Anomalous Pulmonary Venous Connection Research Articles

RISK OF CARDIAC ANOMALIES IN ABERRANT RIGHT SUBCLAVIAN ARTERY RELATIVE AORTIC ARCH ANOMALIES FOR PEDIATRICS: A CROSS-SECTIONAL STUDY

Abstract Introduction: An aberrant right subclavian artery with a normal-sided left aortic arch is the most common congenital anomaly of the aorta. This study aims to determine the risk of cardiac anomalies associated with aberrant right subclavian artery in relation to aortic arch anomalies among pediatric patients. Material and methods: This prospective cross-sectional study was performed between 2008 and 2011. Patients with either clinical or echocardiographic findings of congenital heart disease were referred for complementary evaluation with computed tomography angiography. Results: Finally, 203 patients were included in this study. The prevalence of an aberrant right subclavian artery was presented in 24 patients (11.8%). Moreover, the most common aortic arch anomaly was coarctation 40 (19.7%). The only double outlet right ventricle cardiac abnormality was associated with a higher risk of an aberrant right subclavian artery (relative risk, 1.192; 95% CI, 0.179 – 7.95) relative to other aortic arch anomalies. Discussion: In conclusion, this study showed that the incidence of aberrant right subclavian artery in an extensive series of Aortic Arch Anomalies had been performed (11.8%). Furthermore, pulmonary atresia, total anomalous pulmonary venous return, aortic coarctation, cervical arch, aortic interruption, tubular hypoplasia, and pulmonary sling were significantly higher in the aberrant right subclavian artery group in contrast with Other Aortic Arch Anomalies. However, just double outlet right ventricle was associated with a higher risk of aberrant right subclavian artery.

Surgical repair of neonatal total anomalous pulmonary venous connection: A single institutional experience with 241 cases

ObjectiveChallenges persist in surgery for neonatal total anomalous pulmonary venous connection (neoTAPVC), with the high mortality risk not mitigated over time. MethodsA prospectively collected single-center database containing all neonates with TAPVC undergoing biventricular repair in 2012 to 2020 was retrospectively reviewed. The primary outcome was death or postoperative pulmonary venous obstruction (PPVO). Based on the preoperative admission location in our hospital, patients were classified into those being admitted to cardiac intensive care unit versus neonatal intensive care unit or general pediatric intensive care unit. Access to dedicated presurgical care (DPC) was defined as patients who were preoperatively admitted to the cardiac intensive care unit. ResultsOverall, 241 patients with a median age at surgery of 14 days (interquartile range [IQR], 9-21 days) were included. Anomalous return was supracardiac in 38.6%, cardiac in 26.1%, infracardiac in 28.6%, and mixed in 6.6%. Patients receiving DPC had better survival (96.3% vs 84.3%; P = .0028) and lower incidence of PPVO (15.2% vs 28.6%; P = .011) compared with those without DPC. Patients in the DPC group were less likely to undergo operation within 24 hours on presentation (27.1% vs 40.3%; P = .041), had improved lactate clearance (1.5 [IQR, 1.0-2.2] vs 2.8 [IQR, 1.8-4.1]; P < .001), and had lower incidence of postoperative pulmonary hypertension crisis (2.8% vs 18.7%; P < .001) compared with those in no-DPC group. After matching, no difference in PPVO could be observed in patients undergoing conventional versus sutureless repair (22.6% vs 12.9%; P = .29). ConclusionsAccess to DPC potentially improves outcomes in the neoTAPVC setting; freedom from PPVO were similar using conventional versus sutureless repair.

Cor Triatriatum Sinister Associated with Total Anomalous Pulmonary Venous Connection.

Cor triatriatum sinister is rare congenital heart disease. It is defined as the presence of an abnormal septum dividing the left atrium into two chambers, and in some cases may be associated with a total abnormal pulmonary venous connection. Prenatal diagnosis of cor triatriatum sinister may be possible with fetal echocardiography and the diagnosis can be made earlier than postnatal imaging techniques. In the fetal echocardiography performed in our patient, cor triatriatum sinister was present and the main pathology was the flow of right and left pulmonary vein return from the accessory atrium to the right atrium.

Fetal Pulmonary Venous Return: From Basic Research to the Clinical Value of Doppler Assessment.

