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Objectives To retrospectively review 14 cases, describe preoperative imaging, anatomic findings and confirmation at surgery, surgical technique, and outcomes. Materials and Methods We describe anatomic variations in mixed-type total anomalous pulmonary venous return and surgical outcomes in a case series from 2 centers. Mixed-type total anomalous pulmonary venous return is classified based on the pattern of pulmonary venous drainage. Type I refers to '2+2' drainage of venous pairs, type II refers to '3+1' and type III refers to all other variants. Six patients (43%) had type I "2+2" drainage; 2 patients had 2 supra-cardiac and 2 cardiac connections, and 4 patients had 2 infra-cardiac connections and 2 cardiac. 4 patients (29%) had type II "3+1" drainage. Three patients had 3 cardiac and 1 supra-cardiac variants and one had a rare supra-cardiac pulmonary venous combination. 4 patients (29%) had type III morphology. 2 patients of them had 3+2 anatomy with 3 supra-cardiac and 2 cardiac connections, both of which included 3 right-sided pulmonary veins. The remaining 2 patients had unique anatomy, one with tri-level attachment to cardiac, supra-cardiac, and infra-cardiac and the last with all supracardiac pulmonary venous drainage but in a "3+1+1" pattern. Results In previous publications, mixed-type total anomalous pulmonary venous return has a higher mortality rate, with univariate analysis of mixed-type versus cardiac type with statistically significant hazard ratios of 2.88 in previous study and mortality as high as 42-50%. We achieved improved outcomes, with overall survival of 79% and no intraoperative mortality. Both patients who had complex intracardiac lesions (1 with ventricular septal defect and 1 with tetralogy of fallot) ultimately died. Of the remaining patients, 29% developed pulmonary venous obstruction on echocardiography follow-up with 3 requiring interventions with cardiac catheterization. Conclusion Mixed-type total anomalous pulmonary venous return has a wide variety of connections, which contributes to the complexity of planning and surgical correction. Further studies are needed to better understand the different morphologies of the disease. Keywords:Mixed type, outcome, surgical management, total anomalous pulmonary venous return: [ABSTRACT FROM AUTHOR]

Objectives: To retrospectively review 14 cases, describe preoperative imaging, anatomic findings and confirmation at surgery, surgical technique, and outcomes. Materials and Methods: We describe anatomic variations in mixed-type total anomalous pulmonary venous return and surgical outcomes in a case series from 2 centers. Mixed-type total anomalous pulmonary venous return is classified based on the pattern of pulmonary venous drainage. Type I refers to ‘2+2’ drainage of venous pairs, type II refers to ‘3+1’ and type III refers to all other variants. Six patients (43%) had type I “2+2” drainage; 2 patients had 2 supra-cardiac and 2 cardiac connections, and 4 patients had 2 infra-cardiac connections and 2 cardiac. 4 patients (29%) had type II “3+1” drainage. Three patients had 3 cardiac and 1 supra-cardiac variants and one had a rare supra-cardiac pulmonary venous combination. 4 patients (29%) had type III morphology. 2 patients of them had 3+2 anatomy with 3 supra-cardiac and 2 cardiac connections, both of which included 3 right-sided pulmonary veins. The remaining 2 patients had unique anatomy, one with tri-level attachment to cardiac, supra-cardiac, and infra-cardiac and the last with all supracardiac pulmonary venous drainage but in a “3+1+1” pattern. Results: In previous publications, mixed-type total anomalous pulmonary venous return has a higher mortality rate, with univariate analysis of mixed-type versus cardiac type with statistically significant hazard ratios of 2.88 in previous study and mortality as high as 42-50%. We achieved improved outcomes, with overall survival of 79% and no intraoperative mortality. Both patients who had complex intracardiac lesions (1 with ventricular septal defect and 1 with tetralogy of fallot) ultimately died. Of the remaining patients, 29% developed pulmonary venous obstruction on echocardiography follow-up with 3 requiring interventions with cardiac catheterization. Conclusion: Mixed-type total anomalous pulmonary venous return has a wide variety of connections, which contributes to the complexity of planning and surgical correction. Further studies are needed to better understand the different morphologies of the disease.

