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.
Abstract： Objective?To summarize the clinical experience，surgical technique and indication of coronary artery implantation with double flap extension technique in arterial switch operations （ASO） in D-transposition of the great arteries （D-TGA） and Taussig-Bing anomalies.?Methods?From January 2006 to June 2011， 21 patients （13 males and 8 females；age 110.0±84.5 d；weight 5.4±4.2 kg） with D-TGA or Taussig-Bing anomalies associated with complex coronary artery malformations underwent ASO with double flap extension technique for coronary artery implantation in Shanghai Children’s Medical Center affiliated to Medical College of Shanghai Jiaotong University. All the patients had a main trunk of right coronary artery or dilated right ventricular conus branch originated from the left or right aortic sinus，with abnormal course of anterior looping to the aorta. The double flap extension technique was described as followed： a long coronary button was excised as a flap from the aorta; another pedicle flap on the pulmonary artery （neoaorta） was cut to extend to the button of coronary artery with an equal distance; the side edges of the flap and the button were sutured together to form a lengthened coronary artery tube.?Results?No operative death occurred in hospital. The postoperative duration of mechanical ventilation was 101.6±53.6 h. The duration of ICU stay was 9.5±4.9 d. Postoperatively，low cardiac output syndrome occurred in 9 cases，pulmonary hypertension crisis in 2 cases，pneumonia in 6 cases，and acute kidney failure in 2 cases. Eleven patients underwent delayed sternum closure. All the patients were discharged after proper treatment. Follow-up was complete in 17 cases. The duration of follow-up was 2 months to 5 years. Growth and development were significantly improved in all the patients during follow-up. No patient had ischemic ECG changes. One patient underwent reoperation for supravalvular pulmonary stenosis 2 years after ASO.?Conclusion?Double flap extension technique for coronary implantation in complicated ASO can significantly decrease postoperative death due to coronary artery malformations，especially for patients who have two-stage ASO and patients whose main trunk of right coronary artery or dilated right ventricular conus branch originates from the left or right aortic sinus with abnormal course of anterior looping to the aorta.
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.
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.
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 define the patient characteristics and perioperative management, and to define the mortality and its risk factors after arterial switch operation (ASO).MethodsWe conducted a bidirectional cohort study with 571 consecutive patients undergoing ASO from 1997 to 2016 in our hospital. We enrolled patients who underwent ASO before 2012 retrospectively and after 2012 prospectively and followed up all the patients prospectively. Demographic characteristics, clinical information and mortality of these patients were summarized. Joinpoint regression analysis was used to identify the time trend of the overall mortality. Kaplan-Meier survival analysis was used to evaluate the mid- and long-term survival rate after ASO. Cox proportional hazards regression models were used to explore the potential factors associated with mortality. The cumulative incidence of complications after ASO was predicted using competing risk models.ResultsSeveral aspects of patients’ characteristics and perioperative management in our center differed from those in the developed countries. The overall mortality and in-hospital mortality after ASO was 16.3% and 15.1%, respectively. The overall cumulative survival rate at 5, 10 and 15 years after ASO was 83.3%, 82.8% and 82.8%, respectively. A significant decrease of overall mortality from 1997 to 2016 was observed. Independent risk factors of mortality included earlier ASO (1997-2006), single or intramural coronary anatomy and longer cardiopulmonary bypass time. Ten years after ASO, re-intervention, arrhythmia, pulmonary and anastomotic stenosis were the most common complications with a cumulative incidence over 10%.ConclusionSignificant improvements in the results of the ASO were observed and the postoperative mortality rate is close to reports from developed countries. Nonetheless, we have identified the need for further improvement in the early and late postoperative periods after ASO. Pulmonary stenosis, anastomotic stenosis and arrhythmia should be paid attention to during the long-term follow-up after ASO.
