Anomalous Pulmonary Venous Connection Research Articles

Proximal Interruption of Pulmonary Artery: Spectrum of Radiological Findings With Emphasis on Chest Radiograph and Contrast-Enhanced Computed Tomography (CECT).

Introduction Proximal interruption of pulmonary artery (PIPA) is a congenital anomaly presenting withaberrant termination of the pulmonary artery at the hilum. It results in a variety of radiological andclinical manifestations. Clinically, isolated PIPA can be asymptomatic till late adulthood or can present with dyspnoea, chest discomfort, hemoptysis and recurrent infections. PIPA can be associated with multiple cardiovascular anomalies such as tetralogy of Fallot (TOF), ventricular septal defects (VSD), and scimitar syndrome. We present a spectrum of cases with both isolated proximal interruption of the pulmonary arteryand cases associated with other cardiovascular abnormalities.Typical chest radiographs and chest contrast-enhanced computed tomography (CECT) findings are discussed and demonstrated in detail.Proper and early diagnosis is a crucial step as it can significantly modify the treatment choice, thereby reducing morbidity. Objective To document the different presentations of the proximal arrest of pulmonary arteries, to document associations with cardiovascular and pulmonary manifestations, and to elaborate on and demonstrate the various radiological imaging findings. Material and methods All the cases that were reported with proximal interruption of pulmonary artery on the CECT studies conducted between 2019 and 2022 at a tertiary care hospital in Telangana, India. The demographic data, clinical presentation, chest radiographs, and chest CECT were collected retrospectively. Data analysis was done using Microsoft Excel 2019 to calculate descriptive statistics.A total of 22 cases were identified of which three cases were excluded of as they were previously operated and 19 cases were taken as the study population. Results Nineteen patients were included in the study. Demographic details, clinical history, CECT, and chest radiographs were collected wherever available. The majority of the cases belonged to the ≤ 10 yrs age group with the most common clinical presentation being a previous diagnosis of tuberculosis or recurrent upper respiratory tract infections. The predominant findings on chest radiographs were deviation of the trachea to the affected side, volume loss in the ipsilateral lung field, and compensatory hyperinflation of the contralateral lung field. On the CECT chest, the main findings were interrupted pulmonary artery, hypoplastic lung fields with bronchiectasis, or ground glassing. Associated cardiovascular and pulmonary malformations were identified with notable cases: TOF, right-sided aortic archand scimitar syndrome. Their typical imaging findings have been elucidated and discussed in detail. Conclusions Patients with recurrent respiratory infections or hemoptysis having hypoplastic lung field with hyperinflation of the contralateral lung on chest radiographs should be evaluated for pulmonary artery interruptions. Chest CECT allows evaluation of the bronchial tree and lung parenchyma at the same time which helps distinguish pulmonary interruption from conditions such as Swyer-James-Macleod syndrome, pulmonary hypoplasia, thromboembolismand arteritis. Cases with PIPA can also be associated with cardiovascular and pulmonary anomalies such as TOF, partial anomalous pulmonary venous connection (PAPVC), and VSD. The knowledge of these associations is essential as they can influence the mode of treatment and can help reduce the long-term morbidity and mortality associated with the condition.

Midterm postoperative outcomes of different types of surgical reconstruction of sinus venosus atrial septal defects with anomalous pulmonary venous connection: The Results of Prospective Cohort Study.

Sinus venosus atrial septal defects (SVASDs) constitute a substantial part of atrial septal defects and are usually characterized by anomalous pulmonary venous connection (APVC), causing complications like sinus node dysfunction and arrhythmias. Several surgical approaches are used for treating SVASDs in pediatric patients, including single- and two-patch techniques. The study aimed to prospectively evaluate and compare the safety and efficacy of these two methods with different follow-up periods. Ten patients aged 1-8 years with SVASDs and partial APVC were enrolled in the study at Bhanubhai and Madhuben Patel Cardiac Centre, Karamsad, India, between December 2018 and October2021. The single-patch (sandwich-patch) technique was used in two patients, whereas the two-patch (dual-patch) technique with autologous pericardium was used in seven. Safety was assessed as the absence of complications in the follow-up periods of 6 months, 1, and 2 years, whereas efficacy was estimated by the preserved sinus rhythm and the development of arrhythmias. Electrocardiographic and echocardiographic methods were used to evaluate both parameters. No deaths, reoperations, pulmonary vein, and superior vena cava (SVC) stenosis or phrenic nerve palsy were observed among the 10 patients in the three follow-up periods. Sinus rhythm was arrested in two of the seven patients who underwent two-patch repair, whereas no rhythm disturbances occurred in those who underwent single-patch repair. Both techniques used in SVASD repair with autologous pericardium proved to cause the smaller rate of complications in midterm postsurgical phase. However, there is a potentially great risk of the development of sinus node malfunction after the application of the two-patch technique. Therefore, methods avoiding sinus node interference are preferred in patients with partial APVC involving SVC.

