Anomalous Pulmonary Venous Connection Research Articles

An explainable deep learning model to predict partial anomalous pulmonary venous connection for patients with atrial septal defect

BackgroundPatients with partial anomalous pulmonary venous connection (PAPVC) usually present asymptomatic and accompanied by intricate anatomical types, which results in missed diagnosis from atrial septal defect (ASD). The present study aimed to explore the predictive variables of PAPVC from patients with ASD and constructed an explainable prediction model based on deep learning.MethodsThe retrospective study included 834 inpatients with ASD in Women and Children's Hospital, Qingdao University from January 2018 to January 2023. They were separated into two groups based on the presence of PAPVC. Propensity score matching and SMOTE were used to balance the baseline data between groups. The differential variables between the two groups were determined by univariate logistic regression. The patients were randomly divided into the training set and the validation set in a ratio of 8:2. Support vector machines (SVM), Random forest, Decision tree, XGBoost, and LightGBM were used to build models by differential variables. The classification performance of models was compared. Split, gain and SHAP were used to measure the importance of differential variables and improve the interpretability of the model. Moreover, a portion of the patients was included in the validation set to test the performance of the selected models.ResultsThree hundred twenty-eight patients with ASD and patients with 82 PAPVC were included in the training set and the validation set, respectively. The selection of 10 differential variables was based on univariate logistic regression, including right atrial diameter (longitudinal axis and transverse axis), right ventricular diameter, left atrial diameter, left ventricular end-diastolic diameter, left ventricular end-systolic diameter, P-wave voltage, P-wave interval PR interval, and QRS-wave voltage. In the classification model established based on differential variables, the LightGBM model achieved the highest performance on the validation set (AUC = 0.93). Based on variables importance analysis, the LightGBM-Clinic model was retrained by P-wave voltage, P-wave interval, PR interval, QRS wave interval, and right ventricular diameter, and performed excellently (AUC = 0.90). The AUC of the LightGBM-Clinic model was 0.87 in the test set.ConclusionIn this study, the LightGBM model performs excellently in determining whether patients with ASD are accompanied by PAPVC. ECG parameters such as P-wave voltage were important to predictive value and enhance the explainability of the model.

Analysis and Visualization of Inter-/Intra-surgeon Variability Using Surgical Simulation for Partial Anomalous Pulmonary Venous Connection Repair.

There may be inter-/intra-surgeon variability in the repair of congenital heart defects. The objective was to analyze and visualize inter-/intra-surgeon variability in the level of patch suture lines and in the shape and size of patches developed through surgical simulation for partial anomalous pulmonary venous connection (PAPVC) repair using a patient-specific 3D-printed heart model. A patient with PAPVC and preoperative computed tomography data were selected, and a patient-specific heart model was obtained. Two different exposures on the model were tested. Two surgeons were enrolled. Both surgeons performed a single-patch repair on four heart models with one exposure and four models with the other. On the potential suture line, 20 points common in each model were allocated. The level of patch suture lines was represented as a deviation from the 20 points. The shape and size of the patches were analyzed and visualized using geometric morphometrics approaches, using the 20 points as landmarks to represent the patches. There was inter-surgeon variability in the level of patch suture lines, and the variability was higher in particular locations. There was inter-surgeon variability in the shape and size of patches, which was not affected by the exposures. The inter-surgeon variability in the shape of patches was higher in particular locations. There was intra-surgeon variability in the shape of patches. Inter-/intra-surgeon variability was analyzed and visualized by the surgical simulation using geometric morphometrics approaches. Further studies are needed to scrutinize whether the variability affects postoperative hemodynamics in actual surgery.

Pseudo hepatic vein thrombosis in a newborn with infracardiac total anomalous pulmonary venous connection.

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.

Contemporary prenatal diagnosis of congenital heart disease in a regional perinatal center lacking onsite pediatric cardiac surgery: obstetrical and neonatal outcomes.

