Clinical characteristics of pulmonary vein stenosis in children / 中华实用儿科临床杂志

Objective:To analyze the clinical characteristics of pulmonary vein stenosis (PVS) in children, and to explore its treatment and prognostic factors.Methods:The clinical data of 19 children with PVS treated in Beijing Children′s Hospital, Capital Medical University from October 2016 to March 2022 were analyzed retrospectively.There were 16 males and 3 females.The median age at diagnosis was (2.81±1.95) years.A descriptive analysis of clinical characteristics of children was made.Results:Of the 19 children, 14 cases (73.7%) had primary PVS and 5 cases (26.3%) had secondary PVS after surgery of anomalous pulmonary venous connection (APVC). Thirteen children (68.4%) had hemoptysis.In the hemoptysis children, 5 cases had life-threatening massive hemoptysis, and 11 cases (57.9%) had a history of recurrent respiratory tract infection or pneumonia.Other manifestations of hemoptysis included failure to thrive (6 cases), cyanosis (5 cases), and dyspnea (3 cases). Complications were pulmonary hypertension (6 cases) and right heart failure (3 cases). There were 16 cases (84.2%) of unilateral PVS and 3 cases of bilateral PVS.Interlobular septal thickening, grid shadow and ground glass opacities were found on CT of all PVS cases.Ten cases underwent surgery, and 2 cases of them received angioplasty, but restenosis occurred in both of them.Eight children underwent pulmonary lobectomy, and their clinical symptoms were all relieved after operation.Nine patients were treated conservatively, and 3 cases of them died of bilateral PVS secondary to APVC.The remaining 6 alive cases still had intermittent clinical symptoms during follow-up.Conclusions:Hemoptysis and recurrent respiratory tract infection are the main clinical manifestations of PVS in children, and life-threatening massive hemoptysis can occur.Lobectomy is an effective treatment for unilateral PVS.The prognosis of secondary PVS after APVC is poorer and its mortality is higher, compared with primary PVS.

Integrated management during the perinatal period for total anomalous pulmonary venous connection / 中国当代儿科杂志

OBJECTIVES@#To evaluate the clinical effectiveness of integrated management during the perinatal period for fetuses diagnosed with total anomalous pulmonary venous connection (TAPVC) by prenatal echocardiography.@*METHODS@#Clinical data of 64 cases of TAPVC fetuses diagnosed by prenatal echocardiography and managed with integrated perinatal care in Qingdao Women and Children's Hospital from January 2017 to December 2021 were retrospectively analyzed. Integrated perinatal care included multidisciplinary collaboration among obstetrics, fetal medicine, ultrasound, pediatric cardiology, pediatric anesthesia, and neonatology.@*RESULTS@#Among the 64 TAPVC fetuses, there were 29 cases of supracardiac type, 27 cases of intracardiac type, 2 cases of infracardiac type, and 6 cases of mixed type. Chromosomal analysis was performed in 42 cases, and no obvious abnormalities were found. Among the 64 TAPVC fetuses, 37 were induced labor, and 27 were followed up until term birth. Among the 27 TAPVC cases, 2 cases accepted palliative care, 2 cases were referred to another hospital for treatment and lost to follow-up, while the remaining 23 cases underwent primary repair surgery. One case died within 6 months after the operation due to low cardiac output syndrome, while the other 22 cases were followed up for (2.1±0.3) years with good outcomes (2 cases underwent a second surgery within 1 year after the first operation due to anastomotic stenosis or pulmonary vein stenosis).@*CONCLUSIONS@#TAPVC fetuses can achieve good outcomes with integrated management during the perinatal period.

Patent Vertical Vein Device Closure: Peri Procedural Anesthetic Management

We present the peri?procedural anesthetic management in a case of transcatheter closure of an unligated patent vertical vein (VV) in a 2?year?old male child operated case of obstructed supra cardiac total anomalous pulmonary venous connection (TAPVC) who presented with significant left to right shunt causing symptoms of right heart failure. The procedure was carried out successfully under deep sedation and monitored anesthesia care (MAC) and had some specific clinical implications from the anesthetic management perspective which are highlighted and discussed in this report.