摘要:目的:回顾性研究大动脉转换术同时进行主动脉弓矫治的I期手术治疗完全性大动脉错位或TaussigBing合并主动脉弓畸形的早中期效果。方法:2000年1月至2008年12月,连续对26例存在主动脉弓畸形的完全性大动脉错位或TaussigBing畸形的小婴儿进行了I期手术矫治,其中完全性大动脉错位13例(TGA/VSD 11例,TGA/IVS 2例),TaussigBing 13例;主动脉弓畸形中主动脉弓中断(A型)7例,CoA19例,6例伴有冠状动脉异常类型。平均手术年龄(28±35) d,lt;2个月占62％,手术平均体重为(4.19±1.15) kg。在深低温停循环或深低温低流量下进行主动脉弓畸形矫治,采用自身组织直接吻合扩大或重建弓,伴有弓部发育不良者补片扩大成形。伴有冠状动脉畸形者在大动脉转换手术中冠状动脉移植方法予改良处理。〖HTH〗结果〖HTSS〗:手术住院死亡3例(11.5%),死因与冠脉移植无关。平均插管时间102 h,监护室时间平均8 d。术后早期生存者主动脉瓣上压力阶差gt;30 mm Hg有2例,主动脉瓣反流轻度2例。单因素分析中伴有冠状动脉异常类型者与术后早期死亡或并发症的风险相关,多因素分析示其与手术年龄、肺动脉高压、术前FS、主动脉阻断时间、术后血清乳酸水平相关。随访期3个月~7年,无死亡,术后5年实际生存率为88.5％(95％ 可信度范围CI 76％～96％),术后1年、5年无需介入干预或手术分别为91.4％、87％。结论:TGA和TaussigBing伴有主动脉弓畸形者I 期进行大动脉转换术和主动脉弓畸形矫治早中期效果良好,早期手术并发症和死亡的风险因素为年龄偏大,肺高压严重,把握手术时机是手术成功要则之一。Abstract: Objective: The study was to evaluate earlymid term results after onestage arterial switch operation (ASO) associated with aortic arch repair for D Transposition of the great arteries (DTGA) and TaussigBing Anomaly with arch abnormally in infant. 〖WTHZ〗Methods〖WTBZ〗: Between January 2000 and December 2008, a primary operation including aortic arch repair through a midline sternotomy was performed in 26 patients, 13 patients with DTGA and 13 TaussigBing. Most patients (62%) underwent operation during the first two months. The repair of arch was accomplished under deep hypothermic circulatory arrest or low flow, employing a wide pericardial patch to reconstruction of arch in some patients or direct ananstomosis. Results: There were 3 (11.5%) hospital deaths. The high risk factors for early mortality and morbidity were unsuitable reconstructed arch, higher age, severe pulmonary hypertension and longer aortic crossclamp time. There were no late deaths. Actuarial 5year survival was 88.5% (95% CI 70% to 96%). Actuarial freedom from overall reintervention, reoperation among operative survivors was 91.4% at 1 year and 87% at 5 years, respectively. Conclusion: the singlestage repair for DTGA and TaussigBing with aortic arch abnormally is suitable choice for infant, and followup of operative survivors is favorable. Optimal operative time was as sooner as possible.
Abstract： Objective To evaluate clinical outcomes of pulmonary artery banding for morphologic left ventricular training in corrected transposition of the great arteries.?Methods?A total of 89 patients with corrected transposition of the great arteries underwent surgical repair in Shanghai Children’s Medical Center from January 2007 to December 2011 year. Among them, 11 patients underwent pulmonary artery banding, whose clinical records were retrospectively analyzed. Except that one patient was 12 years, all other patients were 3 to 42 （16.40±11.67） months old and had a body weight of 6 to 32 （11.70±7.20）kg. All the patients were diagnosed by echocardiogram and angiocardiogram.?Results?There was no postoperative death after pulmonary artery banding in 11 patients. The pulmonary arterial pressure/systemic blood pressure ratio （Pp/Ps） was 0.3 to 0.6 （0.44±0.09） preoperatively and 0.6 to 0.8 （0.70±0.04） postoperatively with statistical difference （P＜0.01）. Tricuspid regurgitation was mild in 2 （18.2%） patients, moderate in 5 （45.4%）, severe in 4 （36.4%）preoperatively，and none in 2（18.2%）patients, mild in 7 （63.6%），and mild to moderate in 2 （18.2%）postoperatively. Five patients underwent staged double-switch operation after pulmonary artery banding at 15.20±8.31 months, and 1 patient died. The other 6 patients were followed up for 18.83±3.43 months, and echocardiogram showed tricuspid regurgitation as trivial in 2 （33.3%）, mild in 3 （50.0%）, and moderate in 1 （16.7%）patient.?Conclusions?In patients with corrected transposition of the great arteries, pulmonary artery banding is helpful to reduce tricuspid regurgitation, and morphologic left ventricle can be trained for staged double-switch operation with good clinical outcomes. It is important to follow up these patients regularly to evaluate their morphologic left ventricular function and tricuspid regurgitation after pulmonary artery banding.
A 23-year-old male patient was diagnosed with congenitally corrected transposition of the great arteries (ccTGA) at the age of 3 during a routine physical examination. Due to the absence of significant symptoms, the patient was managed conservatively with follow-up without surgical treatment. He developed chest tightness one year prior to admission, with symptom exacerbation in the recent month. Echocardiography demonstrated significantly worsened morphological tricuspid regurgitation. The patient presented to Guangdong Provincial People’s Hospital for surgical evaluation. Given the patient’s age and clinical presentation, a totally thoracoscopic morphological tricuspid valve replacement was performed. Postoperative assessment showed good prosthetic valve function, with no regurgitation or paravalvular leakage; the electrocardiogram confirmed sinus rhythm without atrioventricular block. Extubation occurred within 12 hours postoperatively, and the patient was transferred from the intensive care unit (ICU) on postoperative day 2. At the three-month follow-up, the patient was asymptomatic and had resumed normal activities. This report describes the first case of a totally thoracoscopic morphological tricuspid valve replacement in a ccTGA patient in China. This initial experience suggests that the totally thoracoscopic approach for morphological tricuspid valve replacement in ccTGA patients is a feasible, safe, and effective minimally invasive option associated with rapid recovery.
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.