We report an interesting incidental liver finding during ECG-gated cardiac computed tomography (CT) in a newborn with infracardiac total anomalous pulmonary venous connection to the portal vein. This case shows a unique abnormality in hepatic perfusion that was initially mistaken for hepatic vein thrombosis. We review the altered hepatic blood flow distribution in this pathologic anatomy to help explain the observed hepatic perfusion abnormality on CT. This understanding will enable an imager to anticipate hepatic perfusion patterns in similar patients, potentially avoiding misdiagnosis and unnecessary further testing. [ABSTRACT FROM AUTHOR]

Introduction: Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect in which there is no direct connection between pulmonary veins and the left atrium. Hospital mortality in patients with TAPVR, depending on the anatomical type of the defect, ranges from 14% to 35%. Without surgical correction, children with obstructive TAPVR die in the neonatal period. The mortality rate of children with nonobstructive TAPVR reaches 80% by the first year of life.Objective: To evaluate the capabilities of transthoracic echocardiography (TTE) and cardiac computed tomography (CT) in TAPVR diagnosis and surgical planning of its correction.Materials and methods: The study included 17 children with an initial diagnosis of TAPVR. The age of the patients was 28.5 (11; 91) days. All the infants underwent TTE; 15 patients underwent cardiac CT, and 4 patients, cardiac catheterization with conventional angiography. We evaluated and compared the capabilities of these methods in the diagnosis and determination of the TAPVR type and concomitant anomalies. All data were compared with conventional angiography findings and intraoperative data. The interval between TTE, cardiac CT, and angiography or open surgery was less than 5 days.Results: The accuracy of TTE in diagnosing TAPVR was not significantly inferior to that of cardiac CT (88% vs 100%); erroneous results of TTE were found in 2 patients with heterotaxy syndrome (right isomerism). Both methods showed 100% diagnostic efficiency in determining the TAPVR type. The accuracy of TTE in diagnosing obstructive TAPVR was 81.2%, whereas that of cardiac CT, 94%. There was no statistically significant difference in diagnostic efficiency between CT and TTE in the diagnosis of TAPVR and its obstructive forms.Discussion: Our findings demonstrate the high efficiency of TTE in diagnosing TAPVR, determining its anatomical type, and identifying obstructive forms. This method has limited diagnostic capabilities in determining TAPVR in patients with heterotaxy syndrome and other congenital anomalies of the atria. Cardiac CT has proven to be a highly accurate tool in diagnosing TAPVR and determining obstructive forms and associated heart anomalies.Conclusions: In children with isolated TAPVR and normal arrangement of the organs (situs solitus) with good acoustic window, TTE is the initial and final tool for surgical planning of TAPVR correction. In children with TAPVR and concomitant heart defects, heterotaxy syndrome and other atrial anomalies, TTE should be supplemented by cardiac CT. Cardiac CT in children with TAPVR is an alternative to invasive cardiac catheterization with conventional angiography and should be a preferred diagnostic tool. Conventional angiography should be used in children with TAPVR for assessment of functional, not anatomical characteristics of the defect.

This article comprehensively reviews the literature concerning prenatal ultrasound findings of isolated total anomalous pulmonary venous return (TAPVR) and the application of specific sonographic markers to differentiate among the TAPVR types. These markers can be categorized as direct and indirect, based on either morphological features or hemodynamic changes in TAPVR. Indirect markers include the ventricular disproportion, an increased distance between the left atrium (LA) and the descending aorta, as well as the dilatation of superior vena cava or coronary sinus for supracardiac or cardiac TAPVR, along with abnormal pulmonary venous spectral Doppler patterns. Direct markers predominantly focus on the absence of a connection between the pulmonary veins and the LA. Some direct markers are specific to certain TAPVR types, such as the vertical vein. Diagnosing isolated TAPVR can pose challenges, but following a sequential approach can improve detection rates and outcomes. In cases with equivocal findings, additional follow-ups are recommended. The sequential approach described in the current article provides a step-wise methodology and sonographic markers for prenatal diagnosis of TAPVR, which can be utilized by fetal–maternal medicine specialists, obstetricians, and radiological technicians to ensure timely interventions.