Transcatheter management of post-surgical superior vena cava obstruction

Obstruction of the superior vena cava (SVC) is a rare complication after cardiac surgery in infants and children. We present the case of a 2-year-old male child who underwent surgery to repair a mixed total anomalous pulmonary venous connection. After 18 months of surgery, the child developed SVC syndrome. Transcatheter stenting was performed to relieve the SVC obstruction detected with transthoracic echocardiography. The patient was discharged after 5 days of hospitalization. The patient was doing well at follow-up appointments, with good laminar flow through the stent. In conclusion, transcatheter management of post-surgical complications of SVC obstruction was successful in this patient.

Treatment of supracardiac total anomalous pulmonary venous connection in a single center / 中国胸心血管外科临床杂志

@#Objective    To analyze the surgical results of patients with supracardiac total anomalous pulmonary venous connection (TAPVC) in a single pediatric cardiac center. Methods    A retrospective study was conducted on 98 pediatric patients with supracardiac TAPVC receiving surgical repair from 2014 to 2019 in our center. There were 64 males and 34 females with a median surgical age of 3.0 (1.5, 7.0) months and a median weight of 5.0 (4.0, 6.0) kg. Twenty-three (23.5%) patients had preoperative pulmonary vein obstruction. Ninety-two (93.9%) patients received conventional surgical repair, while six (6.1%) patients were treated with the sutureless technique. The Cox regression model was used to analyze the data. Results    The median follow-up time was 26.50 (5.75, 44.25) months. There were 9 (9.2%) deaths. Lower weight at the time of repair (P=0.013) and prolonged cardiopulmonary bypass time (P=0.007) were associated with mortality. Postoperative pulmonary vein obstruction was observed in 8 (8.2%) patients. Associated risk factors for postoperative pulmonary vein obstruction included lower weight at the time of repair (P=0.042) and prolonged cardiopulmonary bypass time (P=0.002). Conclusion    Surgical repair of supracardiac TAPVC has achieved satisfactory results in our center. Risk factors such as lower weight at the time of repair and prolonged cardiopulmonary bypass time are associated with a poor prognosis.

A modified sutureless technique treating total anomalous pulmonary venous connection / 中国胸心血管外科临床杂志

@#Objective    To introduce a modified sutureless technique and its surgical results in the treatment of total anomalous pulmonary venous connection (TAPVC). Methods    Clinical data of 11 patients with TAPVC who underwent the modified sutureless technique treatment from 2014 to 2019 in our center were retrospectively analyzed, including 4 males and 7 females. The median surgical age was 1.4 (0.3, 27.0) months. The median weight was 4.3 (3.5, 8.5) kg. Six (54.5%) patients were of supracardiac subtype, and five (45.5%) patients were of infracardiac subtype. Five (45.5%) patients had preoperative severe pulmonary hypertension, and three (27.3%) patients had preoperative pulmonary vein obstruction. The surgical results were compared with those of 10 patients treated with conventional surgical technique. Results    The median follow-up was 12 (range, 1-65) months. During the follow-up, no death or postoperative pulmonary vein obstruction occurred in the modified sutureless technique group. The perioperative data and relief of re-obstruction were superior in the modified sutureless technique group, but the difference was not statistically significant (P>0.05). The postoperative survival of the the modified sutureless technique group was better than that of the traditional surgery group (P=0.049). Conclusion    The modified sutureless technique which includes partial suture and then incising, and eversion of pulmonary vein incision, is a safe and reliable method for the treatment of TAPVC with satisfactory short-term results.