Determine obstetrical and neonatal outcomes in neonates with major CHD delivered at a level IV neonatal intensive care units (NICU) center lacking onsite pediatric cardiac surgery. A 10- year retrospective review of all neonates admitted to our level IV NICU, with CHD between January 1st, 2011 and December 31st, 2021. Births and NICU charts were cross queried with those from our perinatal center which include pediatric cardiology records. Terminations and stillbirths were excluded. A total of 285 neonates with major CHD and 78 withminor defects were included. In the major CHD group, 82.8 % had an isolated cardiac anomaly and 17.2 % had an extracardiac anomaly. Type of extracardiac anomaly had noimpact on neonatal survival. Prenatal diagnosis of aneuploidy did not impact survival in major CHD. Truncus arteriosus had the highest NICU mortality at 34.0 % followed by hypoplastic left heart syndrome (HLHS) at 31.6 %. Double outlet right ventricle with transposition of the great vessels and interrupted aortic arch (both types) had a 25 % mortality. Neonates with truncus arteriosus and total anomalous pulmonary venous returns were likely to have 5-min Apgar score<7. Transfer rate of neonates with major CHD for cardiac surgery was 58.6 %. Of those 81.5 % were discharged home, 14.3 % expired before discharge, and 1 % were transferred elsewhere post-operatively for higher level of care. Neonates with major CHD can deliver safely at a level IV NICU lacking onsite pediatric cardiac surgery. Our neonatal mortality was high for HLHS and truncus arteriosus, however comparable to other centers with proximate pediatric cardiac surgery.

Disney to Pixar Transition Causing Epilepsy: A Case Report of Reflex Epilepsy.

This case report describes a rare instance of reflex seizures in a 4-year-old boy with a complex medical history, including total anomalous pulmonary venous connection and developmental disabilities. The patient experienced seizures triggered exclusively by a specific visual stimulus: the transition scene from the Disney castle to the Pixar lamp in Disney-Pixar movies. Video electroencephalography (EEG) revealed biparasagittal rhythmic delta waves and diffuse slowing, suggesting parietal involvement and complex cortical processing. The findings highlight the individualized nature of reflex epilepsy and highlight the need for a nuanced understanding of specific seizure triggers. Effective management included avoiding the identified visual stimulus and adjusting medication based on the patient's response. This case emphasizes the intricate relationship between sensory processing and epileptogenic mechanisms, contributing to our knowledge of cortical excitability and guiding targeted treatment strategies for reflex seizures.

Transcatheter correction of a rare combined anomalous pulmonary and systemic venous return in an adult.

Transcatheter correction of a rare combined anomalous pulmonary and systemic venous return in an adult.

A Comparison for Infantile Mortality of Crucial Congenital Heart Defects in Korea over a Five-Year Period.

Background: Nearly half of congenital heart defects (CHDs) related to mortality occur during infancy although advancements in treatments have increased the survival rates. This study comprehensively examined overall and surgical mortality in CHD infants with the highest mortality rates in an effort to improve our understanding of CHD epidemiology. Methods: Participants were drawn from a dataset of 1,964,691 infants born between 2014 and 2018 in Korea. Crucial CHDs are defined here as including diverse categorical defects and classical critical CHDs but excluding simple shunt defects. Overall mortality (procedural and natural mortality) and procedural mortality (interventional and surgical mortality) for infants were analyzed. Results: The performance rate for multiple procedures in infants with crucial CHDs was 16%. The overall and surgical mortalities of crucial CHDs were 8% and 7%. The mortalities of palliative procedures were relatively high. Procedural mortalities for infants were significantly decreased in the tetralogy of Fallot (TOF), atrioventricular septal defects, and total anomalous pulmonary venous return (TAPVR) compared with overall mortalities for infants. Surgical mortalities for infants involving TOF and TAPVR were significantly lower, but those for infants involving hypoplastic left heart syndrome (HLHS) were higher than those for all ages. Conclusions: Palliative procedural techniques in infants must be improved to obtain better outcomes, particularly in the palliative surgery of HLHS. The infantile procedural outcomes for TOF and TAPVR are excellent and important in order to overcome disastrous circumstances during infancy. This comprehensive study of the overall and procedural mortalities of CHDs may have laid a cornerstone for CHD epidemiology in Korean infants.

