Anomalous Pulmonary Venous Connection Research Articles

A Prospective Randomised Observational Study of Obstructed Total Anamalous Pulmonary Venous Connection (TAPVC) Repair Patients with Milrinone Versus Milrinone and Inhaled Nitric Oxide

Abstract Background: Obstructed total anomalous pulmonary venous connection (OTAPVC) typically presents with severe cardiovascular decompensation and requires urgent surgical management. Pulmonary arterial hypertension (PAH) is a major risk factor affecting mortality. Perioperative management focuses on providing inotropic support and managing potential pulmonary hypertensive episodes. The aim of this study was to determine the outcome of patients with high pulmonary arterial pressure (PAP) with milrinone alone and a combination of milrinone and inhaled nitric oxide (INO). Material and Methods: After the approval of the ethical committee, this single-centre prospective randomised and observational study was conducted over a period of two years among eighty-six patients with obstructed TAPVC repair with severe PAH. Group-I patients received milrinone, and Group-II patients received both milrinone (after aortic cross clamp removal) and INO during the post-operative period at the cardiac care unit (CCU). Clinical outcomes such as ventilation time, length of stay (LOS) in the CCU, LOS in the hospital, complications, and hospital mortality were compared between the two groups. Result: The average ventilation time, LOS in CCU, and LOS in hospital for group I were 96.82 ± 19.46 hours, 10.91 ± 7.53 days, and 14.46 ± 7.58 days, respectively, and for group II, it was 85.14 ± 15.79 hours, 7.28 ± 3.68 days, and 10.21 ± 3.14 days, respectively, which was statistically significantly lower for group II. Reintubation, RV dysfunction, and hospital mortality were 16.3%, 37.2%, and 6.9% in group I, and 4.8%, 14.6%, and 2.4% in group II, respectively. The P value for each variable was significant < 0.05 (except mortality). Conclusion: Preoperative obstruction is a risk factor for postoperative obstruction, as 235 patients with obstructed TAPVC had severe PAH (39.98%) in this study. Management of severe PAH with a combination of milrinone and INO had a better outcome than milrinone alone.

Transcatheter stenting of superior vena cava to treat postoperative SVC syndrome in a child: a case report

BackgroundSuperior vena cava (SVC) obstruction leading to SVC syndrome is an uncommon but potential complication of cardiac surgeries that involve dissection and anastomosis around the great vein. We present a case of iatrogenic SVC obstruction that was initially treated with transcatheter balloon angioplasty, which provided temporary relief, and ultimately resolved by stenting the affected segment.Case presentationThe index case underwent total anomalous pulmonary venous connection (TAPVC) repair and presented 3 months after surgery with features of SVC obstruction. Initially, transcatheter balloon angioplasty was performed, providing relief from the obstruction; however, the condition recurred within one month. Finally, the patient was treated with percutaneous stenting of superior vena cava, through femoral venous route, using 8 mm × 30 mm balloon-expandable bare metal stent (Formula 418, Cook Medical, Bloomington, IN). Remarkable relief of obstruction was established with decrease in mean gradient across SVC–right atrium junction to 2 mm Hg (from 12 mm Hg before balloon angioplasty and 18 mm Hg before stenting).ConclusionPercutaneous treatment for iatrogenic SVC obstruction developing after cardiac surgery appears to be effective. Close monitoring is required in the postoperative period for early diagnosis and timely intervention.

Outcomes of Adults With Unrepaired Partial Anomalous Pulmonary Venous Return and Intact Atrial Septum.