The management of total anomalous pulmonary venous connection and risk factors for postoperative pulmonary venous obstruction / 中华实用儿科临床杂志

Objective:To discuss the treatment strategies and experiences of total anomalous pulmonary venous connection (TAPVC) and study the risk factors for postoperative pulmonary venous obstruction (PVO).Methods:Clinical data of 125 children with TAPVC in the Children Heart Center of Fuwai Central China Cardiovascular Hospital from January 2018 to December 2020 were included in this study and analyzed retrospectively.Of the 125 cases, 116 were treated surgically.They were divided into 2 groups according to whether PVO appeared after repair of TAPVC.The t-test or chi- square test was used to compare the clinical data of the 2 groups, and Logistic regression analysis was adopted to analyze the risk factors for postoperative PVO. Results:Among the 116 patients undergoing surgery, including 73 males (62.9%) and 43 females (37.1%), and according to Darling′s classification, there were 58 cases (50.0%) of supracardiac, 35 cases(30.2%)of intracardiac, 15 cases(12.9%) of infracardiac and 8 cases (6.9%) of mixed.The median age and body weight during surgery were 3.0 (1.2, 7.0) months and 5.0 (4.0, 6.8) kg.There were 13 deaths (11.2%), and recurrent PVO was observed in 18 patients (15.9%). Statistically significant diffe-rences were observed in terms of Darling′s classification (supracardiac 27.8% vs.56.6%, intracardiac 27.8% vs.31.6%, infracardiac 27.8% vs.10.5%, mixed 16.6% vs.5.3%, χ2=8.571, P=0.036) of PVO group and non-PVO group after operation preoperative PVO (83.3% vs.21.1%, χ2=25.293, P<0.01), with confluence (55.6% vs.81.1%, χ2=6.049, P=0.014), preoperative critical state (83.3% vs.31.6%, χ2=12.938, P<0.01), cardiopulmonary bypass time[ (128.800±47.254) min vs.(106.700±37.288) min, t=-2.094, P=0.039 ], delayed thoracic closure(27.8% vs.5.3%, χ2=6.227, P=0.013), and anastomotic velocity at 1 day after operation[1.0(0.9, 1.4) m/s vs.0.9(0.8, 1.1) m/s, Z=-2.004, P=0.045], anastomotic velocity at 1 week after operation[1.4 (1.3, 1.8) m/s vs.0.9 (0.7, 1.1) m/s, Z=-4.446, P<0.001], and mechanical ventilation time[121.0 (76.8, 246.9) h vs.91.5 (60.4, 135.9) h, Z=-1.989, P=0.047]. All of these data were included in the Logistic regression analysis.The results showed that preoperative PVO ( OR=797.179, 95% CI: 8.074-78 712.270, P=0.004) and increased anastomotic flow velocity at 1 week after operation ( OR=11 848.376, 95% CI: 23.746-5 912 017.803, P=0.003) were associated with postoperative PVO. Conclusions:Surgical correction in patients with TAPVC with a biventricular anatomy in this center is satisfactory.Preoperative PVO and early anastomotic flow velocity increase after operation are the high risk factors for postoperative PVO.

Comparison of intracardiac and upturned approach for total anomalous pulmonary venous connection / 中华胸心血管外科杂志

Objective:Analyze the effect of intracardiac method and upturning method in the treatment of infracardiac total anomalous pulmonary venous connection(TAPVC), to explore the surgical method of infracardiac TAPVC.Methods:From July 2011 to August 2019, 20 patients with infracardiac TAPVC were treated, including 12 cases with upturning method and 8 cases with intracardiac method. The cardiopulmonary bypass time, aortic cross-clamp time, delayed thoracic closure, ICU time, mechanical ventilation time, postoperative days and anastomotic flow rate were compared between the two groups.Results:There was no significant difference in cardiopulmonary bypass time, aortic cross-clamp time, delayed thoracic closure, ICU time and mechanical ventilation time between the two groups. The postoperative hospital stay in upturning group was significantly lower than that in intracardiac group [(14.7±2.9)days vs.(16.1±6.2)days, P<0.05], and the postoperative anastomotic velocity > 120 cm/s in intracardiac group was significantly less than that in upturning group(1 case vs. 7 cases, P<0.05). Two patients died in upturning group, but there was no significant difference compared with the intracardiac group. Conclusion:There is no significant difference between the two methods in the treatment of subcardiac TAPVC. The authors think that the exposure of the upturning methods is difficult, and the distortion of the anastomosis may be hidden trouble. The in situ anastomosis of the intracardiac method is not easy to make mistakes.

