Preoperative Pulmonary Venous Obstruction Research Articles

Total anomalous pulmonary venous connection in 80 patients: Primary sutureless repair and outcomes.

Total anomalous pulmonary venous connection (TAPVC) is a rare but critical cardiac anomaly, in which pulmonary veins are connected to an abnormal location rather than the left atrium. The prognosis can be extremely poor without intervention, with a mortality of 80% during infancy. The purpose of this research is to summarize the outcomes and relevant risk factors of 80 total anomalous pulmonary venous connection (TAPVC) patients who underwent primary TAPVC sutureless repair and discuss the indications and benefits of primary sutureless repair. This retrospective review included 80 patients with TAPVC who underwent primary sutureless repair at a single institution between January 2015 and December 2020. Patients were subdivided into 4 groups according to Darling's classification. Risk factors that increase the postoperative pulmonary vein flow velocity were explored by Multiple Linear regression. Anatomic TAPVC subtypes included supracardiac 35 (43.8%), cardiac 24 (30%), infracardiac 17 (21.2%), and mixed 4 (5%). Median age at repair was 16.5 days and median weight was 3.5 kg. Preoperative pulmonary venous obstruction (PVO)was presented in 20 (25%) patients. There were 2 early deaths and 1 late death. 2 patients developed postoperative PVO and none required reintervention. Prolonged cardiopulmonary bypass time (CPB) (p = 0.009), preoperative pneumonia (p = 0.022) and gender (p = 0.041) were found to be associated with the increase of postoperative pulmonary vein flow velocity. Under the primary sutureless technique, no statistical difference was observed among the 4 subgroups in terms of postoperative pulmonary vein flow velocity (p = 0.589). The primary sutureless technique may eliminate the differences between subtypes while decrease the postoperative PVO rate, which makes it applicable in any subtypes of TAPVC. Following the favorable outcomes in preventing postoperative PVO in all subtypes in this study, we advocate the indications for primary sutureless repair may expand further to all the TAPVC patients.

Outcomes of treatment for right atrial isomerism with functional single ventricle and extracardiac total anomalous pulmonary venous connection beyond neonatal period: Delayed surgical treatment, improving outcomes.

Total anomalous pulmonary venous connection (TAPVC) is frequently associated with right atrial isomerism (RAI), which is commonly complicated with an unbalanced atrioventricular canal with contralateral hypoplasia, complex systemic and pulmonary venous anatomy, and conotruncal abnormalities, resulting in increased risk of mortality. This study aimed to review the outcomes of delayed surgical treatment for patients with RAI complicated with functional single ventricle (FSV) and TAPVC at a single center. In this retrospective study, we reviewed the medical records of 24 consecutive patients with RAI complicated with FSV and TAPVC who underwent initial surgical palliation after 5-month old between September 2008 and June 2019. Demographic data, concomitant anomalies, age at initial palliation, and surgical interventions were extracted and analyzed using the Cox proportional hazard model to assess risk factors for mortality and the Kaplan-Meier method to assess survival. The in-hospital mortality was 12.5% (three out of 24). The causes of death were pulmonary arterial hypertension and low cardiac output syndrome. Average follow-up was 65.2 ± 40.3 months (7-137 months). Another 4 patients died during the follow-up due to low cardiac output syndrome, protein-losing enteropathy and pulmonary arterial hypertension, respectively. Kaplan-Meier estimated survival at 1 and 5 years were 83.1 and 69.4%, respectively. Fontan completion was 45.8% (11/24). The mortality for patients with pulmonary venous obstruction (PVO) was 66.7% (4/6). Cox multivariate regression analysis indicated that preoperative PVO was the only risk factor for mortality (p = 0.032; hazard ratio, 10.000; CI 1.222-81.811). Outcomes of delayed surgical treatment for patients with RAI complicated with FSV and TAPVC have improved significantly. The survival and Fontan completion were higher. However, preoperative PVO was still the risk factor for mortality.