The purpose of this study was to describe the correlates and outcomes in adults with unrepaired partial anomalous pulmonary venous return and intact atrial septum (PAPVR-IAS). We identified adults with PAPVR-IAS who received care at the Mayo Clinic, while those with unrepaired PAPVR-IAS comprised the reference group. Clinical indices (New York Heart Association class, peak oxygen consumption, and NT-proBNP [N-terminal pro-B-type natriuretic peptide]) and echo-derived right heart indices (right atrial [RA] volume, RA reservoir strain, right ventricular [RV] free wall strain, RV end-diastolic area, and RV systolic pressure) were assessed at baseline and 3-year and 5-year follow-up. There were 80 patients and 38 patients with unrepaired versus repaired PAPVR-IAS, respectively. The clinical predictors of surgical repair were the number of anomalous veins, RA volume, and RV end-diastolic area. The PAPVR-IAS risk score, derived from these clinical predictors, was associated with surgical repair (adjusted odds ratio, 1.37 [95% CI, 1.24-1.65] per unit increase in risk score; area under the curve, 0.742). Among patients with unrepaired PAPVR-IAS with 3-year (n=73) and 5-year follow-up (n=36), there was no temporal change in clinical indices (New York Heart Association class, predicted peak oxygen consumption, and NT-proBNP) and right heart indices (RA volume index, RA reservoir strain, RV end-diastolic area index, RV free wall strain, and RV systolic pressure). The PAPVR-IAS risk score can be used to assess the odds of requiring surgical repair. Furthermore, there was no temporal deterioration in clinical and right heart indices during follow-up in adults with unrepaired PAPVR-IAS.

Minimally Invasive Surgical Repair of Simple Congenital Heart Defects Using the Right Vertical Infra-Axillary Thoracotomy Approach.

To evaluate the safety and efficacy of surgical repair for patients diagnosed with simple congenital heart defects (CHD) using the minimally invasive right vertical infra-axillary minithoracotomy (RVIAT) approach. We retrospectively reviewed the clinical data of consecutive patients who underwent minimally invasive RVIAT for repair of CHD between August 2019 and August 2022. There were 382 patients who underwent 8 primary procedures and were included in this study. The median age of the patients was 16.2 (interquartile range [IQR], 7.2 to 41.9) months, and the median weight of the patients was 8.8 (IQR, 6.5 to 14) kg. The preoperative diagnoses were as follows: ventricular septal defect, atrial septal defect, partial anomalous pulmonary venous return, partial atrioventricular septal defect, cor triatriatum, complete atrioventricular septal defect, and myxoma. The mean aortic cross-clamp time, bypass time, and operation time were 45.4 ± 19.3 min, 65.6 ± 23.1 min, and 154.5 ± 29.7 min, respectively. There was no in-hospital mortality or conversion to median sternotomy. Two patients (0.5%) required early reoperation; 1 due to postoperative bleeding and 1 for permanent pacemaker implantation. Other complications included trivial residual shunts (23 of 382, 6%), pleural effusion (3 of 382, 0.8%), pneumothorax (0.8%), and wound infection (4 of 382, 1%). There were 2 late noncardiac deaths. Late reoperation was performed on 1 patient with progressive aortic valve regurgitation who required aortic valvuloplasty. RVIAT is a minimally invasive approach that can be safely performed on patients with simple CHDs. RVIAT may be a good alternative approach for median sternotomy and cardiac intervention.

Surgical Repair of Total Anomalous Pulmonary Venous Connection into the Superior Vena Cava: A Case Report.

Total anomalous pulmonary venous connection is a rare congenital anomaly that has four anatomical subtypes. In the supracardiac type, the common pulmonary vein confluence usually drains into the left innominate vein via a vertical vein; however, it may drain into the superior vena cava. Herein, we present a successful surgical repair of a rare type of total anomalous pulmonary venous connection in which the common pulmonary vein confluence was draining directly into the superior vena cava without a vertical vein.

Diagnostic Capabilities of Transthoracic Echocardiography and Cardiac Computed Tomography in Preoperative Planning of Total Anomalous Pulmonary Venous Return Correction

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.

De Novo and Progressive Pulmonary Vein Stenosis Following Pediatric Heart Transplantation: A Multicenter Retrospective Study.