A Double Connection of Mixed Total Anomalous Pulmonary Venous Connection : Successful Patch Closure of Residual Shunt / 日本心臓血管外科学会雑誌

Double drainage sites from a common venous confluence of the pulmonary veins of a mixed total anomalous pulmonary venous connection is a rare condition that is called a “double connection.” There have been very few reports of reoperation for minor drainage of a double connection. A 28-year-old male with double connection type (Ia: major drainage + IIa: minor drainage) mixed total anomalous pulmonary venous connection (TAPVC) was referred to our institution. He had undergone TAPVC type Ia (major drainage) repair at the age of 1 year. Postoperative enhanced computed tomography showed residual TAPVC IIa (minor drainage) at the age of 15 years. Therefore, the definitive diagnosis was double connection-type mixed TAPVC. Residual shunt gradually increased, and cardiac catheterization revealed an increased pulmonary blood flow/systemic blood flow ratio. Echocardiography showed enlarged shunt vessel and mild tricuspid regurgitation. Catheter intervention was considered too risky and, therefore, we performed patch closure of the residual shunt through a right atriotomy. The postoperative course was uneventful, and follow-up computed tomography showed shrinkage of the shunt vessel without any thromboembolic events.

Clinical value of left atrium-descending aorta distance measured on ultrasound images in the diagnosis of fetal total anomalous pulmonary venous connection / 中国基层医药

Objective:To investigate the clinical value of ultrasonic measurement of left atrium-descending aorta distance in the diagnosis of fetal total anomalous pulmonary venous connection (TAPVC).Methods:Pregnant women who underwent fetal anatomy scans in the second trimester of pregnancy in Central Hospital of Panyu District between January 2018 and June 2019 were included in this study using prospective and case-control study methods. The GE Voluson E8 and Philips EPIQ 7 ultrasound machines were used to measure the left atrium-descending aorta distance in the four-chamber view of the fetal heart. Pulmonary vein was carefully examined. Fetuses with isolated TAPVC were included in the positive group. The pregnancy outcomes were followed up during all participants. 200 healthy fetuses were randomly selected and included in the control group. The correlation between left atrium-descending aorta distance in normal fetuses and gestational weeks was analyzed. The average value of left atrium-descending aorta distances was compared between positive and control groups.Results:A total of 2 156 pregnant women received fetal anatomy scans, with the completion rate of 100%. Among them, 1 786 pregnant women were successfully followed up and 370 were lost to follow up. Among the 1 786 pregnant women, four fetuses were diagnosed with isolated TAPVC, consisting of three fetuses with intracardiac type TAPVC and one fetus with supracardiac type TAPVC, as confirmed by prenatal ultrasound. The left atrium-descending aorta distance in 200 normal fetuses was weakly related to gestational weeks ( r2 = 0.35, P < 0.000 1). The mean left atrium-descending aorta distance in the positive group was significantly greater than that in the control group (5.4 mm vs. 2.1 mm). Conclusion:Ultrasonic measurement of left atrial posterior spatial distance is simple and it is hardly affected by gestational weeks. It is innovative to diagnose TAPVC through quantitative analysis. The widening of left atrium-descending aorta distance has a certain value in suggesting fetal TAPVC. The sample size is small in this study. Multi-center studies involving larger sample sizes are needed to further validate the clinical significance of widened left atrium-descending aorta distance.