Cardiac-type total anomalous pulmonary venous return is not benign

ObjectiveThe objective of this study was to investigate the association between morphological variation and postsurgical pulmonary vein (PV) stenosis (PPVS) in patients with cardiac total anomalous pulmonary venous connection (TAPVC). MethodsThis single-center, retrospective study included 168 pediatric patients who underwent surgical repair of cardiac TAPVC from 2013 to 2019 (connection to the coronary sinus [CS], n = 136; connection directly to the right atrium [RA], n = 32). Three-dimensional computed tomography modeling and geometric analysis were performed to investigate the morphological features; their relevance to the PPVS was examined. ResultsThe connection type had no association with PPVS (CS type: 18% vs right atrial type: 19%; P = .89) but there was a higher incidence of PPVS in patients with a single PV orifice than > 1 orifice (P < .001). Confluence-to-total PV area ratio (hazard ratio, 4.78, 95% CI, 1.86-12.32; P = .001) and length of drainage route (hazard ratio, 1.22; 95% CI, 1.14-1.31; P < .001) had a 4- and 1-fold increase in the risk for PPVS in the CS type after adjustment for age and preoperative pulmonary venous obstruction. In the right atrial type, those with anomalous PV return to the RA roof were more likely to develop PPVS than to the posterior wall of the RA (P < .001). ConclusionsThe number of inter-junction PV orifice correlated with PPVS development in cardiac TAPVC. The confluence-to-total PV ratio, length of drainage route, and anomalous PV return to the RA roof are important predictors for PPVS. Morphological subcategorization in this clinical setting can potentially assist in surgical decision-making.

Sutureless Closure Versus Conventional Technique in the Primary Surgery of Total Anomalous Pulmonary Venous Connection: A Systematic Review and Meta-analysis.

Sutureless closure has been used for primary repair of total anomalous pulmonary venous connection (TAPVC) for over 20years but its superiority over conventional technique is still uncertain. This systematic review was conducted to compare the effectiveness of sutureless closure and conventional surgery as the primary repair for TAPVC. Systematic search was performed in June 2021 on 12 databases. All studies comparing sutureless and conventional surgery for TAPVC were included. The primary endpoints were early mortality, overall mortality, postoperative pulmonary venous stenosis (PVS), and reoperation. Meta-analysis of two-arm studies was performed with several sensitivity and subgroup analyses. Six retrospective studies with 767 patients were included in meta-analyses. Sutureless closure significantly reduced the risk of early mortality, overall mortality, postoperative PVS, and reoperation by 53%, 45%, 77%, and 67% compared to conventional technique, respectively. No heterogeneity was found and presence of publication bias was non-significant. The results were consistent in all sensitivity analyses. Subgroup analyses revealed that sutureless closure was superior to conventional technique in patients with and without preoperative pulmonary venous obstruction, and neonates and non-neonates. Sutureless closure is better than conventional closure as the primary surgery for TAPVC patients. We advocate using sutureless closure for patients with TAPVC. Future large-scale observational studies or clinical trials are required to confirm our findings.

Mid-Term Results and Risk Factors For 10 Years of Functional Single Ventricle Associated With Total Anomalous Pulmonary Venous Connection

There are few surgical treatment results in elderly patients with functional single ventricle (FSV) and total anomalous pulmonary venous connection (TAPVC). We retrospectively analyzed 10 years of mid-term surgical treatment results and risk factors of these age-specific people. Between March 2008 and December 2018, 43 consecutive patients with FSV and TAPVC received initial surgical palliation in our center. There were 20 cases of supracardiac TAPVC, 21 of cardiac type, and two cases of mixed type. Initial surgical palliation procedures involved pulmonary artery banding (PAB) for patients, modified Blalock-Taussing shunt (mBTs) for five patients, and bidirectional Glenn (BDG) for 34 patients. TAPVC repair was performed in 12 patients during BDG. The 1-year and 5-year overall survival rates were 69.7% and 62.8%, respectively. In TAPVC repair group and non-TAPVC repair group, the 1-year overall survival rates after initial surgical palliation were 41.7 and 80.5%, respectively, and the 3-year ones were 25% and 77%, respectively. There were significant differences in the type of TAPVC (P < 0.001), preoperative pulmonary venous obstruction (P = 0.001), and overall mortality (P = 0.001) between these two groups. Cox univariate and multivariable analysis indicated concomitant TAPVC repair was the only risk factor for mortality. The mid-term results of surgical treatment of FSV and TAPVC, especially for patients who underwent concomitant TAPVC repair, remain poor. TAPVC repair may be a priority over single-ventricular palliative surgery for patients with FSV and TAPVC.