Primary sutureless repair concomitant with the Warden procedure.

A 2-month-old girl who had supracardiac total anomalous pulmonary venous connection (Darling classification type 1b) was referred to our institution. Computed tomography showed that multiple right upper pulmonary veins drained into the vertical vein, near the entry to the superior vena cava. The common pulmonary venous chamber was located lower right than usual, and right upper pulmonary veins were far from the common chamber. We successfully performed primary sutureless repair concomitant with the Warden procedure. Postoperative computed tomography showed unobstructed pulmonary veins and superior vena cava routes, and the vertical vein between right upper and lower pulmonary veins shrank slightly.

Outcomes of treatment for right atrial isomerism with functional single ventricle and extracardiac total anomalous pulmonary venous connection beyond neonatal period: Delayed surgical treatment, improving outcomes.

Total anomalous pulmonary venous connection (TAPVC) is frequently associated with right atrial isomerism (RAI), which is commonly complicated with an unbalanced atrioventricular canal with contralateral hypoplasia, complex systemic and pulmonary venous anatomy, and conotruncal abnormalities, resulting in increased risk of mortality. This study aimed to review the outcomes of delayed surgical treatment for patients with RAI complicated with functional single ventricle (FSV) and TAPVC at a single center. In this retrospective study, we reviewed the medical records of 24 consecutive patients with RAI complicated with FSV and TAPVC who underwent initial surgical palliation after 5-month old between September 2008 and June 2019. Demographic data, concomitant anomalies, age at initial palliation, and surgical interventions were extracted and analyzed using the Cox proportional hazard model to assess risk factors for mortality and the Kaplan-Meier method to assess survival. The in-hospital mortality was 12.5% (three out of 24). The causes of death were pulmonary arterial hypertension and low cardiac output syndrome. Average follow-up was 65.2 ± 40.3 months (7-137 months). Another 4 patients died during the follow-up due to low cardiac output syndrome, protein-losing enteropathy and pulmonary arterial hypertension, respectively. Kaplan-Meier estimated survival at 1 and 5 years were 83.1 and 69.4%, respectively. Fontan completion was 45.8% (11/24). The mortality for patients with pulmonary venous obstruction (PVO) was 66.7% (4/6). Cox multivariate regression analysis indicated that preoperative PVO was the only risk factor for mortality (p = 0.032; hazard ratio, 10.000; CI 1.222-81.811). Outcomes of delayed surgical treatment for patients with RAI complicated with FSV and TAPVC have improved significantly. The survival and Fontan completion were higher. However, preoperative PVO was still the risk factor for mortality.

Cardiac surgical outcomes of patients with heterotaxy syndrome

ObjectivesThe study objectives were to analyze the outcomes of pediatric patients with heterotaxy syndrome undergoing cardiovascular surgery and to determine the predictors of mortality. MethodsA retrospective analysis of 82 patients diagnosed with heterotaxy syndrome who underwent cardiovascular surgery between January 2008 and December 2017 was performed. Univariate and multivariable Cox regression analyses to determine risk factors for mortality and Kaplan–Meier analysis for survival were performed. ResultsPatient mortality in the cohort was 34% (28/82), including 36% (20/55) for single ventricle palliation and 30% (8/27) for biventricular repair. At 5 years, the probability of survival did not differ between the groups by log-rank testing (P = .829). Multivariable analysis found extracorporeal membrane oxygenation support (hazard ratio, 10.4; 95% confidence interval, 4.3-25.4; P < .001), total anomalous pulmonary venous return (hazard ratio, 4.3; 95% confidence interval, 1.7-10.8; P = .002), and birth weight 2.5 kg or less (hazard ratio, 2.4; 95% confidence interval, 1.0-5.4; P = .041) to be independent risk factors for mortality in all-comers. Pulmonary vein stenosis was a univariate predictor of mortality among all patients with heterotaxy (hazard ratio, 3.0; 95% confidence interval, 1.4-6.4; P = .005) and in the subgroup of patients with single ventricles (hazard ratio, 4.0; 95% confidence interval, 1.7-9.7; P = .002). Overall survival was 66% (54/82) at a median follow-up time of 2.2 years (0.4-4.1) from the initial surgery. ConclusionsOutcomes of children with heterotaxy syndrome, irrespective of the operative pathway, remain suboptimal in the current era. Risk factors for mortality in this population include birth weight 2.5 kg or less, extracorporeal membrane oxygenation, pulmonary vein stenosis, and total anomalous pulmonary venous return, which may help to further optimize surgical decision making. Multiorgan system involvment is frequently encountered in these patients.