Pulmonary vein stenosis (PVS) is a rare condition in which neointimal proliferation leads to venous and arterial hypertension. Little is known about PVS after heart transplant (HTx) in children. We sought to describe the characteristics and outcomes of children who develop PVS after HTx. We performed a retrospective review of patients ≤18 years old who underwent HTx at two HTx centers between April 2012 and October 2023. Patients with PVS were identified via database queries. Cardiac diagnosis, PVS location and extent, and outcomes were recorded. Over 11.5 years, 422 patients underwent HTx across both centers. Nineteen patients with PVS (10 male) were identified, 15 with de novo PVS. Sixteen had underlying congenital heart disease (CHD), two with anomalous pulmonary venous return. PVS was diagnosed at a median of 2 months (range 2 weeks to 14 years) after HTx. At time of initial diagnosis, 13 patients had one-vessel PVS. At final follow-up, 7/19 (37%) had increases in the number of vessels involved. Six patients underwent surgery, and nine patients had stent or balloon angioplasty. Two patients were treated for pulmonary hypertension following PVS diagnosis. Three patients died from right heart failure secondary to PVS. This is the largest study to describe the characteristics of post-HTx PVS in children. PVS occurs in 4.5% of HTx, and underlying CHD is a strong risk factor. Multiple vessels can be involved and may require catheter-based or surgical intervention. Clinicians must be vigilant in monitoring the development of PVS in this population.

Early outcomes of minimal invasive surgery via the axillary approach for complex congenital heart disease at the Cardiovascular Center - E hospital

Objective: This study aims to outline the initial outcomes of implementing minimally invasive surgery via the right axillary approach to completely repair complex congenital heart diseases. Summary: Minimally invasive surgical techniques have gained widespread adoption in managing simple congenital heart diseases such as atrial septal defect, ventricular septal defect, partial atrioventricular septal defect, cor triatriatum, and partial anomalous pulmonary venous return. Postoperative results have demonstrated equivalence, if not superiority, to traditional sternotomy procedures while offering enhanced cosmetic benefits. However, for more intricate congenital heart conditions such as tetralogy of Fallot (TOF), complete atrioventricular septal defect (CAVSD), and Ebstein syndrome, this study evaluates the early outcomes of employing minimally invasive surgery via the right axillary approach, a technique that remains relatively uncommon globally. Our study seeks to assess the initial outcomes of this approach in managing complex congenital heart diseases. Subjects and methods: descriptive method based on a series of cases. From April 2023 to December 2023, 45 patients diagnosed with complex congenital heart disease underwent minimally invasive surgery utilizing the axillary approach. This cohort comprised 40 patients with tetralogy of Fallot, 3 patients with complete atrioventricular septal defect, and 2 patients with Ebstein syndrome. Results: All procedures were technically successful, without requiring sternotomy. The cardiopulmonary bypass times for the TOF, complete AVSD, and Ebstein syndrome groups were 81 minutes, 91 minutes, 96 minutes, respectively. Corresponding aortic cross - clamp times were 70 minutes, 72 minutes, and 74 minutes. There were no postoperative fatalities or significant complications. Postoperative echocardiographic evaluations demonstrated favorable outcomes. Conclusion: Minimally invasive surgery via the axillary approach for managing complex congenital heart diseases yields promising early results and offers notable cosmetic advantages.

Clinical Outcomes, Predictors, and Surgical Management of Infracardiac Total Anomalous Pulmonary Venous Connection.

