Infracardiac Total Anomalous Pulmonary Venous Connection Research Articles

Clinical Outcomes, Predictors, and Surgical Management of Infracardiac Total Anomalous Pulmonary Venous Connection.

This study sought to find the risk factors of postoperative pulmonary venous obstruction (PVO), PVO-related reintervention, and postoperative mortality, determine the relationship between pulmonary venous confluence (PVC) morphology and the clinical outcomes, and provide the basis for the selection of the optimal surgical strategy for infracardiac total anomalous pulmonary venous connection (TAPVC). From December 2009 to December 2023, 101 patients with infracardiac TAPVC undergoing surgical repair in our institution were included [Conventional surgery (CS) group, n = 73; Sutureless technique (ST) group, n = 28]. The Kaplan-Meier curve was used to demonstrate the survival estimates. The Cox proportional hazard model was used to identify risk factors for primary endpoints. There were 8 in-hospital deaths and 4 late deaths. The survival rates at 1, 5, and 10years were 88.8%, 87.6%, and 87.6%, respectively, in the whole cohort. Multivariable analysis showed that prolonged cardiopulmonary bypass (CBP) time was associated with overall mortality and Y-shaped PVC has a trend for a higher mortality rate. Postoperative PVO occurred in 27 patients. Freedom from postoperative PVO at 1, 5, and 10years were 81.8%, 69.9%, and 69.9%, respectively. Y-shaped PVC and preoperative PVO were incremental risk factors for PVO, but ST repair was a protective factor. Sixteen patients underwent PVO-related reintervention. Y-shaped PVC was the only independent risk factors for PVO-related reintervention while ST repair was a protective factor. Sutureless technique can achieve satisfactory outcomes for infracardiac TAPVC repair. Compared with CS repair, ST repair is significantly associated with decreased postoperative PVO and PVO-related reintervention. Y-shaped PVC is associated with a higher risk of PVO, PVO-related reintervention, and mortality, respectively. Patients with preoperative PVO are more likely to develop postoperative PVO.

Risk factors and outcomes for surgical repair of obstructed total anomalous pulmonary venous connection.

Surgical repair of obstructed total anomalous pulmonary venous connection (TAPVC) is a high-risk surgical cohort. This study aimed to assess surgical risk factors and outcomes in infants with TAPVC treated at a single centre. This was a prospective single-centre, observational study that included consecutive patients presenting with obstructed TAPVC. Patients with obstructed supra-cardiac, cardiac, and infra-cardiac TAPVC were included in the study. In-hospital mortality was the primary outcome. Multivariable logistic regression was carried out to identify risk factors for in-hospital mortality. Of the 41 patients, 31 (75.6%) were males. Ages were distributed as follows: 20 (48.8%) between 1 and 3 months, 11 (26.88%) between >3 months, and 10 (24.4%) neonates. The in-hospital mortality was 19.5% (n=8). Of the remaining 33 patients, there were no deaths during the follow-up. Short-term follow-up was achieved for 100% of cases (up to 6 months post-operatively). The mean duration of follow-up was 43.6± 3.6 months. One (3%) early and 2 (6%) late pulmonary vein obstructions were observed during follow-up. Age less than 30 days and pre-operative ventilation were associated with significantly increased risk of in-hospital mortality. Despite logistical challenges, reasonable surgical outcomes are attainable in obstructed TAPVC cases. Age less than 30 days and need for pre-operative ventilation were independent predictors of in-hospital mortality, while pulmonary venous obstruction in the post-operative period contributed to re-interventions.

Prenatal diagnosis and postnatal verification in fetuses with total anomalous pulmonary venous connection.