Surgery for pulmonary venous stenosis after total anomalous pulmonary venous connection repair: Midterm results of 9 patients / 中国胸心血管外科临床杂志

@#Objective    To review our experience of reoperations for pulmonary venous stenosis (PVS) after total anomalous pulmonary venous connection (TAPVC) repair for the past decade in Fuwai Hospital. Methods    Nine patients underwent reoperation for PVS between 2009 and 2019 in Fuwai Hospital, including 4 males and 5 females with an average age of 5.10±5.00 years. The patients were divided into a sutureless group (n=3) and a non-sutureless group (n=6). Clinical data were reviewed and analyzed. Results    For primary TAPVC type, 4 patients were supracardiac, 2 patients were cardiac, 1 patient was infracardiac, and 2 patients were mixed-type anomaly. The median cardiopulmonary bypass time was 95 (63, 208) min, aortic clamping time was 58 (30, 110) min, ICU stay was 24 (24, 2 136) h. Early hospital death occured in 1 (11.1%) patient. One (11.1%) patient with single ventricle physiology had hospital comorbidity, who underwent hemofitration therapy. The follow-up time was 11.9 (2.2, 18.0) months, during which 1 patient died of restenosis of pulmonary vein and another patient died of stroke. No statistically significant difference was found between the sutureless group and non-sutureless group in postoperative or follow-up results (P>0.05). Conclusion    Surgery is effective for treatment of PVS after repair of TAPVC, yet with a realatively high morbidity and mortality. The advantage of sutureless repair over conventional repair for this particular group of patients is yet to be verified.

Surgical experience of total anomalous pulmonary venous connection in adults / 中华胸心血管外科杂志

Objective:To explore the therapeutic strategy and result of adult total anomalous pulmonary venous connection(TAPVC).Methods:From November 2011 to November 2019, 6 adult patients with TAPVC underwent surgical correction. The Darling types include 4 cases of supracardiac , 1 case of intracardiac and 1 case of mixed type. There were 1 male and 5 female. The mean age was(28.6±4.8) years old and the mean weight was(47.3±3.67) kg. Preoperative oxygen saturation was 0.91±0.05.Results:All patients underwent primary repair successfully without perioperative death and complications. The average cardiopulmonary bypass time was(122.0±35.9) min, and the aortic cross-clamp time was(78.2±20.4) min. The mean postoperative hospitalization was(9.7±2.9) days, and the mean intensive care unit time was(3.5±1.4) days.The mean mechanical ventilation was(17.1±2.9) h. There were no later left heart dysfunction and pulmonary vein obstruction during the follow-up of 6-100 months.no pulmonary artery hypertension was identifed.Conclusion:TAPVC can be repaired savely in adult and satisfied result can be anticipated.

Risk factors for postoperative pulmonary venous obstruction after correction of total anomalous pulmonary venous connection / 中华胸心血管外科杂志

Objective:To analyze the risk factors for postoperative pulmonary venous obstruction after correction of total anomalous pulmonary venous connection(TAPVC).Methods:We retrospectively reviewed all patients undergoing operative repair of TAPVC in our institution from December 2013 to January 2018. Patients with functionally univentricular circulations or atrial isomerism were excluded. Patients were divided into two groups according to whether there was pulmonary vein obstruction. The clinical variables of the two groups were compared. Variables for the multivariable analysis were chosen if there was statistical significance on univariable analysis.Results:145 patients were included, 91(63%) males, aged 4(2, 8)months and weight 5.5(4.5, 7.5)kg. Mean follow-up interval was(51±23) months. Postoperative obstruction developed in 27 patients(18.6%). The differences of anatomic type[supracardiac 18(67%) vs.59(50%), cardiac 4(15%) vs. 50(42%), infracardiac 3(11%) vs. 1(1%), mixed 2(7%) vs. 8(7%), P=0.003], preoperative obstruction[yes 19(70%) vs. 37(31%), no 8(30%) vs. 81(69%), P<0.001], associated cardiac lesions[yes 13(48%) vs. 27(23%), no 14(52%) vs. 91(77%), P=0.008] and bypass time[109(89, 129)min vs. 88(70, 110)min, P=0.002] between two groups were statistical significant. A multivariable model showed preoperative obstruction( P<0.001) and bypass time( P=0.009) were associated with postoperative obstruction. Conclusion:The incidence of pulmonary vein obstruction after correction of TAPVC was still high. If there was preoperative obstruction, or the bypass time was too long during operation, the surveillance of pulmonary vein obstruction should be strengthened after operation.

