Total Anomalous Pulmonary Venous Connection Repair Research Articles

Modified L-shaped incision technique for supracardiac total anomalous pulmonary venous connection as an alternative to sutureless technique.

The modified L-shaped incision technique (MLIT) was successfully applied to the repair of supracardiac total anomalous pulmonary venous connection (TAPVC) with promising mid-term outcomes. It is, however, unclear whether or not MLIT could be an alternative to sutureless technique (ST). All patients ( n =141) who underwent MLIT or ST repair for supracardiac TAPVC between June 2009 and June 2022 were included and a propensity score-matched analysis was performed to reduce the heterogeneity. MLIT was performed in 80.9% (114/141), whereas ST was performed in 19.1% (27/141). Patients who underwent MLIT repair had a lower incidence of pulmonary veinous obstruction (PVO)-related reintervention (1.8 vs. 18.5%, P =0.002), and late mortality (2.6 vs. 18.2%, P =0.006). Overall survival at 10 years was 92.5% (87.7-97.7%) for MLIT and 66.8% (44.4-100%) for ST ( P =0.012). Freedom from postoperative PVO at 10 years was 89.1% (83.2-95.5%) for MLIT and 79.9% (65.6-97.4%) for ST ( P =0.12). Cox proportional hazards regression identified prolonged mechanical ventilation duration, postoperative PVO, respiratory dysfunction, and low cardiac output syndrome were associated with postoperative death and PVO-related reintervention. The MLIT strategy is a safe, technologically feasible, and effective approach for supracardiac TAPVC, which is associated with more favorable and promising freedom from death and PVO-related reintervention.

Risk factors associated with prolonged intensive care unit stay following surgery for total anomalous pulmonary venous connection: a retrospective study

BackgroundProlonged intensive care unit (ICU) stays consume medical resources and increase medical costs. This study identified risk factors associated with prolonged postoperative intensive care unit (ICU) stay in children with total anomalous pulmonary venous connection (TAPVC).MethodsThe medical records of 85 patients who underwent surgical repair of TAPVC were retrospectively analyzed. The patients were divided into prolonged-stay and standard-stay groups. The prolonged stay group included all patients who exceeded the 75th percentile of the ICU stay duration, and the standard stay group included all remaining patients. The effects of patient variables on ICU stay duration were investigated using univariate and logistic regression analyses.ResultsPatient median age was 41 (18–103) days, and median weight was 3.80 (3.30–5.35) kg.Postoperative duration of ICU stay was 11–68 days in the prolonged stay group (n = 23) and 2–10 days in the standard stay group (n = 62). Lower preoperative pulse oximetry saturation (SpO2), higher intraoperative plasma lactate levels, and prolonged postoperative mechanical ventilation were independent risk factors for prolonged ICU stay. Preoperative SpO2 < 88.5%, highest plasma lactate value > 4.15 mmol/L, and postoperative mechanical ventilation duration was longer than 53.5 h, were associated with increased risk of prolonged ICU stay. Young age, low body weight, subcardiac type, need for vasoactive drug support, emergency surgery, long anesthesia time, low SpO2 after anesthesia induction, long cardiopulmonary bypass (CPB) and aortic clamp times, high lactate level, low temperature, large volume of ultrafiltration during CPB, large amounts of chest drainage, large red blood cells (RBCs) and plasma transfusion, and postoperative cardiac dysfunction may be associated with prolonged ICU stay.ConclusionsLower preoperative SpO2, higher intraoperative plasma lactate levels, and prolonged postoperative mechanical ventilation were independent risk factors for prolonged ICU stay in children with TAPVC. When SpO2 was lower than 88.5%, the highest plasma lactate value was more than 4.15 mmol/L, and the postoperative mechanical ventilator duration was longer than 53.5 h, the risk of prolonged ICU stay increased. Improved clinical management, including early diagnosis and timely surgical intervention to reduce hypoxia time and protect intraoperative cardiac function, may reduce ICU stay time.