This study sought to find the risk factors of postoperative pulmonary venous obstruction (PVO), PVO-related reintervention, and postoperative mortality, determine the relationship between pulmonary venous confluence (PVC) morphology and the clinical outcomes, and provide the basis for the selection of the optimal surgical strategy for infracardiac total anomalous pulmonary venous connection (TAPVC). From December 2009 to December 2023, 101 patients with infracardiac TAPVC undergoing surgical repair in our institution were included [Conventional surgery (CS) group, n = 73; Sutureless technique (ST) group, n = 28]. The Kaplan-Meier curve was used to demonstrate the survival estimates. The Cox proportional hazard model was used to identify risk factors for primary endpoints. There were 8 in-hospital deaths and 4 late deaths. The survival rates at 1, 5, and 10years were 88.8%, 87.6%, and 87.6%, respectively, in the whole cohort. Multivariable analysis showed that prolonged cardiopulmonary bypass (CBP) time was associated with overall mortality and Y-shaped PVC has a trend for a higher mortality rate. Postoperative PVO occurred in 27 patients. Freedom from postoperative PVO at 1, 5, and 10years were 81.8%, 69.9%, and 69.9%, respectively. Y-shaped PVC and preoperative PVO were incremental risk factors for PVO, but ST repair was a protective factor. Sixteen patients underwent PVO-related reintervention. Y-shaped PVC was the only independent risk factors for PVO-related reintervention while ST repair was a protective factor. Sutureless technique can achieve satisfactory outcomes for infracardiac TAPVC repair. Compared with CS repair, ST repair is significantly associated with decreased postoperative PVO and PVO-related reintervention. Y-shaped PVC is associated with a higher risk of PVO, PVO-related reintervention, and mortality, respectively. Patients with preoperative PVO are more likely to develop postoperative PVO.

The use of minimally invasive surgical techniques ın pediatric patients with partial anomalous pulmonary venous return

Aim The use of minimally invasive procedures in low-risk congenital heart surgeries has been increasing recently. Compared to traditional median sternotomy, minimally invasive techniques offer better cosmetic results and provide more satisfaction to both the patient and their parents. Partial anomalous pulmonary venous return (PAPVR) can be safely repaired using these techniques. Material and Metods The perioperative data of right- sided PAPVR patients who were operated on using minimally invasive approaches in our clinic between March 2019 and January 2023 were reviewed retrospectively. The perioperative data of the patients' including , type of surgery , cardiopulmonary bypass time, cross-clamp time, postoperative cardiac rhythm, total operation time, total drainage, total intensive care unit time, and total hospital stay time were obtained. The results were compared with the data in the current literature. Results During the study period, 14 patients underwent surgical repair. Five (36%) of them were female and nine (64%) were male. Patients undergoing right infraaxillary vertical thoracotomy (RIAVT) and right anterolateral thoracotomy (RALT) had mean ages of 48±26.6 and 42±18.2 month, respectively. High venosum type atrial septal defect (ASD) was detected in 12 (85.7%) of the patients. The most frequently used surgical treatment was the double patch technique which was used in 12 patients (85.7%). This was followed by single patch repair with one patient (7.1 %). The Warden procedure was used in one patient (7.1%) who was operated on with the RIAVT technique. Patients’ cross-clamp, cardiopulmonary bypass (CPB), and total operation times were comparable to those reported in the medical literature. Conclusion RIAVT and RALT are two of the oftenly used minimally invasive surgical techniques in congenital heart surgery. After gaining sufficient experience, both of these methods can be safely applied to PAPVR repair.

Diagnostic value of reversed differential cyanosis in (supra)cardiac total anomalous pulmonary venous return.

