Clinical outcomes and follow-up results of 300 cases of transposition of great arteries intervened by the arterial switch operation / 中华实用儿科临床杂志

Objective:To summarize and analyze the efficacy, experience and follow-up results of 300 cases of transposition of the great arteries (TGA) intervened by arterial switch operation.Methods:It was a retrospective, single-center study involving 300 TGA patients intervened by arterial switch operation between January 2010 and December 2017 in Guangdong Provincial People′s Hospital.Their clinical data were retrospectively analyzed.There were 236 male patients and 64 females.Among them, 128 cases (42.7%) were TGA with ventricular septal defect (TGA/VSD), and 172 cases (57.3%) were TGA with intact ventricular septal defect (TGA/IVS). The mean age and weight at operation were (23.8±39.2) cases days, and (3.5±0.8) kg, respectively.There were 193 cases (64.3%) with usual coronary artery patterns, and 107 cases (35.7%) with unusual coronary artery patterns.Among the 107 cases with unusual coronary artery patterns, 21 cases (7.0%) were involved with the intramural coronary artery, and 17 (5.7%) presented the single-ostium coronary pattern.Non normal distribution data were used the Mann- Whitney U test.Categorical measures were compared by Chi- square test or Fisher′ s exact test.Survival probability and freedom from events were calculated by the Kaplan-Meier method, and difference in survival probability by the Log Rank test. Results:All patients were successfully intervened by arterial switch operation, 73.3% of patients with TGA/IVS underwent the surgery within 3 weeks after birth, and 85.9% of patients with TGA/VSD underwent surgery within 3 months.The mean cardiopulmonary bypass time and aortic occlusion time were (193±68) min, and (122±39) min, respectively.Twenty-five patients (8.3%) died in hospital.Thirty cases had low cardiac output syndrome, 1 implanted with a permanent pacemaker due to complete atrioventricular block.A total of 254 patients were followed up for 1 month to 10 years.Three patients with single-ostium coronary pattern died at the follow-up period.The 5-year and 10-year survival rates were both 90.7%.During the follow-up, 49 cases (49/254 cases, 19.3%) had pulmonary artery stenosis, 66 cases (66/254 cases, 26.0%) had aortic valve regurgitation, 47 cases (47/254 cases, 18.5%) had pulmonary valve regurgitation, and 4 (4/254 cases, 1.6%) had aortic anastomotic stenosis.Among the 21 patients (21/254 cases, 8.3%) requiring reintervention, 17 patients (17/254 cases, 6.7%) underwent a total of 18 reinterventions, including 12 interventions of pulmonary artery plasty, 4 of percutaneous balloon pulmonary valvuloplasty, 1 of aortic reconstruction at anastomosis and 1 of pacemaker exchange due to battery exhaustion.Conclusions:Arterial switch operation is the optimal treatment for TGA.The long-term follow-up results of arterial switch operation are satisfactory in TGA children, with a low risk of long-term reoperation.

Double artery transposition: Coronary pattern and great arteries / Doble transposición arterial: Patrón coronario y grandes arterias

Extensive anatomic variability of the coronary arteries in hearts with transposition of great arteries has been described. Usual coronary pattern is defined as right coronary artery arising from posterior facing sinus and left coronary artery from anterior facing sinus. Results of prior case series have suggested that certain coronary patterns are associated with adverse outcomes in surgery (arterial switch operation). We present the case of a neonate with prenatal diagnosis of transposition of great arteries. Postnatal transthoracic echocardiography showed great arteries in a side-by-side relationship (fig. 1). Coronary pattern was described as inverted or with double coronary looping, which represents less than 3% of the anatomical variants. The entire left coronary artery system arose from the posterior-facing sinus, whereas the right coronary artery arose from the aorta anteriorly. Left coronary artery pursued a retropulmonary course, dividing posteriorly into the circumflex and anterior descending arteries (fig. 2). The patient underwent arterial switch operation uneventfully.