The Outcomes of Total Anomalous Pulmonary Venous Connection in Neonates-10-Year Experience at a Single Center.

Background: Recent developments in surgical techniques and hospital care have led to improved outcomes following repair of total anomalous pulmonary venous connection (TAPVC). However, surgical repair of neonatal TAPVC remains associated with a high risk of postoperative mortality and pulmonary venous obstruction (PVO). We conducted this retrospective study to identify risk factors associated with surgical outcomes in the neonatal population.Methods: A retrospective review was conducted for all 127 neonates who underwent operations for isolated TAPVC from January 2009 to January 2019.Results: Preoperative PVO occurred in 33 (26.0%) of the 127 patients. Fifty patients (39.4%) required tracheal intubation before the operation. Twenty-three patients (18.1%) underwent emergency surgery. There were 11 (8.7%) early deaths. Significant risk factors were prolonged cardiopulmonary bypass (CPB) time (p = 0.013) and increased postoperative central venous pressure (CVP, p = 0.036). There were 5 (4.3%) late deaths within 1 year of repair. The risk factors for overall death were preoperative acidosis (p = 0.001), prolonged CPB time (p < 0.001) and increased postoperative CVP (p = 0.007). In particular, mortality was significantly higher (p = 0.007) with a postoperative CVP > 8 mmHg. With an increase in use of sutureless techniques (p = 0.001) and decrease in deep hypothermic circulatory arrest (p = 0.009) over the past 5 years, postoperative mortality greatly decreased (21.2%: 6.7%, p = 0.016). Postoperative PVO occurred in 15 patients (11.8%). Risk factors were mixed TAPVC (p = 0.037), preoperative acidosis (p = 0.001) and prolonged CPB time (p = 0.006).Conclusion: Although postoperative mortality of neonatal TAPVC has dropped to 6.7% over the past 5 years, it is still relatively high. Risk factors for postoperative death include preoperative acidosis, prolonged CPB time and increased postoperative CVP. Mortality was significantly higher for neonates with an average CVP > 8 mmHg 24 h after surgery.

Totally Anomalous Pulmonary Venous Connection Outcomes from A Single Center

Introduction: There is limited data on totally anomalous pulmonary venous connection (TAPVC) repair results in our country. The aim of this study is to evaluate the early postoperative results of a large series of TAPVC patients operated in our clinic. Patients and Methods: The data of the patients who underwent TAPVC repair in our clinic between May 2005 and May 2021 were retrospectively reviewed using hospital records. Results: A total of 150 TAPVC patients aged between 0 days and 39 years (median 3 months, IQR: 18 days-9 months), 92 males (61%) and 58 females (39%) underwent surgical intervention. The median length of hospi- tal stay was 12 (IQR: 7-16) days. Overall mortality was 14% (21 patients). Seventeen out of 32 patients with additional cardiac anomalies (53%) and 4 out of 118 patients with isolated TAPVC (3%) died. The presence of additional cardiac anomalies was associated with mortality (p&lt; 0.00001). Mortality rate in univentricular patients was 59% (10 patients). This rate was higher in comparison to the mortality rate of biventricular patients (8%; p&lt; 0.00001). Three patients were reoperated due to postoperative pulmonary venous stenosis. Conclusion: The TAPVC outcomes in isolated and biventricular patients were favorable with a low mortality and postoperative pulmonary venous stenosis in this cohort. Patients with a single ventricle physiology and/or heterotaxia had a significantly increased risk of mortality, which might be due to the intrinsic challenges of the univentricular physiology regarding the balance between the pulmonary and systemic circulations. Tendency for increased mortality in the cases with preoperative pulmonary venous obstruction is a potential target for improvement.

Factors Associated with Early Postoperative Results of Total Anomalous Pulmonary Venous Connection Repair: Findings from Retrospective Single-Institution Data in Vietnam.