We present the case of a 7-month-old female infant with a history of recurrent respiratory infections and symptoms of respiratory distress during feeding. Echocardiography isolated revealed supracardiac-type total anomalous pulmonary venous connection with a large ostium secundum atrial septal defect and severe pulmonary hypertension. Computed tomographic angiography confirmed the diagnosis and identified stenosis at the level of the venous confluence. The surgical intervention involved a novel approach using dual anastomoses between the pulmonary venous confluence and the left atrium, alongside atrial septal defect repair with a bovine pericardial patch. Postoperative recovery was uneventful, with successful weaning from mechanical ventilation on Day 9 and discharge on Day 12. The patient showed optimal venous drainage and hemodynamic stability, indicating a successful surgical outcome. This case highlights the importance of early surgical intervention in total anomalous pulmonary venous connection with complex anatomical presentations. [ABSTRACT FROM AUTHOR]

Background: The vertical vein (VV) ligation during the total anomalous pulmonary venous return (TAPVR) correction is still controversial. Our study aimed to define the potential risk factors for VV persistence and their percutaneous occlusion. Methods: The retrospective cohort study included 40 patients (26 males) with TAPVR treated at the tertiary referral center from 2005 to 2024. Results: The average days of age at diagnosis was two (IQR 1–8). Complex congenital heart disease with TAPVR was diagnosed in eight patients. A supracardiac type of TAPVR was found in 47% of them. The patients underwent the operation on their eighth day of life (IQR 5–57). The follow-up period was 32 months (IQR 8–99). The early postoperative mortality rate was 17.5%, significantly frequent in the patients’ group with combined CHD (p = 0.002). Four were reoperated on—three due to a postoperative obstruction between the pulmonary venous confluence and the left atrium (LA), while in one patient, a redirection of the VCI was performed. Four patients, aged 12.3 on average (IQR 8.9–14.7), underwent vertical vein embolization. All patients achieved complete occlusion with AVP2. The LA diameter Z score was lower than −4, an increased risk for VV persistence of almost 19 times (OR 18.6, 95% CI 1.6–216.0). Conclusions: We found that an LA diameter Z score of lower than −4 was a major risk factor for VV persistence. Percutaneous VV embolization is a safe and effective procedure in adolescents.

Objectives: The sutureless repair technique has been favoured due to its purported reduction in post-operative pulmonary venous obstruction rates. This study aims to compare the outcomes of conventional versus sutureless repair techniques in Total Anomalous Pulmonary Venous Drainage., Methods: In this retrospective single-centre analysis (2012-2022), we evaluated children who underwent conventional or sutureless repair for isolated total anomalous pulmonary venous drainage, excluding complex cardiac anomalies and incomplete data. Patients were categorised into conventional (Group C, n = 58) and sutureless (Group S, n = 41) groups. Primary outcomes included mortality, morbidity, and post-operative complications. Statistical analysis included Mann-Whitney U, chi-square, and Fisher's exact tests where appropriate., Results: Supracardiac type predominated in both groups (53.4% in Group C and 70.7% in Group S), with higher cardiac type frequency in Group C (24.1% versus 2.4%, p = 0.016). Early complications occurred in 58.5% versus 53.4% of cases in Groups S and C, respectively (p = 0.767). The mortality rate (17.2% versus 14.6%, p = 0.944) and post-operative pulmonary venous obstruction (21.2% versus 19.0%, p = 0.809) were higher in Group C, though not significantly. Mean cardiopulmonary bypass times were comparable between groups (105 versus 89 minutes, p = 0.424)., Conclusions: In this comprehensive analysis of paediatric Total Anomalous Pulmonary Venous Drainage repair, both conventional and sutureless techniques demonstrated comparable safety profiles and clinical outcomes. These findings suggest that surgical approach selection should be individualised based on patient characteristics and surgeon expertise. Further prospective studies with larger cohorts are needed to validate these observations.