The fetal pulmonary circulation represents less than 25% of the fetal cardiac output. In comparison with the pulmonary arteries, studies on pulmonary veins are few and limited, and many questions remain to be answered. The literature reports that pulmonary veins play an important role in regulating vascular flow, forming an active segment of the pulmonary circulation. The development of more sophisticated ultrasonography technology has allowed the investigation of the extraparenchymal pulmonary veins and their waveform. The recognition of the pulmonary vein anatomy in echocardiography is important for the diagnosis of anomalous pulmonary venous connections, with a significant impact on prognosis. On the other hand, the identification of the normal pulmonary vein waveform seems to be a reliable way to study left heart function, with potential applicability in fetal and maternal pathology. Thus, the goal of this narrative review was to provide a clinically oriented perspective of the available literature on this topic.

Sutureless technique combined with vertical vein incision and pulmonary veins unroofed for correction of infracardiac total anomalous pulmonary venous connection.

We report a surgical method (sutureless technique), combined with vertical vein incision and pulmonary veins unroofed (semisutureless technique), to correct infracardiac total anomalous pulmonary venous connection (TAPVC). The clinical characteristics of 21 patients, who were diagnosed with infracardiac TAPVS between February 2017 and March 2022, were retrospectively analyzed. These patients were divided into three groups according to different surgical methods: conventional surgery group, sutureless technique group, and semisutureless technique group. The conventional surgery group enrolled five patients with a median age of 16 days (interquartile range, 9-27 days) and a median weight of 3.25 kg (interquartile range, 3.1-3.42 kg). In this group, no preoperative pulmonary vein obstruction (PVO), preoperative ventilator support, or emergency surgery were reported. The sutureless technique group enrolled seven patients with a median age of 12 days (interquartile range, 5-16 days) and a median weight of 3.04 kg (interquartile range, 2.76-3.20 kg). In this group, two patients with preoperative PVO, four patients with preoperative ventilator support, and seven patients requiring emergency operation were found. The semisutureless technique group enrolled nine patients with a median age of 14 days (interquartile range, 7-24 days) and a median weight of 3.22 kg (interquartile range, 3.15-3.50 kg). In this group, four patients with preoperative PVO, two patients with preoperative ventilator support, and seven patients requiring emergency operation were noted. In the conventional surgery group, two patients with postoperative supraventricular tachycardia, one patient with postoperative low cardiac output syndrome, one patient with PVO, and no case of postoperative death were reported. In the sutureless technique group, two patients with postoperative low cardiac output syndrome, one patient with postoperative supraventricular tachycardia, one patient with postoperative PVO, and no postoperative deaths were determined. In the semisutureless technique group, three patients had low cardiac output syndrome, two patients had supraventricular tachycardia after the operation, and one patient, who had been admitted to the hospital after cardiopulmonary resuscitation in the emergency room, died early after the operation. No case of death or PVO was noted after the operation. The semisutureless technique has positive effects. This surgery method can enlarge the anastomotic stoma, increase the volume of the left atrium, reduce the tension of the anastomotic stoma, fix the pulmonary vein to avoid distortion, and prevent postoperative hemorrhage.

Outcomes and Risk Factors of Interventions for Pediatric Post-operative Pulmonary Vein Stenosis.

Pulmonary vein stenosis (PVS) in children is a challenging condition with poor outcomes. Post-operative stenosis can occur after repair of anomalous pulmonary venous return (APVR) or stenosis within native veins. There is limited data on the outcomes of post-operative PVS. Our objective was to review our experience and assess surgical and transcatheter outcomes. Single-center retrospective study was performed including patients < 18years who developed restenosis after baseline pulmonary vein surgery that required additional intervention(s) from 1/2005 to 1/2020. Non-invasive imaging, catheterization and surgical data were reviewed.We identified 46 patients with post-operative PVS with 11 (23.9%) patient deaths. Median age at index procedure was 7.2months (range 1month-10years), and median follow-up was 10.8months (range 1day-13years). Index procedure was surgical in 36 (78.3%) and transcatheter in 10 (21.7%). Twenty-three (50%) patients developed vein atresia. Mortality was not associated with number of affected veins, vein atresia, or procedure type. Single ventricle physiology, complex congenital heart disease (CCHD), and genetic disorders were associated with mortality. Survival rate was higher in APVR patients (p = 0.03). Patients with three or more interventions had a higher survival rate compared to patients with 1-2 interventions (p = 0.02). Male gender, necrotizing enterocolitis, and diffuse hypoplasia were associated with vein atresia. In post-operative PVS, mortality is associated with CCHD, single ventricle physiology, and genetic disorders. Vein atresia is associated with male gender, necrotizing enterocolitis, and diffuse hypoplasia. Multiple repeated interventions may offer a patient survival benefit; however, larger prospective studies are necessary to elucidate this relationship further.