Ductus arteriosus banding to regulate excessive pulmonary blood flow in a neonate with necrotizing enterocolitis and complex congenital heart disease, including pulmonary atresia and total anomalous pulmonary venous return: a case report

BackgroundPatients with right isomerism have accompanying complex congenital heart disease, which is characterized by pulmonary atresia and total anomalous pulmonary venous return. Balanced regulation of the systemic and pulmonary circulation is essential for successful management, especially for cases complicated with necrotizing enterocolitis (NEC).Case presentationA 6-day-old male neonate with a single ventricle, pulmonary atresia, patent ductus arteriosus (DA), and total anomalous pulmonary venous return associated with right isomerism was admitted because of dyspnea, cyanosis, and melena. The patient presented circulatory incompetence due to excessive pulmonary blood flow, resulting in NEC. The patient underwent DA banding and colectomy following continuous intravenous infusion of prostaglandin E1 at six days. Subsequently, his condition improved, reaching a systemic oxygen saturation of around 80%. He underwent a bidirectional Glenn procedure and closure of colectomy at the ages of 5 and 6 months, respectively.ConclusionDA banding can be an alternative to placing an aortopulmonary shunt, which is conventional in patients with ductus-dependent pulmonary circulation, because DA banding is feasible without cardiopulmonary bypass.

Echocardiographic evaluation of supracardiac anomalous pulmonary venous connection in children: comparison with multilayer spiral CT.

Objective To explore the clinical value of transthoracic echocardiography (TTE) in the differentiation of Supracardiac Anomalous Pulmonary Venous Connection (SAPVC) in children. Materials and methods A total of 118 children with concurrent TTE and CT databases of cases diagnosed with SAPVCs were included. We analyzed the consistency between the two for the ability to diagnose the classification of SAPVC, drainage sites, ectopic pulmonary veins and the segments of superior vena cava (SVC). Results The consistency between TTE and CT in diagnosing the existence of SAPVC and the classification were 88.1% (95% CI: 80.9-93.4%) and 91.0% (95% CI: 84.1-95.6%), respectively. The error rate of partial type diagnosed by TTE was significantly higher than that of total and mixed type (20.5% vs. 2.8%, P = 0.003). The consistency between TTE and CT to determine drainage sites was 91.9% (95% CI: 85.2-96.2%). TTE had a significantly higher error rate in determining pulmonary vein drainage to the SVC than in those draining into the left innominate vein (17.5 vs. 2.5%, P = 0.007). The consistency of TTE and CT in judging the number of veins was 87.4% (95% CI: 79.7-92.9%). The error rate in determining the presence of 2 and 5 ectopic pulmonary veins was significantly higher than those of 1 and 4 veins (P < 0.05). Conclusion TTE for diagnosing partial SAPVC and identifying the drainage site of SVC has a high error rate of misdiagnosis and missed diagnosis. The extra attention should be given to these factors in clinical practice to improve the accuracy of TTE in diagnosing SAPVC.

