Early and mid-term results of Fontan operation versus anatomic correction for congenitally corrected transposition of the great arteries: A retrospective cohort study in a single center / 中国胸心血管外科临床杂志

@#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.

The myocardial protection of HTK versus del Nido cardioplegia solutions in neonates with surgeries for transposition of the great arteries : A propensity score matching study / 中国胸心血管外科临床杂志

@#Objective    To compare and analyze the effect of myocardial protection between HTK and del Nido cardioplegia solutions in neonates with surgeries for transposition of the great arteries. Methods    The clinical data of 208 neonates with complete transposition of the great arteries in our institution from 2014 to 2020 were retrospectively analyzed. According to the cardioplegia solutions utilized in the operations, the patients were divided into two groups: a HTK group and a del Nido group. Propensity score matching was conducted to eliminate the biases. The cardiopulmonary bypass time, aortic cross-clamping time, total amount of cardioplegia solutions, transfusion frequency of cardioplegia, ICU stay time, mechanical support time, inotropic score, hospital stay, left ventricular ejection fraction, N-terminal proBNP and troponin I were compared and analyzed between the two groups after matching. Results    After 1:1 propensity score matching, a total of 54 patients were analyzed with 27 patients in each group. In the HTK group, there were 22 males and 5 females with a median age of 7.0 (2.0, 11.0) d. In the del Nido group, there were 23 males and 4 females with a median age of 8.0 (3.0, 11.0) d. A total of 3 children died after the surgery: 2 (7.4%) patients in the HTK group and 1 (3.7%) patient in the del Nido group. There was no significant difference in hospital mortality between the two groups (P=1.000). The total amount of cardioplegia solutions in the HTK group was significantly higher than that of del Nido group (P<0.001). Transfusion frequency of cardioplegia in del Nido group was significantly higher than that of the HTK group (P=0.043). There was no significant difference in the postoperative ICU time, mechanical support time, length of hospital stay, inotropic score, left ventricular ejection fraction, N-terminal B-type natriuretic peptide precursor or troponin I between the two groups (P>0.05). Conclusion    For neonates with surgeries for complete transposition of the great arteries, HTK cardioplegia solutions can provide effective and safe myocardial protection, which is similar to del Nido cardioplegia solutions.

Ultrasonographic diagnosis and prognosis of transposition of the great arteries at 11-13 + 6 weeks gestation / 中华超声影像学杂志

Objective:To investigate the value of ultrasonography in diagnosis of transposition of great arteries of the fetus at 11-13 + 6 weeks gestation. Methods:A prospective study was conducted on fetuses screened by ultrasound in the first trimester in Maternal and Child Health Hospital of Guangxi Zhuang Autonomous Region between January 2015 and March 2022. Fetal heart structure was screened by three-section screening method. Fetuses with suspected transposition of the great arteries at 11-13 + 6 weeks gestation underwent followed-up ultrasound examination, chromosome and gene test results. The ultrasound characteristics and prognosis pregnancy outcomes were summarized. Results:Twenty-one cases of transposition of the great arteries were detected by ultrasonography, including complete transposition of great arteries (20 cases) and congenitaly corrected transposition of the great arteries (1 case). Two cases were miss diagnosed. Twenty-one cases showed parallel signs of two major arteries on grayscale outflow section at 11-13 + 6 weeks gestation. There were 6 cases with aneuploid ultrasonographic soft markers abnormality, 2 cases with extracardiac malformation. Chromosome and microarray analysis were performed in 13 cases. 4 cases with chromosomal abnormality. Four cases of chromosomal abnormalities were associated with ultrasonographic soft markers abnormality, and 1 case with extracardiac malformation.In the 23 cases, 20 cases were induced, 1 miscarried, and 2 delivered to term. Among the fetuses delivered at term, 1 case died before neonatal operation and 1 case survived. Conclusions:Standardized ultrasound scan at 11-13 + 6 weeks has high accuracy in diagnosis of transposition of the great arteries. And the incidence of chromosomal abnormality is high with ultrasonographic soft markers abnormality or extracardiac malformation.