Treatment considerations in total anomalous pulmonary venous connection

Background Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic abnormality that accounts for about 1%–3% of congenital heart disease malformations. TAPVC is a condition in which there is no direct connection between all four pulmonary veins and the left atrium but makes abnormal connections to the right atrium or systemic venous system. TAPVC caused a high mortality rate of 80% in the first year of life, and 50% of them die within 3 months after birth without intervention. Objectives The aim of this review is to elucidate the various treatment considerations of TAPVC. Methods A literature search was conducted on PubMed, ScienceDirect and Google Scholar using various combinations of keywords related to treatment of TAPVC. The citations from all selected articles were reviewed for additional studies. Results and Conclusion TAPVC intervention, including medical and surgical, is tailored to each type of TAPVC. Catheter-based interventions are frequently used to temporize neonates and provide time to optimize patients medically prior to definite repair. Corrective surgery is required for all patients with this condition. Several latest catheter-based or surgical intervention technique modifications have also been reported.

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.

Prevalence of Partial Anomalous Pulmonary Venous Return (PAPVR) Amongst Adults Undergoing Multimodality Cardiac Imaging and Predictive Features on Transthoracic Echocardiography

Prevalence of Partial Anomalous Pulmonary Venous Return (PAPVR) Amongst Adults Undergoing Multimodality Cardiac Imaging and Predictive Features on Transthoracic Echocardiography

Long-Term Outcomes of Unrepaired Isolated Partial Anomalous Pulmonary Venous Connection With an Intact Atrial Septum

The natural history of an unrepaired isolated partial anomalous pulmonary venous connection(s) (PAPVC) and the absence of other congenital anomalies remains unclear. This study aimed to expand the understanding of the clinical outcomes in this population. Isolated PAPVC with an intact atrial septum is a relatively uncommon condition. There is the perception that patients with isolated PAPVC are usually asymptomatic, that the lesion generally has a limited hemodynamic impact, and that surgical repair is rarely justified. For this retrospective study, we reviewed our institutional database to identify patients with either 1 or 2 anomalous pulmonary veins that drain a portion of but not the complete ipsilateral lung. Patients with previous surgical cardiac repair, coexistence of other congenital cardiac anomalies that would result in either pretricuspid or post-tricuspid loading of the right ventricle (RV), or scimitar syndrome were excluded. We reviewed their clinical course over the follow-up period. We identified 53 patients; 41 with a single and 12 with 2 anomalous PAPVC. A total of 30 patients (57%) were men, with a mean age at the latest clinic visit of 47 ± 19years (18 to 84 years). Turner syndrome (6 of 53, 11.3%), bicuspid aortic valve (6 of 53, 11.3%), and coarctation of the aorta (5 of 53, 9.4%) were commonly associated anomalies. A single anomalous left upper lobe vein was the most commonly identified variation. More than half of the patients were asymptomatic. Cardiopulmonary exercise test demonstrated a maximal oxygen consumption of 73 ± 20% expected (36 to 120). Transthoracic echocardiography demonstrated a mean RV basal diameter of 4.4 ± 0.8cm, RV systolic pressure of 38 ± 13 (16 to 84) mm Hg. A total of 8 patients (14.8%) had ≥moderate tricuspid regurgitation. Cardiac magnetic resonance in 42 patients demonstrated a mean RV end-diastolic volume index of 122 ±3 0ml/m2 (66 to 188ml/m2), of which in 8 (14.8%), it was >150ml/m2. Magnetic resonance imaging-based Qp:Qs was 1.6 ± 0.3. A total of 5 patients (9.3%) had established pulmonary hypertension (mean pulmonary artery pressure ≥25mm Hg). In conclusion, isolated single or dual anomalous pulmonary venous connection is not necessarily a benign congenital anomaly because a proportion of patients develop pulmonary hypertension and/or RV dilation. Regular follow-up and on-going patient surveillance with cardiac imaging is advised.

