Supracardiac Total Anomalous Pulmonary Venous Connection Research Articles

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.

Total Anomalous Pulmonary Venous Connection in Adults: Should We Offer Surgery?

Background: We aim to determine the surgical outcomes of adult patients with total anomalous pulmonary venous connection (TAPVC) and examine the regression of pulmonary artery (PA) pressures after the procedure. Methods: We reviewed the hospital records from 2003 to 2022 and identified 49 adult patients with TAPVC. We assessed their surgical outcomes and the trend of PA pressures after the procedure. Continuous data are presented as mean ± SD or median (interquartile range) and categorical variables are presented as percentages. Results: The median age of the patients was 23 years (range 18-42) and 31 (63.3%) were male. Thirty-six patients (73.5%) had supracardiac TAPVC. The mean systolic PA pressure was 65.8 ± 16.4 mm Hg and it decreased by 47.9% (34%, 61.8%) after surgery. Moderate or more tricuspid regurgitation was seen in 27 (55.1%) patients before surgery; however, it was present in only 3 (6.1%) patients during early follow-up. There was no intraoperative or 30-day mortality, and the median hospital length of stay was six days. Long-term follow-up data were available for 29 patients with the average duration of follow-up being 5.6 years (range 6 months to 15 years) and the mean systolic PA pressures of this cohort was 29.8±7.9 mm Hg. Forty-six (93.1%) patients were asymptomatic; four women had uneventful pregnancies and delivered healthy children. Conclusion: Surgical repair of the naturally selected group of adult TAPVC patients can be performed safely with good results. Regression in flow-related pulmonary hypertension and an improvement in functional quality of life are seen in nearly all patients.

Risk factors and outcomes for surgical repair of obstructed total anomalous pulmonary venous connection.

Surgical repair of obstructed total anomalous pulmonary venous connection (TAPVC) is a high-risk surgical cohort. This study aimed to assess surgical risk factors and outcomes in infants with TAPVC treated at a single centre. This was a prospective single-centre, observational study that included consecutive patients presenting with obstructed TAPVC. Patients with obstructed supra-cardiac, cardiac, and infra-cardiac TAPVC were included in the study. In-hospital mortality was the primary outcome. Multivariable logistic regression was carried out to identify risk factors for in-hospital mortality. Of the 41 patients, 31 (75.6%) were males. Ages were distributed as follows: 20 (48.8%) between 1 and 3 months, 11 (26.88%) between >3 months, and 10 (24.4%) neonates. The in-hospital mortality was 19.5% (n=8). Of the remaining 33 patients, there were no deaths during the follow-up. Short-term follow-up was achieved for 100% of cases (up to 6 months post-operatively). The mean duration of follow-up was 43.6± 3.6 months. One (3%) early and 2 (6%) late pulmonary vein obstructions were observed during follow-up. Age less than 30 days and pre-operative ventilation were associated with significantly increased risk of in-hospital mortality. Despite logistical challenges, reasonable surgical outcomes are attainable in obstructed TAPVC cases. Age less than 30 days and need for pre-operative ventilation were independent predictors of in-hospital mortality, while pulmonary venous obstruction in the post-operative period contributed to re-interventions.

Modified L-shaped incision technique for supracardiac total anomalous pulmonary venous connection as an alternative to sutureless technique.

The modified L-shaped incision technique (MLIT) was successfully applied to the repair of supracardiac total anomalous pulmonary venous connection (TAPVC) with promising mid-term outcomes. It is, however, unclear whether or not MLIT could be an alternative to sutureless technique (ST). All patients ( n =141) who underwent MLIT or ST repair for supracardiac TAPVC between June 2009 and June 2022 were included and a propensity score-matched analysis was performed to reduce the heterogeneity. MLIT was performed in 80.9% (114/141), whereas ST was performed in 19.1% (27/141). Patients who underwent MLIT repair had a lower incidence of pulmonary veinous obstruction (PVO)-related reintervention (1.8 vs. 18.5%, P =0.002), and late mortality (2.6 vs. 18.2%, P =0.006). Overall survival at 10 years was 92.5% (87.7-97.7%) for MLIT and 66.8% (44.4-100%) for ST ( P =0.012). Freedom from postoperative PVO at 10 years was 89.1% (83.2-95.5%) for MLIT and 79.9% (65.6-97.4%) for ST ( P =0.12). Cox proportional hazards regression identified prolonged mechanical ventilation duration, postoperative PVO, respiratory dysfunction, and low cardiac output syndrome were associated with postoperative death and PVO-related reintervention. The MLIT strategy is a safe, technologically feasible, and effective approach for supracardiac TAPVC, which is associated with more favorable and promising freedom from death and PVO-related reintervention.