218 PERSISTENT LEFT SUPERIOR VENA CAVA IN AN ADULT PATIENT WITH AN IMPLANTED PACEMAKER: A CASE REPORT

Abstract Introduction Persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly, although infrequent in the general population (prevalence estimates as 0.2-3%). It begins at the junction of the left subclavian and internal jugular veins, passes through the left side of the mediastinum adjacent to the aortic arch, and mostly drains into the right atrium via the coronary sinus (CS). For the majority of cases it is asymptomatic and it is mostly detected incidentally. More rarely it can be associated with other congenital abnormalities, such as aortic bicuspid valve, aortic coartation or atrial septal defects. Besides, in less than 10% of the cases the left superior vena cava drains in the left atrium, leading to a right-to-left shunt. Lastly, it can lead to arrhythmic problems. Case presentation A 85-year-old man was admitted to the emergency department for syncope. Previous medical history was unknown and there was no clinical documentation available. The electrocardiogram showed an atrial fibrillation and some pacemaker-induced beats. An echocardiogram was performed, which showed dilation and dysfunction of the right chambers and a dilated CS. Moreover, the same exam revealed the two pacemaker leads passing through the CS and ending their course into the right atrium and ventricle. The chest X-ray displayed the pacemaker leads passing to the left side of the aorta. Furthermore, a computed tomography angiography was performed in the suspicion of a pulmonary embolism. The exam showed small vascular filling defects in arteries of the right inferior lung lobe, but also the presence of two superior vena cavas, with the left one draining into the CS. The pacemaker leads passed from the left subclavian vein through the left superior vena cava reaching the right chambers. Therefore, the diagnosis of PLSVC was confirmed. Discussion and Conclusions Despite the benign natural history of this diagnosis, it is important to know the existence of this condition. PLSVC can be suspected when a dilated CS is detected on a transthoracic echocardiogram, with a parasternal long axis view. The best projections to visualize the dilatation of CS are also the apical four-chamber and the subcostal view. Besides, the direct detection of left superior vena cava can be obtained with a suprasternal view. The presence of the right superior vena cava should also be confirmed with a subcostal or suprasternal view. The differential diagnosis for a dilatation of the CS includes anomalous pulmonary venous return with a pulmonary vein draining in the CS, the “unroofed coronary sinus”, a tricuspid regurgitation with a jet directed towards the CS, elevated right-chamber filling pressures. The diagnosis of PLSVC can be confirmed with a saline contrast echocardiography (“bubble-test”) or with other radiological investigations (computed tomography or magnetic resonance). It should be reported in radiological reports even when it is an incidental finding because of its clinical relevance: it is essential to know its presence in advance in invasive procedures, such as pacemaker implantation, ablative procedures, cardiac surgery, because it could affect the proper approaches and could lead to complications. Moreover, in a minority of cases, it can be associated with other congenital abnormalities or with arrhythmic problems.

Arrhythmic Burden of Adult Survivors With Repaired Total Anomalous Pulmonary Venous Connection.

The long-term outcome of adults with repaired total anomalous pulmonary venous connection (TAPVC) is poorly documented. Therefore, the present study aims to provide current clinical data on adult survivors with repaired TAPVC focusing on arrhythmia. Clinical and imaging data (prevalence and type of arrhythmias, symptoms, surgical and medical treatment, echocardiographic and cardiac magnetic resonance haemodynamic parameters) were retrospectively collected from 8 European centres and compared between patients with and without arrhythmias. Fifty-seven patients were included (age 20 [16-67] years [female 28, 49%]). At the last follow-up, that is, 21 (8-51) years after surgery, 79% and 93% of patients were free of symptoms and cardiac medication, respectively. The prevalence of late arrhythmias was 21%; 9 (16%) patients showed intra-atrial re-entrant tachycardia (IART) and 2 (4%) ventricular arrhythmias. Patients with IART were older (P= 0.018) and 4 (7%) required antiarrhythmic medication. Three patients (5%) underwent an electrophysiological study, and another 3 (5%) underwent pacemaker implantation within 36 months after surgical correction, which were removed in 2 patients after 7 years. Early postoperative arrhythmias (P= 0.005), right ventricular dilatation (P= 0.003), and valvulopathy (P= 0.009) were more often present in patients with late IART. Adult survivors after isolated-TAPVC repair presented a high prevalence of arrhythmias. Age, right ventricular dilatation, early arrhythmias, and valvular lesions are risk factors for IART. Long-term follow-up is important as some of these currently asymptomatic patients will probably develop arrhythmias in the future.

Total anomalous pulmonary venous connection with another major congenital cardiac defect in biventricular hearts.

The association of total anomalous pulmonary venous connection (TAPVC) with major congenital cardiac anomalies is rare. This coexistence has an impact on the diagnosis and management strategies. The objective of this single centre study was to analyse the outcomes of such association in bi-ventricular hearts. This is a single-centre retrospective review of all patients who underwent repair for TAPVC associated with major congenital cardiac anomalies in bi-ventricular hearts from February 2015 to March 2020. Data were obtained from the hospital database. The primary aim was to assess the surgical outcomes. In addition, we described the incidence, pathophysiology, morphology and surgical strategies in this cohort of patients. Out of a total of 274 patients who underwent repair for TAPVC during the study period, 16 (5.8%) patients had one or more major associated cardiac defect anomalies. The major associated lesions were ventricular septal defects (9), tetralogy of Fallot (2), transposition of the great arteries (1), aorto-pulmonary window (1), partial atrioventricular canal defect (1), pulmonary stenosis (1) and severe tricuspid valve regurgitation due to flail chord (1). The mean follow-up period is 32 ± 13 months. There were two deaths in the post-operative period and no deaths in the follow-up period. The incidence of major associated cardiovascular defects with TAPVC in bi-ventricular morphology is 5.8%. The presence of a major associated lesion with TAPVC significantly alters the pathophysiology and clinical presentation with an impact on diagnosis and treatment.