Background Total anomalous pulmonary venous return is a rare cyanotic congenital heart disease in which pulmonary veins connect to the systemic veins, right atrium, or coronary sinus. Given its variability, it is essential to define the pathway and drainage site. Case summary We describe a 3-day-old patient with total anomalous pulmonary venous return, in which the four pulmonary veins drained into a common vertical vein that had a double connection at the cardiac (coronary sinus) and infracardiac (portal vein) levels. The echocardiographic diagnosis was confirmed by computed tomography scan. At 1.5 months, she underwent surgery by anastomosis of the venous collector with the left atrium, unroof of the coronary sinus, and clousure of its communication with right atrium. The vertical vein was ligated. There were no complications after 1 year of follow-up. Discussion This is an unusual anatomy that has not been described in the literature. In this article, we discuss the embryological origin. Furthermore, we highlight the importance of an accurate diagnosis of the venous pathway and its impact on surgical planning and patient prognosis. [ABSTRACT FROM AUTHOR]

Total anomalous pulmonary vein return is a critical heart defect with 4 anatomic forms. It belongs to the severe category of complexity of the STAD scale with an incidence of about 0.013% among all newborns, most of them requiring urgent surgical treatment in the first days and hours of life. Currently, there are many surgical techniques for its correction. This paper presents a comparative evaluation of two groups of patients with previously classical and "sutureless technique" of surgery. It was proved that "sutureless technique" has a number of advantages in comparison with the previously performed methods, the main ones being reduction of myocardial ischemia time, artificial circulation and universalism in application to any form of malformation.

Total anomalous pulmonary venous return (TAPVR) is a life-threatening condition that requires emergency surgical correction. The diagnosis of TAPVR can be challenging, especially in non-tertiary centers lacking pediatric cardiology support. Extracorporeal membrane oxygenation (ECMO) has been used in some patients as a bridge to cardiac surgery or for postoperative support. We describe a term neonate with severe pulmonary hypertension who was placed on ECMO, which facilitated the diagnosis and subsequent surgical correction of obstructive TAPVR with excellent outcomes.

In a newborn in whom cor triatriatum was missed on echocardiography, infracardiac total anomalous pulmonary venous connection was successfully repaired with the aid of cardiac computed tomography (CT). In rare combinations, as in this case, an accurate diagnosis prior to surgery, which is of vital importance for successful repair, can be made through a high index of suspicion and the use of a supplemental imaging modality such as CT.