Congenitally Corrected Transposition of the Great Arteries in the Adult

ABSTRACT Introduction: Congenitally corrected transposition of the great arteries (CCTGA) is a rare anomaly. Current data available regarding adult cases is derived from small series, information simultaneously presented in pediatric publications, and one classical multicenter study. This review, not aimed to exhaust the subject, has the purpose to examine the literature addressing presentation, diagnostic methodology, and management of afflicted adult patients. Methods: A comprehensive search was undertaken in three major databases (PubMed, Cochrane, SciELO), using the keywords "congenitally corrected transposition of the great arteries" and "adults". Relevant articles in English, Spanish, and Portuguese were extracted and critically appraised in this review. Steps for study selection were: (1) identification of titles of records through databases searching, (2) removal of duplicates, (3) screening and selection of abstracts, (4) final inclusion in the study. Results: Four hundred sixty-five publications on CCTGA in adult patients were retrieved, and 166 were excluded; 299 studies were used for this review including 76 full-text articles, 70 studies related to general aspects of the subject, and, due to the small number of publications, 153 case reports. Sixty-one articles referring to combined experiences in pediatric and adult patients and judged to be relevant, but retrieved from another sources, were also included. Conclusion: Albeit clinical presentation and diagnostic criteria have been well stablished, there seems to be room for discussion related to clinical and surgical management of CCTGA in adults. Considering the rarity of the disease, well designed multicenter studies may provide answers.

Analysis of early death and long-term reoperation after 11-year single-center arterial switch operation / 中华胸心血管外科杂志

Objective:To analyze the early postoperative mortality and long-term reoperation of ASO and to explore the surgical risk and the reasons of long-term reoperation.Methods:The clinical data and follow-up data of ASO children undergoing surgery in Shanghai Children Medical Center from January 2010 to December 2020 were analyzed retrospectively. Four groups were divided into transposition of the great vessels(TGA/IVS) , transposition of the great vessels with ventricular septal defect(TGA/VSD) , Taussig-bing anomaly(TBA) , and two stage ASO(Ⅱ-ASO) groups. χ2 test was used to analyze the early mortality and long-term reoperation rates of ASO in different groups. Results:A total of 861 ASO patients were included in this study and 108 died early(12.5%, 108/861) . Seven hundred and fifty three cases were followed up and 102 cases were lost(13.5%, 102/753) . The median follow-up time was 7.23 years and the quartile interval was 4.74-9.37 years old. Sixty six patients(10.1%, 66/651) underwent long-term reoperation. Four patients(6%, 4/66) died after reoperation. In 241 cases of TGA/IVS, 24 cases(10%) were performed reoperation. In 256 cases of TGA/VSD, 23 cases(9%) had reoperation. In 126 cases of TBA, 18 cases(14.3%) for reoperation. And in 28 cases of Ⅱ-ASO only 1 case(3.6%) had reoperation. Among all the reoperation cases, there were 36 cases(2 deaths) for pulmonary angioplasty, 2 cases for pulmonary stenting, 10 cases for right ventricular outflow tract obstruction(RVOTO) repair, 2 cases for aortic valvularplasty, 5 cases for aortic valve replacement(2 deaths), 4 cases for aortic anastomotic stenosis repair, 3 cases for left ventricular outflow tract obstruction(LVOTO) repair, 2 cases for VSD residual shunt repair and 2 cases for coarctation of the aorta(CoA) correction.Conclusion:The early mortality rate of ASO surgery is still higher than that of developed countries. Long-term follow-up after ASO surgery should focus on right ventricular outflow tract stenosis and aortic valve insufficiency.

Surgical treatment of infants with transposition of the great arteries and intramural coronary artery / 中国胸心血管外科临床杂志

@#Objective    To 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. Methods    We 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. Results    There 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. Conclusion    For 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.