Intracardiac thrombosis after congenital heart disease surgeries in neonates: a report of two cases

BackgroundIntracardiac thrombosis (ICT) is a rare complication after the cardiopulmonary surgery for interrupted aortic arch (IAA) or total anomalous pulmonary venous connection (TAPVC) without previous records. There are still no general guidelines regarding as the mechanism or management of postoperative ICT in neonates and younger infants.Case presentationWe reported the conservative and surgical therapies in two neonates with intra-ventricular and intra-atrial thrombosis after the anatomical repair for IAA and TAPVC, respectively. There were no risk factors for ICT in both patients, except for the use of blood product and prothrombin complex concentrate. The surgery was indicated after TAPVC correction due to the worsening respiratory status and rapidly decreased mixed venous saturation. Anticoagulation combined with antiplatelet therapies was adopted in another patient. These two were both finally recovered, and three-month, six-month, and one-year follow-up echocardiography revealed no abnormality.ConclusionsICT is uncommon in pediatric population after the surgery for congenital heart disease. Single ventricle palliation, heart transplantation, longer central line use, post-extracorporeal membrane oxygenation, and massive blood product use are major risk factors for postcardiotomy thrombosis. The causes of postoperative ICT are multifactorial, and the immaturity of thrombolytic and fibrinolytic system in neonates may serve as a prothrombotic factor. However, no consensus reached regarding as the therapies for postoperative ICT, and the large-scale prospective cohort study or randomized clinical trial is needed.

A Rare Combination of Transposition of Great Arteries and Total Anomalous Pulmonary Venous Connection

The association of total anomalous pulmonary venous connection (TAPVC) with transposition of great arteries (TGA) is a very rare combination of 2 congenital cyanotic heart diseases. TAPVC, in the case of TGA with the intact interventricular septum (IVS), is a cause of early regression of the left ventricle (LV). Very few cases of this rare combination (TAPVC and TGA with or without VSD) have been reported in the world of literature. We found 10 case reports in the literature. The minimum age was 6 days and the maximum age of 16 years. 5 of them, which were operated before 1990, underwent atrial switch with TAPVC repair. Other 4 of them, operated after 1995, underwent arterial switch with TAPVC repair. In this case report we are reporting 2 such rare cases. In both cases, large VSD prevented LV regression.

Sutureless Repair of Extracardiac Univentricular Total Anomalous Pulmonary Venous Connection

BackgroundThis study aimed to evaluate the results of sutureless repair of extracardiac total anomalous pulmonary venous connection (TAPVC) with a functional single ventricle at a single institution, including changes in the anastomotic site over time. MethodsThe database contained 98 patients with single-ventricle anatomy who underwent extracardiac TAPVC repair from 1996 to 2022. The median age and body weight at surgery were 59 days and 3.8 kg, respectively. Eighty-seven patients had heterotaxy syndrome, and 42 had preoperatively obstructed TAPVC. Primary sutureless repair was performed in 18 patients, 13 of whom were neonates. The cross-sectional area of the anastomotic site of the atrium and pericardium was divided by the body surface area, and the changes in this value over time were assessed. The median follow-up was 5.2 years (range, 0-19.4 years). ResultsOperative mortality and late mortality occurred in 2 (2.0%) and 38 (38.8%) patients, respectively. The actuarial survival rate at 5 years postoperatively was 56.2%. Multivariate analysis identified preoperatively obstructed TAPVC as a risk factor for mortality. Recurrent pulmonary venous stenosis (PVS) developed in 25 patients, thus giving a 5-year rate of freedom from PVS of 64.9%. Multivariate analysis revealed that sutureless repair significantly decreased the incidence of recurrent PVS. The cross-sectional anastomotic area tended to grow in accordance with the patients’ growth. ConclusionsSutureless repair of extracardiac TAPVC with univentricular anatomy achieved acceptable results. The anastomotic site tended to grow over time and contributed to a decline in the rate of recurrent PVS.

Total Anomalous Pulmonary Venous Connection: Surgical Outcomes and Risk Factors for Postoperative Pulmonary Vein Obstruction.

