To develop a predictive model for optimal anastomosis sizing in TAPVC surgery, focusing on the role of pulmonary venous confluence (PVC) size. A patient-specific fluid-structure interaction (FSI) model simulated blood flow through various anastomosis sizes. Key variables included body weight, anastomosis length, and PVC size. The model's predictions were validated against postoperative echocardiographic measurements from nine TAPVC cases. A strong positive correlation was found between flow velocity and the ratio of body weight to anastomosis length and PVC circumference. Including PVC size significantly improved predictive accuracy. No significant difference was observed between predicted and measured velocities. PVC size is a critical factor for planning TAPVC surgery. Incorporating it into computational models enhances the prediction of flow dynamics and supports personalized surgical decision-making.

Mixed Total Anomalous Pulmonary Venous Connection to Superior Cavoatrial Junction.

High risk and low incidence diseases: Cyanotic critical congenital heart disease.

Abstract Background Pre-tricuspid shunts, such as atrial septal defects (ASDs) and partial anomalous pulmonary venous return (PAPVR), result in left-to-right volume overload and may progress to pulmonary arterial hypertension (PAH). Cardiac magnetic resonance (CMR)-based RV feature tracking (RV-FT) has emerged as a non-invasive modality to evaluate RV longitudinal strain and function. Purpose To investigate the utility of longitudinal RV feature tracking in the assessment of RV function in patients with pre-tricuspid shunts. Methods We retrospectively analyzed a cohort of patients with pre-tricuspid shunts and compared them to age- and sex-matched healthy controls. Inclusion criteria comprised eligibility for CMR and absence of claustrophobia. The imaging protocol included cine sequences in the short-axis, long-axis (3CH, 4CH, RV inflow/outflow views), axial stack, velocity-encoded phase contrast imaging of the pulmonary artery, aorta, and atrial septum, as well as late gadolinium enhancement (LGE) sequences. Post-processing was performed using dedicated software (Circle CVI) to derive biventricular volumes and function, Qp:Qs ratio, and longitudinal RV strain from 4CH cine images. Statistical analyses included non-parametric testing (Wilcoxon rank-sum), correlation matrices, and Fisher’s exact test. Results Sixteen participants were included: 8 patients with pre-tricuspid shunts (4 males, 4 females; median age 49 years [IQR: 31–68; range: 18–75]) and 8 controls (5 males; median age 39 years [IQR: 28–56; range: 25–57]). Among the shunt group, three patients had sinus venosus ASDs with PAPVR, three had ostium secundum ASDs, and one had PAPVR involving both the superior and inferior vena cava. Median RV functional parameters were as follows: for the shunt group—EF 54%, RV end-diastolic volume (RVEDV) 121 mL/m², RV stroke volume (RVSV) 100 mL; and for the control group—EF 56%, RVEDV 75 mL/m², RVSV 83 mL. In the shunt group, significant negative correlations were observed between LV stroke volume and Qp:Qs ratio (r = –0.643), right atrial volume (r = –0.731), and main pulmonary artery (MPA) diameter (r = –0.323). A strong positive correlation was found between MPA diameter and age (r = 0.970). While RV-FT values did not significantly differ between patients and controls (p = 0.373, Holm-adjusted), RV longitudinal strain was significantly correlated with RV ejection fraction (r = 0.738) in the shunt group. Conclusions CMR-based longitudinal RV feature tracking may provide valuable insight into RV function in patients with pre-tricuspid shunts. Although RV-FT did not demonstrate significant intergroup differences, its strong correlation with RVEF in the shunt group underscores its potential utility in clinical risk stratification, particularly in patients with incidentally diagnosed congenital heart defects.

A neonate who underwent corrective surgery for cardiac-type total anomalous pulmonary venous connection (TAPVC) was suspected of having a pseudoaneurysm of the left ventricular (LV) free wall on transthoracic echocardiography (TTE) on postoperative day 11. Emergency surgery was performed the following day, revealing LV rupture due to a congenital partial defect of the LV free wall. The defect was successfully repaired using double patches closure reinforced with BioGlue. The postoperative course was uneventful. This case highlights that left ventricular rupture may occur due to an unrecognized congenital defect after neonatal cardiac surgery, particularly in conditions such as TAPVC where the left ventricle is underfilled preoperatively.