Clinical outcomes of arterial switch operation in infants with transposition of the great arteries--A single center clinical experience / 中华胸心血管外科杂志

Objective:To retrospectively analyze the efficacy of arterial switch operation in infants with transposition of the great arteries and to explore the prognostic related factors.Methods:The clinical data of 381 children with transposition of the great arteries from October 2001 to December 2017 were collected, including anatomical diagnosis, age of surgery, preoperative status, coronary artery malformation, aortic arch disease, etc. The relevant factors of postoperative mortality and reintervention were analyzed.Results:The overall mortality rate is about 4.5%, and the reintervention rate is about 3.7%. The postoperative mortality of every 100 cases droped significantly ( P<0.05), early surgery did not increase the risk of surgical death, but the mortality rate in the emergency surgery group was higher than that in the non-emergency surgery group. The mortality in the combined coronary artery abnormality group was significantly higher than that in the normal coronary artery group. Patients with Taussig-Bing anomaly and abnormal aortic arch were significantly more likely to get worse outcomes than those without aortic arch abnormality. In the whole group, 14 patients were re-intervened due to pulmonary valve or supra-valvular stenosis, aortic arch constriction, left ventricular outflow obstruction, and new aortic valve regurgitation. One patient died after operation. There was no coronary reintervention in the middle and long-term follow-up. Conclusion:The clinical outcome of early diagnosis and treatment of transposition of great arteries was good, preoperative status affects the outcome of surgery, coronary artery malformation, Taussig-Bing combined with aortic arch abnormalities were associated with increased operative mortality.

Delated Senning Procedure for Transposition of the Great Arteries with Severe Pulmonary Hypertension / 日本心臓血管外科学会雑誌

We report a case of transposition of the great arteries (TGA) with severe pulmonary hypertension from the right to left shunt in the right modified Blalock-Taussig shunt. The patient was diagnosed with TGA with a small ventricular septal defect, restrictive patent foramen ovale, and patent ductus arteriosus. Balloon atrial septostomy was performed, and an arterial switch operation (ASO) was planned. However, ASO was delayed during the neonatal period due to cerebral bleeding. Moreover, left outflow tract obstruction was noted ; hence, the surgical strategy was shifted to an atrial switch operation or Rastelli type operation. The patient was palliated at the age of 5 months with a right 4-mm Gore-Tex modified Blalock-Taussig shunt. After this procedure, he was followed up at the outpatient clinic with good saturation level. However, at 9 months, he revisited the hospital due to septic shock. His SpO2 was 60% in the upper right limbs and 40% in the upper left and lower limbs. Nitric oxide inhalation and 100% oxygen were administered to improve pulmonary hypertension and subsequent differential cyanosis. Pulmonary hypertension decreased from over-systemic to 70% of the systemic arterial pressure. The Senning procedure with a fenestration in an atrial baffle was successfully performed at the age of 1 year.

Clinical characteristics and risk factors for prolonged recovery after one-stage arterial switch operation for patients with transposition of the great arteries / 中国胸心血管外科临床杂志

@#Objective    To summarize the early clinical features and perioperative management strategies for patients with transposition of the great arteries (TGA) after one-stage arterial switch operation (ASO), and investigate the risk factors for prolonged recovery in ICU, with a focus on the age structure and deformity complexity. Methods    The clinical data of 231 consecutive TGA patients who underwent one-stage ASO were retrospectively analyzed. There were 165 males and 66 females, aged from 3 d to 10 years. The patients were sequenced by the length of ICU stay. The time at the 75th percentile was defined as the critical value for grouping. Patients with an ICU stay time over this point were allocated to a prolonged recovery group (n=54), while the rest were allocated to a normal recovery group (n=177). The perioperative clinical data were compared between the two groups, and the risk factors for prolonged recovery were evaluated. Results    About half (49.6%) of the patients received late operation. The mean ICU stay time was 23.9±15.6 d in the prolonged recovery group, and 4.9±2.3 d in the normal recovery group. Complication of aortic arch lesion, delayed chest closure and postoperative pulmonary infection were independent risk factors for prolonged recovery after ASO in ICU. However, late operation had no significant effect on the overall recovery. Conclusion    With strict surgery indications and excellent postoperative management, most patients can have satisfactory early-stage outcomes, but are confronted with  increased complications, which is associated with prolonged recovery. Complication of aortic arch lesion, delayed chest closure and postoperative pulmonary infection are independent factors for delayed recovery of ASO.