Cardiovascular health in women with turner's syndrome: a 3-year single centre audit

Abstract Background Turner’s syndrome (TS) is the most common genetic abnormality affecting females and is characterised by complete or partial absence of the X chromosome. Mortality is predominantly related to cardiovascular disease (1). Risk of life-threatening aortic dissection is 40 per 100,000 (2). Indexed aorta size (ASI) to body surface area (BSA) &amp;gt;20mm/m² is considered abnormal. An ASI of ≥25mm/m² is associated with increased risk of aortic dissection (3). Despite this there is a paucity of data in the literature regarding TS cardiovascular disease and providing evidence-based care can be challenging. Purpose Define cardiovascular disease, with focus upon aortopathy in our TS Cardiovascular Clinic (TCC). Methods Single centre retrospective medical record analysis of 248 women with TS (2021-2024) Results Patient baseline characteristics; mean age 37 years (range 18-75yrs), height 150cm (132-169cm), weight 62kg (37-130kg), BSA 1.55m² (1.19-2.17m²). 65 patients (29%) had bicuspid aortic valve (BAV), 19 (8%) and 5 (3%) had repaired and un-repaired aortic coarctation (CoA) respectively. 30 (13%) had other congenital heart disease, with most common was partial anomalous pulmonary venous connection (12 patients: 5%). 22/248 patients had previous aortic root surgery; 20 elective (mean ASI 29mm/m², range 26-33mm/m², mean age at time of surgery 42 years); 2 presented with aortic dissection (ASI 21mm/m²; pregnant, BAV &amp; 24mm/m²; tri-leaflet aortic valve, TAV). Data was available for 221 unoperated patients (figure 1). The site of maximal dimension was observed at the sinus of Valsalva in 70% compared to 30% at the ascending aorta. 130 patients (59%) had maximal ASI of &amp;lt;20mm/m². Dimensions of 20-23mm/m² and 23-25mm/m² were seen in 64 (29%) and 18 (8%) patients respectively. 7 patients (3%) had a maximal ASI of &amp;gt;25mm/m². 4 patients (2%) had an absolute maximal dimension of 40mm with ASI of 23mm/m² for all four. 9/11 patients with dimension ≥25mm/m² or 40mm had a body mass index (BMI) &amp;gt;25kg/m². Review of medical history for patients at and above threshold for risk of aortic dissection revealed referral to or previous discussion in an Aortic multi-disciplinary meeting (MDT) with a plan for ongoing surveillance. 57 patients (35%) with TAV had significant aortopathy (mean age 46years, range 19-75years) compared with 33 (51%) with BAV (mean age 39yrs, range 22-72 years) (figure 2). Review of patients &amp;gt;55years of age demonstrated a very similar burden of aortopathy in both TAV (14%) and BAV (15%) populations. Conclusions A high burden of cardiovascular disease exists within our TS population. There is a very high prevalence of aortopathy, increasingly with age and both with and without the presence of a bicuspid aortic valve. More published data is needed to help better define those at risk.

Appraisal of partial anomalous pulmonary venous drainage through a lumped-parameter mathematical model: a new pathophysiological proof of concept.

Haemodynamic determinants of the ratio between pulmonary and systemic flow (Qp/Qs) in partial anomalous pulmonary venous return (PAPVR) are still not fully understood. Indeed, among patients with the same number of lung segments draining anomalously, a great variability is observed in terms of right ventricular overload. The aim of this study was to test the hypothesis that the anatomic site of drainage, affecting the total circuit impedance, independently influences the magnitude of shunt estimated by Qp/Qs. A zero-dimensional (0D) lumped parameter mathematical model was developed and validated on a sample of patients. We developed a 0D lumped parameter model, using time varying elastances for heart chambers, RLC Windkessel circuits for the systemic and pulmonary circulations. Patients, were categorized into vena cava (VC) type (including left drainage to anomimous vein) and right atrium type (RA). The mathematical model is a system of ordinary differential equations that are numerically solved by means of the ode15s solver in the Matlab environment. The model showed an increase of Qp/Qs with the increase of the number of anomalous veins. With the same number of anomalous veins, Qp/Qs was lower in patients with anomalous drainage to the vena cava (VC) as compared with right atrium (RA) The validation sample consisted of 49 patients (27, 55% females). As predicted by the model, patients with PAPVR with VC type displayed a lower invasive and CMR Qp/Qs as compared with drainage to RA: 1.4 (1.2-1.7) and 1.45 (1.25-1.6) versus 2 (1.75-2.1) and 1.9 (1.6-2) p < 0.05. After stratifying for number of lung territories a lower Qp/Qs was measured in patients with VC PAPVR as compared with RA. In patients with PAPVR, the site of anomalous drainage modulates the Qp/Qs. According to the model, this effect is mediated by the post-capillary impedance of the circuit and significantly decreases with the increase of pulmonary vascular resistances.