Follow-up and further intervention for postoperative pulmonary venous obstruction of total anomalous pulmonary venous connection / 中华胸心血管外科杂志

Objective:Postoperative venous obstruction (PVO) is the most severe complication of total anomalous pulmonary venous connection (TAPVC), and facing challenging re-intervention with high mortality. We aimed to review and analyze the follow-up and management of postoperative PVO in our center.Methods:We conducted a retrospective study of the patients with isolated TAPVC admitted in our center from October 2013 to October 2019. All available data and images of PVO patients were reviewed, such as the initial perioperative medical records, patients’ follow-up records, results of patients’ echo and CT angiography. Re-intervention including hybrid technique, sutureless technique, and patch augmentation, were carried out for postoperative PVO patients. The results were reviewed and analyzed to find the risk factors for adverse prognosis.Results:A series of 174 isolated TAPVC patients were admitted in our center and 169 received surgical treatment and 26 (26/169, 15.4%) had postoperative PVO. The diagnosis was made at a median time of 11.5 (0-77) weeks after initial operation and within 6 months of surgery in 22 (22/26, 84.6%) of the 26 patients. The subtype of TAPVC patients with postoperative PVO were: supracardiac 11 cases (11/26, 42.3%), cardiac 7 cases (7/26, 26.9%), infracardiac 5 cases (5/26, 19.2%), and mixed 3 cases (3/26, 11.5%). Bilateral obstruction and stenosis with diffusely small pulmonary veins were in 12 (12/26, 46.2%) and 3 cases (3/26, 11.5%) respectively. PVO progressed to worse condition in all the 26 cases during follow-up period. 8 (8/26, 30.8%) postoperative PVO patients underwent 10 re-interventions: one cases had 3 re-interventions. Five-year survival for patients with postoperative PVO was worse than those without postoperative PVO ( HR=6.46, 95% CI: 2.34-17.85, P<0.01). Risk factors for death or re-intervention in postoperative PVO patients were earlier presentation after TAPVC repair ( HR=0.85, 95% CI: 0.73-0.99, P=0.04) and an increased number of lung segments affected by obstruction ( HR=1.74, 95% CI: 1.01-2.99, P=0.04). Conclusion:Risk factors for death or re-intervention in postoperative PVO patients were earlier presentation after TAPVC repair and an increased number of lung segments affected, which should be focused on during strict follow-up period. Early re-intervention should be taken before irreversible secondary changes occur in these patients.

Surgical repair for simple total anomalous pulmonary venous connection in neonates / 中华胸心血管外科杂志