Comparative study of rapid and long-term two-stage arterial switch operation in treatment of transposition of the great arteries with intact ventricular septal / 中国胸心血管外科临床杂志

@#Objective    To summarize the experience of surgical treatment of transposition of the great arteries with intact ventricular septal (TGA-IVS) after left ventricular regression by comparing the characteristics of rapid and long-term two-stage arterial switch operation (ASO). Methods    Forty-one patients who were mainly diagnosed with TGA-IVS from January 2007 to January 2019 and underwent two-stage ASO were included. They were divided into a rapid two-stage ASO group (19 patients) and a long-term two-stage ASO group (22 patients) according to the interval of left ventricular training surgery and ASO. The clinical effectiveness of the two groups was compared. Results    There was a statistical difference in age, body weight, blood oxygen saturation before ASO, end diastolic diameter of left ventricle before training, and thickness of posterior left ventricular wall before ASO (P<0.05). Children older than 1 year was an independent risk factor for long-term two-stage ASO. Conclusion    Long-term two-stage ASO is suitable for children who are older than 1 year and who have severe left ventricular regression.

Arterial switch operation: A double cohort study of 20 years’ outcomes of 571 patients in a single center / 中国胸心血管外科临床杂志

@#Objective    To define the patient characteristics and perioperative management, and to define the mortality and its risk factors after arterial switch operation (ASO). Methods    We 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. Results    Several 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%. Conclusion    Significant 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.

Early and midterm results of the Rastelli operation in Fuwai hospital / 中华胸心血管外科杂志

Objective@#To describe eraly and midterm outcome of the Rastelli repair in Fuwai hospital Patients.@*Methods@#From May 2010 to March 2017, 71 patients with transposition of the great arteries(TGA)with ventricular septal defect(VSD)and right ventricular outflow tract obstruction(RVOTO) or double outlet right ventricle(DORV)with VSD and RVOTO underwent Rastelli repair. 48 cases male , 23 cases female . Age at operation is(4.7±2.7) years. There are 10 TGA cases, 27 DORV cases, 34 CTGA cases in this group. 30 patients(42.3% , 30/71)received palliative operation prior to the Rastelli procedure, including 13 BT shunt and 17 bi-Glenn operation. 31 patients(43.7%, 31/71 )underwent the Rastelli procedure with VSD enlargement. Right ventricle-to-pulmonary artery connection were created with the use of 9 homografts, 56 valved bovine jugular vein, 6 man-made valved Gore-Tex conduit. The overall mean right ventricle-to-pulmonary artery conduit size was(17.9±3.3)mm.@*Results@#CPB time was(209.0±83.4)minutes, aortic crossclamping time was(132.0±71.1)minutes, mechanical ventilation time was(102.6±81.7)h. Early mortality was 1.4%(1/71). morbidity in hospital was 16.9%, 4 patients with Ⅲ AVB implanted permanent pacemaker, Subxiphoid pericardial window drainage in 3 cases, delayed sternal closure in 3 and re-thoratomy for hemaostsis in 2.Follow up is from 4 months to 6.8 years. Overall survival was 97.2% and 97.2% at 1 and 5 years, respectively. Freedom from RVOTO was 98.6% and 84.1% at 1 and 5 years, respectively. Freedom from reintervention was 98.6% and 90.0% at 1 and 5years, respectively. 1 patients performed a conduit replacement. Seven patients performed 10 times balloon dilatation . Time-related freedom from recurrent LVOTO on echocardiogram in all patients, and the pressure gradient of the LV to the aorta was(10.5±8.8 )mmHg at the most recent follow-up.@*Conclusion@#The Rastelli operation remains the preferred procedure for part of the DORV , CTGA , TGA with VSD and severe fixed valvular or subvalvular PS. The Rastelli procedure can be performed with low early mortality. There is frequent need for late reoperation, especially for conduit replacement.