Partial anomalous pulmonary venous return in adults: Insight into pulmonary hypertension

BackgroundPartial anomalous pulmonary venous return (PAPVR) is a rare condition that may lead to pulmonary arterial hypertension (PAH). We sought to determine the prevalence of PAPVR, the follow-up rate of incidentally discovered PAPVR, the repercussions of volume and pressure overload on the right ventricle (RV), and the prevalence and predictors of PAH. Methods and resultsFifty PAPVR patients aged ≥18 years were included. All underwent transthoracic echocardiography and multislice imaging. PAPVR prevalence was 0.2% (23/13,606 chest computed tomography reports). Of the 26 incidentally diagnosed patients, 14 (54%) did not have a cardiac follow-up. Seven (14%) patients had PAH, of whom one third (28%) had Eisenmenger syndrome. The left-to-right shunt ratio (Qp/Qs) was the only predictor of PAH. PAPVR-induced volume overload led to increased right chamber volumes (RV basal diameter 45±8 mm, RV mid-diameter 38±9 mm, RV/left ventricle ratio 1.2 ± 0.2, indexed RV end-diastolic area 14±4 cm2/m2, indexed RV end-systolic area 8.6 ± 3.2 cm2/m2) and increased RV functional parameters (tricuspid annular plane systolic excursion 27±5 mm, fractional area change [FAC] 42 ± 10%, global longitudinal strain [GLS] −22 ± 5). In contrast, PAH-induced pressure overload had no impact on right heart volumes, but on RV functional parameters, which were decreased compared to non-PAH patients (FAC 35 ± 13% vs. 43 ± 10%, p = 0.049; GLS -18 ± 5 vs. −23 ± 4%, p = 0.01). ConclusionsPAPVR prevalence was 0.2%. When incidentally diagnosed, its management was often neglected despite potentially serious consequences (14% PAH). Only the Qp/Qs ratio was predictive of PAH. PAPVR-induced volume overload was shown to increase right heart volumes, while PAH-induced pressure overload caused alterations of RV functional parameters.

Birth defects associated with paternal firefighting in the National Birth Defects Prevention Study.

Few studies have evaluated birth defects among children of firefighters. We investigated associations between birth defects and paternal work as a firefighter compared to work in non-firefighting and police officer occupations. We analyzed 1997-2011 data from the multi-site case-control National Birth Defects Prevention Study. Cases included fetuses or infants with major structural birth defects and controls included a random sample of live-born infants without major birth defects. Mothers of infants self-reported information about parents' occupations held during pregnancy. We investigated associations between paternal firefighting and birth defect groups using logistic regression to estimate odds ratios (ORs) and 95% confidence intervals (CIs). Referent groups included families reporting fathers working non-firefighting and police officer jobs. Occupational groups included 227 firefighters, 36,285 non-firefighters, and 433 police officers. Twenty-nine birth defects were analyzed. In adjusted analyses, fathers of children with total anomalous pulmonary venous return (TAPVR;OR = 3.1; 95% CI = 1.1-8.7), cleft palate (OR = 1.8; 95% CI = 1.0-3.3), cleft lip (OR = 2.2; 95% CI = 1.2-4.2), and transverse limb deficiency (OR = 2.2; 95% CI = 1.1-4.7) were more likely than fathers of controls to be firefighters, versus non-firefighters. In police-referent analyses, fathers of children with cleft palate were 2.4 times more likely to be firefighters than fathers of controls (95% CI = 1.1-5.4). Paternal firefighting may be associated with an elevated risk of birth defects in offspring. Additional studies are warranted to replicate these findings. Further research may contribute to a greater understanding of the reproductive health of firefighters and their families for guiding workplace practices.

Surgical Outcomes of Three Repair Techniques for Partial Anomalous Pulmonary Venous Connection in Adult Patients

Objectives: To investigate primary and secondary surgical outcomes following transcaval repair (TCR), modified Warden repair, and transatrial repair techniques for partial anomalous pulmonary venous connections (PAPVCs) and sinus venosus atrial septal defects (ASDs). Methods: This is an observational cohort clinical study. Patients who underwent TCR, modified Warden repair, and transatrial surgical repair for PAPVC and ASD between January 2003 and October 2019 at our institution were included in the study. Patients had one of the surgical procedures based on the anatomy of the defect. Results: Ten patients, seven (70%) males and three (30%) females, were included in the analysis. Seven patients underwent TCR, two patients the modified Warden technique, and one patient underwent transatrial surgical repair. Mean age was 57 years ± 14.7. Mean EuroScore II was 3.4 ± 3.5. The baseline left ventricle ejection fraction was 45 ± 6.5%. No patient had previous stroke, pacemaker (PM) implantation, or myocardial infarction. Total cardiopulmonary bypass and cross-clamping time were 123 ± 72.5 and 100 ± 48.5 min, respectively. Mean mechanical ventilation, mean intensive care unit, and mean hospital length of stay for the transcaval, modified Warden, and transatrial groups were 4.6 ± 10.7, 5.7 ± 8.8, and 10.5 ± 9.2 days, respectively. Superior caval or pulmonary venous obstruction, sinus node dysfunction, and PM implantation were not present at follow-up. The patient who underwent transatrial repair had died at 5.5-year follow-up due to myocardial infarction. Total survival rate at 6 years was 90%. Conclusions: The findings from this study elicit that all three techniques have low postoperative morbidity and are feasible and reliable procedures.