To systematically verify the accuracy of a four-step prenatal ultrasonography in diagnosing fetal total anomalous pulmonary venous connection (TAPVC). A total of 62 TAPVC fetuses received prenatal ultrasonography and were confirmed by postnatal echocardiography, surgery, or postabortion autopsy. The suspected TAPVC fetuses were further screened by a four-step prenatal ultrasonography for TAPVC classification, pulmonary venous obstruction, and the associated malformations, and followed postpartum. The sonographic features, clinical data, and prognosis of the TAPVC fetuses were retrospectively analyzed. Of the 62 TAPVC fetuses, supracardiac TAPVC was found in 20 cases, intracardiac TAPVC in 12, infracardiac TAPVC in 21, and mixed TAPVC in 9. A total of 30 cases with right atrium isomerism were correctly diagnosed. Of the 11 cases with other intracardiac and extracardiac malformations, 1 case was missed to be diagnosed. Of the 21 isolated TAPVC cases, 6 were missed prenatally and 1 case was prenatally diagnosed as intracardiac and postnatally proved to be mixed (intracardiac type + supracardiac type) by echocardiography. Of the 13 TAPVC live births, 4 infants died in the neonatal period without operation. Of the nine infants undergoing the operation, five recuperated and survived; one survived but had complications with superior vena cava obstruction and collateral circulation formation, and three died postoperatively. The four-step prenatal ultrasound procedure can comprehensively and systematically evaluate fetal TAPVC, detailing the classification, potential obstruction, and associated malformations. It provides substantial support for subsequent prenatal counseling and neonatal assessment. The retrospective analysis also reveals that isolated TAPVC is more prone to be missed in diagnosis.

Three-dimensional spatiotemporal imaging correlation in the diagnosis of isolated infracardiac total anomalous pulmonary venous connection in fetal life.

Total anomalous pulmonary venous connection (TAPVC) is a critical congenital heart disease which is often missed on prenatal echocardiography because of the decreased pulmonary blood flow in fetal life. Improvement in technology has resulted in increasing prenatal diagnosis of this condition. We report a foetus with infra cardiac TAPVC in whom prenatal diagnosis was facilitated by the use of STIC technology.

Invited commentary: Total anomalous pulmonary venous connection remains a challenging pediatric disease.

Cervantes-Salazar and colleagues report the long-term surgical outcomes of 414 patients with total anomalous pulmonary venous connection (TAPVC) from January 2003 to June 2019. With an overall survival rate of 87.2% from 2003 to 2019, the authors found that an increased mortality risk was associated with infracardiac TAPVC, pulmonary venous obstruction, and postoperative mechanical ventilation. Their comprehensive study with a large sample size of varying age groups, and patients with late referrals for surgery, provide valuable insight into TAPVC surgical outcomes. Improved survival for these patients continues to be a major goal of clinical teams striving to transform treatment paradigms. The promising result of the study reported by Cervantes-Salazar and colleagues gives our field hope for a better future for these patients.

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.

Total anomalous pulmonary venous connection: 16 years of surgical results in a single center.

The aim of the study was to analyze the surgical outcome of patients with total anomalous pulmonary venous connection (TAPVC) who underwent cardiac surgery. A retrospective study was carried out. Patients with diagnosis of TAPVC undergoing cardiac surgery at the National Institute of Cardiology Ignacio Chávez, from January 1, 2003 and June 30, 2019 were included. Descriptive statistics were calculated, as well as a bivariate analysis of the variables associated with mortality. A logistic regression model was included to determine risk factors associated with the main outcome and survival was analyzed using the Kaplan-Meier method. A total of 5314 patients diagnosed with congenital heart disease (CHD) underwent surgery, 414 (7.8%) were patients with TAPVC, with an average age of 17.1 ± 34.6 months, 58.2% were male. It was frequent in infants (61.6%) and preschool (19.6%). Predominant type was supracardiac TAPVC (47.4%). Pulmonary venous obstruction (PVO) occurred in 32.1%. Risk factors associated with mortality were infracardiac TAPVC (odds ratio [OR]: 3.26; 95% confidence interval [CI]: 1.17-9.03; p = .02), PVO (OR: 2.56; 95% CI: 1.05-6.22; p = .03) and postoperative mechanical ventilation (OR: 1.005; 95% CI: 1.002-1.008; p = .01). Overall survival was 87.2%, with better outcomes in adolescents (100%), children (94.1%), mixed TAPVC (96%), and cardiac TAPVC (91.9%;p < .001). The survival of our institution after surgical correction of TAPVC is similar to that of other referral centers, where patients with infracardiac TAPVC and newborns worse outcomes. All patients must undergo a rigorous evaluation to determine an adequate repair strategy.

Stepwise Treatment for Heterotaxy Syndrome and Functional Single Ventricle Complicated by Infra-Cardiac Total Anomalous Pulmonary Venous Connection with Ductus Venosus Stent Placement and Subsequent Occlusion.