Anatomic correction for congenitally corrected transposition of the great arteries and 10-year follow-up: A retrospective cohort study / 中国胸心血管外科临床杂志

@#Objective    To evaluate the effects of anatomic correction for congenitally corrected transposition of the great arteries (ccTGA) and 10-year follow-up. Methods    From 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. Results    There 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. Conclusion    According 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.

Trasplante cardíaco en adultos con cardiopatías congénitas / Heart Transplantation in Adult Patients with Congenital Heart Disease

RESUMEN Introducción: En las últimas décadas los pacientes con cardiopatías congénitas (CC) han presentando nuevos desafíos en el manejo de las complicaciones, tanto de la patología originaria como de las cirugías correctoras que les han permitido llegar a la adultez. Objetivo: Comunicar los resultados y la evolución de los pacientes con CC que hayan sido evaluados para trasplante en un centro de alta complejidad. Se utilizó la base de datos institucional, y se analizaron los datos de 11 pacientes evaluados para trasplante con diversas patologías congénitas. Accedieron al trasplante 5 de ellos con una sobrevida a 1,6 años del 80%. Los pacientes que se hallaban en lista y no se trasplantaron tuvieron una mortalidad del 66%, y los descartados por comorbilidades presentaron una mortalidad del 35%. Conclusión: El trasplante cardíaco en adultos con CC tiene un riesgo periprocedimiento más elevado que para las cardiopatías adquiridas (CA). No obstante, los que sobreviven el primer año postrasplante tienen una excelente evolución a largo plazo.

ABSTRACT Background: Over recent decades, congenital heart disease (CHD) patients have posed new challenges in the management of complications, both of the original condition as of the corrective surgeries that have allowed them to reach adulthood. Objective: The aim of this study was to report the outcomes and evolution of CHD patients who had been evaluated for transplantation in a tertiary care center. Using the institutional database, data from 11 patients with different congenital diseases were evaluated for transplantation. A total of 5 patients underwent transplantation, with a 1.6-year survival rate of 80%. Mortality rate was 66% for patients who were on the waiting list but were not transplanted, and 35% for those who were ruled out due to comorbidities. Conclusion: Heart transplantation in CHD adult patients present a higher periprocedural risk than in patients with acquired heart diseases. However, those who survive the first post-transplant year have an excellent long-term outcome.

Congenitally corrected transposition of the great arteries:anatomic repair or fontan pathway? / 中华胸心血管外科杂志

Objective To review the early-and mid-term results of anatomic repair or Fontan pathway for congenitally corrected transposition of the great arteries(ccTGA) in a single institution of China.Methods Hospital records over a 9-year period(2009-2017) were reviewed to identify patients with ccTGA who underwent anatomic repair or Fontan pathway.Pa-tient-and procedure-related variables were reviewed.Results We identified 37 patients.Group 1 consisted of 10 anatomic re-pairs, of which 4 required prior pulmonary artery banding.Median age at anatomic repair was 1.0 years( range: 0.3 -7.8 years).There was one early death, and one patients experienced ECMO support.The mean follow-up was(3.0 ±2.8)years (range:0.7-8.4 years).Five(55.5％) patients showed arrhythmias, and one required permanent pacemaker implantation during follow-up.Group 2(27 patients) underwent Fontan palliation, of which 23(85.2％) underwent prior bidirectional Glenn shunt.Median age at Fontan completion was 3.8years(range:2.2-14.3 years).there was one early death with a mor-tality of 3.7％.The mean follow-up was(2.8 ±1.6) years(range:0.8-8.2 years).There was 4(15.4％) cases of arrhyth-mias, but none required reintervention.The arrhythmias incidence in Fontan group was significantly lower than the anatomic re-pair group.The early-and mid-term survival rate were 90.0％ and 96.3％in the two groups.The difference was not statistical-ly significant(P=0.458).Conclusion Patients with ccTGA do well with both anatomic repair and the Fontan pathway in the medium term.Pulmonary artery banding can be used effectively for morphological left ventricular retraining , and extenuate tri-cuspid regurgitation.Excellent outcomes with reduced early complication and arrhythmias incidence can be achieved for this co-hort of patients when a strategy of avoiding complex anatomic repair in favor of the Fontan pathway is used .