Echocardiographic features and influencing factors of pulmonary hypertension of total anomalous pulmonary venous connection in adults.

Patients with total anomalous pulmonary venous connection (TAPVC) generally have symptoms during the neonatal period and infancy, and the fatality rate is extremely high. Most patients do not survive to adulthood. This study analyzed the clinical and transthoracic echocardiographic (TTE) manifestations of adult patients with TAPVC, summarized the echocardiographic characteristics of TAPVC, and identified the factors influencing pulmonary hypertension. Data from adult patients with TAPVC from Beijing Anzhen Hospital, China, were retrospectively collected for analyses, including sex, age, history of gestation, clinical manifestations, echocardiographic parameters, and blood oxygen levels. Patients were grouped for comparative analyses based on their pulmonary artery systolic pressure (PASP) (≥60 vs. <60 mmHg); 32 atrial septal defect (ASD) patients were included as a control group. (I) Sixteen patients were identified with TAPVC (11 women and 5 men; mean age: 32.2±9.5 years), including 8, 4, and 4 patients with supra-cardiac, mixed, and intracardiac type TAPVC, respectively. Furthermore, 10 patients had moderate or severe tricuspid regurgitation, and 6 had a PASP of ≥60 mmHg. Echocardiography misdiagnosed 2 patients with an ASD. (II) The TAPVC group patients had a smaller left atrium (LA) and a lower aorta/pulmonary artery ratio than ASD-only group patients. However, the right ventricular diameter (RVd) and right atrium were larger in patients with TAPVC than in those with only ASD. (III) The RVd was larger and the LA was smaller in patients with a PASP of ≥60 mmHg than in those with a PASP of <60 mmHg. (IV) Of those with a PASP of ≥60 mmHg, TAPVC patients had a smaller LA and a larger RVd than those with only ASD. (V) Pregnancy affected the PASP (adjusted odds ratio: 15.000, 95% confidence interval: 1.031-218.300, P=0.047). (VI) Echocardiography indicated that TAPVC patients with ASD had a right to left shunt at the atrial level and the pulmonary vein (PV) was not connected to the LA. Searching for the PV by TTE is necessary for patients with ASDs, which may help avoid misdiagnosis. Moreover, pregnancy affects the PASP. Patients with TAPVC may present with a larger right heart, smaller LA, and lower aorta/pulmonary artery ration than those with only ASD.

Prenatal diagnosis and postnatal verification in fetuses with total anomalous pulmonary venous connection.

To systematically verify the accuracy of a four-step prenatal ultrasonography in diagnosing fetal total anomalous pulmonary venous connection (TAPVC). A total of 62 TAPVC fetuses received prenatal ultrasonography and were confirmed by postnatal echocardiography, surgery, or postabortion autopsy. The suspected TAPVC fetuses were further screened by a four-step prenatal ultrasonography for TAPVC classification, pulmonary venous obstruction, and the associated malformations, and followed postpartum. The sonographic features, clinical data, and prognosis of the TAPVC fetuses were retrospectively analyzed. Of the 62 TAPVC fetuses, supracardiac TAPVC was found in 20 cases, intracardiac TAPVC in 12, infracardiac TAPVC in 21, and mixed TAPVC in 9. A total of 30 cases with right atrium isomerism were correctly diagnosed. Of the 11 cases with other intracardiac and extracardiac malformations, 1 case was missed to be diagnosed. Of the 21 isolated TAPVC cases, 6 were missed prenatally and 1 case was prenatally diagnosed as intracardiac and postnatally proved to be mixed (intracardiac type + supracardiac type) by echocardiography. Of the 13 TAPVC live births, 4 infants died in the neonatal period without operation. Of the nine infants undergoing the operation, five recuperated and survived; one survived but had complications with superior vena cava obstruction and collateral circulation formation, and three died postoperatively. The four-step prenatal ultrasound procedure can comprehensively and systematically evaluate fetal TAPVC, detailing the classification, potential obstruction, and associated malformations. It provides substantial support for subsequent prenatal counseling and neonatal assessment. The retrospective analysis also reveals that isolated TAPVC is more prone to be missed in diagnosis.