Total anomalous pulmonary venous connection (TAPVC) is a fatal congenital cardiac anomaly that requires urgent surgical intervention. The development of postoperative pulmonary vein obstruction (PVO) negatively impacts long-term survival. The present study aimed to evaluate the surgical outcomes of TAPVC repair and risk factors associated with postoperative PVO. Patients who underwent primary TAPVC repair at our institute between 2004 and 2022 were retrospectively enrolled, and those with right atrial isomerism and single ventricle physiology were excluded. Factors associated with survival and postoperative PVO were analyzed. A total of 116 patients were enrolled in the present study. The early mortality rate was 6.9%. Nineteen patients (16.4%) developed postoperative PVO within a median time of 59 days of the primary repair, of whom 10 were successfully relieved without any recurrent obstruction. In long-term follow-up, patients with postoperative PVO had significantly lower long-term survival rates than those without postoperative PVO [57.9%, 95% confidence interval (CI) = 34.8-79.5%; vs. 90.4%, 95% CI = 83-96.6% at 10 years, p < 0.001]. Risk factors for postoperative PVO development included lower body weight, younger age, preoperative mechanical ventilation, preoperative inotrope use, and emergency operation. Postoperative PVO was significantly associated with a higher long-term mortality rate after primary TAPVC repair, with the risk being higher in patients with critical preoperative status. The long-term outcome was good for patients in whom the obstruction was successfully relieved. Early detection and prompt intervention for postoperative PVO after TAPVC repair can improve overall survival in these patients.

Cardiopulmonary Vascular Odyssey and Haemodynamic Conundrum: A Case Report of Total Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection (TAPVC) is a cyanotic congenital heart disease where all the pulmonary veins drain directly or indirectly into the right atrium. Each of these types can be further classified into two types based on the pulmonary veins – obstructive and non-obstructive. This case report describes a rare case of a 2-month-old male infant with an unobstructed supra-cardiac TAPVC. The patient presented with tachypnoea, cyanosis and feeding difficulties, which prompted a thorough examination. Unobstructed supra-cardiac TAPVC was revealed by the diagnostic evaluation using two-dimensional echocardiography. Supra-cardiac TAPVC repair surgery was performed, following which post-operative evaluations indicated that the patient had satisfactory biventricular functionality. This case emphasises the relevance of early detection of TAPVC, thereby highlighting the need for meticulous and prompt postnatal care. The primary insight lies in the crucial role of prenatal diagnosis in tertiary care facilities, which allows timely planning of delivery, postnatal surgical interventions and a better prognosis of the disease. By underlining the significance of early intervention in the management of such rare congenital cardiac diseases such as TAPVC, this case report offers valuable insights into the scientific community.

Ectopic Atrial Tachycardia in Infants Following Congenital Heart Disease Surgery.

Although ectopic atrial tachycardia (EAT) is common following surgery for congenital heart disease (CHD), there are limited data regarding this arrhythmia. This study assessed risk factors and outcomes for patients less than one year of age with post-operative EAT. This was a retrospective analysis of infants undergoing CHD surgery from 2007 to 2020. Patients and surgeries with EAT were compared to controls without EAT. Out of 5372 infant CHD surgeries, EAT developed in 129 (2.5%). Compared to controls, the EAT cohort was younger (median 7 vs 85days, p < 0.01), weighed less at time of surgery (3.3 vs 4.2kg, p < 0.01), and was more likely to have DiGeorge syndrome (7.7% vs 3.0%, p < 0.01). Multivariate analysis revealed total anomalous venous connection (TAPVC) repair (odds ratio [OR] 2.8; 95% confidence interval 1.5-5.2), DiGeorge syndrome (OR 2.4; 1.1-5.2), Society of Thoracic Surgeons-European Association for Cardio-Thoracic surgery (STAT) category ≥ 4 (OR 2.1; 1.0-4.4), and longer cardiopulmonary bypass times (OR 1.1; 1.0-1.2) as independent risk factors for EAT. The onset of EAT occurred a median of 9days (IQR 5-14days) after CHD surgery. Antiarrhythmic treatment was initiated in 109/129 patients (84%) with propranolol (71%) and amiodarone (24%) the most commonly used medications. Although 15 (11.6%) patients did not survive to hospital discharge, EAT was not directly implicated in any deaths. EAT occurred after 2.5% of infant CHD surgeries. In addition to TAPVC repair, longer and more complex surgeries were associated with an increased the risk for the development of post-operative EAT.

Total anomalous pulmonary venous connection with another major congenital cardiac defect in biventricular hearts.