Resumen: El drenaje venoso pulmonar anómalo total (DVPAT) es una cardiopatía congénita poco frecuente que se caracteriza por la ausencia de conexión entre todas las venas pulmonares y la aurícula izquierda. En este defecto, las venas pulmonares llegan a un confluente detrás de la aurícula izquierda que conecta con el retorno venoso sistémico a través de una vena vertical. Existen cuatro tipos anatómicos (supracardiaco, cardiaco, infracardiaco y mixto), siendo el supracardiaco el más frecuente. Cuando existe una obstrucción al retorno venoso pulmonar constituye una auténtica emergencia quirúrgica. El objetivo de la reparación es conseguir un drenaje de los efluentes pulmonares a la aurícula izquierda sin obstrucción. Se ha descrito la suplementación de la anastomosis con parche de pericardio, así como la utilización de suturas reabsorbibles para minimizar el riesgo de estenosis de la conexión durante el seguimiento.Presentamos un caso de DVPAT supracardiaco y obstructivo que requirió de tratamiento quirúrgico emergente neonatal. La reparación se llevó a cabo a los dos días de vida mediante anastomosis directa con sutura reabsorbible entre las venas pulmonares y la cara posterior de la aurícula izquierda. La porción derecha del confluente y la aurícula izquierda fueron suplementadas con un parche de pericardio. El postoperatorio transcurrió sin incidencias significativas y el paciente fue dado de alta el 20° día postoperatorio. Seis años tras la cirugía, este se encuentra asintomático, ha tenido un desarrollo neurológico normal y no existen signos de obstrucción en el drenaje venoso por ecocardiografía. Abstract: Total anomalous pulmonary venous return (TAPVR) is a rare congenital heart disease that is characterized by lack of conection between all four pulmonary veins and the left atrium. In this defect, the pulmonary veins make a confluence behind the left atrium which connects with the systemic venous return. Four anatomic subtypes (supracardiac, cardiac, infracardiac and mixed) exist, being the supracardiac the most common one. Obstruction of the pulmonary venous return constitutes a surgical emergency. The goal of surgical repair is to get unobstructed egress from the pulmonary veins int the left atrium. Patch supplementation of the anastomosis as well as use of bioabsorbible sutures have been reported to minimize the risk of stenosis of the conection through follow-up.We present a case of supracardiac and obstrictive TAPVR who required emergent neonatal surgical treatment. Repair was undertaken at two days of life by means of direct anastomosis with bioabsorbable suture between the pulmonary veins and the posterior wall of the left atrium. The anastomosis was supplemeted with a pericardial patch. Postoperative course was uneventful and the patient was discharged home on the 20th postoperative day. Six years after surgery, our patient is asymptomatic, with a normal neurologic development and shows no signs of obstruction on the venous return by echocardiography.

Surveillance of patients with congenital heart disease is difficult in some clinical settings. This case study follows the progress of a patient, born with prenatally missed diagnoses of tetralogy of Fallot (TOF) and total anomalous pulmonary venous return (TAPVR), for over two years. The patient acquired pulmonary vein stenosis following initial TOF/TAPVR repair, which made the case an interesting longitudinal study. Multiple cardiac catheterizations for stents and balloon angioplasties of the pulmonary veins were necessary. This case study illustrates this patient's success with treatment options such as chemotherapy medications, as well as the failure of each treatment. It was also important to note that the interval of time between each interventional treatment weighed heavily upon results of frequent echocardiograms. [ABSTRACT FROM AUTHOR]

Total anomalous pulmonary venous return (TAPVR), also known as total anomalous pulmonary venous connection, is a congenital cardiovascular malformation that presents itself in the neonatal period, with cyanosis and tachypnea. There are 4 types of TAPVR with the mixed type being the least common. Any type of TAPVR may be associated with obstruction as result of flow redirection through the liver parenchyma before it may return to the heart, but infracardiac is the most common one. We report a case of a 10-hour-old female, with a mixed (cardiac and infracardiac) TAVPR with obstruction, that showed drainage to the coronary sinus and the portal vein, as the other classic findings in TAVPR, made with computed tomography angiography and echocardiogram. The patient was taken to surgical repair, but unfortunately died during the procedure because of multiple complications.