Cardiopulmonary Vascular Odyssey and Haemodynamic Conundrum: A Case Report of Total Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection (TAPVC) is a cyanotic congenital heart disease where all the pulmonary veins drain directly or indirectly into the right atrium. Each of these types can be further classified into two types based on the pulmonary veins – obstructive and non-obstructive. This case report describes a rare case of a 2-month-old male infant with an unobstructed supra-cardiac TAPVC. The patient presented with tachypnoea, cyanosis and feeding difficulties, which prompted a thorough examination. Unobstructed supra-cardiac TAPVC was revealed by the diagnostic evaluation using two-dimensional echocardiography. Supra-cardiac TAPVC repair surgery was performed, following which post-operative evaluations indicated that the patient had satisfactory biventricular functionality. This case emphasises the relevance of early detection of TAPVC, thereby highlighting the need for meticulous and prompt postnatal care. The primary insight lies in the crucial role of prenatal diagnosis in tertiary care facilities, which allows timely planning of delivery, postnatal surgical interventions and a better prognosis of the disease. By underlining the significance of early intervention in the management of such rare congenital cardiac diseases such as TAPVC, this case report offers valuable insights into the scientific community.

Anatomical attention-based prediction of postoperative pulmonary venous obstruction via CTA images.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease, with which some patients suffer from postoperative pulmonary venous obstruction (PPVO), requiring particular follow-up strategies and treatments. PPVO prediction has important clinical significance, while building a PPVO prediction model is challenging due to limited data and class imbalance distribution. Inspired by the anatomical evidence of PPVO, which is related to the structure of the left atrium (LA) and pulmonary vein (PV), we design an effective multi-task network for PPVO classification. The proposed method incorporates clinical priors and merits of the segmentation-based network into the classification task. The features learned from segmenting LA and PV are concatenated into the PPVO classification branch to constrain the learning of discriminative features. Anatomical-guided attention is applied in the aggregation of these features to restrict them focusing on TAPVC-related regions. To deal with the imbalance classification problem of PPVO, a novel classification loss derived by masked class activation map (MCAM) is designed to improve the classification performance. Computed tomography angiography (CTA) images of 146 patients diagnosed with supracardiac TAPVC in Shanghai Children's Medical Center and Guangdong Provincial People's Hospital were enrolled in this work. The comprehensive experiments demonstrate the effectiveness and generalization of our proposed method. The automatic PPVO prediction model shows the potential application in helping clinicians develop follow-up strategies, thereby improving the survival rate of TAPVC patients.

Prenatal diagnosis of total anomalous pulmonary venous connection using 2D and HDlive flow combined with spatiotemporal image correlation.

The objective of this study is to examine the application value of two-dimensional (2D) and high-definition live (HDlive) flow combined with spatiotemporal image correlation (STIC) in diagnosing fetal total anomalous pulmonary venous connection (TAPVC). Seventeen cases of fetal TAPVC were diagnosed using 2D and HDlive Flow combined with STIC. These cases were then retrospectively analyzed to examine the value of using 2D and HDlive Flow combined with STIC in the diagnosis of TAPVC. 2D and HDlive Flow combined with STIC detected 13 cases of supracardiac TAPVC (two isolated cases, seven cases with right atrial isomerism (RAI), four cases with other complex malformations), one case of isolated intra-cardiac TAPVC, and three cases of cardiac TAPVC (two isolated cases and one case with complex congenital heart anomaly). Small left atrium (LA), the absence of PVs drainage into the LA and the increased retroatrial distance between LA and the descending aorta (DAo) were significant signs that should raise the suspicion of fetal TAPVC. HDlive Flow combined with STIC can dynamically display the TAPVC which may assit the prenatal diagnosis of TAPVC. 2D and HDlive Flow combined with STIC can assit the diagnosis of fetal TAPVC abnormalities and has important clinical value.

Window anastomosis technique for repair of supracardiac total anomalous pulmonary venous connection in infants.

Outcomes of operations for total anomalous pulmonary venous connection (TAPVC) have improved. However, postoperative pulmonary venous obstruction remains the most significant complication with high morbidity and mortality. We introduce a window anastomosis technique for repair of supracardiac TAPVC in infants. The mainstay of the surgical technique is to resect the anterior wall of the pulmonary vein confluence and part of the posterior wall of the left atrium to form a large and undistorted "window to window" anastomosis.