The association of total anomalous pulmonary venous connection (TAPVC) with major congenital cardiac anomalies is rare. This coexistence has an impact on the diagnosis and management strategies. The objective of this single centre study was to analyse the outcomes of such association in bi-ventricular hearts. This is a single-centre retrospective review of all patients who underwent repair for TAPVC associated with major congenital cardiac anomalies in bi-ventricular hearts from February 2015 to March 2020. Data were obtained from the hospital database. The primary aim was to assess the surgical outcomes. In addition, we described the incidence, pathophysiology, morphology and surgical strategies in this cohort of patients. Out of a total of 274 patients who underwent repair for TAPVC during the study period, 16 (5.8%) patients had one or more major associated cardiac defect anomalies. The major associated lesions were ventricular septal defects (9), tetralogy of Fallot (2), transposition of the great arteries (1), aorto-pulmonary window (1), partial atrioventricular canal defect (1), pulmonary stenosis (1) and severe tricuspid valve regurgitation due to flail chord (1). The mean follow-up period is 32 ± 13 months. There were two deaths in the post-operative period and no deaths in the follow-up period. The incidence of major associated cardiovascular defects with TAPVC in bi-ventricular morphology is 5.8%. The presence of a major associated lesion with TAPVC significantly alters the pathophysiology and clinical presentation with an impact on diagnosis and treatment.

Window anastomosis technique for repair of supracardiac total anomalous pulmonary venous connection in infants.

Outcomes of operations for total anomalous pulmonary venous connection (TAPVC) have improved. However, postoperative pulmonary venous obstruction remains the most significant complication with high morbidity and mortality. We introduce a window anastomosis technique for repair of supracardiac TAPVC in infants. The mainstay of the surgical technique is to resect the anterior wall of the pulmonary vein confluence and part of the posterior wall of the left atrium to form a large and undistorted "window to window" anastomosis.

Application of sutureless technique in total anomalous pulmonary venous connection repair.

The sutureless technique was initially used for the treatment of postoperative pulmonary vein obstruction (PVO) in patients with total anomalous pulmonary venous connection (TAPVC). However, as the effectiveness of the sutureless technique has been confirmed and widely developed, the sutureless technique has been used for the primary treatment of high-risk children with TAPVC who are at high risk of PVO. Compared with traditional surgery, the sutureless technique significantly reduces the incidence of postoperative PVO, re-intervention rate due to PVO, and postoperative mortality, but there are potential complications such as pericardial and confluent venous anastomosis, phrenic nerve damage, air embolism. In addition, the sutureless technique is not effective in dealing with the progressive stenosis of the pulmonary veins after sutureless surgery and diffuse stenosis of extrapulmonary proximal veins and intrapulmonary veins. These make the efficacy and safety of this procedure controversial. This paper reviews the research status of the sutureless technique in TAPVC repair at home and abroad over the years.

A Risk Prediction Model for Reintervention After Total Anomalous Pulmonary Venous Connection Repair

BackgroundOutcomes after total anomalous pulmonary venous connection (TAPVC) repair remain suboptimal due to recurrent pulmonary vein (PV) obstruction requiring reinterventions. We sought to develop a clinical prediction rule for PV reintervention after TAPVC repair. MethodsData from consecutive patients who underwent TAPVC repair at a single institution from January 1980 to January 2020 were retrospectively reviewed after Institutional Review Board approval. The primary outcome was postdischarge (late) unplanned PV surgical or transcatheter reintervention. Echocardiographic criteria were used to assess PV residual lesion severity at discharge (class 1: no residua; class 2: minor residua; class 3: major residua). Competing risk models were used to develop a weighted risk score for late reintervention. ResultsOf 437 patients who met entry criteria, there were 81 (18.5%) reinterventions at a median follow-up of 15.6 (interquartile range, 5.5-22.2) years. On univariable analysis, minor and major PV residua, age, single-ventricle physiology, infracardiac and mixed TAPVC, and preoperative obstruction were associated with late reintervention (all P < .05). The final risk prediction model included PV residua (class 2: subdistribution hazard ratio [SHR], 4.8; 95% CI, 2.8-8.1; P < .001; class 3: SHR, 6.4; 95% CI, 3.5-11.7; P < .001), age <1 year (SHR, 3.3; 95% CI, 1.3-8.5; P = .014), and preoperative obstruction (SHR, 1.8; 95% CI, 1.1-2.8; P = .015). A risk score comprising PV residua (class 2 or 3: 3 points), age (neonate or infant: 2 points), and obstruction (1 point) was formulated. Higher risk scores were significantly associated with worse freedom from reintervention (P < .001). ConclusionsA risk prediction model of late reintervention may guide prognostication of high-risk patients after TAPVC repair.