Objective:This study aimed at analyzing risk factors associated with surgical outcomes of neonatal total anomalous pulmonary venous connection (TAPVC) in our center.Methods:A total of 105 neonates who underwent surgical repair for TAPVC from January 1st, 2009 to January 1st, 2018 were retrospectively analyzed. The anatomical types of TAPVC included supracardiac 42(40%, 42/105), cardiac 21(20%, 21/105), infracardiac 36(34.3%, 36/105), and mixed 6(5.7%, 6/105). The Cox proportional hazards analysis was used to analyze the risk factors related to postoperative pulmonary venous obstruction (PVO) and mortality. Kaplan- Meier analysis was used to analyze the overall survival rates. Results:Twenty-six patients (24.8%, 26/105) were diagnosed with preoperative PVO. The 30-day, 1 year, and 5 years survival rate was 92.4%, 86.7%, and 86.7% respectively. Postoperative PVO occurred in 17 patients (16.2%, 17/105). Preoperative acidosis, low surgical weight, prolonged duration of cardiopulmonary bypass time, increasing postoperative central venous pressure (CVP), and reoperation were risk factors associated with mortality. Preoperative acidosis ( P<0.001), prolonged duration of cardiopulmonary bypass time ( P<0.001), and increasing postoperative CVP ( P=0.005) were independent risk factors for mortality. Mixed TAPVC, preoperative acidosis, low surgical age, prolonged cardiopulmonary bypass time, postoperative pulmonary arterial hypertension were risk factors associated with postoperative PVO. Prolonged cardiopulmonary bypass time ( P=0.029), postoperative pulmonary arterial hypertension ( P<0.001), and mixed TAPVC ( P=0.017) were independent risk factors associated with postoperative PVO. Conclusion:The surgical outcomes of neonatal TAPVC in our center were acceptable, with low mortality rate and incidence of PVO. However, neonates with preoperative acidosis, prolonged duration of cardiopulmonary bypass time, and increased postoperative CVP had a poor prognosis. Patients with mixed TAPVC were at increased risk for postoperative PVO.

Surgical treatment and mid-term results of functional single ventricle associated with total anomalous pulmonary venous connection / 中国胸心血管外科临床杂志

@#Objective    To evaluate the mid-term results of surgical treatment for functional single ventricle associated with total anomalous pulmonary venous connection. Methods    We reviewed the clinical data and follow-up results of 12 patients in our hospital who underwent both single ventricle series palliation and total anomalous pulmonary venous connection correction from 2008 to 2018. There were 6 males and 6 females at age of 2.3 (1-21) years. Univariable and multivariable Cox proportional hazard regression methods were performed. The Kaplan-Meier method was used to estimate the survival rate. Results    All patients were successfully removed from extracorporeal circulation. The cardiopulmonary bypass time was 113.8±42.5 min. The myocardial block time was 57.7±31.7 min. There were 3 in-hospital deaths and 4 late deaths. The causes of death in hospital were pulmonary hypertension, pulmonary hemorrhage, and hypoxemia. During follow-up, the cause of death was heart failure. The survival rate at 1 year and 3 years was 58.3%(95%CI 27% to 80%) and 40% (95%CI 13% to 65%), respectively. Median follow-up was 48.3 months (range: 1 to 118 months). Conclusion    Functional single ventricle combined with complete pulmonary venous drainage is a serious clinical disease, with poor therapeutic effect and high overall mortality.

Comparative analysis of the treatment of partial anomalous pulmonary vein connection by double-patch method or Warden Technique / 中华胸心血管外科杂志

Objective:To compare the surgical and long-term follow-up results of partial anomalous pulmonary vein connection treated by double-patch method or Warden Technique.Methods:There were 33 cases of right pulmonary vein connected with the superior vena cava from May 2010 to May 2019 in our center treated by double-patch method or Warden technique. 21 cases were treated by double-patch method and 12 cases by Warden technique. Echocardiography and electrocardiogram were followed up regularly to observe the occurrence of arrhythmia, superior vena cava stenosis and pulmonary vein stenosis postoperatively.Results:All patients were discharged uneventfully, and were followed up for 1~8 years. In double-patch group, 2 cases with arrhythmia, 1 of whom was junctional arrhythmia which was automatically converted to sinus rhythm 1 day after surgery. The other had an early second degree atrioventricular block after surgery, and sinus rhythm was restored 3 days later with temporary pacemaker. 1 case had superior vena cava stenosis by echocardiography(PD 8 mmHg). No arrhythmia was found in long-term follow-up in Warden group. 2 cases had superior vena cava stenosis by echocardiography(PD 6 mmHg). Right pulmonary vein stenosis(PD 8 mmHg) was found in 1 case by echocardiography, no obvious aggravation was found in long-term follow-up.Conclusion:The double-patch method and Warden Technique are both safe and effective in the treatment of partial anomalous pulmonary venous connection.