Multisystem Inflammatory Syndrome in Children (MIS-C) in Pediatric Patient Post Cardiac Surgery: A Case Report

Introduction: Multisystem inflammatory syndrome in children (MIS-C) is a relatively new disease associated with recent infection of severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2). Case Report: We reported a 1-year-3-month-old girl with congenital heart disease since 1-month-old. Peripheral oxygen saturation was 89%, and systolic murmur was found from physical examination. Patient was assessed with ASA III physical status and planned to undergo re-route partial anomalous pulmonary venous return (PAPVR) and surgical atrial septal defect (ASD) closure under general anesthesia and cardiopulmonary bypass. During surgery, hemodynamic fluctuations were controlled. Post-operatively, she was transferred to the ICU, given sedation and analgesic. On night time, patient started to have fever with temperature 37.9⁰C. On the second day, patient had bradypneu, bradycardia, re-intubated and was given CPR. On the third day, patient’s SARS-CoV-2 Antibody was found to be reactive and diagnosis of MIS-C was made. On the fourth day, patient’s condition begin to deteriorate on even though she was given of Dobutamine, Epinephrine, Norepinephrine and Vasopressin. Four hours later, patient did not respond after CPR and declared deceased. Discussion: Early recognition of MIS-C is challenging because the initial symptoms appear in previously healthy patient, with several symptoms such as fever, abdominal discomfort, mucocutaneous involvement, neurologic symptoms, cardiorespiratory symptoms (shock, myocarditis, dyspnea) with evidence of a marked inflammatory state. Immunomodulators are the mainstay of the treatment, with additional therapy such as steroids and anticoagulant. This severe disease’s mortality rate could be as high as 15% and more research must be done to better understand this disease. Conclusion: MIS-C is a novel pediatric syndrome characterized by fever, multi-organ involvement and marked inflammatory state. Further study is required to provide consistent diagnosis criteria, therapy regimens, and long-term prognosis since this is a relatively new pediatric syndrome. Keywords: Multisystem inflammatory syndrome in children (MIS-C), SARS-CoV-2, pediatric

Characterization of ultrasound and postnatal pathology in fetuses with heterotaxy syndrome.

To explore the diagnostic clues and abnormality spectrum of heterotaxy syndrome by prenatal ultrasonography and postnatal verification. The prenatal ultrasonic data of 88 heterotaxy syndrome fetuses were analyzed retrospectively as left isomerism (LI) and right isomerism (RI). Prenatal ultrasound compared with the anatomical casting of the fetal body after labor induction, and the confirmatory postnatal diagnosis after delivery. Fetal LI showed typical malformations of gastric vesicles on different sides from the heart, absence of hepatic segment of the inferior vena cava (IVC), abdominal aorta (AO) parallel with the azygos vein (AV), bilateral left bronchus, bilateral left atrial appendages, and polysplenia; intracardiac malformations of AV septal defects (AVSD), single atrium (SA), left ventricular outflow tract obstruction (LVOTO), and double-outlet right ventricle (DORV); and cardiac conduction abnormalities of sinus bradycardia and AV blockage. Fetal RI reported typical malformations of gastric vesicles on different sides from the heart, juxtaposition of the IVC with AO, anomalous pulmonary venous connection (APVC), asplenia, and bilateral right atrial appendages; intracardiac malformations of AVSD, SA, single ventricle, pulmonary atresia and stenosis, and DORV. The postnatal verification revealed 3 malformations misdiagnoses and 4 malformations missed diagnoses in LI fetuses and 10 misdiagnoses and 8 missed diagnoses in RI fetuses. The proposed five-step prenatal ultrasonography has an important diagnostic value for the identification and classification of heterotaxy syndrome. The different sides of gastric vesicles and cardiac apex are important diagnostic clues for heterotaxy syndrome, featuring disconnected or hypoplastic IVC, typical complex cardiac malformation, and atrioventricular block in fetal LI, and shown APVC, juxtaposition of IVC and AO, and intracardiac malformations such as AVSD, DORV, and LVOTO in fetal RI.