Fetal Doppler Echocardiographic Assessment Predicts Severe Postnatal Obstruction in Total Anomalous Pulmonary Venous Connection

Obstructed total anomalous pulmonary venous connection (TAPVC) is a form of critical congenital heart disease that usually requires urgent postnatal intervention. Knowing which patients have severe obstruction can aid delivery planning. The authors previously developed a novel quantitative metric of pulmonary venous flow, the pulmonary venous variability index (PVVI). The aim of this study was to test the hypothesis that fetal PVVI and vertical vein Doppler velocities are associated with severe pulmonary vein obstruction postnatally. A retrospective cohort study of neonates with prenatally diagnosed TAPVC was performed. Patients who underwent fetal echocardiography at the Children's Hospital of Philadelphia with Doppler interrogation of the vertical vein were included for analysis. Twenty-nine patients met criteria (21 with heterotaxy, 18 with supracardiac TAPVC). The latest gestation fetal echocardiogram was used. Severe pulmonary vein obstruction was defined as preoperative death or urgent surgery or catheter-based intervention (first day of life). Measurements of PVVI, defined as (maximum velocity-minimum velocity)/mean velocity, were made offline. Wilcoxon rank sum models were used to assess the associations of severe obstruction and PVVI and maximum, mean, and minimum velocities. The mean gestational age at the latest fetal echocardiographic examination was 35weeks (range, 30-39weeks). Twelve of the 29 patients (41%) met criteria for severe pulmonary vein obstruction. Lower PVVI was associated with greater risk for severe pulmonary venous obstruction (P=.008). The maximum, mean, and minimum velocities in the vertical vein were all significantly associated with severe pulmonary venous obstruction (P=.03, P=.03, and P=.007, respectively). Qualitative assessment of obstruction was not significantly associated with the outcome. Interobserver reliability for all vertical vein Doppler metrics was high (intraclass correlation coefficient &gt; 0.9). Fetal PVVI and maximum, mean, and minimum velocities are associated with severe postnatal pulmonary vein obstruction in TAPVC. Accurate prediction of obstructed TAPVC could allow safer delivery planning. Further research with larger sample sizes is needed to identify the ideal cutoff values for these Doppler measures.

Total anomalous pulmonary venous connection: 16 years of surgical results in a single center.

The aim of the study was to analyze the surgical outcome of patients with total anomalous pulmonary venous connection (TAPVC) who underwent cardiac surgery. A retrospective study was carried out. Patients with diagnosis of TAPVC undergoing cardiac surgery at the National Institute of Cardiology Ignacio Chávez, from January 1, 2003 and June 30, 2019 were included. Descriptive statistics were calculated, as well as a bivariate analysis of the variables associated with mortality. A logistic regression model was included to determine risk factors associated with the main outcome and survival was analyzed using the Kaplan-Meier method. A total of 5314 patients diagnosed with congenital heart disease (CHD) underwent surgery, 414 (7.8%) were patients with TAPVC, with an average age of 17.1 ± 34.6 months, 58.2% were male. It was frequent in infants (61.6%) and preschool (19.6%). Predominant type was supracardiac TAPVC (47.4%). Pulmonary venous obstruction (PVO) occurred in 32.1%. Risk factors associated with mortality were infracardiac TAPVC (odds ratio [OR]: 3.26; 95% confidence interval [CI]: 1.17-9.03; p = .02), PVO (OR: 2.56; 95% CI: 1.05-6.22; p = .03) and postoperative mechanical ventilation (OR: 1.005; 95% CI: 1.002-1.008; p = .01). Overall survival was 87.2%, with better outcomes in adolescents (100%), children (94.1%), mixed TAPVC (96%), and cardiac TAPVC (91.9%;p < .001). The survival of our institution after surgical correction of TAPVC is similar to that of other referral centers, where patients with infracardiac TAPVC and newborns worse outcomes. All patients must undergo a rigorous evaluation to determine an adequate repair strategy.