Introduction: Total Anomalous Pulmonary Venous Connection (TAPVC) is an uncommon congenital heart defect. They are classified into cardiac, supracardiac, infracardiac and mixed subtypes. It has a very high mortality without surgical correction. Aim: To assess the results and early outcomes of surgical repair in patients with isolated total anomalous pulmonary venous drainage with biventricular anatomy. Materials and Methods: This was a prospective study on 27 patients with isolated TAPVC, who underwent surgical correction between January 2012 and December 2018 at a tertiary cardiac care centre in Chennai, Tamil Nadu, India. This study involved detailed pre-operative Echocardiography and CT pulmonary angiography. Analysis of surgical repair, post-operative intensive care unit stay and up to 1 year follow-up of patients was done. Parametric variables were expressed as mean±standard deviation. Chi-square test was used to analyse categorical variables and calculate p-value. Results: The mean age of 27 patients was 4.86±10.08 months and mean weight was 4.69±4.13 kg. The subtype of anomalous connection decided the specific technique of surgical correction. The mean cardio-pulmonary bypass time for all TAPVC types was 111.04±39.82 minutes, mean aortic cross clamp time was 61.44±25.95 minutes and mean hospital stay of the patients was 9.11±2.08 days. There was one early post-operative death in supracardiac TAPVC variant (72 hours after surgery). There were three late post-operative deaths in infracardiac TAPVC variant due to post-operative pulmonary venous obstruction. One patient died three months after surgery and the other two died six months after surgery. The cumulative proportion of patients surviving at the end of one year was 0.88 (standard error of 0.07). Conclusion: Good early outcomes after surgical correction for TAPVC can be achieved. The duration of cardio-pulmonary bypass in infracardiac type of TAPVC is significantly higher than supracardiac and cardiac variants. Infracardiac TAPVC and preoperative Pulmonary Venous Obstruction (PVO) are associated with a poorer prognosis. This subset of patients represents the extreme end of TAPVC spectrum where they often require prolonged cardiopulmonary bypass support.

We report an uncommon case report of total anomalous pulmonary venous returns into the right atrium at the base of the superior caval vein's ostium without a sinus venosus defect, in situs solitus, without vertical vein or a posterior pulmonary venous confluence.

For some congenital heart lesions, higher institutional surgical volume has been associated with better survival than in lower volume centers. The relationship between institutional surgical volume and mortality in infants after total anomalous pulmonary vein return (TAPVR) repair has not been well explored. The Texas Inpatient Public Use Data File was queried for hospitalizations including TAPVR repair in infants between January 1, 1999 and December 31, 2016. We first evaluated the change in mortality over the study period. We then evaluated associations between institutional TAPVR surgical volume and mortality using univariable analysis and multivariable analysis accounting for center effects. For secondary analyses, we evaluated the association between volume and mortality among non-mutually exclusive TAPVR subsets, including isolated TAPVR, TAPVR with other congenital heart disease (CHD), TAPVR with heterotaxy, and TAPVR with single ventricle anatomy. Of 971 surgical hospitalizations that met inclusion criteria, 62% were male. Mortality after TAPVR repair decreased over the study period from 15.1% (1999–2004) to 7.6% (2012–2016) with an odds ratio per increasing year of 0.96 (95% CI 0.92–0.99, p = 0.030). By univariable analysis, earlier era, preterm birth, lower institutional surgical volume, heterotaxy, and additional CHD were associated with increased mortality. Institutional surgical volume remained significant in multivariate analysis with an odds ratio per increase in surgical volume of every 10 patients of 0.93 (95% CI 0.90–0.96, p < 0.001). When examining by subgroup, isolated TAPVR had the lowest mortality (n = 606, mortality = 6%), compared to TAPVR with other CHD (n = 359, mortality = 20%), TAPVR with heterotaxy (n = 135, mortality = 21%), and TAPVR with single ventricle (n = 128, mortality = 23%). In all groups except those with single ventricle, higher surgical volume was associated with lower mortality in multivariate analyses (isolated TAPVR p = 0.001, TAPVR with other CHD p = 0.009, TAPVR with heterotaxy p < 0.001, TAPVR with single ventricle p = 0.161). This is the first study to demonstrate an association between institutional surgical volume and mortality after TAPVR repair. Higher volume centers are associated with lower hospital mortality after TAPVR repair, including TAPVR with other CHD. [ABSTRACT FROM AUTHOR]