Sutureless Technique for Primary Total Anomalous Pulmonary Venous Connection Repair: An Updated Meta-Analysis.

BackgroundAn updated meta-analysis was performed to explore the clinical outcomes following the sutureless repair in patients with total anomalous pulmonary venous connection (TAPVC) and compare outcomes between the sutureless technique and conventional surgery.MethodsA systematic search of PubMed, Ovid-Embase, and Cochrane Library (CENTRAL) for relevant published studies that reported the clinical outcomes of the sutureless technique in children with TAPVC was performed in February 2022. The publication language was restricted to English.ResultsEleven studies were included involving 771 patients in total. Following the sutureless technique, the incidences of postoperative pulmonary venous obstruction (PVO) and reoperations due to PVO were 3.3% [95% confidence interval (CI), 1.3–5.3%] and 1.8% (95% CI, 0.3–3.3%), respectively. The early and late mortality rates were 3.2% (95% CI, 1.0–5.3%) and 2.5% (95% CI, 0.7–4.3%), respectively. Compared with conventional surgery, the sutureless technique significantly reduced the incidences of postoperative PVO [odds ratio (OR), 0.16; 95% CI, 0.08–0.33; P < 0.00001], reoperations due to PVO (OR, 0.25; 95% CI, 0.10–0.63; P = 0.003), and early mortality (OR, 0.40; 95% CI, 0.21–0.79; P = 0.008). However, no significant difference was found between conventional surgery and the sutureless technique concerning late mortality (OR, 0.63; 95% CI, 0.13–3.00; P = 0.58).ConclusionThe sutureless technique is superior to conventional surgery for the primary repair of TAPVC concerning postoperative PVO, reoperations due to PVO, and early mortality. However, the level of evidence is of low quality. Prospective cohort studies or randomized control trials (RCTs) should be performed to evaluate the effectiveness of sutureless techniques for primary TAPVC repair.

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.

Essential role of cardiac computed tomography for surgical decision making in children with total anomalous pulmonary venous connection and single ventricle.

Total anomalous pulmonary venous connection (TAPVC) is a major risk factor in infants with single ventricle (SV). Exact definition of TAPVC anatomy is crucial for surgical planning. To evaluate the role of cardiac computed tomography (CT) in this setting. Retrospective review of 13 infants who underwent TAPVC repair associated with SV from May 2016 to October 2021. Anatomy, incidence, and mechanisms of pulmonary venous obstruction (PVO) were described. Cardiac CT diagnostic yield was compared to echocardiography (echo). Of 13 infants, median age and weight were 24 days (range 2-303 days) and 3.2 (range 2.6-9.1) kg, 8 (62%) were male, 4 (31%) premature, and 11 (85%) had heterotaxy syndrome. All infants had pre- and postoperative echo; 13 had preoperative and 8 (62%) had postoperative cardiac CT. Type of TAPVC: six (46%) supracardiac, two (15%) intracardiac, one (8%) infracardiac, and four (31%) mixed, with pulmonary veins draining in >1 confluence in nine (69%). PVO was present in 6/13 (46%) preoperatively and 5/13 (31%) postoperatively. Mechanisms of PVO: 9/11 (82%) stenosis, 1/9 (9%) membrane formation, and 1/9 (9%) external compression. The sensitivity to diagnose PVO was 45.5% for echo and 100% for cardiac CT, the specificity was 100% for both. No discrepancy was found between cardiac CT and intraoperative findings, but echo had a complete preoperative diagnosis in 1/13 (8%) (p < .00001, Fisher exact test). Cardiac CT is essential to evaluate pre- and postoperative TAPVC in SV for surgical decision making and long term follow up.

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.