To investigate the occurrence of reversed differential cyanosis (RDC) in case of (supra)cardiac total anomalous pulmonary venous return (TAPVR), we explored the hemodynamic changes and oxygen saturation levels during the fetal-to-neonatal transition in (supra)cardiac TAPVR, thereby revealing determinant factors of RDC. A computational model was used to simulate the cardiovascular fetal-to-neonatal transition up to 24 h after birth. Abnormalities associated with TAPVR, like patent ductus arteriosus (PDA) and persistent pulmonary hypertension of the neonate (PPHN), were imposed on the model. Hemodynamic impact on flow distribution and right-sided pressures as well as oxygen saturations were assessed. Model findings demonstrated that RDC in (supra)cardiac TAPVR was dependent on two key factors: (1) the type of pulmonary venous connection being supracardiac or cardiac, and (2) the presence of a patent ductus arteriosus exhibiting right-to-left shunting. Persistence of RDC was mainly determined by the latter; an increase in pulmonary-to-systemic pressure difference by PPHN or PDA-induced pulmonary over-circulation contributed to persistence of RDC. This study highlights the significance of RDC in (supra)cardiac TAPVR and suggests to incorporate early screening ( < 24 h after birth) and to consider RDC as an immediate fail in screening protocols to ensure prompt detection of (supra)cardiac TAPVR. Utilizing a validated computational model for the cardiovascular fetal-to-neonatal transition, this study sheds light on the complex hemodynamics in neonates with (supra)cardiac Total Anomalous Pulmonary Venous Return (TAPVR). Model findings suggest that the often-present pulmonary over-circulation in neonates with TAPVR might significantly contribute to the anomaly's frequent omission during pulse-oximetry screening beyond the first 24 h after birth. This study highlights the diagnostic value of reversed differential cyanosis in early screenings within the first 24 h after birth. By including RDC as an immediate fail in early pulse-oximetry screenings, the likelihood of missing (supra)cardiac TAPVR cases could be reduced.

Early and Midterm Outcome of Surgical Repair of Total Anomalous Pulmonary Venous Connection

Early and Midterm Outcome of Surgical Repair of Total Anomalous Pulmonary Venous Connection

Assessing the risk of reoperation for mild pulmonary vein obstruction post-TAPVC repair: a retrospective cohort study.

This study investigates the impact of mild pulmonary vein obstruction, detected via echocardiography before hospital discharge, on the likelihood of reoperation in patients who have undergone repair for Total Anomalous Pulmonary Venous Connection (TAPVC). Utilizing a single-center, retrospective cohort approach, we analyzed 38 cases from October 2017 to December 2023, excluding patients with functionally univentricular circulations or atrial isomerism. Our primary outcome was the necessity for reoperation within one year due to anatomical issues related to the initial TAPVC repair. Mild obstruction was defined as a pulmonary vein flow velocity ≥1.2 m/s. Our findings revealed that 31.6% of patients exhibited pre-discharge mild obstruction. During the median follow-up of 10 months, reoperations were notably higher in the mild obstruction group compared to the normal group, with a significant association between pre-discharge mild obstruction and increased risk of reoperation. Specifically, in the fully adjusted model, mild obstruction was linked to a 13.9-fold increased risk of reoperation. Our results suggest that a pre-discharge echocardiography Doppler velocity threshold of 1.2 m/s could serve as a critical predictor for reoperation, emphasizing the need for targeted follow-up strategies for at-risk patients.

Long-Term Outcomes of Surgical Repair of Supracardiac Total Anomalous Pulmonary Venous Connection.