Even today, when the surgical outcome of congenital heart disease in the neonatal period has improved, the prognosis for heterotaxy syndrome and functional single ventricle complicated with total anomalous pulmonary venous connection (TAPVC), especially the infra-cardiac type, is catastrophic. We describe a strategy that combines percutaneous ductus venosus (DV) stent placement and occlusion after TAPVC repair to ensure survival from initial surgery to bidirectional cavopulmonary shunt (BCPS) procedure and facilitate subsequent treatment. Three consecutive patients with heterotaxy syndrome and functional single ventricle complicated by infra-cardiac TAPVC treated with our own strategy were retrospectively studied. In two infants, DV stent placement was performed on the day of birth. In one case at 11days of age. The risk of pulmonary vein obstruction was reduced, and on-pump surgery, including TAPVC repair, was performed on a standby basis. Since the rapid increase in hepatic enzymes occurred on postoperative day 0 to 1 in all cases, percutaneous stent occlusion was performed until postoperative day 3. The procedure improved liver function. One patient died due to severe atrioventricular valve regurgitation, one case underwent BCPS, and one patient was waiting to undergo. DV stent placement can avoid TAPVC repair in the early neonatal period. After TAPVC repair, the portosystemic shunt remained, resulting in hepatic dysfunction, but this could be improved by stent and vertical vein occlusion. A series of stepwise treatments can be useful to help such critically ill infants survive the high-risk neonatal period and achieve good BCPS circulation.

Infra-cardiac TAPVC with an intrahepatic portosystemic shunt: A rare association.

Total anomalous pulmonary venous connection (TAPVC) is an uncommonly encountered cyanotic congenital heart disease characterized by anomalous drainage of the pulmonary veins. Concomitant venous anomalies are rarely found in these patients. We present a case of an infra-cardiac TAPVC with an intrahepatic portosystemic shunt, where a vascular channel was seen between the middle hepatic vein and the left branch of the portal vein with fusiform dilatation of the latter, diagnosed on computed tomography angiography.

Short-term results of sutureless closure for primary total anomalous pulmonary venous connection: seventeen cases

Total anomalous pulmonary venous connection (TAPVC) is a rare condition. Mortality of TAPVC has improved dramatically in the present. Sutureless closure had initially used for congenital pulmonary venous stenosis (PVS) or post-repair PVS. Recently, it had been adopted for primary TAPVC to prevent postoperative pulmonary venous obstruction, a leading cause of death. This technique had gradually applied at our institute for primary TAPVC since 2018. Prospective case series was conducted from mid-2019. Including criteria consists of patients with primary TAPVC. These are all supracardiac, infracardiac, mixed type and intracardiac TAPVC with pulmonary venous stenosis. Excluding measures are TAPVC with single ventricle, isomerism or heterotaxy. End-point outcomes were mortality and short-term PVS. Seventeen TAPVC cases were enrolled in this study from 06/2019-12/2020 at Children’s Hospital 1. Male/female ratio was 12/5. Median age at admission was 44 (3-1010) days old. The median operative weight was 3.9 kg (2.4-11). Of those, fourteen (82.4%) cases were supracardiac TAPVC and 3 (17.6%) cases were infracardiac TAPVC. Nine (52.9%) cases were emergency operations. Median aortic clamp time was 66 (32-138) mins. Median bypass time was 112 (86-212) mins. There were 9 (52.9%) cases with arrhythmias, 2 (11.8%) cases with chylothorax. Mean ventilation time was 3.6±0.5 days; mean CICU time was 6.5 (1-20), median LOS was 14.5 (8-39). In-hospital mortality was 5.9% (1). Mean follow-up time was 12.3±6.4 months (range 4-24), there was no PVS with median pulmonary venous score of 0 (range 0-1). Sutureless technique could be a safe option for primary TAPVC repair.

