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Objective:This study aimed at reviewing surgical experiences and exploring risk factors for mortality and postoperative complications in patients with infracardiac total anomalous pulmonary venous connection (TAPVC).Methods:This retrospective study included 74 infants who underwent conventional repair (28 cases) and sutureless repair (46 cases) in one hospital from February 2009 to December 2022. Clinical data were reviewed to assess risk factors for mortality and postoperative pulmonary venous obstruction (PVO). Kaplan- Meier curves and cox regressions were applied to analyze the overall survival. Cumulative incidence curve and sub-distribution hazard models were used to evaluate postoperative PVO. Results:There were 4 early deaths and 3 late deaths, and the overall survival rate was 90.5%. A total of 12 patients complicated postoperative PVO. The median follow-up was 39.4 months ( IQR: 13.3 to 73.7 months). The overall survival rate was higher in the sutureless group than the conventional group ( P=0.003). The incidence rate of postoperative PVO in the conventional group was higher than that in the sutureless group ( P= 0.008). Risk factors for recurrent PVO included longer cardiopulmonary bypass time, the increase of direct bilirubin level and international normalized ratio level before surgical repair. Conclusion:Both sutureless and conventional repairs for patients with infracardiac TAPVC can achieve acceptable postoperative outcomes. Sutureless repair has a higher survival rate and a lower incidence of re-stenosis in pulmonary veins and anastomosis.
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@#Objective To evaluate the safety and mid- to long-term efficacy of surgical correction of isolated partial anomalous pulmonary venous connection (IPAPVC). Methods We retrospectively collected consecutive patients who were diagnosed with IPAPVC and underwent surgical correction at Fuwai Hospital of Chinese Academy of Medical Sciences and Fuwai Yunnan Cardiovascular Hospital from June 2009 to May 2019, summarized the basic preoperative and intraoperative data of patients, analyzed the postoperative and mid- to long-term follow-up results. Results A total of 54 patients were enrolled, including 29 males and 25 females, with an average age of 16.20±2.40 years, ranging from 1 month to 62 years. There were 28 (51.9%) patients with varying degrees of arrhythmia, 22 (40.7%) patients with cardiac insufficiency, and 39 (72.2%) patients with pulmonary hypertension. According to Bordy's typing, 14 (25.9%) patients were classified as type A, 23 (42.6%) type B, 4 (7.4%) type C, 5 (9.3%) type D and 8 (14.8%) mixed type. Transthoracic echocardiography was performed in the whole group of patients and the accuracy of staging diagnosis was 66.7% (36/54), and cardiac CT angiography (CTA) was performed in 37 patients and the accuracy of staging diagnosis was 94.6% (35/37). All surgical procedures were assisted with cardiopulmonary bypass, aortic cross-clamping time was 0-219 (67.02±5.23) min, cardiopulmonary bypass time was 40-261 (105.09±5.23) min, and there was no serious intraoperative complication. Postoperative tracheal intubation time was 0-230 (13.33±4.20) h, intensive care unit stay was 0-13 (1.89±0.28) days, postoperative hospital stay was 5-18 (7.20±0.38) days, and follow-up time was 16-140 (62.58±5.12) months. There were 2 (3.7%) all-cause postoperative deaths, including 1 in-hospital death and 1 death during the follow-up, and there was no intraoperative death. Among the survivors, there were 3 patients with surgery-related complications: 1 patient had atrial septal defect with the second surgical treatment, 1 early obstruction of the superior vena cava and 1 arrhythmia. Two patients had complications of IPAPVC (atrial fibrillation, collateral circulation) prior to surgery and underwent the second surgery with a poor prognosis, and 1 patient had preoperative cardiac insufficiency and atrial fibrillation, whose symptoms persisted for a long time during the follow-up. Conclusion IPAPVC accounts for a lower percentage of partial anomalous pulmonary venous connection, transthoracic echocardiography combined with CTA improves diagnostic accuracy, and IPAPVC should be treated with elective surgery after diagnosis. The surgical approach should be individualized with imaging features such as disease staging, number of drains and drainage location. Surgical treatment of IPAPVC is safe and effective, and regular follow-up is warranted.
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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.
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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.
Тема - темы
Female , Humans , Pregnancy , Infant, Newborn , Echocardiography , Heart Defects, Congenital/surgery , Pulmonary Veins/surgery , Retrospective Studies , Scimitar Syndrome/surgeryРеферат
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.
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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.
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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.
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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.
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@#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.
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@#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.
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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.
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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.
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@#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.
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@#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.
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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.
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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.
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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.
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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.
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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.
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The patient was a 34-year-old woman who had been routinely monitored after receiving a childhood diagnosis of partial anomalous pulmonary venous connection, but unilaterally discontinued follow-up examinations after the age of 18. At 33 years of age, she was admitted to our hospital after a physical examination revealed an abnormal shadow on a chest X-ray. Transthoracic echocardiography detected an atrial septal defect (ASD), and contrast-enhanced computed tomography showed that the right lower pulmonary vein drained to the inferior vena cava. The patient was diagnosed with scimitar syndrome with ASD. Cardiac catheterization showed a pulmonary/systemic flow ratio (Qp/Qs) of 2.48 and a left-to-right shunt rate of 59.7%. Surgical treatment was deemed to be indicated. The right lower pulmonary vein was anastomosed to the anterolateral wall of the right atrium, and an intra-atrial baffle repair was performed from the orifice within the right atrium to the left atrium through the existing ASD using untreated fresh autologous pericardium. Two years after the operation, good blood flow was maintained within the baffle with no stenosis at the anastomotic site. This report describes a rare case of scimitar syndrome with ASD in an adult woman, and provides a review of the existing literature.