Impact and Problems of Fetal Echocardiography: A Single-Institution Study in Japan.

The purpose of this study was to assess the current status of prenatal diagnosis and the benefit of fetal echocardiography for complex congenital heart disease (CHD) in Kagawa prefecture. We reviewed 152 cases of CHDs who were born between 2012 and 2020 and performed cardiovascular surgery or catheter intervention in our hospital. They were dividedinto two groups: the fetal diagnosis group (FD) and the no-fetal diagnosis group (nFD). We compared patientdata between FD and nFD groups. Of the 152 patients, 63 patients were FD group and 89 patients were nFD group. The fetal detection rate was lower than 40% between 2012 and 2014, its ratio raise to about 50% after 2015. The ratio of poor general conditions at admission was significantly higher in the nFD group (p<0.01). When focused on hypoplastic left heart syndrome, the number of patients with poor general conditionat admission was significantly small in the FD group. Although, the first operation day of life and neonatal death did not show a significant difference between both groups. In total anomalous pulmonary venous connection (TAPVC) cases, 14 cases out of 15 had not been diagnosed prenatally. Among them, the number of cases with poor general conditionwas nine (64%) and inappropriate usage of oxygen or nitric oxide was 10 cases (71%). This study suggests that prenatal diagnosis significantly reduces poor general condition at admission. Although the prenatal detection rate grew up, that of TAPVC remains low and their clinical course is poor. In addition, inappropriate therapies were performed. It is desirable to increase the detection rate of TAPVC.

Influenza vaccination during pregnancy and risk of selected major structural congenital heart defects, National Birth Defects Prevention Study 2006-2011.

Although results from studies of first-trimester influenza vaccination and congenital heart defects (CHDs) have been reassuring, data are limited for specific CHDs. We assessed associations between reported maternal influenza vaccination, 1 month before pregnancy (B1) through end of third pregnancy month (P3), and specific CHDs using data from a multisite, population-based case-control study. Analysis included 2,982 case children diagnosed with a simple CHD (no other cardiac involvement with or without extracardiac defects) and 4,937 control children without a birth defect with estimated delivery dates during 2006-2011. For defects with ≥5 exposed case children, we used logistic regression to estimate propensity score-adjusted odds ratios (aORs) and 95% confidence intervals (CIs), adjusting for estimated delivery year and season; plurality; and maternal age at delivery, race/ethnicity, low folate intake, and smoking and alcohol use during B1P3. Overall, 124 (4.2%) simple CHD case mothers and 197 (4.0%) control mothers reported influenza vaccination from 1 month before through the third pregnancy month. The aOR for any simple CHD was 0.97 (95% CI: 0.76-1.23). Adjusted ORs for specific simple CHDs ranged from 0.62 for hypoplastic left heart syndrome to 2.34 for total anomalous pulmonary venous return (TAPVR). All adjusted CIs included the null except for TAPVR. Although we cannot fully exclude that exposure misclassification may have masked risks for some CHDs, findings add to existing evidence supporting the safety of inactivated influenza vaccination during pregnancy. The TAPVR result may be due to chance, but it may help inform future studies.

Abstract 10439: Assessment of Left Atrial Deformation in Patients With Total Anomalous Pulmonary Venous Connection by Two-Dimensional Speckle-Tracking Echocardiography - A Single-Center Retrospective Cohort Study