Utility of a modified vascular corrosion casting technique in the diagnosis of fetal total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiac malformation, and prenatal detection of TAPVC malformation remains a challenging. TAPVC can be easily missed or misdiagnosed in prenatal examinations. This study was aimed to use the modified vascular corrosion casting technique to prepare fetal cardiovascular casts with TAPVC and investigate the utility of cardiovascular casting for the demonstration of fetal TAPVC. The retrospective study enrolled twenty fetuses (22 to 29 + 4 gestational weeks) with TAPVC diagnosed by prenatal echocardiography and casting technique from May 2015 to May 2020. Pre- and postnatal medical records, including results obtained by prenatal ultrasound, postpartum computed tomography angiography, as well as anatomic and cardiovascular casting findings were carefully reviewed and analyzed. In twenty cases, 80% (16/20) had intra- or extracardiac malformations. The TAPVC types were supracardiac (n = 8), cardiac (n = 6), infracardiac (n = 4), and mixed (n = 2). The diagnosis of 1 case each of supracardiac and cardiac TAPVC was modified to partial anomalous pulmonary venous connection; additionally, 4 malformations were missed and 2 were misdiagnosed, including an anomalous left brachiocephalic vein in supracardiac TAPVC, abnormal inflow of the hepatic vein and a double inferior vena cava in infracardiac TAPVC; and bilateral ductus arteriosus in infracardiac TAPVC; a tetralogy of Fallot in cardiac TAPVC that was corrected to right ventricular double outlet; and an absence of ductus arteriosus that was misdiagnosed as slim ductus arteriosus. Comparing with ultrasound, casting technique has its own superiority in exhibiting TAPVC abnormalities, especially in certain types such as course, origin and absence abnormalities of ductus. Postpartum cardiovascular casts can accurately depict the branch structure of the heart’s larger vessels, and may be used as a clinical assessment and teaching method in complex cardiac malformations.

Primary sutureless repair for infracardiac total anomalous pulmonary venous connection.

This study aimed to review surgical experiences in patients with infracardiac total anomalous pulmonary venous connection (TAPVC). This retrospective study included 63 infants who underwent conventional repair (n = 27) and sutureless repair (n = 36) from 1 February 2009 to 31 June 2019. Kaplan-Meier curves and Cox regressions were applied to analyse the overall survival and risk factors. Cumulative incidence curves and competing risk models were used to evaluate postoperative pulmonary venous obstruction (PVO). There were 4 hospital deaths and 2 late deaths, and 8 patients experienced postoperative PVO. The survival rates at 30 days, 1 year and 5 years were 95.2%, 90.5% and 90.5%, respectively. The overall survival rate was significantly higher in the sutureless group than that in the conventional group. The cumulative incidence of postoperative PVO in the conventional group was higher than that in the sutureless group. Univariable Cox regression analyses showed that lower surgical weight, increase in preoperative international normalized ratio, prolonged cardiopulmonary bypass time and aortic cross-clamp time and longer duration of postoperative ventilation were associated with higher mortality. Longer cardiopulmonary bypass time, lower preoperative prothrombin activity and the increasing preoperative international normalized ratio before surgical repair were associated with a higher incidence of postoperative PVO. Both sutureless and conventional repairs for patients with infracardiac TAPVC achieved favourable postoperative outcomes. There was no death in the sutureless repair group. Compared to conventional repair, sutureless repair was associated with lower mortality and lower incidence of restenosis in pulmonary veins and anastomosis.

Atypical perioperative management for duodenal obstruction in an infant with heterotaxy syndrome: a case report

BackgroundHeterotaxy syndrome (HS) is characterized by a wide variety of cardiac and extra-cardiac malformations, including pulmonary valve stenosis, interruption of the inferior vena cava, total anomalous pulmonary venous connection (TAPVC), asplenia, polysplenia, intestinal malrotation, and preduodenal portal vein (PDPV). We report the case of a heterotaxic infant with an infracardiac TAPVC and preduodenal portal vein who experienced repetitive hemodynamic instability during urgent laparotomy for duodenal obstruction.Case presentationA 3-day-old boy with HS was planned to undergo urgent laparotomy for duodenal atresia. Echocardiogram showed an interrupted inferior vena cava, single right ventricle, pulmonary valve stenosis, and infracardiac TAPVC. On exploratory laparotomy, intestinal malrotation characterized by Ladd’s band was found. During further exploration, repetitive severe hypotension and hypoxia occurred. Thorough examination revealed a greatly dilated PDPV crossing over and compressing the proximal duodenum externally. Finally, we considered the possibility that surgical manipulation directly compressed the dilated PDPV into which the TAPVC had pulmonary venous drainage, leading to repetitive pulmonary venous obstruction (PVO). Computed tomography, which was examined after laparotomy, indicated that the vertical vein from pulmonary venous confluence drained into the portal vein.ConclusionPDPV is a rare anomaly associated with HS. In case of intestinal malrotation and duodenal obstruction in HS with infracardiac TAPVC, both the presence of PDPV and the possibility of pulmonary venous drainage into the PDPV should be considered by pediatric surgeons and anesthesiologists performing laparotomy to avoid catastrophic PVO.