Introduction: Total Anomalous Pulmonary Venous Connection (TAPVC) is an uncommon congenital heart defect. They are classified into cardiac, supracardiac, infracardiac and mixed subtypes. It has a very high mortality without surgical correction. Aim: To assess the results and early outcomes of surgical repair in patients with isolated total anomalous pulmonary venous drainage with biventricular anatomy. Materials and Methods: This was a prospective study on 27 patients with isolated TAPVC, who underwent surgical correction between January 2012 and December 2018 at a tertiary cardiac care centre in Chennai, Tamil Nadu, India. This study involved detailed pre-operative Echocardiography and CT pulmonary angiography. Analysis of surgical repair, post-operative intensive care unit stay and up to 1 year follow-up of patients was done. Parametric variables were expressed as mean±standard deviation. Chi-square test was used to analyse categorical variables and calculate p-value. Results: The mean age of 27 patients was 4.86±10.08 months and mean weight was 4.69±4.13 kg. The subtype of anomalous connection decided the specific technique of surgical correction. The mean cardio-pulmonary bypass time for all TAPVC types was 111.04±39.82 minutes, mean aortic cross clamp time was 61.44±25.95 minutes and mean hospital stay of the patients was 9.11±2.08 days. There was one early post-operative death in supracardiac TAPVC variant (72 hours after surgery). There were three late post-operative deaths in infracardiac TAPVC variant due to post-operative pulmonary venous obstruction. One patient died three months after surgery and the other two died six months after surgery. The cumulative proportion of patients surviving at the end of one year was 0.88 (standard error of 0.07). Conclusion: Good early outcomes after surgical correction for TAPVC can be achieved. The duration of cardio-pulmonary bypass in infracardiac type of TAPVC is significantly higher than supracardiac and cardiac variants. Infracardiac TAPVC and preoperative Pulmonary Venous Obstruction (PVO) are associated with a poorer prognosis. This subset of patients represents the extreme end of TAPVC spectrum where they often require prolonged cardiopulmonary bypass support. [ABSTRACT FROM AUTHOR]

Background: Recent developments in surgical techniques and hospital care have led to improved outcomes following total anomalous pulmonary venous return (TAPVR) repair. However, the surgical repair of TAPVR remains associated with a high risk of mortality and need for reoperation. We conducted this retrospective study to evaluate mid-term outcomes following in situ TAPVR repair without total circulatory arrest (TCA), and to identify the risk factors associated with surgical outcomes. Methods: We retrospectively reviewed 29 cases of surgical intervention for TAPVR conducted between April 2000 and July 2015. All patients were newborns or infants who underwent in situ TAPVR repair without TCA. Results: Four anatomic subtypes of TAPVR were included in this study: supracardiac (20 cases, 69.0%), cardiac (4 cases, 13.8%), infracardiac (4 cases, 13.8%), and mixed (1 case, 3.4%). The median follow-up period for all patients was 42.9 months. Two (6.9%) early mortalities occurred, as well as 2 (6.9%) cases of postoperative pulmonary venous obstruction (PVO). Preoperative ventilator care (p=0.027) and preoperative PVO (p=0.002) were found to be independent risk factors for mortality. Conclusion: In situ repair of TAPVR without TCA was associated with encouraging mid-term outcomes. Preoperative ventilator care and preoperative PVO were found to be independent risk factors for mortality associated with TAPVR repair.

We report a term female infant with congenital heart block and total anomalous of pulmonary venous return. The results of single nucleotide polymorphism oligonucleotide microarray analysis showed an interstitial duplication of approximately 818 Kb, which involved 11 genes, including the entire LAMB3 gene which is known to associate with cardiac conduction defect. Our report adds to the collective knowledge that the cardiac conduction defect is a clinical feature of chromosome 1q32.2 duplication.