Background: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease of newborns characterized by insufficient left ventricle growth and diastolic dysfunctions. We hypothesized that reduced blood flow into the left heart in the uterus could also affect left atrium (LA) growth, leading to anomalies after birth. We compared the age-related changes in LA parameters detected using two-dimensional speckle-tracking echocardiography (2DSTE) in patients with TAPVC. Method: The LA datasets of 28 neonates with isolated TAPVC were analyzed before surgery and during follow-ups and compared with those of age-matched healthy controls (January 2009–2022). The LA strain (ε), indicating LA function, was analyzed using QLAB represented by reservoir (εR), conduit (εCD), and contractile (εCT) functions. LA pressure was determined by performing catheterization. Results: Compared to the control group, the TAPVC group had significantly smaller LA maximum size preoperatively and normal and larger time-dependent increase in the LA maximum and minimum size, respectively. All 2DSTE-determined LA functions showed significant reductions at all time points in the TAPVC group compared to those in the control group (median εR, εCD, and εCT; before surgery: 17.0 vs. 26.0, 12.9 vs. 15.9, and 6.3 vs. 10.4; follow up at 1-2 years: 30.0 vs. 45.7, 23.2 vs. 29.6, and 6.1 vs. 16.3; follow up at 5-7 years: 31.2 vs. 43.1, 25.0 vs. 31.2, and 5.2 vs. 10.8, respectively; p &lt; .01). However, compared to before surgery, εR and εCD did represent a significantly increased trend at follow-ups (median εR and εCD: 16.8→27.5 and 1.1→23%, respectively). Patients with pulmonary venous obstruction (PVO) at birth showed significantly decreased εR and εCD and higher LA pressure compared to those without PVO. Conclusion: This study showed that LA impairments existed even after repair in patients with TAPVC, suggesting that LA function loss mediated by less blood flow persist in childhood.

Abstract 15712: Imaging Conundrum: The Diagnosis of Unusual Partial Anomalous Pulmonary Venous Return With Dual Connections

Case: A 68 year old male with a past medical history of hypertension, hyperlipidemia and chronic kidney disease presented for progressive dyspnea on exertion and bilateral lower extremity edema over three weeks. He was found to be in decompensated heart failure and new onset Atrial Flutter with rapid ventricular rate. Transthoracic echocardiography revealed severely impaired left ventricular systolic function with ejection fraction &lt;20%, enlarged right ventricle, moderate right ventricular systolic dysfunction, biatrial enlargement, mild pulmonary hypertension. He was started on guideline directed medical therapy and referred for transesophageal echocardiogram (TEE) with cardioversion for new onset Atrial Flutter and possible tachycardia induced cardiomyopathy. Imaging findings: During TEE, routine doppler of right upper pulmonary vein showed markedly dilated superior vena cava (SVC) (3.6 cm) and unusual predominant outflow with brief mid late systolic inflow (Fig A and B), suggestive of partial anomalous pulmonary venous return (PAPVR). All three other pulmonary veins appeared normal. Also noted to have positive bubble study likely related to PAPVR. Subsequent right heart catheterization left to right shunt with Qp/Qs 1.6, step up noted between SVC and right atrium. CTA revealed partial anomalous right upper lobe pulmonary vein (RUPV) had dual connection to both SVC via window like communication and left atrium (Fig C), no sinus venosus defect seen. Conclusion: This case illustrates an unusual example of PAPVR with bidirectional flow, as well as the importance of thorough evaluation even on routine transesophageal echocardiograms especially for patients with right heart enlargement.

Abstract 9409: Risk Factors and Outcomes Associated With Loss of Follow-Up in Children With Congenital Heart Disease

Introduction: Adults with congenital heart disease (CHD) benefit from cardiology follow-up. However, benefit for children is less clear as studies of follow-up patterns in children with CHD are limited. Hypothesis: There are identifiable risks for loss to cardiology follow-up in children with CHD, and loss to follow-up changes healthcare utilization and outcomes. Methods: Our cohort included children &lt;10 years old with CHD and a healthcare encounter from 2008 to 2013 at one of four North Carolina hospitals. Using a Cox proportional hazards model, we assessed associations between demographics, presence of a severe lesion (conotruncal, endocardial cushion, and single ventricle lesions, aortic arch interruption, and total anomalous pulmonary venous return), and healthcare access, including educational isolation (EI) via geo-coded neighborhood of residence, and at least one cardiology follow-up visit within two years of initial encounter. We analyzed healthcare utilization by follow-up using a negative binomial landmark analysis. We described incidence of death and cardiac transplantation. Results: Of the 6,969 children, 24.8% were non-Hispanic Black and 14.1% were Hispanic. Overall, 60.4% received follow-up within 2 years, including 53.1%, 58.1%, and 79.0% of those with valve, shunt, and severe lesions respectively. Factors associated with loss to follow-up were increased drive time to the nearest cardiology clinic (Hazard Ratio 0.92 per 15-minute increase), EI (HR 0.94 per 0.2-unit increase), lower lesion severity (HR 0.48 for shunt/valve vs severe), and older age at initial encounter (HR 0.95 per month if &lt;1 year old and 0.94 per year if &gt;1 year old; p&lt;0.05 ). Children lost to follow-up had more emergency department (ED) visits (Rate Ratio 1.59) and fewer inpatient encounters and procedures (RR 0.51, 0.35; p&lt;0.05 ). Only 0.8% of children died or received a cardiac transplant after the 2-year landmark with no significant difference between follow-up groups. Conclusions: We found novel factors associated with loss to cardiology follow-up in children with CHD and worse utilization with loss to follow-up. Our findings demonstrate a need to mitigate healthcare barriers and generate clear cardiology follow-up guidelines for children.