Coronary Sinus Anomalies with Case Illustrations

Abstract Coronary sinus (CS) is the largest coronary vein that receives blood from all the venous tributaries of the heart. Its variations in anatomy are significant from the surgical point of view, especially in patients with complex congenital heart diseases (CHDs). CS anomalies are rare, but with increasing advancements in imaging, they are often diagnosed with cardiac computed tomography (CT). These are associated with other CHDs, so the radiologists are alerted to look further for different anomalies. The data in this study were collected from the routine contrast-enhanced CT examinations of the patients referred to the Department of Radiology, Narayana Hrudayalaya Hospital, Bengaluru, from January 2019 to August 2023. It was observed that the anomalies were seldom seen as a single entity and instead were seen as a part of the spectrum of anomalies such as persistent left superior vena cava (LSVC), Raghib complex, total anomalous pulmonary venous connection, and interrupted inferior vena cava. Out of these, the most common associated anomaly was found to be persistent LSVC. These anomalies are essential to detect in the preoperative assessment because they may cause significant morbidity in some cases if left uncorrected. Therefore, we must search for other cardiac anomalies when encountering CS anomalies.

Successful repair of infracardiac total anomalous pulmonary venous connection with nonconfluent bilateral pulmonary veins.

We report a rare case of an infracardiac-type total anomalous pulmonary venous connection with nonconfluent bilateral pulmonary veins in a patient diagnosed with heterotaxy syndrome with right atrial isomerism, mitral valve atresia, a single atrium, and double-outlet right ventricle. On the fourth day of life, the patient underwent successful repair using a sutureless technique. Two years after the surgery, the patient remained well without any signs of pulmonary venous obstruction.

Outcomes of the Modified Warden Procedure for Partial Anomalous Pulmonary Venous Connection Drainage to a High Location in the Superior Vena Cava

To report the safety and effectiveness of a modified Warden procedure. Twenty-six (26) patients underwent modified Warden surgery at our centre from September 2017 to September 2021. In all patients, the superior vena cava (SVC) was reconstructed by turning the atrial wall in the main body of the right atrium upwards and applying fresh autologous pericardial patches. There were 13 male patients and 13 female patients, and the median age of the patients was 7.0 (range, 0.3-47.0) years. The median follow-up time after surgery was 47.0 (range, 32.0-80.0) months. The aortic cross-clamp time was 108.50±34.72 minutes, the cardiopulmonary bypass time was 154.81±41.65 minutes, the median postoperative mechanical ventilation time was 8.5 (range, 0.0-91.0) hours, the median intensive care unit stay was 43.5 (range, 15.0-352.0) hours, and the median length of postoperative hospital stay was 7.0 (range, 6.0-19.0) days. All patients were safely and successfully discharged from the hospital. No deaths or sinus node dysfunction occurred during the follow-up period. The mean gradient of the reconstructed SVC and/or the right pulmonary vein (PV) increased in eight patients and decreased to less than 1 mmHg in six patients. One (1) adult patient had already undergone two vascular interventions, and one child had persistent stenosis but did not undergo reoperation at the time of the study. In addition, two patients were subjected to pericardial windowing. A modified Warden technique using a right atrial flap and autologous pericardium effectively corrected the high-plane connection between the PVs and SVC. This technique effectively avoids sinus node dysfunction; however, a short-term increase in the differential pressure of the re-established SVC or PV after the procedure is acceptable, and reintervention is required only in rare cases.

Multimodality assessment of a child with Turner syndrome and a late diagnosis of mixed partial anomalous pulmonary venous return without atrial septal defect. Case report

Multimodality assessment of a child with Turner syndrome and a late diagnosis of mixed partial anomalous pulmonary venous return without atrial septal defect. Case report

A Rare Case of Total Anomalous Pulmonary Venous Connection in an Adult

A Rare Case of Total Anomalous Pulmonary Venous Connection in an Adult

Numerical modeling assisting in surgical treatment of total anomalous pulmonary venous connection in children

ObjectiveTo develop a model using patient-specific computational fluid dynamics (CFD) to predict the required anastomotic size for total anomalous pulmonary venous connection (TAPVC) surgery and to forecast surgical outcomes.MethodsBased on...