Rare association of obstructed supracardiac total anomalous pulmonary venous connection with coarctation of aorta: case report

Coexistence of TAPVC (total anomalous pulmonary venous connection) and coarctation of aorta rarely occurs. Newborn weighing 2.4 kg admitted in Sangli civil hospital NICU with respiratory distress with cyanosis. Chest X-ray of baby was suggestive of ground glass appearance of bilateral lung fields and cardiomegaly. SpO2 of baby was fluctuating between 85 to 90% on nasal CPAP. 2D echo showed baby was having obstructed supracardiac TAPVC with severe discrete post-ductal coarctation of aorta. There was stretched PFO shunting R-L and small PDA shunting R-L. Baby was planned for emergency surgery but couldn’t make it to operation theatre and succumbed within 30 hours of life. Till now there are very few case reports of TAPVC with coarctation but no case of obstructed supracardiac TAPVC with coarctation has been reported.

Mid-Term Results and Risk Factors For 10 Years of Functional Single Ventricle Associated With Total Anomalous Pulmonary Venous Connection

There are few surgical treatment results in elderly patients with functional single ventricle (FSV) and total anomalous pulmonary venous connection (TAPVC). We retrospectively analyzed 10 years of mid-term surgical treatment results and risk factors of these age-specific people. Between March 2008 and December 2018, 43 consecutive patients with FSV and TAPVC received initial surgical palliation in our center. There were 20 cases of supracardiac TAPVC, 21 of cardiac type, and two cases of mixed type. Initial surgical palliation procedures involved pulmonary artery banding (PAB) for patients, modified Blalock-Taussing shunt (mBTs) for five patients, and bidirectional Glenn (BDG) for 34 patients. TAPVC repair was performed in 12 patients during BDG. The 1-year and 5-year overall survival rates were 69.7% and 62.8%, respectively. In TAPVC repair group and non-TAPVC repair group, the 1-year overall survival rates after initial surgical palliation were 41.7 and 80.5%, respectively, and the 3-year ones were 25% and 77%, respectively. There were significant differences in the type of TAPVC (P < 0.001), preoperative pulmonary venous obstruction (P = 0.001), and overall mortality (P = 0.001) between these two groups. Cox univariate and multivariable analysis indicated concomitant TAPVC repair was the only risk factor for mortality. The mid-term results of surgical treatment of FSV and TAPVC, especially for patients who underwent concomitant TAPVC repair, remain poor. TAPVC repair may be a priority over single-ventricular palliative surgery for patients with FSV and TAPVC.

Short-term results of sutureless closure for primary total anomalous pulmonary venous connection: seventeen cases

Total anomalous pulmonary venous connection (TAPVC) is a rare condition. Mortality of TAPVC has improved dramatically in the present. Sutureless closure had initially used for congenital pulmonary venous stenosis (PVS) or post-repair PVS. Recently, it had been adopted for primary TAPVC to prevent postoperative pulmonary venous obstruction, a leading cause of death. This technique had gradually applied at our institute for primary TAPVC since 2018. Prospective case series was conducted from mid-2019. Including criteria consists of patients with primary TAPVC. These are all supracardiac, infracardiac, mixed type and intracardiac TAPVC with pulmonary venous stenosis. Excluding measures are TAPVC with single ventricle, isomerism or heterotaxy. End-point outcomes were mortality and short-term PVS. Seventeen TAPVC cases were enrolled in this study from 06/2019-12/2020 at Children’s Hospital 1. Male/female ratio was 12/5. Median age at admission was 44 (3-1010) days old. The median operative weight was 3.9 kg (2.4-11). Of those, fourteen (82.4%) cases were supracardiac TAPVC and 3 (17.6%) cases were infracardiac TAPVC. Nine (52.9%) cases were emergency operations. Median aortic clamp time was 66 (32-138) mins. Median bypass time was 112 (86-212) mins. There were 9 (52.9%) cases with arrhythmias, 2 (11.8%) cases with chylothorax. Mean ventilation time was 3.6±0.5 days; mean CICU time was 6.5 (1-20), median LOS was 14.5 (8-39). In-hospital mortality was 5.9% (1). Mean follow-up time was 12.3±6.4 months (range 4-24), there was no PVS with median pulmonary venous score of 0 (range 0-1). Sutureless technique could be a safe option for primary TAPVC repair.