Prepregnancy exposure to dietary arsenic and congenital heart defects.

Arsenic crosses the placenta and accumulates in fetal tissues. In the United States, diet is the predominant route of arsenic exposure, but epidemiologic data are sparse regarding this exposure and development of birth defects. Using data from a large case-control study, we explored associations between maternal dietary arsenic exposure and congenital heart defects (CHDs), the most prevalent birth defects. We used maternal self-reported dietary assessments and arsenic concentration estimates in food items to estimate average daily exposure to dietary arsenic during the year before pregnancy for mothers of 10,446 unaffected control children and 6,483 case children diagnosed with CHDs. Using tertiles of dietary exposure to total arsenic (all species) and inorganic arsenic, we applied logistic regression analysis to estimate associations for middle and high tertiles, compared with the low tertile. Positive associations (odds ratio [OR] ≥ 1.2) for total arsenic were observed in both tertiles for perimembranous ventricular septal defect (VSD) and high tertile only for double outlet right ventricle-transposition of the great arteries (DORV-TGA), partial anomalous pulmonary venous return (PAPVR), and tricuspid atresia. Positive associations were also observed in both tertiles (tricuspid atresia) and high tertile only (DORV-TGA, conoventricular VSD, PAPVR, and pulmonary atresia) for inorganic arsenic. Most remaining associations were near or below unity. Exploration of maternal dietary exposure to total and inorganic arsenic and CHDs produced few positive associations but was limited by available food item concentrations. Future research requires expanded collection of dietary data, improved estimates of concentrations, and consideration of nondietary sources of arsenic exposure.

Minimally invasive versus sternotomy approach for double-patch repair of partial anomalous pulmonary venous connection and sinus venosus defect in pediatric and adult patients: Mid to long-term outcomes.

Several surgical techniques have been proposed to repair right partial anomalous pulmonary venous connection (PAPVC) along with sinus venosus defect (SVD). This study aimed to compare the perioperative data and outcomes of double-patch repair using a minimally invasive approach versus conventional sternotomy in pediatric and adult patients. This retrospective study was conducted on 48 minimally invasive cases and 35 sternotomy cases, undergoing surgery by a single surgeon between July 2002 and August 2020. For all patients, repair was performed using the double-patch technique. In the minimally invasive approach, right mini-thoracotomy was performed with central cannulation for children and with peripheral cannulation for adults. The patients were classified into two pediatric and adult groups, and each group was categorized into minimally invasive and sternotomy approaches. They were followed-up by transthoracic echocardiography and electrocardiography before and early after surgery, 3 and 6 months after surgery, and then annually. The relative data were compared between the two approaches in terms of perioperative findings, postoperative pulmonary vein or superior vena cava (SVC) stenosis, and sinus node dysfunction. This study included 25 minimally invasive cases and 19 sternotomy cases in the pediatric group (mean age, 4.99 ± 4.28 and 6.10 ± 4.39 years, respectively) and 23 minimally invasive cases and 16 sternotomy cases in the adult group (mean age, 35.73 ± 8.06 and 32.62 ± 9.80 years, respectively). The mean and median follow-ups were 6.31 ± 4.92 years and 6 years (range: 6 month-18 year) in the pediatric group and 6.15 ± 4.53 years and 5 years (range: 6 month-18 year) in the adult group, respectively. The mean chest tube drainage was significantly lower in the minimally invasive pediatric group (p = .03), and the mean blood transfusion volume was significantly lower in the minimally invasive adult group compared to the other groups (p = .03). No stenosis occurred in the pulmonary veins. Mild SVC stenosis occurred in one patient in the minimally invasive pediatric group, with no need for reintervention. All patients had a normal sinus rhythm, except for the mentioned case with a transient, first-degree atrioventricular block, which spontaneously reverted to the normal sinus rhythm. The minimally invasive approach can be a safe and practical alternative for the double-patch repair of PAPVC and SVD. It ensures a repair with comparable quality to sternotomy, but with better cosmetic and psychological outcomes.