A missense variant at the RAC1-PAK1 binding site of RAC1 inactivates downstream signaling in VACTERL association

RAC1 at 7p22.1 encodes a RAC family small GTPase that regulates actin cytoskeleton organization and intracellular signaling pathways. Pathogenic RAC1 variants result in developmental delay and multiple anomalies. Here, exome sequencing identified a rare de novo RAC1 variant [NM_018890.4:c.118T > C p.(Tyr40His)] in a male patient. Fetal ultrasonography indicated the patient to have multiple anomalies, including persistent left superior vena cava, total anomalous pulmonary venous return, esophageal atresia, scoliosis, and right-hand polydactyly. After birth, craniofacial dysmorphism and esophagobronchial fistula were confirmed and VACTERL association was suspected. One day after birth, the patient died of respiratory failure caused by tracheal aplasia type III. The molecular mechanisms of pathogenic RAC1 variants remain largely unclear; therefore, we biochemically examined the pathophysiological significance of RAC1-p.Tyr40His by focusing on the best characterized downstream effector of RAC1, PAK1, which activates Hedgehog signaling. RAC1-p.Tyr40His interacted minimally with PAK1, and did not enable PAK1 activation. Variants in the RAC1 Switch II region consistently activate downstream signals, whereas the p.Tyr40His variant at the RAC1-PAK1 binding site and adjacent to the Switch I region may deactivate the signals. It is important to accumulate data from individuals with different RAC1 variants to gain a full understanding of their varied clinical presentations.

#4339 CARDIORENAL SYNDROME TYPE 1 IN CHILDREN AFTER SURGICAL TREATMENT OF CONGENITAL HEART DISEASE USING CARDIOPULMONARY BYPASS

Abstract Background and Aims According to recent data, acute kidney injury (AKI) after pediatric cardiac surgery is a significant postoperative complication that occurs in 35-64% of children (1,2) and is associated with increased hospital mortality and unfavorable short-term outcomes. The problem of acute kidney injury in children after cardiac surgery remains relevant, despite the development of modern technologies and methods. Complications after pediatric cardiac surgery have serious negative consequences, prolonging the days of stay in the pediatric intensive care unit (PICU) and increasing mortality (3). The aim of the study is to analyze the frequency of AKI in children after open-heart surgery for congenital heart defects (CHD) using cardiopulmonary bypass (CBP). Method We conducted a retrospective analysis of 402 medical records of patients hospitalized in the ICU of JSC NRCSC Astana, Kazakhstan, from January 2018 to December 2022. Inclusion criteria: Children under 18 with CHD who underwent open-heart surgery with the following diagnosis - transposition of the great vessels, total anomalous pulmonary venous connection, interrupted aortic arch, coarctation of the aorta, ventricular septal defect. Exclusion criteria: The presence of AKI and terminal chronic kidney failure. Results The analysis included the total number of patients - 402 patients. The frequency of AKI was 15.6% (63 out of 402). Among the patients who developed AKI: boys - 45 (71.4%), girls - 18 (28.5%). The age distribution is as follows: children under 1 month among AKI - 50 (79.3%), under 6 months among AKI – 13 (20.6%), under 1 year among AKI – 5 (7.9%). According to the statistical analysis results, the complication depends on the type of operation, as there is a statistical significance of the differences in the groups (p = 0.046). However, the type of operation (p = 0.35) does not matter for the outcome. Also, the children's weight (p = 0.082) has no statistical significance on the number of complications and the outcome. The sex of children has a statistical significance for complications; in boys, it is significantly higher (p = 0.05). In addition, the lethal outcome in male children is higher than in girls (p = 0.000). The total number of patients with peritoneal dialysis is 63 (15.6%). The total mortality was 80 patients (19.9%). Mortality in the intensive care unit in patients with AKI after open-heart surgery for CHD was 43 (68.2%). Conclusion According to our data, the frequency of AKI developed in 15.6% of patients after surgical treatment of CHD using CBP. The overall mortality rate is 19.9%, with boys having a higher risk. Thus, the frequency of AKI is influenced by many factors before, during and after surgery. Therefore, developing preventive measures is a priority for improving the cardiac surgery results in children.

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.

Intracardiac thrombosis after congenital heart disease surgeries in neonates: a report of two cases