IntroductionThere are scanty reports of the risk factors for pulmonary hypertensive crisis and low cardiac output syndrome after the operative repair of total anomalous pulmonary venous connection (TAPVC). We aim to evaluate early surgical outcomes of TAPVC and risk factors for pulmonary hypertensive crisis and low cardiac output syndrome.MethodsWe conducted a retrospective medical record review for all patients undergoing operative repair of TAPVC within 5 years. Outcome variables included pulmonary hypertensive crisis, low cardiac output syndrome and early mortality.ResultsOf 58 patients, we documented 77.59% supracardiac, 20.69% cardiac and 1.72% mixed site of connection. About 86.21% patients underwent elective surgery, and 13.79% patients required emergency surgery. Incidence rates were 27.59% for pulmonary hypertensive crisis and 6.90% for low cardiac output syndrome. Body weight below 6 kg, pneumonia, tachycardia, hepatomegaly, preoperative pulmonary congestion on chest x-ray, preoperative elevated mean pulmonary artery pressure, preoperative pulmonary venous obstruction, emergency surgery and prolonged aortic cross-clamping time were significant risk factors for postoperative pulmonary hypertensive crisis. Significant risk factors for postoperative low cardiac output syndrome included pneumonia, prolonged duration of preoperative mechanical ventilation and prolonged aortic cross-clamping time.ConclusionThe early outcome of surgical repair of TAPVC was acceptable, with 96.55% survival rate. This current analysis suggests that a thorough evaluation of all preoperative and operative characteristics is imperative to achieve best medical and surgical outcomes.

Outcomes of Surgical Repair of Total Anomalous Pulmonary Venous Drainage: Role of Primary Sutureless Technique.

To evaluate the surgical outcomes of total anomalous pulmonary venous drainage focusing on survival, postoperative and pulmonary venous obstruction. Further investigate the role of primary sutureless technique in patients with preoperative pulmonary venous obstruction. Consecutive patients underwent total anomalous pulmonary venous drainage repair in our institution during Jan 2000 to Dec 2019 were enrolled into this retrospective analysis. Since 2016, sutureless repair was regularly applied in patients with preoperative pulmonary venous obstruction. All patients with preoperative pulmonary venous obstruction referred before 2016 had underwent traditional repair. A total of 95 patients were included. During follow-up time of 85months, main endpoints were documented in 21 patients, including 9 (9.5%) early deaths, 3 (2.3%) late deaths and 9 (9.5%) postoperative pulmonary venous obstructions. Preoperative pulmonary venous obstruction was presented in 26 (27.4%) patients with more emergent surgery (14/26 vs 3/69, P < 0.001) was required. Main endpoints occurred more in patients with preoperative pulmonary venous obstruction (4/26 vs 5/69, P = 0.004). Patients experienced sutureless technique had a lower incidence of postoperative PVO at follow-up (0/11 versus 4/11, P = 0.045). Outcomes of surgical repair for total anomalous venous drainage are satisfactory. However, preoperative pulmonary venous obstruction may be accompanying unfavorable early deaths and postoperative pulmonary venous obstruction. Propensity matching analysis showed that sutureless technique was benefit for postoperative pulmonary venous obstruction without longer cardiopulmonary bypass and aortic cross-clamp time.

Venous Flow Variation Predicts Preoperative Pulmonary Venous Obstruction in Children with Total Anomalous Pulmonary Venous Connection

Identifying preoperative pulmonary venous obstruction in total anomalous pulmonary venous connection is important to guide treatment planning and risk prognostication. No standardized echocardiographic definition of obstruction exists in the literature. Definitions based on absolute velocities are affected by technical limitations and variations in pulmonary venous return. The authors developed a metric to quantify pulmonary venous blood flow variation: pulmonary venous variability index (PVVI). The aim of this study was to demonstrate its accuracy in defining obstruction. All patients with total anomalous pulmonary venous connection at a single institution were identified. Echocardiograms were reviewed, and maximum (Vmax), mean (Vmean), and minimum (Vmin) velocities along the pulmonary venous pathway were measured. PVVI was defined as (Vmax-Vmin)/Vmean. These metrics were compared with pressures measured on cardiac catheterization. Echocardiographic measures were then compared between patients with and without clinical preoperative obstruction (defined as a need for preoperative intubation, catheter-based intervention, or surgery within 1day of diagnosis), as well as pulmonary edema by chest radiography and markers of lactic acidosis. One hundred thirty-seven patients were included, with 22 having catheterization pressure recordings. Vmax and Vmean were not different between patients with catheter gradients ≥ 4 and<4mm Hg, while PVVI was significantly lower and Vmin higher in those with gradients ≥ 4mm Hg. The composite outcome of preoperative obstruction occurred in 51 patients (37%). Absolute velocities were not different between patients with and without clinical obstruction, while PVVI was significantly lower in patients with obstruction. All metrics except Vmax were associated with pulmonary edema; none were associated with blood gas metrics. The authors developed a novel quantitative metric of pulmonary venous flow, which was superior to traditional echocardiographic metrics. Decreased PVVI was highly associated with elevated gradients measured by catheterization and clinical preoperative obstruction. These results should aid risk assessment and diagnosis preoperatively in patients with total anomalous pulmonary venous connection.

Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection.

A few studies have described the outcomes of systemic-to-pulmonary artery shunt (SPS) for functional single ventricular heart with extracardiac total anomalous pulmonary venous connection (TAPVC). This study aimed to examine the outcomes of SPS with extracardiac TAPVC and identify the predictors of mortality before bidirectional Glenn operation (BDG). Medical records of 41 children with single ventricular heart and extracardiac TAPVC who underwent SPS between 1998 and 2019 were reviewed retrospectively. The median age and body weight at SPS were 36days and 3.4kg, respectively. Surgical outcomes and predictors of mortality were investigated. Four operative deaths (10%) and 10 late deaths (27%) occurred before BDG. Of all the children, 19 underwent BDG at a median of 10months since SPS and eight are waiting for BDG. In the multivariate analysis, preoperative pulmonary venous obstruction (p = 0.01) at initial surgery was most predictive of death before BDG. Patients who underwent simultaneous SPS and TAPVC repair were younger, had high preoperative rate of pulmonary venous obstruction, and more deaths before BDG. Survival outcomes of SPS for SVH with extracardiac TAPVC were improved as a whole due to the increase in knowledge and technique of management SPS. However, the patients who have preoperative pulmonary venous obstruction (PVO) and need SPS and TAPVC repair concomitantly in the early postnatal period have poor outcomes and still challenging. In such a case, staged TAPVC repair and SPS may be beneficial.

Predictors of In-Hospital Mortality in Isolated Total Anomalous Pulmonary Venous Connection.

We assessed risk factors for early mortality in isolated total anomalous pulmonary venous connection over a modern era excluding emergent cases to eliminate the influence of associated factors on surgical outcome. Methods: 70 patients with isolated total anomalous pulmonary venous connection who were repaired electively between January 2013 and February 2018 were included. In-hospital mortality was encountered in 4 patients (5.7%). Upon univariate analysis, low age (P = .003) and weight (P = .001) at surgery, preoperative pulmonary venous obstruction (P = .010), preoperative low oxygen saturation (P = .031), long cardiopulmonary bypass (P = .001) and aortic cross clamp (P = .003) times, long duration of mechanical ventilation (P = .001), chest infection (P = 0.041), postoperative low CO syndrome (P < .001) and long postoperative inotropic support (P = .015) were significant predictors of in-hospital mortality. In multivariate analysis postoperative low cardiac output syndrome (OR: 1.060; 95% CI: 1.008-1.116) and prolonged postoperative mechanical ventilation (OR: 1.772; 95% CI: 1.141-2.751) were independent factors of in-hospital mortality. Surgical repair of TAPVC is now performed with acceptable results. According to our study, postoperative low cardiac output syndrome and prolonged postoperative mechanical ventilation were the most significant predictors for early mortality.

Surgical results of total anomalous pulmonary venous connection repair in 256 patients.