Partial anomalous pulmonary venous connection in a dog.

Partial anomalous pulmonary venous connection in a dog.

Outcomes of the Lateral Caval Flap and Conventional Techniques for Repair of Right-Sided Partial Anomalous Pulmonary Venous Connection in Adults

Outcomes of the Lateral Caval Flap and Conventional Techniques for Repair of Right-Sided Partial Anomalous Pulmonary Venous Connection in Adults

Right-sided partial anomalous pulmonary venous connection with intact atrial septum and small left atrium: in a dextroverted heart

Right-sided partial anomalous pulmonary venous connection with intact atrial septum and small left atrium: in a dextroverted heart

Plasma PVD by small spiral Ta hollow cathodes

Spiral hollow cathodes represent interesting options for local PVD applications. Radio frequency powered small diameter spiral hollow cathodes made from 0.45 mm diameter Ta wire rolled around 0.5 mm diameter rod were tested in PVD regimes on silicon substrates at the gas pressure of 400 Pa (3 Torr). The PVD of Ta and reactive PVD of Ta-N resulted in deposition rates of about 130 nm/min with maximum thickness in the center of the coating spots. However, part of the coating spots can be heavily eroded. At higher RF powers droplets from the melted Ta tip of the spiral can damage the coating and melt the Si substrate. The PVD rates of Ta in argon were similar as those for TaN. However, lower number of droplets of the melted Ta were formed in argon. The heating of the spiral outlet and its effect on the coating was also more intense in nitrogen than in argon. The temperature of the Si substrate table reached about 500 °C in 20 min in the nitrogen plasma and up to 400 °C in argon. This heating was higher on electrically grounded substrates than on the floating substrates. The effect of sharp outlet on possible eroding of the sample was confirmed by a sharp ended 1 mm diameter stainless steel medical needle used as a hollow cathode.

Birth in the Operating Room for Immediate Cardiac Surgery: A Rare but Effective Strategy.

Background: With significant advancements in fetal cardiac imaging, patients with complex congenital heart disease (CHD) carrying a high risk for postnatal demise are now being diagnosed earlier. We sought to assess an interdisciplinary strategy for delivering these children in an operating room (OR) adjacent to a cardiac OR for immediate surgery or stabilization. Methods: All children prenatally diagnosed with CHD at risk for immediate postnatal hemodynamic instability and cardiogenic shock who were delivered in the operating room (OR) between 2012 and 2023 in which the senior author was consulted were included. Results: Eight patients were identified. Six (75%) patients were operated on day-of-life zero, all requiring obstructed total anomalous pulmonary venous return (TAPVR) repair. Of these six patients, 2 (33%) required a simultaneous Norwood procedure, 2 (33%) required pulmonary artery unifocalization and modified Blalock-Taussig-Thomas shunt, and 2 (33%) patients had repair of obstructed mixed TAPVR. The remaining 2 patients potentially planned for immediate surgery had nonimmune hydrops fetalis and went into cardiogenic shock at 12 and 72 hours postnatally, requiring a novel Norwood procedure with left-ventricular exclusion for severe aortic/mitral valve insufficiency. The median ventilation and inpatient durations were 19 [IQR: 11-26] days and 41 [IQR: 32-128] days, respectively. Three(38%) patients required one or more in-hospital reoperations. Subsequent staged procedures included Glenn (n = 5), Fontan (n = 3), biventricular repair (n = 2), ventricular assist device placement (n = 1), and heart transplant (n = 1). Median follow-up was 5.7 [IQR:1.3-7.8] years. The five-year postoperative survival was 88% (n = 7/8). Conclusion: While children with these diagnoses have historically had poor survival, the strategy of birth in the OR adjacent to a cardiac OR where emergent surgery is planned is a potentially promising strategy with excellent clinical outcomes. However, this is a high-resource strategy whose feasibility in any program requires thoughtful assessment.