The conventional surgery (CS) of supracardiac total anomalous pulmonary venous connection (TAPVC) is not always effective particularly in the setting of complex anatomy such as the mixed variety of TAPVC. This study aimed to analyze the outcomes of surgical treatment of supracardiac TAPVC and determine the optimal strategy. From December 2009 to December 2023, patients with supracardiac TAPVC undergoing surgical repair in our institution were included. The Kaplan-Meier curve was used to demonstrate the survival estimates. The Cox proportional hazard model was used to identify risk factors for death and postoperative pulmonary venous obstruction (PVO). One hundred and eighty-three patients with supracardiac TAPVC underwent surgical repair [CS group, n = 102; modified L-shaped incision technique (MLIT) group, n = 81]. There were 8 in-hospital deaths and 16 late deaths. The survival rates at 1, 5, and 10 years were 89.0%, 85.0%, and 85.0%, respectively in the whole cohort. Multivariable analysis showed that lower weight (P = 0.031), prolonged CBP time (P = 0.007), preoperative PVO (P = 0.020), and emergency surgery (P = 0.001) were incremental risk factors for death, but using the MLIT was a protective factor for death (p = 0.028). In the CS group, patients with emergency operation had worse survival than patients with elective surgery (P < 0.001). However, in the MLIT group, patients with emergency operation had comparable survival to patients with elective surgery (P = 0.332). Postoperative PVO occurred in 30 patients. Fourteen patients underwent PVO-related reintervention. In the whole cohort, freedom from postoperative PVO at 1, 5, and 10 years were 87.5%, 80.6%, and 80.6%, respectively. Patients who underwent MLIT repair had a lower incidence of postoperative PVO (P < 0.001), and PVO-related reintervention (P = 0.019). Neonates(P = 0.033), aortic cross-clamp time (P = 0.012), preoperative PVO (P = 0.002), and using the CS (P = 0.005) were associated with postoperative PVO. In terms of postoperative PVO, MLIT had a protective effect compared with CS. In the CS group, Infant and Children patients had better freedom from postoperative PVO than Neonate patients (P < 0.001). However, in the MLIT group, Neonate patients had comparable freedom from postoperative PVO to Infant and Children patients (P = 0.332). The MLIT can achieve satisfactory outcomes for supracardiac TAPVC repair. Compared with CS, the MLIT was significantly associated with decreased death, postoperative PVO, and PVO-related reintervention. It is especially significant in improving the survival rate of patients undergoing emergency surgery and reducing the incidence of postoperative PVO in neonatal patients.

Quantification of 3-Dimensional Confluence-Atrial Morphology in Supracardiac Total Anomalous Pulmonary Venous Connection

BackgroundPulmonary vein stenosis (PVS) continues to be a major complication after surgical repair of total anomalous pulmonary venous connection (TAPVC). Recent studies suggest that the morphology of pulmonary venous confluence and the left atrium (LA) is associated with PVS. However, there are limited data on the prognostic value of integrating quantitative confluence-atrial morphology into risk stratification. ObjectivesThis study sought to evaluate the prognostic impact of novel imaging metrics derived from 3-dimensional (3D) computed tomography angiography (CTA) modeling on postsurgical PVS (PPVS) in the supracardiac TAPVC (sTAPVC) setting. MethodsPatients undergoing sTAPVC repair in 2017 to 2022 from 3 centers were retrospectively reviewed. Study investigators developed 3D CTA modeled geometric features to quantify confluence-atrial morphology that were analyzed with regard to PPVS. ResultsOf the 162 patients (median age 61 days; 55% having preoperative pulmonary venous obstruction [prePVO]) included, 47 (29%) with PPVS at a median of 1.5 months ([quartile 1-quartile 3: 1.5-3.0 months]). In the univariable analysis, the indexed total volume of the LA and confluence (iTVLC) and the ratio of the corresponding confluence length to the mean distance between the LA and confluence (CCL/mDBLC ratio) were significantly associated with PPVS. In a multivariable model adjusting for prePVO and age, the iTVLC and CCL/mDBLC ratio independently predicted PPVS (HR: 1.15; 95% CI: 1.06-1.25; and HR: 1.20; 95% CI: 1.08-1.35, respectively, all P < 0.01). Specifically, an iTVLC ≥20 cm3/m2 and a CCL/mDBLC ratio ≥7.7 were significantly associated with a reduced risk of PPVS. ConclusionsQuantification of 3D confluence-atrial morphology appears to offer a deeper and better metric to predict PPVS in patients with sTAPVC.