Two wrongs make a right: A case report on the anesthetic management of a pediatric patient with congenitally corrected transposition of great arteries

@#Congenitally corrected transposition of the great arteries (ccTGA) or ventricular inversion, is a rare form of congenital heart disease (CHO) representing approximately 0.5% of all CHO. It is characterized by atrioventricular and ventriculoarterial discordance, in which the atria are connected to the opposite ventricle, and the ventricles are connected to the incorrect great artery. The defect is termed "corrected" because of the physiologic flow of blood through the body despite the malformation. ccTGA can be associated with other cardiac anomalies like ventricular septa! defect (VSO), pulmonary outflow tract (LVOT) obstruction, tricuspid valve lesions, and coronary artery anomalies. This paper aims to discuss the anesthetic management unique to patients with ccTGA in which the ultimate goal is to prevent hemodynamic instability that could potentially lead to cardiac failure. Here, we report the anesthetic management of a 6 year old child with ccTGA with mild tricuspid regurgitation who underwent plastic repair of cleft lip under general endotracheal tube anesthesia (inhalational). With use of balanced anesthesia to produce minimal to no cardiovascular effects, the operation concluded successfully.

Arterial switch operation: 10-year experience at a single center / 中华胸心血管外科杂志

Objective To summarize the outcomes and experience of arterial switch operation (ASO) in the past 10 years in our center.Methods From September 2008 to July 2017,238 patients underwent ASO at Guangzhou Women and Children Medical Center for repair of transposition of the great arteries and Taussig-Bing anomaly.There were 193 male and 45 female.Median age at operation was 2 months (2 days to 10 years) and mean body weight was(4.2 ± 1.7) kg (1.8-20.6 kg).There were TGA and VSD 91 cases,TGA/IVS 110 cases,and Taussig-Bing anomaly 37 cases.Among them 24 patients had an aortic arch anomaly.Intramural coronary artery was found in 8 patients.Results All patients successfully completed the operation,one-stage ASO was perfonned in 232 patients.Two-stage ASO was performed in 6 patients.The mortality was 14.2％.The follow-up duration was 1-10 years (median time,46 mouths).There were 3 died.Two suffered sudden death,and another one arrhythmia.10-year survival rate was 92.8％.Conclusion The outcomes of ASO were satisfactory.The long-term reoperation rate was rare.

Clinical Analysis of Eleven Pregnancies with Transposition of Great Arteries / 实用妇产科杂志

Objective:To analyze the management and outcome of pregnant woman with transposition of great arteries and to investigate the effect of multidisciplinary management during pregnancy on the pregnancy outcome.Methods:The clinical data of 11 pregnant women with transposition of great arteries under going treatment in Beijing Anzhen Hospital during January 2007 and December 2017 was studied retrospectively.The cardiac function, cardiac index, management during pregnancy and outcomes were assessed.Results:Eight patients (No.1～No.8) were complicated with corrected transposition of the great arteries (CTGA).one (No.9) was complicated with complete transposition of the great arteries (TGA).Two were complicated with double outlet right ventricle (DORV).Enlargement of left atrial enlargement and increase of left functional ventricular diameter were observed in all eight patients with CTGA.The left atrioventricular valve regurgitation was reported in these patients except the patient No.7 who had a mechanical valve replacement.Three cases had elevated BNP.Patient No.9 who was with TGA showed an enlargement of the right heart and a narrow inner diameter of left heart.Fetal death occurred in second trimester.The left ventricular enlargement, BNP elevation and atrioventricular valve regurgitation were observed in the two patients with DORV.The mean pregnancy length was 34.6±5.0 weeks.Nine had cesarean deliveries.One received cesarean section with fetal death.One had vaginal delivery.Average hemorrahge volume was 245±110 ml.One was fetal loss with cesarean section, while six were term delivery, and the other four cases were premature delivery.All eleven patients were discharges with an average hospitalization days 9.4±4.2 days.The average weight of fetus was 2659±1012 g.Three were low birth weight infants.Two newborns had mild asphyxia.No birth defects or fetal complications were reported.Conclusions:Pregnancy could be attempted in CTGA patients with normal cardiac function, no obvious cardiac valve regurgitation orventricular enlargement.They should be followed by multidisciplinary management during pregnancy.It is pregnancy contraindication for woman with TGA and DORV without surgical treatment.