BackgroundIntracardiac thrombosis (ICT) is a rare complication after the cardiopulmonary surgery for interrupted aortic arch (IAA) or total anomalous pulmonary venous connection (TAPVC) without previous records. There are still no general guidelines regarding as the mechanism or management of postoperative ICT in neonates and younger infants.Case presentationWe reported the conservative and surgical therapies in two neonates with intra-ventricular and intra-atrial thrombosis after the anatomical repair for IAA and TAPVC, respectively. There were no risk factors for ICT in both patients, except for the use of blood product and prothrombin complex concentrate. The surgery was indicated after TAPVC correction due to the worsening respiratory status and rapidly decreased mixed venous saturation. Anticoagulation combined with antiplatelet therapies was adopted in another patient. These two were both finally recovered, and three-month, six-month, and one-year follow-up echocardiography revealed no abnormality.ConclusionsICT is uncommon in pediatric population after the surgery for congenital heart disease. Single ventricle palliation, heart transplantation, longer central line use, post-extracorporeal membrane oxygenation, and massive blood product use are major risk factors for postcardiotomy thrombosis. The causes of postoperative ICT are multifactorial, and the immaturity of thrombolytic and fibrinolytic system in neonates may serve as a prothrombotic factor. However, no consensus reached regarding as the therapies for postoperative ICT, and the large-scale prospective cohort study or randomized clinical trial is needed.

A Rare Combination of Transposition of Great Arteries and Total Anomalous Pulmonary Venous Connection

The association of total anomalous pulmonary venous connection (TAPVC) with transposition of great arteries (TGA) is a very rare combination of 2 congenital cyanotic heart diseases. TAPVC, in the case of TGA with the intact interventricular septum (IVS), is a cause of early regression of the left ventricle (LV). Very few cases of this rare combination (TAPVC and TGA with or without VSD) have been reported in the world of literature. We found 10 case reports in the literature. The minimum age was 6 days and the maximum age of 16 years. 5 of them, which were operated before 1990, underwent atrial switch with TAPVC repair. Other 4 of them, operated after 1995, underwent arterial switch with TAPVC repair. In this case report we are reporting 2 such rare cases. In both cases, large VSD prevented LV regression.

Fast-Track Virtual Reality to Facilitate 3D Reconstruction in Congenital Heart Disease.

A limitation of the clinical use of 3D reconstruction and virtual reality (VR) systems is the relatively high cost and experience required to use hardware and software to effectively explore medical images. We have tried to simplify the process and validate a new tool developed for this purpose with a novel software. Five patients with right partial anomalous pulmonary venous return with adequate preoperative imaging acquired by magnetic resonance were enrolled. Five volunteers with no previous experience in the field of 3D reconstruction were instructed to use the software after a short video tutorial. Users were then asked to create a 3D model of each patient's heart with DIVA and their results were compared quantitatively and qualitatively with a benchmark reconstruction performed by an experienced user. All our participants recreated 3D models in a relatively short time, maintaining a good overall quality (average quality score ≥ 3 on a scale of 1-5). The overall trend of all the parameters analysed showed a statistical improvement between Case 1 and Case 5, as users become more and more experienced. DIVA is a very simple software that allows accurate 3D reconstruction in a relatively short time ("fast-track" VR). In this study, we demonstrated the potential use of DIVA by inexperienced users, with a significant improvement in quality and time after a few cases were performed. Further studies are needed to confirm the potential application of this technology on a larger scale.

ISUOG Practice Guidelines (updated): fetal cardiac screening.

ISUOG Practice Guidelines (updated): fetal cardiac screening.

The 100 most cited papers on total anomalous pulmonary venous connection: a bibliometric analysis

BackgroundThe number of citations a paper receives reflects its impact on the scientific community. We aimed to identify and explore the characteristics of the most cited papers on total anomalous pulmonary venous connection (TAPVC).MethodsWeb of Science Core Collection Expanded Science Citation Index (1900 to present) was searched and papers on TAPVC were reviewed. Articles were ranked by the number of citations and the 100 most cited papers were analyzed.ResultsThe 100 most cited papers were published between 1952 and 2018 with a mean number of citations of 52 (range 26 to 148). The 1990s was the most productive decade. All articles except one were written in English. The 100 most cited articles were published in 24 journals, led by Journal of Thoracic and Cardiovascular Surgery (21 articles), followed by Annals of Thoracic Surgery (20 articles), and Circulation (16 articles). The United States of America contributed most of the 100 most cited papers (60 articles). Hospital for Sick Children, Toronto led the list of citation classics with six papers. Christopher A. Caldarone, John W. Kirklin, and P. E. F. Daubeney were the most productive authors with 3 articles each. More than half of the papers were cohort studies (51 articles). Surgery, radiology and etiology were the main topics. Thirty-one articles were funded by public foundations, and none received support from commercial companies.ConclusionsThe bibliometric analysis gives a historical perspective on scientific progress in the field of TAPVC and lays the foundation for future research.