Double left brachiocephalic vein in a paediatric patient with CHD: a case report

AbstractAnomalous brachiocephalic vein (ABCV) is a rare entity of head and neck venous channel variations and malformations. Amongst the five subtypes of ABVC, double left brachiocephalic vein (DLBCV) is the rarest. We present the case of a 1-year-11-month-old syndromic child, who had global developmental delay (GDD) with Sprengel deformity and failure to thrive (suspected Klippel Feil phenotype), who presented to us for the cardiac evaluation. Her 2D echocardiography revealed unobstructed total anomalous supra-cardiac pulmonary venous connection. However, for the delineation of individual pulmonary venous course, CT-pulmonary angiography was advised. CTPA revealed supra-cardiac total anomalous pulmonary venous connection (TAPVC) with incidentally noted DLBCV. Importance of recognition of DLBCV enables us to prevent accidental venous injury during cardiac surgery and to avoid intraoperative technical issues during transvenous pacemaker insertion.

Anatomical and physiological diagnostic discrepancies in fetuses with single-ventricle congenital heart disease in a contemporary cohort.

Image quality of fetal echocardiography (FE) has improved in the recent era, but few recent studies have reported the accuracy of FE, specifically in single ventricle (SV) congenital heart disease (CHD). This study aimed to assess the ability of FE to correctly predict SV-CHD postnatal anatomy and physiology in a contemporary cohort. The contemporary clinical reports of patients with SV-CHD, in which FE was performed between July 2017 and July 2021, were compared with postnatal echocardiograms from a formal quality assurance program. SV fetuses were grouped by anatomical subtype. Diagnostic errors were designated as major if the error would have caused significant alteration in parental counseling or postnatal management. The remaining errors were classified as minor. Physiological discrepancies, including prostaglandin-E (PGE) dependency, atrioventricular valve regurgitation (AVVR), pulmonary venous obstruction, and restrictive atrial septum (RAS) were assessed by chart review of the postnatal course. A total of 119 subjects were analyzed. SV subtypes in the cohort included hypoplastic left heart syndrome (HLHS) (n = 68), tricuspid atresia (n = 16), double-inlet left ventricle (n = 12), unbalanced atrioventricular canal (UAVC) (n = 11), heterotaxy (n = 9) and other (n = 3). The rate of major anatomical and physiological errors was low (n = 6 (5.0%)). A higher proportion of minor errors was noted in HLHS and tricuspid atresia, but the differences were not statistically significant. Physiological discrepancies were uncommon, with three major discrepancies, including underestimation of the degree of venous obstruction in one non-HLHS fetus with total anomalous pulmonary venous return, overestimation of RAS in one HLHS fetus and incorrect prediction of PGE dependency in one case false-negative for pulmonary blood flow. No discrepancy in degree of AVVR or RAS affected postnatal care. Minor physiological discrepancies included two false-positive predictions of PGE dependency with one false-positive for ductal-dependent systemic flow and one false-positive for pulmonary blood flow. In this contemporary review of FE at our center, there was high accuracy in describing anatomical and physiological findings in SV-CHD. Major physiological discrepancies were uncommon but included important cases of false-negative prediction of PGE dependency and underestimation of obstruction of total anomalous pulmonary venous return. These data can inform more accurate counseling of families with SV-CHD fetuses and guide diagnostic improvement efforts. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Long-term outcomes of repair in children with total anomalous pulmonary venous connection.

The clinical data of patients with total anomalous pulmonary venous connection who underwent repair in our centre in the past 13 years were reviewed. In this study, we systemically reviewed our experience in the optimal surgical strategy for patients with total anomalous pulmonary venous connection, aiming to provide evidence for clinical decision-making. From January 1, 2009, to December 31, 2021, 122 patients undergoing surgical treatment for total anomalous pulmonary venous connection in our hospital were enrolled. Among them, 18 patients with single ventricle repair were excluded from the study. Multivariate analysis was used to determine the risk factors for early and late death and the risk factors for pulmonary vein obstruction. There were 64 males and 40 females. The median age at surgery was 107 days (range, 25 days-788 days), the median weight at surgery was 4.8 kg (range, 3 kg-22 kg), and the median follow-up was 59 months (range, 0-150 months). Seven patients died early after surgery and six died late after discharge. Multivariable analysis indicated that prolonged cardiopulmonary bypass time was the only independent risk factor for early postoperative mortality. Multivariate analysis did not identify risk factors for late death. Emergency surgery, preoperative moderate and severe pulmonary hypertension, and prolonged cardiopulmonary bypass time were independent risk factors for postoperative pulmonary vein obstruction. Early and long-term late outcomes of repair in patients with total anomalous pulmonary venous connection have been encouraging. Postoperative pulmonary vein obstruction remains a major problem for specialists worldwide. Pulmonary vein obstruction should be considered in children with preoperative emergency surgery, moderate to severe pulmonary hypertension and prolonged cardiopulmonary bypass time, and regular follow-up is necessary.