Prognosis of the complete transposition of great arteries with left ventricular outflow tract obstruction after intraventricular repair / 中国胸心血管外科临床杂志

@#Objective     To compare the clinical characteristics and prognosis of patients who received two different intraventricular repair. Methods     We retrospectively analyzed the clinical data of 24 complete transposition of the great arteries (TGA)/left ventricular outflow tract obstruction (LVOTO) patients who all received intraventricular repair. The patients were allocated into two groups including a REV group and a Rastelli group. There were 13 patients with 9 males and 4 females at median age of 25.2 (6, 72) months in the REV group. There were 11 patients with 10 males and 1 female at median age of 47.9 (14, 144) months in the Rastelli group. Results     The age at operation (P=0.041), pulmonary valve Z value (P=0.002), and LVOT gradient (P=0.004), rate of multiphase operation between the REV group and the Rastelli group was statistically different. The mean follow-up time was 17.3 months. And during the follow-up, 1 patient had early mortality, 2 patients had early reintervention, 7 patients had postoperative RVOTO, and received Rastelli and larger VSD inner diameter were associated with postoperative RVOTO. Conclusion     As the traditional surgery for TGA/LVOTO patients, the intraventricular repair has a low early mortality and low early reintervention. Modified REV is associated with postoperative peripheral pulmonary vein isolation (PVIS). Patients who received Rastelli operation and with larger VSD inner diameter are more likely to have postoperative RVOTO, but the reintervention for PVI and   RVOTO during follow up is very low.

Changes of blood flow in posterior cerebral artery in fetus with complete transposition of great arteries by pulsed Doppler ultrasound / 中华超声影像学杂志

Objective To study the changes of blood flow in posterior cerebral artery ( PCA ) in complete transposition of great arteries (CTGA) through the application of the pulsed Doppler . Methods Twenty CTGA fetuses ( CTGA group) and 20 healthy control fetuses ( control group) were involved ,the blood flow indexes peak systolic velocity ( Vs) ,end-diastolic velocity ( Vd) ,pulsatility index ( PI) ,resistance index ( RI) ,velocity-time integral ( VTI) of PCA-S1 ,PCA-S2 and MCA of the fetuses in the two groups were detected by pulsed Doppler . The differences in blood flow indexes between CTGA fetuses and healthy controls were analyzed by independent t -test . The rates of abnormal resistance in PCA-S1 and MCA in CTGA fetuses were compared through Chi-square test ( χ2 test) . Results Compared with control group ,the MCA-PI ,MCA-RI ,PCA-S1-PI and PCA-S1-RI of CTGA group decreased significantly( all P < 0 .05) ,MCA-VTI ,PCA-S1-VTI ,PCA-S2-VTI increased significantly ( all P < 0 .05) ,but no significant difference was found in PCA-S2-PI ,PCA-S2-RI ,Vs and Vd of the MCA and the PCA ( all P > 0 .05 ) . The rate of abnormal resistance in the MCA was significantly lower than that in the PCA-S1 in CTGA group ( P <0 .05) . Conclusions The pulsed Doppler can be used to study the changes of blood flow in PCA of CTGA fetuses and the differences of specific hemodynamic alterations may occured in different segments of the PCA in CTGA fetuses ,indicating a tendency to protect the PCA-supplying areas of the brain when ischemia and hypoxia .

The clinical research of double switch operation with Glenn and hemi-Mustard in CC-TGA in children / 中华胸心血管外科杂志

Objective To avoid complications associated with the complete Senning procedures and to assist right-heart hemodynamics, this study was undertaken to assess the risks and benefits of the double-switch operation using a hemi-Mustard atrial switch procedure and the bidirectional Glenn operation for congenitally corrected transposition of the great arteries. we fa-vor a modified atrial switch procedure. Methods Between January 2014 and March 2016, anatomic repair was achieved in 26 patients. A hemi-Mustard procedure was the atrial switch procedure for 26. 9％(7/26). The Rastelli-atrial switch procedure was performed in 5 patients( RAS) and the arterial-atrial switch procedure was performed in 2 patients( AAS) . Results There was no in-hospital death after anatomic repair. There were no late deaths in a median follow-up of 7-21 months. Glenn opera-tion complications were uncommon limited to the perioperative period, atrial baffle-related reoperations or sinus node dysfunc-tion had not been observed. Tricuspid regurgitation decreased to mild for 1 case and mild to moderate for 6 cases. Right ventri-cle-pulmonary artery conduit longevity was significantly improved. Conclusion We recentely describe an experience with the double-switch operation using a modified atrial switch procedure with favorable earlyterm results. The risks of the hemi-mustard and bidirectional Glenn operation are minimal and are limited to a well-defined patient subset. The benefits include reduced baffle-and sinus node-related complications, technical simplicity and inspiring results.