The coexistence of multiple complex congenital cardiac anomalies in a single infant - particularly unbalanced Atrioventricular Septal Defect; Double Outlet Right Ventricle; Infracardiac Total Anomalous Pulmonary Venous Connection, situs inversus, and pulmonary hypertension - is exceptionally rare and presents significant surgical and physiological challenges. A full-term male neonate was diagnosed at 25 days of age with this rare combination of defects. Despite low-flow oxygen, the baseline oxygen saturation remained at 60%-65%, and growth was severely impaired. Initial management at a tertiary center included diuretics, captopril, and high-calorie feeding, with discharge on home oxygen therapy. Recurrent cyanotic spells worsened, leading to readmission at 7 months due to profound hypoxemia (SpO2 35%). Escalated respiratory and nutritional support, along with optimized heart failure therapy, achieved only transient stability. Two multidisciplinary teams concluded that local surgical or palliative options were infeasible and recommended urgent referral to a supraregional center for treatment. Before transfer could occur, the infant suffered a terminal cyanotic spell at 8 months. Resuscitation efforts were unsuccessful, and the infant succumbed to death at 8 months of age. This case illustrates how anatomical and physiological complexity, coupled with limited regional surgical infrastructure, can preclude curative repair. Early antenatal or neonatal detection, rapid multidisciplinary coordination, and timely transfer to specialized cardiac centers are essential for improving survival in such cases.

Development of a Patient-Specific Fontan Failure Risk Calculator Using Machine Learning - A Step Towards Personalized Medicine.

Total anomalous pulmonary venous connection (TAPVC) is one of the most severe congenital heart defects; however, prenatal diagnosis remains suboptimal. A normal fetal heart has a junction between the pulmonary venous (PV) and left atrium (LA). In contrast, no junctions are observed in patients with TAPVC. In the present study, we attempted to visualize and detect fetal PV-LA connections using artificial intelligence (AI) trained on the fetal cardiac ultrasound videos of 100 normal cases and six TAPVC cases. The PV-LA aggregate area was segmented using the following three-dimensional (3D) segmentation models: SegResNet, Swin UNETR, MedNeXt, and SegFormer3D. The Dice coefficient and 95% Hausdorff distance were used to evaluate segmentation performance. The mean values of the shortest PV-LA distance (PLD) and major axis angle (PLA) in each video were calculated. These methods demonstrated sufficient performance in visualizing and detecting the PV-LA connection. In terms of TAPVC screening performance, MedNeXt-PLD and SegResNet-PLA achieved mean area under the receiver operating characteristic curve values of 0.844 and 0.840, respectively. Overall, this study shows that our approach can support unskilled examiners in capturing the PV-LA connection and has the potential to improve the prenatal detection rate of TAPVC.

Circular shunt is a very rare, albeit critical, condition. We present a case of a post-surgical circular shunt that resulted after correction of an anomalous total pulmonary venous connection to the coronary sinus with a persistent left superior vena cava. A left cavo-pulmonary anastomosis was performed; however, she developed heart failure during follow-up. The anastomosis was closed by interventional cardiac catheterisation without complications that led to resolution of heart failure symptoms.

Perinatal transition is uniquely complex in newborns with critical congenital heart disease (CCHD), whose cardiopulmonary physiology often diverges from the standard neonatal resuscitation framework. We developed lesion-specific clinical practice guidelines (CPGs) for delivery room (DR) management of six high-risk CCHD diagnoses - hypoplastic left heart syndrome, dextro- transposition of the great arteries, Ebstein anomaly, congenital complete heart block, total anomalous pulmonary venous return, and tetralogy of Fallot with absent pulmonary valve. Developed collaboratively between neonatologists, cardiologists, and cardiac intensivists, these guidelines standardize DR stabilization for high-risk, low frequency events, promote a shared mental model among multidisciplinary teams, and provide structured escalation thresholds for oxygen, respiratory support, intubation, and medication use. Beyond clinical standardization, these CPGs enhance provider education, team preparedness, and family counseling. They offer a scalable framework adaptable to centers with or without on-site cardiac care, bridging physiologic principles with practical implementation.