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.

Repair of a simple total anomalous pulmonary venous connection coexisting with a persistent left superior vena cava.

A simple total anomalous pulmonary venous connection (TAPVC) coexisting with a persistent left superior vena cava (PLSVC) is extremely rare. Connection of the PLSVC with the coronary sinus behind the left atrium induces coronary sinus dilatation. This reduces the free posterior wall space to which the common pulmonary vein is anastomosed for repairing the anomalous connection. Postoperative recurrent pulmonary venous obstruction (PVO) is the most important complication. To prevent PVO, sufficient tension-free anastomosis is necessary. When dilated, the coronary sinus becomes an obstacle for obtaining sufficient incision length in the left atrial cavity. We encountered two cases of a simple TAPVC with a PLSVC in infants weighing 1.8 and 2.9 kg, respectively. To obtain sufficient incision length, we extended the incision line to the right atrium for an atypical supracardiac TAPVC and incised from the left atrium to the coronary sinus via the right atrium for an infracardiac TAPVC. Moreover, we recreated the atrial septum with a rightward shift using a tanned pericardium in both cases. The postoperative courses were uneventful, without recurrent PVO.

Surgical treatment of infra-cardiac total anomalous pulmonary venous connection in 13 neonates

Objective To evaluate the effectiveness of urgent surgical correction for infra-cardiac total anomalous pulmonary venous connection(TAPVC) in neonates. Methods From October 2009 to January 2015, 13 patients with infra-cardiac TAPVC received surgical correction.The age of patients ranged from 6 to 28 days[(15.08±7.42)days], the body weight ranged from 2.5 to 4.8 kg[(3.34±0.67) kg]. A median sternotomy was performed.Continuous cardiopulmonary bypass using bicaval cannulation with aortic cross-clamping and mild systemic hypothermia were used in all patients.Bi-atrial incision technique making anastomosis between the posterior left atrial wall and the vertical vein were employed in 3 cases.In the other 10 cases, the heart was elevated upward and to the right to expose the anomalous descending vertical vein to facilitate the anastomosis between the posterolateral left atrial wall and vertical vein. Results Emergent or subemergent operations were performed in all patients without surgical death.In the early stage after operation, delayed sternal closure was employed in 11 patients, pulmonary hypertension crisis occurred in 3 patients, small atrial septal defect was kept open for hemodynamic stabilization in 6 patients, temporary cardiac pacemaker for proper heart rate in 3 patients.All patients were uneventfully discharged except 2 patients died of severe low cardiac output syndrome.During the period of follow up, 2 patients presented with recurrent pulmonary infection and signs of pulmonary venous obstruction including the increased flow speed at the site of anastomosis.One of 2 patients was lost of follow up and the other patient was uneventfully discharged after the second operation.The rest 9 patients received postoperative follow up for 1 month to 5 years, echocardiography, X ray chest radiography, and electrocardiogram were performed during this period.As a result, all children had good cardiac function and with sinus rhythm on electrocardiogram and apparently reduced pulmonary congestion on radiography.No obstructive pulmonary venous return was observed on echocardiography. Conclusion Corrective operation for infra-cardiac TAPVC in neonate on urgent basis may provide favorite outcomes. Key words: Anomalous pulmonary venous connection; Cardiac surgical procedures; Congenital heart disease; Neonate

Conexión anómala total de venas pulmonares a la vena porta: Valor de la angiotomografía multicorte. Informe de tres casos

We present three female patients with infracardiac total anomalous pulmonary venous connection (TAPVC) with the portal vein. Angio CT scan reconstructions and a correlation be- tween tomographic and echocardiographic findings were conducted. Multi-slice angio CT scan has been a valuable tool for the study of vessels in children with congenital heart disease. It is performed rapidly and it obtains high quality images, showing the vessels and the adjacent organs, which supply excellent information. The term TAPVC includes a group of malformations in which the four pulmonary veins connect in such a way that the arterialized blood in the lungs drains into the right atrium, either directly or by way of tributary veins. Thus, systemic and pulmonary blood mix in the right atrium; and atrial septal defect must exist in order for patients to survive. These malformations represent 0.4 to 2.0 % of all congenital heart diseases, with an incidence of 6.8 patients in every 100,000 births. Sixty eight percent of the cases are diagnosed in newborns, which indicates that most patients are symptomatic early in life. The infracardiac TAPVC represent 15 to 25% of all TAPVC.