This study was performed to analyse the surgical results of total anomalous pulmonary venous connection (TAPVC) repair at a single institution and to identify trends and variables associated with mortality and morbidity, particularly predictors of recurrent pulmonary venous obstruction (PVO). Our surgical database contained 256 patients with biventricular anatomy who underwent surgical repair for TAPVC from 1981 to 2016. The anatomic TAPVC subtypes in this study were as follows: 114 supracardiac (44.5%), 56 cardiac (21.9%), 64 infracardiac (25.0%) and 22 mixed (8.6%) types. The follow-up for the entire study ranged from 1.6 months to 28.2 years (median 10.4 years). Preoperative PVO was present in 128 patients (50.0%). All patients with TAPVC with single-ventricle anatomy were excluded from the analysis. Data were retrospectively reviewed. Seven (2.7%) early deaths and 26 (10.1%) late deaths occurred. The actuarial survival rate at 20 years postoperatively was 85.3%. The preoperative predictors of operative mortality were younger age and the era of TAPVC repair (before 1998). In addition to these variables, associated cardiac anomalies were predictors of late mortality. Those for postoperative PVO were younger age, lower weight and being an emergency case. The actuarial survival rate at 20 years was 38.6% for patients with postoperative PVO and 92.2% for patients without postoperative PVO (P < 0.001). The long-term outcomes after TAPVC repair in patients with biventricular anatomy were satisfactory. Mortality was significantly associated with the rate of progression of postoperative PVO, and careful follow-up was required especially within 6 months after the primary operation.

Neonatal Outcomes in Total Anomalous Pulmonary Venous Return: The Role of Prenatal Diagnosis and Pulmonary Venous Obstruction.

The objective of this study is to evaluate neonatal outcomes of total anomalous pulmonary venous return (TAPVR) and identify fetal echocardiography findings associated with preoperative pulmonary venous obstruction (PPVO). This retrospective study evaluated TAPVR cases from 2005 to 2014 for preoperative and postoperative outcomes based on prenatal diagnosis, PPVO, and heterotaxy syndrome. Fetal pulmonary and vertical vein Dopplers were analyzed as predictors of PPVO. Of 137 TAPVR cases, 12% were prenatally diagnosed; 60% had PPVO, and 21% had heterotaxy. Of the prenatally diagnosed patients, 63% also had heterotaxy. TAPVR repair was performed in 135 cases and survival to discharge was 82% (112/137). Heterotaxy was the only independent predictor of mortality on multiple regression analysis [OR 5.5 (CI 1.3-16.7), p = 0.02]. PPVO was associated with preoperative acidosis, need for inhaled nitric oxide, and more emergent surgery, but not postoperative mortality. Fetal vertical vein Doppler peak velocity > 0.74m/s mmHg predicted PPVO (93% sensitivity; 83% specificity) while pulmonary vein Doppler did not. TAPVR has severe neonatal morbidity and mortality with low prenatal diagnosis rates in the absence of heterotaxy. Patients with obstructed TAPVR had greater preoperative morbidity, but only heterotaxy was independently associated with increased postoperative mortality. Vertical vein velocity helped prenatally identify those at risk of PPVO.

Superior Approach for Supracardiac Total Anomalous Pulmonary Venous Connection

Studies on the outcomes of surgical repair for supracardiac total anomalous pulmonary venous connection through the superior approach are uncommon. From January 2004 to January 2016, 198 patients with supracardiac total anomalous pulmonary venous connection underwent side-to-side anastomosis between the common pulmonary vein and left atrium through the superior approach. Kaplan-Meier curve was used to demonstrate the survival estimates. Cox proportional hazard model and competing risk regression model were used to identify risk factors for death and postoperative pulmonary venous obstruction. There were six in-hospital deaths and no late deaths. The survival rates at 30 days, 1 year, and 12 years were 97%, 97%, and 97%, respectively. Follow-up was completed in 92.2% of the survivors. Median follow-up was 47 months (range: 0 to 136 months). Twenty-seven patients (14.1%, 27 of 192) required reoperation for pulmonary venous obstruction, residual atrial septal defect, or superior cava vena syndrome. Multivariable analysis showed that preoperative pulmonary venous obstruction (p= 0.012), longer duration of ventilation (p= 0.011), and emergency operation (p= 0.010) were incremental risk factors for death. Aortic cross-clamp time (p < 0.001) and preoperative pulmonary venous obstruction (p= 0.002) were associated with postoperative pulmonary venous obstruction. Side-to-side anastomosis through a superior approach in surgical repair of supracardiac total anomalous pulmonary venous connection can achieve satisfactory results.

Surgery for total anomalous pulmonary venous connection: primary sutureless repair vs. conventional repair.