Introduction: Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital heart defect where one or more pulmonary veins fail to connect to the left atrium. Left-sided PAPVC accounts for only 10% of cases, often involving the left upper pulmonary vein (LUPV). While many patients remain asymptomatic, prolonged volume overload in the right heart can lead to symptoms like dyspnea. Non-invasive imaging (TTE/CTA) is critical for diagnosis. Case presentation: An 11-year-old female presented with dyspnea and recurrent respiratory infections. Imaging revealed isolated LUPV drainage into the innominate vein via an ascending vertical vein, with right heart dilation. Surgical repair involved anastomosing the transected vertical vein to the left atrial appendage under cardiopulmonary bypass. Postoperative recovery was uneventful, with normalized pulmonary venous drainage and symptom resolution at 1-year follow-up. Clinical discussion: Left-sided PAPVC is rare (10–15% of PAPVC cases) and often asymptomatic, delaying diagnosis. Symptomatic patients require surgery to prevent right heart failure and pulmonary vascular complications. Non-invasive imaging (CTA/MRI) has replaced invasive diagnostics. Surgical success hinges on creating a gradient-free anastomosis; our on-pump approach achieved this, aligning with favorable outcomes reported in the literature. Conclusion: Early surgical intervention in symptomatic PAPVC prevents irreversible cardiac complications. Accurate imaging and meticulous surgical technique, as demonstrated in this case, are vital for optimal long-term outcomes.

BackgroundApproximately half of the patients with Turner syndrome (TS) have congenital or acquired cardiovascular diseases. The objectives of this study were to improve the early diagnosis of TS and to predict the risk of severe cardiovascular diseases in patients with TS by analyzing the main features of cardiovascular diseases in these patients.MethodsThis study included 107 patients with TS who underwent echocardiography and were admitted to the Shanghai Children’s Medical Center between November 2019 and November 2024. In this study, the height, weight, age, karyotype, cardiac imaging data, and electrocardiograms of the patients were collected. The main features of the cardiovascular diseases in patients with TS were assessed, and the correlations between the height, body mass index (BMI), karyotype, and cardiovascular diseases were analyzed.ResultsOverall, 107 patients with TS were included, with an average age of 9.68 ± 4.23 years at diagnosis. Bicuspid aortic valve (BAV), aortic coarctation (CoA), and persistent left superior vena cava (PLSVC) were the most prevalent cardiovascular diseases, with prevalence rates of 11.2% (12/107), 8.4% (9/107), and 9.3% (10/107), respectively. The partial anomalous pulmonary venous return (PAPVC) was of the ‘supra cardiac type’ in all cases. Patients with CoA had lower BMI-for-age Z-score (BMIAZ) (-0.81 ± 1.17 vs. 0.61 ± 0.99; P = 0.003) than those without CoA. The best cutoff for predicting CoA was calculated using a sensitivity of 0.857 and specificity of 0.697 with a BMIAZ of 0.115 and an area under the curve of 0.825 (P = 0.004). Moreover, CoA (63.3%, 7/11) was the leading cause for surgical and interventional procedures in children and adolescents with TS. The 45,X karyotype was found to be associated with congenital heart disease (CHD) (47.4% vs. 24.6%; P = 0.016).ConclusionsGirls with BAV or CoA should undergo karyotyping to facilitate the early diagnosis of TS. CoA is the leading cause for surgical and interventional procedures in children and adolescents with TS. The BMIAZ is a reliable predictor of CoA. Cardiovascular magnetic resonance (CMR) or computed tomography (CT) should be performed at the time of diagnosis for girls with TS, especially those with a 45,X karyotype, BMIAZ < 0.115, or CHD detected by echocardiography.

Inadvertent Central Venous Catheter Placement into a Partial Anomalous Pulmonary Venous Connection: A Case Report

Background: Automated technologies have become increasingly vital in fetal cardiac assessment. Fetal heart quantification (HQ), which utilizes two-dimensional speckle tracking, provides quantitative measures of morphology and function. This study aims to identify the progression of many fetal cardiac function parameters during pregnancy in normal pregnant women and to determine which parameters are relevant to fetal well-being and worth considering.Methods: We conducted a prospective observational study involving fetuses of 26 normal pregnant women to evaluate 27 fetal HQ parameters (including strain, sphericity indices, and four-chamber view measurements) across gestation. Factor analysis was used to identify indicators of fetal and neonatal well-being. Additionally, we analyzed a case of fetal growth restriction (FGR) complicated by a double-outlet right ventricle (DORV) and total anomalous pulmonary venous connection (TAPVC).Results: Four parameters, which were tricuspid valve width, tricuspid valve length, the area of the four-chamber view, and right ventricular end-diastolic area (RVEDA), might be particularly informative. It is possible to perform serial assessments of fetal cardiac contractile development throughout gestation. In cases of FGR with DORV and TAPVC, fetal HQ detected abnormal right ventricular loading and impaired contractility. In cases of FGR with DORV and TAPVC, fetal HQ detected abnormal right ventricular loading and impaired contractility.Conclusion: Fetal HQ provides a reproducible, quantitative approach to fetal cardiac assessment. These key indices may serve as potential indicators of fetal well-being and facilitate the early detection of cardiac dysfunction in high-risk pregnancies.