Current topics in surgery for isolated total anomalous pulmonary venous connection

Surgical correction of total anomalous pulmonary venous connection (TAPVC) remains a challenge, with reported early mortality rates of up to 20%. In this review article, we describe several topics, including surgery for neonates, diagnoses with multidetector computed tomography (MDCT), and primary sutureless repair. Several studies have reported mortality rates of around 10%, and demonstrated unchanged hospital mortality in neonates, despite improvement of the overall mortality of cohorts including older patients. Previous reports identified a low body weight at the time of the operation, preoperative pulmonary venous obstruction (PVO), and a prolonged cardiopulmonary bypass time as risk factors for hospital mortality. With the development of new technologies, MDCT has become a good diagnostic modality for use in the pre- and post-operative evaluation. MDCT delineates the drainage site of the vertical vein and the atypical vessel into the systemic vein, and it can also evaluate the existence of obstruction in the vertical vein. Following favorable experiences with post-repair PVO, the indications for sutureless repair as a primary operation have been expanded for infants, including those at risk of developing PVO after the repair of TAPVC. Primary sutureless repair has proven especially useful for difficult patient groups, such as those with congenital PVO, infracardiac TAPVC with small pulmonary veins, or mixed-type TAPVC.

The research on operation of obstructed total anomalous pulmonary venous connection in neonates.

Objectives. Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease. This study aimed to evaluate the outcomes of TAPVC repair in neonates, controlling for anatomic subtypes and surgical techniques. Methods. Between 1997 and 2013, 88 patients (median age: 16 days) underwent repair for supracardiac (31), cardiac (18), infracardiac (36), or mixed (3) TAPVC. All the patients underwent emergency operation due to obstructed drainage. Supracardiac and infracardiac TAPVC repair included a side-to-side anastomosis between the pulmonary venous confluence and left atrium. Coronary sinus unroofing was preferred for cardiac TAPVC repair. Results. The early mortality rate was 2.3% (2/88 patients). The echocardiogram showed no obstruction in the pulmonary vein anastomosis, and flow rate was 1.1–1.42 m/s in the 3-year follow-up period. Conclusions. The accurate preoperative diagnosis, improved protection of heart function, use of pulmonary vein tissue to anastomose and avoid damage of the pulmonary vein, and delayed sternum closure can reduce the risk of mortality. The preoperative severity of pulmonary vein obstruction, the timing of the emergency operation, and infracardiac or mixed-type TAPVC can affect prognosis. Using our surgical technique, the TAPVC mortality among our patients was gradually reduced with remarkable results. However, careful monitoring of the patient with pulmonary vein restenosis and the timing and method of reoperation should also be given importance.

Total anomalous pulmonary venous connection repair; risk factors and outcome

Background Repair of total anomalous pulmonary venous connection (TAPVC) is associated with high mortality and morbidity. Age of presentation, low birth weight, type of TAPVC, pre-operative infection, clinical situation, pulmonary venous obstruction, pulmonary hypertension and associated lesions are very important factors affect the outcome of TAPVC repair. Methods Seventy patients underwent TAPVC repair from 2000 to 2012 with a median age of 33 days and mean weight of 4.6 kg. Twenty-six patients (48.6%) had supracardiac type, 23 patients (32.8%) had intracardiac type, and nine patients (8.6%) had infracardiac type, while seven patients (10%) had mixed TAPVC. Results Operative mortality was 5.7% (4 patients), while late deaths was 4.3% (3 patients). Four patients out of these 7 deaths were due to pulmonary hypertensive crises (4/7, 57.1%). Obstruction and single-ventricle physiology were associated with a higher mortality rate (3/7, 42.8%).Mean CPB time was 104.3 min (range, 70–190 min) and mean cross-clamp time was 42.4 min (range, 29–70 min). Three patients had recurrent pulmonary venous obstruction. (4.3%) which required early re-intervention. Conclusion Mortality rate in TAPVC repair is higher in those patients who had single-ventricle physiology or pulmonary venous obstruction at time of repair. Pulmonary hypertension is another risk factor affecting the post-operative course of infants with TAPVC, particularly patients with obstructed, infracardiac TAPVC.