Despite recent advances in surgical technique and perioperative care, the surgical correction of total anomalous pulmonary venous connection (TAPVC) remains a challenge. The major complication and the main cause of reoperation in TAPVC surgery are the occurrence of pulmonary venous obstruction (PVO). In the 1990s, sutureless repair was introduced as a technique to relieve PVO after TAPVC repair. Following the favorable outcomes for postoperative PVO, the indications for sutureless repair as a primary operation have been expanded to include infants who have preoperative PVO or those at risk of developing PVO after the repair of TAPVC. However, the indications of "prophylactic" primary sutureless repair still remain controversial. Recent studies have shown that normal-risk patients have excellent early and long-term outcomes and a low incidence of reoperation for postoperative PVO. Most patients who survived beyond 2years after TAPVC surgery were in NYHA class I and offered good outcomes. Although favorable early and mid-term outcomes of primary sutureless repair are reported, the long-term outcomes of this technique are still unclear. The influence of non-contractile pericardial tissue interposed between the PV vessel wall and LA myocardium on the atrial function is also unclear in patients who undergo sutureless repair. Another disadvantage of primary sutureless repair is potential bleeding from the gap between the confluence and pericardium into the posterior mediastinum or pleural cavity. Thus, it might be best for primary sutureless repair to be indicated for high-risk infants, such as those with TAPVC associated with single-ventricular physiology, mixed-type TAPVC, or small PV confluence.

Outcomes of Surgery for Mixed Total Anomalous Pulmonary Venous Drainage in Children

Total anomalous pulmonary venous drainage (TAPVD) is an uncommon cardiac defect in children. The mixed subset accounts for 5%-10% of the TAPVD and is variable in its anatomy. The outcomes associated with this subset of patients are rarely reported. A retrospective review of all patients with mixed TAPVD undergoing repair at a single institution (1984-2014) was conducted. A descriptive analysis was performed. Twenty-four patients underwent repair for mixed TAPVD (6 univentricular physiology, 18 biventricular physiology). The mixed TAPVD anatomy included 8 patients in group I (2 + 2 veins), 11 patients in group II (3 + 1 veins), and 5 patients in group III (atypical). Preoperative pulmonary venous obstruction occurred in 8 patients (33%). The median age at repair was 2.2 months (range 2 days to 3 years) and median weight was 4.2 kg (range 1.9 to 12.5 kg). Operative mortality was 13% (3 of 24), 33.3% (2 of 6) for patients with univentricular physiology, and 5.6% (1 of 18) for patients with biventricular physiology. There have been no operative deaths in the biventricular group since 1997 (n = 11). Survival at 30-days was 83% ± 15% (95% confidence interval: 27%-97%) and 94% ± 5% (95% confidence interval: 67%-99%) for the univentricular and biventricular groups, respectively. Reoperation for recurrent pulmonary venous obstruction was required in 2 patients (8.3%) where the sutureless technique was used. The average follow-up after surgery was 9.3 ± 6.4 years (5 months to 21 years), and all surviving patients were asymptomatic. Mixed TAPVD can be repaired with good results in children, particularly in those undergoing biventricular repair.

Total Anomalous Pulmonary Venous Connection: The Current Management Strategies in a Pediatric Cohort of 768 Patients.

Total anomalous pulmonary venous connection (TAPVC) is a rare form of congenital heart disease. This study describes current surgical treatment strategies and experiences in a cohort of patients from 2 congenital cardiac centers in Shanghai and Guangdong in China. This retrospective study included 768 patients operated on between 2005 and 2014. Although most patients (n=690) underwent conventional repair, a sutureless technique was used in 10% (n=78) of cases. A multilevel mixed-effects parametric survival model and a competing-risk analysis were used to analyze associated risk factors for death and recurrent pulmonary venous obstruction (PVO), respectively. Kaplan-Meier analysis was used to analyze the overall survival. The Nelson-Aalen cumulative risk curve was used to compare distributions of time with recurrent PVO. The mean surgical age and weight were 214.9±39.2 days and 5.4±3.6 kg, respectively. Obstructed TAPVC (PVO) was documented in 192 (25%) of the 768 patients. There were 38 intraoperative deaths and 13 late deaths. A younger age at the time of repair (P=0.001), mixed (P=0.004) and infracardiac (P=0.035) TAPVC, preoperative PVO (P=0.027), prolonged cardiopulmonary bypass time (P<0.001), and longer duration of ventilation (P=0.028) were associated with mortality. The median follow-up was 23.2 months (range; 1-112 months). Among the 717 survivors, recurrent PVO was observed in 111 patients (15%). Associated risk factors for recurrent PVO included preoperative PVO (P<0.001), infracardiac TAPVC (P<0.001), mixed TAPVC (P=0.013), and prolonged cardiopulmonary bypass time (P<0.001). Sutureless technique was associated with a lower restenosis rate compared with conventional repair in patients with preoperative PVO (P=0.038), except in newborn patients (P=0.443). Reintervention for restenosis was performed in 24 patients. The function of most survivors (91%) was classified according to the New York Heart Association as functional class I or II. Surgical correction in patients with TAPVC with a biventricular anatomy can achieve an acceptable outcome. Risk factors such as a younger age at the time of repair, infracardiac and mixed TAPVC, and preoperative PVO were associated with a poorer prognosis.

