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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.

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.

In infracardiac, infradiaphragmatic total anomalous pulmonary venous connection, all four pulmonary veins connect to a descending vertical vein that usually drains to the portal vein or one of its tributaries. Obstruction is common, and definitive treatment is surgical repair. We present a case of late-diagnosed infradiaphragmatic total anomalous pulmonary venous connection in a premature neonate who was too high risk for surgery and underwent palliative stenting of the venous duct. We demonstrate the feasibility of a transhepatic approach when umbilical access is no longer available.

Mortality outcomes of children with isolated neonatally operated congenital heart defects (CHDs) born with a low (LBW), moderately low (MLBW) or very-low birthweight (VLBW) remain ambiguous. We searched Medline and Embase (inception until October 2021) and included studies that evaluated early mortality. The risk of bias was assessed using the Critical Appraisal Skills Program cohort checklist. Meta-analysis involved random-effects models. We explored variability in mortality across birthweight subgroups, CHD types, and study designs. From 2035 reports, we included 23 studies in qualitative synthesis, and the meta-analysis included 11 studies (1658 CHD cases), divided into 30 subcohorts. The risk of bias was low in 4/11 studies included in the meta-analysis. Summary mortality before discharge or within one month after surgery was 37% (95%CI 27-47). Early mortality varied by birthweight (VLBW 56%, MLBW 15%, LBW 16%; p = 0.003) and CHD types (hypoplastic left heart syndrome (HLHS) 50%, total anomalous pulmonary venous return (TAPVR) 47%, transposition of the great arteries (TGA) 34%, coarctation of the aorta (CoA) 16%; p = 0.13). Mortality was higher in population-based studies (49% vs. 10%; p = 0.006). One-third of infants born with neonatally operated isolated CHDs and LBW, MLBW, or VLBW died within 30 days after surgery. Mortality varied across infant and study characteristics. These results may help clinicians assess neonatal prognosis. PROSPERO registration CRD42020170289.

Variants in T-box transcription factor 5 (TBX5) can result in a wide phenotypic spectrum, specifically in the heart and the limbs. TBX5 has been implicated in causing non-syndromic cardiac defects and Holt-Oram syndrome (HOS). The present study investigated the underlying molecular etiology of a family with heterogeneous heart defects. The proband had mixed-type total anomalous pulmonary venous return (mixed-type TAPVR), whereas her mother had an atrial septal defect. Genetic testing through trio-based whole-exome sequencing was used to reveal the molecular etiology. A nonsense variant was identified in TBX5 (c.577G>T; p.Gly193*) initially showing co-segregation with a presumably non-syndromic presentation of congenital heart disease. Subsequent genetic investigations and more complete phenotyping led to the correct diagnosis of HOS, documenting the novel association of mixed-type TAPVR with HOS. Finally, protein modeling of the mutant TBX5 protein that harbored this pathogenic nonsense variant (p.Gly193*) revealed a substantial drop in the quantity of non-covalent bonds. The decrease in the number of non-covalent bonds suggested that the resultant mutant dimer was less stable compared with the wild-type protein, consequently affecting the protein's ability to bind DNA. The present findings extended the phenotypic cardiac defects associated with HOS; to the best of our knowledge, this is the first association of mixed-type TAPVR with TBX5. Prior to the current analysis, the molecular association of TAPVR with HOS had never been documented; hence, this is the first genetic investigation to report the association between TAPVR and HOS. Furthermore, it was demonstrated that the null-variants reported in the T-box domain of TBX5 were associated with a wide range of cardiac and/or skeletal anomalies on both the inter-and intrafamilial levels. In conclusion, genetic testing was highlighted as a potentially powerful approach in the prognostication of the proper diagnosis. [ABSTRACT FROM AUTHOR]

This case represents anaesthetic challenges while managing a neonate having a congenital cardiac anomaly with incidental stridor for the non-operating room anaesthesia (NORA). Anaesthesia management of neonates is demanding even for experienced anaesthetists in terms of vascular access, airway management, relatively poor respiratory reservoir and transitional cardiac physiology. Neonate having cyanotic congenital heart disease with abnormal cardiac physiology demands further attention. In children with congenital heart disease, difficult intubation remains a possibility as they are more often associated with airway anomalies. NORA has its own set of anaesthesia challenges related to a lack of adequate anaesthesia equipment and monitoring devices, a limited and unfamiliar workspace, lack of trained support staff along with more medically complex patients. Recently there has been concern regarding the safety of anaesthetic agents in children < 3 years. So, techniques are being developed to promote immobilisation without using anaesthesia in short procedures. One such technique is the "feed and wrap" technique where feeding and swaddling induce natural sleep in infants. Here, we have used the "feed and wrap technique" in a 24-day-old neonate having supracardiac total anomalous pulmonary venous return with incidental stridor posted for contrast-enhanced computed tomography angiography., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Singh et al.)

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., Competing Interests: Conflict of interest: None declared., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology.)

A 4-hour-old infant with profound cyanosis on an alprostadil infusion was urgently transferred to Rady Children's Hospital with suspected CHD. Upon arrival, urgent echocardiography was performed but could not confirm the presence of discrete pulmonary veins or pulmonary venous drainage. Given the difficulty in delineating the anatomy, a cardiac CT scan was performed and demonstrated a nearly atretic common pulmonary vein with multiple small collaterals that drained to systemic veins. Due to the high risk of mortality associated with operative repair, the decision was made to proceed with compassionate withdrawal of care. The described anatomy of common pulmonary vein atresia remains rare, and to our knowledge, fewer than 40 cases have been reported in the literature. Albeit rare, common pulmonary vein atresia should be considered in the differential diagnosis of a severely cyanotic neonate.