Risk factors for prolonged mechanical ventilation in children with transposition of the great arteries and intact ventricular septum that underwent primary arterial switch operation / 中华实用儿科临床杂志

Objective To explore the risk factors for prolonged mechanical ventilation in children with trans-position of great arteries and intact ventricular septum who underwent arterial switch operation. Methods This study was a retrospective,single center study. One hundred and twenty patients with transposition of great arteries and intact ventricular septum who underwent primary arterial switch operation between January 2014 and December 2016 at Fuwai Hospital were eligible for this study. The data of patients from pediatric intensive care unit database and electronic medical records were collected. The data related to postoperative respiratory assist time were collected,including demo-graphic data,preoperative diagnosis,intraoperative data,and postoperative recovery data. The patients were divided into 2 groups according to ventilation time which were prolonged mechanical ventilation group(ventilation time > 72 hours) and non - prolonged mechanical ventilation group(ventilation time ≤72 hours). The data of 2 groups were analyzed by using single factor analysis,and the P≤0. 2 factors were processed into Logistic regression analysis. Results Ninety -six patients were enrolled including 22 patients in prolonged ventilation group and 74 patients in non - prolonged me-chanical ventilation group. No statistical significance was found in 2 groups in gender,age,weight,preoperative lactate, hemoglobin,use of prostaglandin E1,mechanical ventilation,cardiopulmonary time,aortic clamping time,the ratio of left ventricular pressure to right ventricular pressure,immediate postoperative plasma lactate,and vasoactive inotropic score. The weight and postoperative left atrial pressure were significantly different between 2 groups with P < 0. 2. Weight were (3. 5 ± 0. 9)kg in prolonged mechanical ventilation group and (3. 9 ± 1. 0)kg in non - prolonged mechanical ventila-tion group (P = 0. 117). Left atrial pressures were (7. 9 ± 1. 9)mmHg(1 mmHg = 0. 133 kPa)in prolonged mechani-cal ventilation group and (6. 7 ± 2. 0)mmHg in non - prolonged mechanical ventilation group(P = 0. 015). The weight and left atrial pressure were processed into Logistic regression analysis and the results revealed that high left atrial pres-sure was the risk factor for ventilation prolongation(OR = 1. 048,P = 0. 020). Respiratory assist time in prolonged and non - prolonged ventilation group was 112(80,194)h and 26(17,46)h,respectively;ICU time in prolonged and non - prolonged ventilation group was 10(1,14)d and 4(3,6)d,respectively;and all the differences were significant (all P = 0. 000). The number of death in each group was 1 with no significant difference(P = 0. 420). Conclusions High left atrial pressure is the risk factor for prolonged mechanical ventilation in children with transposition of great ar-teries and intact ventricular septum following primary arterial switch operation.

Establishment of Congenital Heart Disease Transposition of Great Arteries in Experimental Mice Model / 中国循环杂志

Objective: To establish a mice model of congenital heart disease transposition of great arteries in order to provide a research reference for the occurrence and development of transposition of great arteries. Methods: A total of 20 pregnant ICR mice at 8-10 weeks of age were divided into 2 groups: Control group, the mice received a single dose of DMSO 70 mg/kg at 8.5 days of gestation,n=5 and Experiment group, the mice received a single dose of all-trans retinoic acid 70 mg/kg at 8.5 days of gestation,n=15. All animals were treated for 18 days and then the embryos were taken to observe cardiac morphology under stereomicroscope. Results: Compared with Control group, Experiment group had obviously increased occurrence rates of premature delivery, abortion and embryo absorption, and 61.2% phenotype for transposition of great arteries; meanwhile, combining with non-heart defect phenotypeas exophthalmos and spinal malformation. Conclusion: All-trans retinoic acid may induce transposition of great arteries in mice embryos, which is a feasible animal model in experimental research.