Risk Factors of Total Anomalous Pulmonary Venous Connection Surgery

Background: Total anomalous pulmonary venous connection is a rare congenital heart disease that needs to be surgically corrected. In our study, we retrospectively evaluated the factors associated with mortality and morbidity in patients undergoing total anomalous pulmonary venous connection surgery. Patients and methods: Retrospective data were collected on 34 patients with isolated total anomalous pulmonary venous connection seen from 1990 to 2015. The median age at time of repair was 36 days (range, 1 to 2520 days) with median weight of 3.465 kg (range, 1.4 to 20.7 kg). Pulmonary venous obstruction, reintervention, associated factors for mortality and overall mortality were recorded and analyzed. Results: There were 16 deaths (13 surgical deaths and 3 late deaths due to pulmonary venous restenosis and reintervention). Preoperative pulmonary venous obstruction was presented in 21 patients (21/34, 61.8%) and was found to be associated with overall mortality (p=0.0052). Postoperative pulmonary venous restenosis was also a significant risk factor for mortality (p=0.0421). Emergent surgery is a risk factor for mortality (p=0.004). The associated risk factors, such as pre-operation ventilator FiO2, pre-operation inotropic use, postoperation ventilator FiO2 and post-operation epinephrine use were also associated with patient mortality. The initial SpO2 when arriving at our hospital was a protective factor for mortality. Conclusion: The overall mortality of total anomalous pulmonary venous connection patients has improved in recent years. However, preoperative pulmonary venous obstruction was still an important risk factor for mortality. Postoperative pulmonary venous restenosis was also a risk factor for mortality. Heart failure and emergent surgery were associated with higher mortality rates.

Comparison of Sutureless and Conventional Techniques to Repair Total Anomalous Pulmonary Venous Connection

Surgical repair of total anomalous pulmonary venous connection (TAPVC) is challenging. This study aimed to compare the outcomes of the sutureless and conventional techniques for primary repair. From October 2007 to December 2013, 179 consecutive patients underwent repair of TAPVC (sutureless, n = 81; conventional, n = 98). Propensity score matching was used to submit 140 patients to stratified analysis by the presence or absence of preoperative pulmonary venous obstruction (pre-PVO). Surgeons׳ performance differences were assessed with multilevel mixture survival analysis. Freedom from death and postoperative PVO (post-PVO) were evaluated with Kaplan-Meier curves. Risk factors for death and post-PVO were explored using Cox proportional hazard model. Surgeons׳ multilevel effects did not exist in this study. Following matching, Kaplan-Meier curves showed that for patients with pre-PVO (totally 38 cases), rates of freedom from death and post-PVO at 1 year were 80.0% (59.8%-100.0%) in the sutureless group, which was significantly better than that in the conventional group (38.3% [15.2%-61.4%], P = 0.02). For patients without pre-PVO (totally 102 cases), rates of freedom from death and post-PVO at 1 year were 96.1% [95% CI: 90.8%-100.0%] and 86.7% [76.5%-96.9%] in the sutureless and conventional groups, respectively (P = 0.15). Conventional technique was a risk factor for death (hazard ratio = 4.14, 95% CI: 1.29-13.28) and post-PVO (hazard ratio = 5.56, 95% CI: 1.18-26.27) adjusting for type of TAPVC, pre-PVO, and other confounders. In conclusions, the sutureless strategy for primary repair of TAPVC is safe and effective. For patients with pre-PVO, this strategy may associate with decreased mortality and post-PVO with statistical significance.