Utility of a modified vascular corrosion casting technique in the diagnosis of fetal total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiac malformation, and prenatal detection of TAPVC malformation remains a challenging. TAPVC can be easily missed or misdiagnosed in prenatal examinations. This study was aimed to use the modified vascular corrosion casting technique to prepare fetal cardiovascular casts with TAPVC and investigate the utility of cardiovascular casting for the demonstration of fetal TAPVC. The retrospective study enrolled twenty fetuses (22 to 29 + 4 gestational weeks) with TAPVC diagnosed by prenatal echocardiography and casting technique from May 2015 to May 2020. Pre- and postnatal medical records, including results obtained by prenatal ultrasound, postpartum computed tomography angiography, as well as anatomic and cardiovascular casting findings were carefully reviewed and analyzed. In twenty cases, 80% (16/20) had intra- or extracardiac malformations. The TAPVC types were supracardiac (n = 8), cardiac (n = 6), infracardiac (n = 4), and mixed (n = 2). The diagnosis of 1 case each of supracardiac and cardiac TAPVC was modified to partial anomalous pulmonary venous connection; additionally, 4 malformations were missed and 2 were misdiagnosed, including an anomalous left brachiocephalic vein in supracardiac TAPVC, abnormal inflow of the hepatic vein and a double inferior vena cava in infracardiac TAPVC; and bilateral ductus arteriosus in infracardiac TAPVC; a tetralogy of Fallot in cardiac TAPVC that was corrected to right ventricular double outlet; and an absence of ductus arteriosus that was misdiagnosed as slim ductus arteriosus. Comparing with ultrasound, casting technique has its own superiority in exhibiting TAPVC abnormalities, especially in certain types such as course, origin and absence abnormalities of ductus. Postpartum cardiovascular casts can accurately depict the branch structure of the heart’s larger vessels, and may be used as a clinical assessment and teaching method in complex cardiac malformations.

Non-obstructed supracardiac total anomalous pulmonary venous connection with giant superior vena cava aneurysm

BackgroundGiant superior vena cava (SVC) aneurysm in non-obstructed supracardiac total anomalous pulmonary venous connection (TAPVC) is even more rare than in obstructed one, and this combination has not been reported.Case presentationHere we reported a 29-year‐old young lady with non-obstructed TAPVC complicated with a giant SVC aneurysm. Routine TAPVC correction and tricuspid valve repair were done. Additionally, venoctomy was carried out to seek for its histological etiology and to avoid potential complications.ConclusionsIf confirmed by further and larger experiences, for patients with non-obstructed supracardiac TAPVC with giant SVC aneurysm, surgical treatment of SVC aneurysm could be more aggressive to prevent cardiovascular complications.

Early and Intermediate-Term Results for the Combined Superior Approach Correction of Supracardiac Total Anomalous Pulmonary Venous Connection in Neonatal Patients

There are different surgical approaches used for repairing a supracardiac total anomalous pulmonary venous connection (TAPVC), with different results. This retrospective study evaluated the outcomes of surgical repair for supracardiac TAPVC through the combined superior approach in neonatal patients. Medical records were retrospectively reviewed and 21 neonates who underwent supracardiac TAPVC repair with the combined superior approach between July 2014 and January 2020 were identified. There were 13 males and eight females. The patients' median age was 20.6±8.9 days (range, 3-27). The median weight was 3.1±0.39 kg (range, 2.5-3.7) The median aortic cross-clamp and cardiopulmonary bypass times were 49.3±19.5 minutes (range, 27-86) and 91.1±23.7 minutes (range, 57-146). They were two deaths during the intensive care unit stay. One (1) patient died 2 months after discharge, the other remaining patients had no pulmonary venous obstruction (PVO) at the 6-month and intermediate-term follow-ups. The combined superior approach is a useful method for repair of neonatal critical supracardiac TAPVC. This technique may be more helpful in preventing early postoperative anastomotic stenosis and contribute to an improved patient outcome.

Ivemark syndrome with obstructed supracardiac TAPVC- case report

Heterotaxy disorder is a disturbance in the usual left and right distribution of the thoracic and abdominal organs. Ivemark syndrome is one such heterotaxy disorder. It is a rare disorder which affects males more than females with majority of cases presenting in the neonatal period mainly due to complex congenital cardiac disease. Here is a case report of rare disorder of the neonate with Ivemark syndrome with obstructed supracardiac total anomalous pulmonary venous connection (TAPVC). Management of obstructed supracardiac TAPVC is complicated as PGE1 infusion is contraindicated and immediate surgical correction is advised.