Comprehensive management strategy for critical newborns with transposition of great arteries / 中华实用儿科临床杂志

Objective To summarize the clinical experience of comprehensive management of critical newborns with transposition of great arteries (TGA) by neonatologists and cardiac surgeons.Methods Seventeen critical newborns with TGA rescued by combined efforts of neonatologists and pediatric cardiac surgeons from Oct.2010 to Oct.2012were retrospectively analyzed.Results Of the 17 newborns(14 cases were male,3 cases were female),9 cases were TGA with ventricular septal defect(TGA/VSD),8 cases TGA with intact ventricular septal defect (TGA/IVS).The babies aged from 1-45 days,weighed from 2.7-4.6 kg at operation.Of the 9 cases TGA/VSD babies,3 cases were treated with prostaglandins E (PGE) for hypoxemia correction and selective operation opportunity ;2 cases underwent emergency arterial switch operation (ASO) for severe cardiac shock.All of the 8 cases TGA/IVS babies received PGE treatment preoperatively for life threatening hypoxemia,and 4 cases of them underwent emergency ASO.There were 12 cases delayed sternal closure and 5 cases of them were emergency babies.Peritoneal dialysis was employed for 6 cases and extracorporeal membrane oxygenation (ECMO) for l case.There were 6 cases with postoperative incision infection,including 5 emergencies.Thirteen newborns survived and 4 cases died at hospital:one case complicated with postoperative septicemia and severe mediastinitis which leaded to pericardium patch rupture and fatal pulmonary arterial hemorrhage;one case with abnormal inferior vena cava connection and severe low cardiac output syndrome;one case with disseminated intravascular coagulation (DIC) due to septicemia and severe mediastinitis postoperatively,one case with abnormal coronary artery during surgery and difficult coronary artery transplantation and insufficient blood supply after aorta was open.Conclusions Correcting hypoxemia and stabilizing the severe babies are critical in NICU to avoid emergency surgery.Emergent ASO is the optimized medical therapy for those with persistent severe hypoxemia and heart failure.

Evaluation of left ventricular geometry and function by echocardiography in patients after arterial switch operation / 中华超声影像学杂志

Objective To study the early and mid-term outcome in terms of left ventricular(LV)geometry and function in patients with transposition of great arteries with intact ventricular septum (TGA/IVS) undergoing arterial switch operation.Methods Twenty patients were followed up and divided into 2 groups according to age:the earlier group and the later group.Age and sexual matched controls were also designed as control group 1 and control group 2.LV posterior wall thickness(PWTd),dimension(LVIDd)and length(L) at end diastolic phase,LV end-diastolic volume (EDV),end-systolic volume (ESV),ejection fraction (EF),mitral valve early (E) and late (A) inflow velocities and E/A,isovolumic relaxation time (IVRT) were obtained by two-dimensional echocardiography,normalized isovolumic relaxation time (IVRTn),wall thickness index (PWTd/LVIDd) and LV geometry index(LVIDd/L) were calculated,and these indexes between groups were compared.Results ①Compared with control group 1,PWTd in earlier group was higher [(0.41 + 0.06)cm vs (0.36 + 0.05)cm,P =0.022],but LVIDd had no significant statistical difference [(2.08 + 0.21)cm vs (2.21 + 0.23)cm,P =0.117],indicating PWTd/ LVIDd differed between groups(0.20 + 0.04 vs 0.16 + 0.01 P =0.001).②No abnormalities were observed in later group.③Compared with different age groups in TGA,EDV,ESV,L,LVIDd,PWTd were significantly higher in later group(P =0.000).There were obvious correlations between EDV,ESV,L,LVIDd and examined age (r =0.90,0.83,0.73,0.90,P =0.000,R2 =0.809,0.694,0.528,0.808) while there were no significant correlation between PWTd and examined age(r =0.56,P =0.000,R2 =0.309).Conclusions Early after operation,LV is undergoing hypertrophy or hyperplasia in TGA/IVS,but will recover in the mid-term period,and the function is normal all the time indicating that the LV myocardium may avoided irreversible pathological changes if operated in time.All in all,LV develops well in mid-term period.

Early Complication of Mustard Procedure after Late Repair

No abstract available.