We report a fetus at 20 + 5 weeks' gestation with an exceptionally rare constellation of congenital anomalies. Prenatal imaging revealed left congenital diaphragmatic hernia, rightward cardiac displacement, possible tracheoesophageal fistula (TEF), transposition of the great vessels, and a large ventricular septal defect. Autopsy confirmed complete absence of tracheoesophageal separation from the larynx to the carina, consistent with Kluth type Xc2, incorporating extralobar pulmonary sequestration with esophageal communication. The findings met criteria for a communicating bronchopulmonary foregut malformation (CBPFM), Srikanth Group III. Additional anomalies included bilateral pulmonary hypoplasia, bilateral unilobar lungs with eparterial bronchi, mirror-image dextrocardia, L-looped ventricles with concordant atrioventricular and ventriculoarterial alignments, right-sided aortic arch, bilateral superior vena cava, cardiac-type total anomalous pulmonary venous connection with drainage into the morphologic right atrium, and anomalous coronary origin from the pulmonary artery. Cytogenetic studies and whole-exome sequencing were normal. To our knowledge, this is the first report of Kluth type Xc2 TEF with Srikanth Group III CBPFM since Stolte's original description in 1952, and the first to combine this unique constellation. This case broadens the recognized spectrum of foregut and cardiopulmonary maldevelopment and underscores the importance of comprehensive evaluation in fetuses with multiple anomalies.

Abstract Background Scimitar syndrome is a rare congenital cardiopulmonary anomaly characterized by partial anomalous pulmonary venous return (PAPVR), most often involving drainage of the right pulmonary veins into the inferior vena cava (IVC) instead of the left atrium. It is commonly associated with right lung hypoplasia, dextroposition of the heart, and other cardiovascular or bronchopulmonary abnormalities. While usually diagnosed in infancy or childhood, adult presentations are uncommon and often manifest with vague or non-specific respiratory symptoms. Case presentation We report the case of a 60-year-old female who presented with intermittent dyspnea and was found to have right upper lobe agenesis, middle lobe aplasia, and anomalous right pulmonary venous drainage to the IVC. The diagnosis of Scimitar syndrome was confirmed based on imaging studies. This case is unique in highlighting the diagnostic complexity and subtlety of adult presentations, in which anatomical abnormalities may be overlooked or misattributed to other causes of dyspnea. Conclusion Scimitar syndrome, though typically diagnosed early in life, should be considered in adults presenting with unexplained respiratory symptoms and radiological anomalies. Awareness of this rare condition and its variable presentations is crucial for timely diagnosis and management. We also present a concise review of the available literature outlining diagnostic approaches and therapeutic considerations in adult cases of Scimitar syndrome.

Bilateral Partial Anomalous Pulmonary Venous Return with Intact Interatrial Septum: A Case Report and Review of Literature

Clinical characteristics, echocardiographic findings, and computed tomography angiography in the diagnosis of sinus venosus atrial septal defect with partial anomalous pulmonary venous connection in 10 dogs.

Total Anomalous Pulmonary Venous Connections.

ABSTRACTPartial anomalous pulmonary venous connection (PAPVC) is a rare congenital anomaly in which one or more pulmonary veins drain into the systemic venous circulation. PAPVC is often misdiagnosed as idiopathic pulmonary hypertension in the adult population. We report a case of a 48‐year‐old woman with long‐standing pulmonary hypertension who presented with recurrent pulmonary oedema and right‐sided heart failure. Transthoracic echocardiography showed severe pulmonary hypertension with preserved left ventricular function. Computed tomographic pulmonary angiography excluded pulmonary embolism but confirmed an anomalous drainage of the right upper pulmonary vein into the superior vena cava, consistent with isolated PAPVC without an atrial septal defect. Right heart catheterization confirmed severe precapillary pulmonary hypertension with a positive vasoreactivity response to adenosine. She was successfully managed medically with diltiazem and diuretics, showing sustained clinical improvement. This case highlights isolated PAPVC as a critical, under‐recognised cause of severe pulmonary hypertension in adults.