We used a paclitaxel-coated drug-eluting balloon (DEB) for pulmonary vein stenosis (PVS) after repair of total anomalous pulmonary venous return with asplenia. No adverse effect of paclitaxel was revealed and plasma concentration of paclitaxel was not elevated after intervention. Although progression of PVS slowed after dilatation with the DEB, stenosis recurred relentlessly, resulting in patient demise. This strategy may be safe for infantile cases, but requires further effective strategy, such as usage of larger sizes of DEBs. < Learning objective: Paclitaxel-coated drug-eluting balloons may be safely used for pulmonary vein stenosis after total anomalous pulmonary venous return repair in an infant with asplenia. However, their effectiveness is still limited.> [ABSTRACT FROM AUTHOR]

Total anomalous pulmonary venous return (TAPVR) is a rare and frequently isolated defect identified in 1% to 3% of all congenital heart diseases. To the best of our knowledge, portal vein thrombosis (PVT) associated with TAPVR has not been reported in the literature. We report a successfully managed PVT in a newborn with infracardiac-type TAPVR and review the literature. Anticoagulation therapies were used during the neonatal period to prevent thrombus progression. PVT should be kept in mind in TAPVR patients who have open heart repair with total correction. The treatment in each neonate should be individualized with consideration of the risk/benefit ratio.

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 > 0.74 m/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. [ABSTRACT FROM AUTHOR]

Objective: Total anomalous pulmonary venous return is an uncommon cyanotic congenital heart defect. Echocardiography is the initial diagnostic tool. Complimentary non-invasive modalities like cardiac computerized tomographic angiography and cardiac magnetic resonance imaging have replaced the need for cardiac catheterization in difficult cases. This study aimed to determine the accuracy of echocardiography in diagnosing total anomalous pulmonary venous return, and to determine the factors that may decrease its sensitivity.Methods: This was a cross-sectional study conducted at the Aga Khan University Hospital Karachi, Pakistan from January 2010 to August 2016. All patients who were diagnosed with Total anomalous pulmonary venous return on echocardiography and had subsequent confirmation either on cardiac CT angiography or surgery were included. The diagnostic accuracy of echocardiography was expressed as sensitivity. Previously described taxonomy was used to define diagnostic error. Univariate and multivariate analysis were done by logistic regression OR (95% CI) were reported to identify factors causing the diagnostic error.Results: High diagnostic sensitivity (81%) was found in isolated total anomalous pulmonary venous return and low (27%) in heterotaxy and mixed (20%) varieties. Poor acoustic windows and right isomerism were found to be significant factors responsible for the diagnostic error on multivariate analysis.Conclusion: Echocardiography can diagnose isolated total anomalous pulmonary venous return with high accuracy. Use of additional modalities may be required for a complete diagnosis in cases with mixed variety, heterotaxy and poor acoustic windows. [ABSTRACT FROM AUTHOR]

Total anomalous pulmonary venous connection (TAPVC) is a potentially devastating form of congenital heart disease in which all pulmonary blood flow returns to the systemic venous circulation rather than the left atrium. Anomalous pulmonary venous flow may be obstructed at birth, and affected infants present with severe cyanosis and poor cardiac output unresponsive to standard resuscitation with prostaglandin. Obstructed TAPVC remains one of the few indications for emergent neonatal cardiac surgery. This review will discuss the physiology and perioperative management of isolated TAPVC without associated cardiac lesions. [ABSTRACT FROM AUTHOR]

The objective of this series was to describe a simple rule for prenatal diagnosis of total anomalous pulmonary venous return (TAPVR). Fourteen fetuses had a prenatal diagnosis of TAPVR by the simple rule, including the following components: (1) the major criterion, which was the absence of a connection between the pulmonary vein and the left atrium; and (2) at least 1 of the following minor criteria: (a) the presence of a vascular confluence behind the atria, (b) abnormal spectral Doppler waveforms in the pulmonary veins, (c) a smooth posterior wall of the left atrium, (d) increased retroatrial space, (e) a dilated coronary sinus (cardiac type), (f) a dilated superior vena cava or brachiocephalic vein, and (g) an additional vessel on the 3‐vessel/3‐vessel and trachea view or a vertical descending vein. All were accurately diagnosed, and none were missed by the diagnosis. In summary, the simple rule described is helpful in increasing the number of accurate prenatal diagnoses of TAPVR. [ABSTRACT FROM AUTHOR]