Value of prenatal ultrasound observation of subaortic conical connection in the diagnosis of complete transposition of great anteries / 中华超声影像学杂志

Objective To investigate the value of prenatal ultrasound observation of subaortic conical connection in the diagnosis of complete transposition of great arteries(TGA).Methods Echocardiography findings of 22 cases with complete TGA confirmed by autopsy were retrospectively analyzed and summarized.Results All cases were shown subaortic conical connection on two-dimensional echocardiography,the two great arteries were parallel in 18 cases on left ventricular long-axis view,the character of normal right ventricular outflow tract surrounding the aortic root disappeared in 17 cases.There were 13 cases with ventricular septal defect and 2 cases with left ventricular outflow tract obstruction.Aorta and pulmonary artery were shown in right anterior and left posterior position among 20 cases,and in anterior and posterior position in 2 cases.Conclusions Prenatal ultrasound observation of subaortic conical connection is of great value in the diagnosis of complete TGA.

Comparison of three dose regimens of aprotinin in infants undergoing the arterial switch operation.

To determine the most effective dose regimen of aprotinin for infants undergoing arterial switch operation for transposition of the great arteries in reducing blood loss and postoperative packed red blood cell (PRBC) requirements. A total of 24 infants scheduled for arterial switch operation for transposition of the great arteries were included in the study. The infants were randomly assigned to one of the three groups. Group I (n = 8) patients received aprotinin in a dose of 20,000 kallikrein inhibiting units (KIU)/kg after induction of anesthesia, 20,000 KIU/kg was added to the pump prime, and 20,000 KIU/kg/hour infusion for three hours after weaning from bypass; group II (n = 8) patients received aprotinin 30,000 KIU/kg after induction of anesthesia, 30,000 KIU/kg was added to the pump prime and 30,000 KIU/Kg/hour infusion for three hours after weaning from bypass; group III patients (n = 8) received aprotinin 40,000 KIU/kg after induction of anesthesia, 40,000 KIU/kg was added to the pump prime and 40,000 KIU/kg/hour infusion for three hours after weaning from bypass. Postoperatively, the cumulative hourly blood loss and PRBC requirements were noted up to 24 hours from the time of admission in the intensive care unit (ICU). Use of blood and blood products were noted. Coagulation parameters such as hematocrit, activated clotting time (ACT), fibrinogen, prothrombin time (PT), international normalized ratio (INR), platelet count, and fibrin degradation products (FDP) were investigated before cardiopulmonary bypass (CPB), after protamine administration, and at four hours postoperatively in the ICU. The number of infants reexplored for increased mediastinal drainage was recorded. Renal functions were monitored by measuring urine output (hourly) and serum urea (mg%) and serum creatinine (mg%) at 24 hours. The sternal closure time was comparable in all the three groups. Cumulative blood loss (ml/kg/24 hours) was greatest in group I (17.30 ± 7.7), least in group III (8.14 ± 3.17), whereas in group II, it was 16.45 ± 6.33 (P = 0.019 group I versus group III; (P = 0.036 group II versus group III). Postoperative PRBC requirements were significantly less in high dose group III (P = 0.008, group I versus III; p = 0.116, group II versus group III) . Tests for coagulation performed at four hours postoperatively, viz. ACT, PT, INR, FDP, and platelets were comparable in the three groups. Urine output on CPB was comparable in all the groups. Serum urea and creatinine showed no significant difference between the three groups twenty four hours postoperatively. Aprotinin dosage regimen of 40,000 KIU/kg at induction, in CPB prime and postoperatively for three hours was most effective in reducing postoperative blood loss and PRBC transfusion requirements. Aprotinin does not have any adverse effect on renal function.

Operative and Mid-Term Results of 107 Corrected Congenital Transposion of Great Arteries / 中国循环杂志