Clinical Nomogram for Predicting the Prognosis of Patients With Pulmonary Venous Obstruction After Total Anomalous Pulmonary Venous Connection Repair

BackgroundThe aim of this study was to establish a nomogram to quantify the risk of postoperative pulmonary venous obstruction (PVO) and to make a scientific decision through the decision curve.MethodsIn total, 151 PVO patients with total anomalous pulmonary venous connection (TAPVC) repair in our hospital from December 2008 to December 2015 were involved in this study. A nomogram was generated based on the contribution weights of variables, which were found out by logistic analysis. The optimal clinical decision point was determined by the decision analysis and clinical impact curve, which could assess the net benefit between the nomogram and each independent risk factor for postoperative PVO.ResultPulmonary venous obstruction with TAPVC repair was found to be positively and independently correlated with preoperative pulmonary hypertension, surgical methods, and preoperative pulmonary venous stenosis.Conclusion(s)The study introduced a novel model to aid in clinical decisions making for the patients with TAPVC individually, which may shed light on the evaluation of PVO risk.

Strategy of delayed repair of total anomalous pulmonary venous connection in right atrial isomerism and functional single ventricle

ObjectiveRepair of total anomalous pulmonary venous connection (TAPVC) in neonates with right atrial isomerism and functional single ventricle is challenging. In our novel strategy, primary draining vein stenting (DVS) was applied to patients with preoperative pulmonary vein obstruction to delay TAPVC repair. This study investigated our initial experience with a strategy of delayed TAPVC repair, incorporating DVS. MethodsTwenty-nine patients with right atrial isomerism and functional single ventricle who had a severe obstruction in the course of draining veins, who required surgical or catheter intervention in their neonatal period were retrospectively reviewed (primary DVS: n = 11; primary TAPVC repair: n = 18). ResultsPatients in the primary DVS group had more mixed type TAPVC (primary DVS: n = 5, 45.5%; primary TAPVC repair: n = 2, 11.1%; P = .03) and required more systemic to pulmonary shunt surgeries during their lifetime (primary DVS: n = 9, 81.8%; primary TAPVC repair: n = 6, 33.3%; P = .047). Kaplan–Meier analysis showed that primary DVS repair was associated with improved survival compared with primary TAPVC repair (survival rates at 90 days, 1 year, 3 years and 5 years: primary DVS: 100%, 80%, 68.6%, and 54.9%; primary TAPVC repair: 55.6%, 38.9%, 38.9%, and 38.9%, respectively [P = .04]). Of the 4 patients who underwent stenting of the ductus venosus, 3 had elevated liver enzymes after surgical repair of TAPVC due to ductus venosus steal, which markedly improved after coil embolization of the stent. ConclusionsFor neonates with obstructive TAPVC and functional single ventricle, our delayed TAPVC repair using primary DVS appeared to improve survival compared with the conventional strategy.

Review of surgical experience in 61 patients with mixed total anomalous pulmonary venous connection.

Prior studies have reported a high mortality and incidence of post-repair pulmonary venous obstruction (PVO) in mixed total anomalous pulmonary venous connection (TAPVC). This study sought to review the surgical outcomes in this entity. A review of 61 patients undergoing surgical repair of mixed TAPVC was conducted. Patients with a single ventricle were excluded. Patients were subdivided into 3 groups according to Chowdhury's classification. Predictors for death and postoperative PVO were explored by Cox regression model. This study trended towards an older cohort with a median age of 88 days (interquartile range, 56.5-177). Twelve patients belonged to '2 + 2' type, 40 belonged to '3 + 1' type and the remaining 9 belonged to bizarre type. There were no early death and 7 late deaths. Follow-up was available in 96.7% of the patients after discharge with a median duration of 53 months (range, 1-177). Nineteen patients developed post-repair PVO among whom 2 required reintervention. Patients with preoperative PVO had a 4-fold higher risk (95% confidence interval, 1.36-12.38) of postoperative PVO than those without and were more likely to die (P = 0.009). No statistical difference was observed among the 3 subgroups in terms of mortality (P = 0.058) and postoperative PVO (P = 0.186). Preoperative PVO was significantly associated with postoperative PVO. There was no statistical difference in terms of death and postoperative PVO among the 3 subtypes of mixed TAPVC. Mid-term results favoured a complete rechanneling of pulmonary veins in '3 + 1' type.