ARHGEF16 variants screening and mutation function analysis for children with total anomalous pulmonary venous connection / 上海交通大学学报（医学版）

Objective:To investigate the pathogenesis of total anomalous pulmonary venous connection (TAPVC), and to identify ARHGEF16 gene through full exon sequencing screening and analyze its mutation function. Methods:The blood, clinical data and auxiliary examination results of 78 children with TAPVC and 100 healthy controls were collected, and the genomic DNA was extracted for ARHGEF16 mutation screening. ARHGEF16 wild-type and mutant expression plasmids were constructed and transfected into 293T cells. mRNA and protein expression levels were detected by quantitative real-time PCR (RT-qPCR) and Western blotting, respectively. Protein-protein interaction exploration was performed by Cytoscape software. Results:Two novel variants c.C236>T (A79V) and c.G619>C (G207R) were found in TAPVC patients and were not found in healthy controls. Compared with the wild type, the mutants ARHGEF16 were up-regulated in both mRNA and protein expression levels. Protein interaction analysis showed that ARHGEF16 and RAC1 were directly associated; RAC1 expression was up-regulated in HEK293 cells with ARHGEF16 overexpression through RT-qPCR. Conclusion:The missense mutations of ARHGEF16 affect the mRNA and protein expression levels of ARHGEF16. Overexpression of ARHGEF16 up-regulates the expression of RAC1, suggesting that it may participate in the development and formation of TAPVC by regulating RAC1.

Severe Aortic Stenosis and Partial Anomalous Pulmonary Venous Connection in a Turner Syndrome Patient / 日本心臓血管外科学会雑誌

A 42-year-old woman with Turner syndrome was admitted to our hospital due to severe aortic stenosis. Transthoracic echocardiography demonstrated severe aortic stenosis with a bicuspid aortic valve. Enhanced computed tomography revealed that the left upper pulmonary vein connected to the innominate vein, and the ascending aorta was enlarged (maximum diameter of 41 mm). Surgical intervention was performed though median sternotomy with cardiopulmonary bypass. After achieving cardiac arrest by antegrade cardioplegia, we performed an anastomosis to connect the left upper pulmonary vein to the left atrial appendage. Then, aortic valve replacement was performed with an oblique aortotomy in the anterior segment of the ascending aorta. The aortic valve was a unicaspid aortic valve. Following completion of aortic valve replacement with a mechanical valve, reduction aortoplasty was performed on the ascending aorta. The postoperative course was uneventful.

Treatment strategies of vertical vein during correction for total anomalous pulmonary venous connection in newborns / 中国胸心血管外科临床杂志

@#Objective    To compare the effect of different treatment strategies of vertical vein on the postoperative efficacy of total anomalous pulmonary venous connection (TAPVC) in newborns. Methods    The clinical data of 72 TAPVC newborns who underwent correction in our hospital from June 2008 to January 2018 were retrospectively analyzed. There were 59 males and 13 females, with an average age of 9.93±8.13 d. According to different vertical vein treatment strategies, the patients were divided into an unligated group (22 patients), a partially ligated group (23 patients) and a completely ligated group (27 patients). The mortality and incidence of common complications among the groups were compared. Results    In the completely ligated group, the aortic cross-clamp time was longer (P=0.001), intraoperative circulatory arrest cases were less (P=0.005), and the early velocity of pulmonary vein was faster (P=0.029), but there was no significant difference in the velocity of pulmonary vein for the last follow-up among three groups (P=0.393). There was no significant difference in other perioperative indicators among groups, including the early mortality, the incidence of pulmonary vein obstruction and the non-closure of vertical vein after surgery (P>0.05). The mean follow-up time was 4.47±2.63 years. The follow-up mortality was not significantly different among groups (P>0.05). The cardiac function (NYHA) of all the 64 survived patients was classⅠ-Ⅱ. Conclusion    Proper vertical vein treatment strategies may be conducive to a smooth transition after surgery, ensuring the success of surgeries.