Objective:To retrospectively analyze the results and mid-term outcomes of 107 patients with corrected congenital transposition of great arteries(CCTGA)who underwent surgical treatment.Methods:A total of 107 CCTGA patients with surgical treatment from January 1996 to October 2005 in our hospital were studied.There were 72 male and 35 female,with the mean age of 11.5±8.4years and mean body weight of 31.6±4.7kg. Among the cohort,81 were levocardia,23 dextrocardia,and 3 medocardia.Initial surgical procedure included single ventricle repair in 14 cases,conventional biventricular repair in 75 and biventricular anatomical repair in 18 cases. 99(93%)patients were followed up with the mean time of 47.8 months.Results:The early operative mortality rate was 4.76% in different procedures.The death rate with single ventricle repair was 0%,conventional biventricular repair 4%,atrial-arterial double switch 0%,atrial-ventricular double switch 40%. The risk factors for operative mortality were lesions with double outlet left ventricle combined with pulmonary stenosis(P<0.01)and Rastelli procedure(P<0.05). 12 patients(12.6%)died during the follow up period,among them,10(83.3%)were in conventional repair group,2(16.7%)in single ventricle repair group,and no death in double switch group. The risk of death during the follow up period of time were conventional biventricular repair,Rastelli procedure and tricuspid regurgitation.Conclusion:Conventional biventricular repair had a disappointing outcome in both operation and in long-term of follow up time. Patients suitable for single ventricular repair had fair short-and mid-term outcomes. Atrial-arterial double switch procedure had good operation and long-term results.

Essential Lessions for Perioperative Care of Transposition of Great Arteries

Of those neonates admitted with TGA, 4.1% died before surgery. 3.7% died due to consequences of inadequate interatrial mixing despite PGE1 infusion. Earlier diagnosis and balloon atrial septostomy are critically important in determining survival. Prenatal diagnosis with delivery in a high-risk obstetrical unit with facilities for immediate BAS and supportive therapy for pulmonary hypertension. A management using inhaled nitric oxide and extracorporeal membrane oxygenation was successfully used in neonates with transposition of the great arteries, intact ventricular septum, and persistent pulmonary hypertension. Early arterial switch operation may improve survival in patients with low birth weight or prematurity. Optimal management of dextrotransposition of the great arteries with intact ventricular septum is currently an arterial switch procedure performed in the first 2 weeks of life. However, a subgroup of patients presents for surgery beyond this time for reasons of necrotizing enterocolitis, low birth weight, or late referral. In this case, arterial switch following LV retraining in TGA IVS is a satisfactory option. In conclusion, meticulous care and harmonious team work between pediatric cardiologist and cardiac surgeon are essential for successful perioperative care of TGA.

Válvula aórtica cuadricúspide (VAC) en transposición de grandes arterias: Reporte de un caso diagnosticado por ecocardiografia bidimensional / Quadricuspid aortic valve in a patient with D- transposition of the great arteries

En este reporte presentamos el caso de un paciente con discordancia ventrículo arterial (d-TGA) en quien se diagnosticó por ecocardiografia bidimensional la presencia de válvula aórtica cuadricúspide, con el propósito de ampliar las lesiones asociadas a d-TGA y manifestar la preocupación de la repercusión clínica de anomalías en el número de velos valvulares en la evolución de este tipo de pacientes.

In this report we present the case of a patient with ventricular-arterial discordance in which a QAV was diagnosed by bidimensional echocardiogram with the purpose of expanding the anatomical spectrum of the anomalies associated with d-TGA and to manifest the clinical awareness of the consequence that the QAV has on the evolution of the patients that undergo to anatomic correction.

Repair of Corrected Transposition of the Great Arteries

PURPOSE: To assess the clinical characteristics and surgical approaches in different anatomical and hemodynamic types of corrected transposition of great arteries(TGA) and learn the surgical results of those patients. METHODS: All 52 patients who were diagnosed as corrected TGA between December 1987 and November 1999 and their medical records were reviewed. Three groups were identified according to associated anomalies', Group 1: TGA with intact ventricular septum(n=7), Group 2: TGA with ventricular septal defect(n=6), Group 3: TGA with ventricular septal defect and pulmonary stenosis(n=39). RESULTS: The clinical manifestations and managements according to associated anomalies were different. The average ages at operation were 190, 8.8 and 47 months in Groups 1, 2, and 3, respectively. The five patients underwent double switch operation and the remainder were managed conventionally without correcting discordant connection. Four patients died and 5 patients were re-operated. Eleven patients developed complete heart block, and 7 of them had permanent pacemakers. Progressive systemic tricuspid valve regurgitation developed in 15 patients and progressive systemic right ventricle dysfunction developed in 3 patients. CONCLUSION: The results of conventional management were disappointing, with significant morbidity of tricuspid failure, right ventricle failure and conduction system failure. Anatomical repair of corrected TGA can be achieved with favorable immediate surgical results but long-term follow up will be necessary.