Factors associated with early postoperative mortality after total anomalous pulmonary venous connection repair: A retrospective cohort study.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital defect where pulmonary venous plexus fails to connect with the left atrium (LA). Surgical repair is the primary treatment for TAPVC, but factors influencing outcomes are not fully understood. This study investigates the early outcomes of surgical repair for TAPVC and associated factors. A retrospective cohort analysis was conducted on TAPVC patients who underwent surgical repair between 2012 and 2022. Data were collected from medical records and supplemented with phone call validation. Demographic characteristics, surgical data, diagnostic tests, and outcomes were analyzed. Statistical analysis included chi-square, t-tests, and multivariate logistic regression using SPSS. A total of 88 patients underwent surgical repair for TAPVC, resulting in a mortality rate of 21.6%. Weight and bypass time were significantly associated with patient survival. Female patients had a higher likelihood of death. The anatomic type did not significantly influence mortality. Patients with pulmonary venous obstruction (PVO) experienced a higher mortality rate. Notably, ligation of the vertical vein in supracardiac and infracardiac types was associated with lower mortality. In conclusion, our study identifies several key factors contributing to higher mortality rates following TAPVC surgery, including low weight, female gender, prolonged bypass time, and preoperative vein obstruction. Highlighting the significance of surgical technique, particularly the sutureless approach, we advocate for its meticulous consideration to achieve improved outcomes. Furthermore, our findings indicate a potential decrease in mortality associated with vertical vein ligation, which may mitigate the risk of post-repair heart failure. We suggest further rigorous studies to gain comprehensive insights into TAPVC surgical interventions.

Scimitar Syndrome in a Pediatric Cohort.

Scimitar syndrome is a rare form of congenital heart disease (CHD) characterized by anomalous pulmonary venous drainage of the right lung to the inferior vena cava. We describe the presentation, diagnosis, therapeutic management and long-term follow-up of 10 pediatric patients with Scimitar Syndrome. We performed a retrospective observational study of all pediatric patients from our institution with scimitar syndrome (March 1996-July 2023). Patients underwent systematic evaluation including medical and family history, chest x-ray, 12-lead electrocardiogram, echocardiogram, angiography and/or computed tomography; or magnetic resonance angiography. Ten patients with scimitar syndrome were included. The median age at diagnosis was 10.4 [0.1-150.2] months and the median follow-up time was 7.7 [1.3-15.3] years. Eight patients presented with aortopulmonary collateral arteries which were embolized. Two patients had dual connections to the inferior vena cava and left atrium; embolization of the inferior vena cava connection was only feasible in one of them. No patients underwent surgery of the scimitar vein. Three patients had surgical correction of CHDs. There were no deaths related to scimitar syndrome during follow-up. All patients with scimitar syndrome need prompt cardiovascular evaluation and follow-up. Our study demonstrates that a conservative approach with aortopulmonary collateral artery embolization, scimitar vein embolization when dual drainage to the left atrium is identified, along with correction of concomitant CHDs might have good results in patients with scimitar syndrome in order to postpone surgical correction of the anomalous pulmonary venous return to an older age when clinically or hemodynamically indicated. Further studies with longer-term follow-up and a larger sample size are needed to more effectively determine treatment strategy.