The association of Nodal signaling pathway and the pathogenesis of transposition of TGA / 中华胸心血管外科杂志

Transposition of the great arteries(TGA) is one of the most common and severely congenital heart defects in the neonatal period,even though we have significantly improvement in prenatal diagnosis and postpartum treatment of TGA,patients with severely TGA still have no promising prognosis.Now,the exactly pathogenesis of TGA is still unknown,in the view of embryonic development,TGA is a kind of conotruncal anomalies,which highly associated with unbalanced development of left-right axis.Nodal signaling pathway is one of the most functional pathway in controlling the formation of left-right axis,so fully investigation of Nodal signaling pathway may help us to find deep pathogenesis of TGA.

Anomalous Origin of the Right Subclavian Artery in a Patient with D-transposition of the Great Arteries

This case report concerns a young patient with an extremely rare combination of d-transposition of the great arteries (d-TGA) and anomalous origin of the right subclavian artery. In our patient, the right subclavian artery originated from the pulmonary artery, which is why he did not show reversed differential cyanosis. We conclude that the presence of an aortic arch anomaly should be considered in patients with d-TGA who do not present with reversed differential cyanosis. A further imaging work-up, including computed tomography or magnetic resonance imaging, might be helpful.

Staged Repair for Transposition of the Great Arteries in a Premature Triplet Weighing 1,336 g / 日本心臓血管外科学会雑誌

<p>Cardiac surgery for very-low-birth-weight infants is rarely reported, especially for a triplet. We herein report the successful staged repair of a premature triplet accompanied with transposition of the great arteries. During pregnancy, the fetuses were diagnosed as dichorionic diamniotic triplets, and the mother entered a hospital for maternal protection and health care from 25 weeks' gestation. The triplets were delivered by Caesarean section at 33 weeks and 5 days of gestation because of intrauterine growth retardation. One of the infants, weighing 1,336 g, was diagnosed with transposition of the great arteries (type II). Since he was deemed unable to endure an intracardiac repair, he received balloon atrial septostomy on the 27th day of life and then bilateral pulmonary artery banding on the 29th day of life. However, further balloon atrial septostomy on day 69 and left pulmonary arterial de-banding on day 73 post-birth were needed because of the progression of hypoxia. He received prolonged intubation and inotropic support after temporary cardiopulmonary stability, and we ultimately decided to perform arterial switch operation on day 110, when he weighed 1,838 g. The patient showed a good recovery. In the field of pediatric cardiac surgery, we occasionally select staged strategies for patients who cannot undergo radical operations all at once because of their general condition or low body weight. However, there are no established guidelines concerning the timing of palliative or radical operations in low-birth-weight infants. At present, we select medical strategies ourselves, on a case-by-case basis. In the present case, although our medical strategy had to be adapted, we still obtained a good recovery for this triplet with extremely low birth weight. We herein report this case with some references from the literature.</p>

The clinical research of operative technique strategies and results for congenitally corrected transposition of the great arteries / 中华胸心血管外科杂志

Objective To review and compare various types of operations for congenitally corrected transposition of the great arteries (ccTGA),to provide more suitable surgical procedure and improve surgical results.Methods Analysing 203 patients with ccTGA between June 1999 and June 2014,there were thirten patients who had undergone palliative procedure.Thirty-nine patients had received conventional repair operation.Eighty-eight patients had received double switch operation in which there were three patients of Double Switch With Hemi-Mustard and Bidirectional Glenn Procedure.Sixty-three patients had received other surgical treatments including functional univentricular repair operation.Results There were 13 hospital deaths in all patients,consisted of 2 by conventional repair operation,9 by double switch operation and 2 by other procedure.Conclusion The operative procedure depend on the anatomy and physiology in ccTGA.The double switch operation have relatively higher mortality,more complication,the indication of Double Switch With Hemi-Mustard and Bidirectional Glenn Procedure should be more acceptable,but the long-term outcomes will be followed-up.