 54.9%±9.5%, t =0.712，P＞0.05），and end-diastolic dimension of . morphological right ventricle （54.3±7.5 mm vs. 56.9±9.2 mm，t =0.988，P＞0.05）. There was statistical difference between postoperative and preoperative average end-diastolic dimension of left atrium（42.1±8.9 mm vs. 53.4±11.1 mm，t =3.286，P＜0.05）and NYHA classification（Z = -2.640，P＜0.05）. Conclusion Intravalvular implantation of morphologic tricuspid prosthesis can protect the physiological structure of morphologic right ventricular and prevent furtherdamage to its function caused by morphologic tricuspid valve insufficiency. Postoperative dimension of morphologic left atrium and cardiac function are significantly improved. The early and mid-term outcomes are satisfactory．
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.