Prevalence, management and efficacy of treatment in portal vein obstruction after paediatric liver transplantation: protocol of the retrospective international multicentre PORTAL registry.

INTRODUCTION: Portal vein obstruction (PVO) consists of anastomotic stenosis and thrombosis, which occurs due to a progression of the former. The aim of this large-scale international study is to assess the prevalence, current management practices and efficacy of treatment in patients with PVO. METHODS AND ANALYSIS: The Portal vein Obstruction Revascularisation Therapy After Liver transplantation registry will facilitate an international, retrospective, multicentre, observational study, with 25 centres around the world already actively involved. Paediatric patients (aged <18 years) with a diagnosed PVO between 1 January 2001 and 1 January 2021 after liver transplantation will be eligible for inclusion. The primary endpoints are the prevalence of PVO, primary and secondary patency after PVO intervention and current management practices. Secondary endpoints are patient and graft survival, severe complications of PVO and technical success of revascularisation techniques. ETHICS AND DISSEMINATION: Medical Ethics Review Board of the University Medical Center Groningen has approved the study (METc 2021/072). The results of this study will be disseminated via peer-reviewed publications and scientific presentations at national and international conferences. TRIAL REGISTRATION NUMBER: Netherlands Trial Register (NL9261).

Sonography for assessment of thoracic duct anatomy and physiology before and after meals.

The interest in clinical anatomy of the thoracic duct (TD) has recently grown, owing to discoveries linking its morphology to pathologies such as heart failure or liver cirrhosis. In the light of this knowledge, a cost-efficient and reliable in-vivo imaging method of TD should be devised. Ultrasonography satisfies these criteria and hence is a promising tool for assessment of TD's anatomy and function. Thirty-one healthy volunteers attended the examination after 6 h of fasting and 2 h without drink. Ultrasound of the left supraclavicular fossa was performed in search of TD's orifice into the venous angle. In each case, the largest diameter, number of orifices, presence of valves, tributaries, and motility of the TD were examined. We performed examinations in three sessions: after fasting, after standardized meal and 1 h after the meal. The statistical significance has varied among the three sessions. The strongest connection was shown in the third examination. The TD was visualized in 31 cases, 35 orifices were found, most of which drained into the venous angle. Multiple orifices were seen in four cases and valves in 15 cases. Tributaries were present in 17 cases. Mean widest and orifice diameter measured 3.23 and 2.0 mm, respectively. Spontaneous peristaltic-like movements of the TD were observed in 25 cases. We demonstrated that ultrasound is useful for assessment of TD's anatomy, allowing to visualize and quantify its key features. Moreover, our study is presumably the first to capture and describe TD's motility in vivo.

Implantation of atrial flow regulator devices in patients with congenital heart disease and children with severe pulmonary hypertension or cardiomyopathy-an international multicenter case series.

Background: The Occlutech Atrial Flow Regulator (AFR) is a self-expandable double-disc nitinol device with a central fenestration. Its use has been approved in the adult population with heart failure and described for pulmonary hypertension (PH). Only case reports and small series have been published about its use in the paediatric population and for congenital heart disease (CHD). Objectives: The authors sought to investigate the feasibility, safety, and short-term follow-up of AFR implantation in patients with CHD or children with PH or cardiomyopathy. Methods: This is a multicenter retrospective study involving 10 centers worldwide. Patients of any age with CHD or patients aged < 18 years with PH or cardiomyopathy needing AFR implantation were included. Results: A total of 40 patients underwent AFR implantation. The median age of the population at the time of the procedure was 58.5 months (IQR: 31.5-142.5) and the median weight was 17 kg (IQR: 10-46). A total of 26 (65.0%) patients had CHD, nine (22.5%) children, a cardiomyopathy, and five (12.5%), a structurally normal heart. The implantation success rate was 100%. There were two early and one late device thrombosis. Two patients (5.0%) with dilated cardiomyopathy on extracorporeal membrane oxygenator (ECMO) died during the hospital stay. At a median follow-up of 330 days (IQR: 125-593), 37 (92.5%) patients were alive. At follow-up, 20 patients improved their New York Heart Association (NYHA) class, 12 patients did not change their NYHA class, and one patient with idiopathic PH worsened. Conclusions: AFR implantation in patients with CHD and children with severe PH or cardiomyopathy is promising and seems to have beneficial effects at short-term follow-up.

Tetralogy of Fallot in the fetus - from diagnosis to delivery. 18-year experience of a tertiary Fetal Cardiology Center.

BACKGROUND: Tetralogy of Fallot (TOF) is a common congenital heart disease but very heterogeneous in terms of detailed cardiac anatomy, associated malformations, and genetic anomalies, especially when assessed prenatally. AIMS: We aimed to analyze the clinical spectrum of TOF in the prenatal period, including detailed cardiac morphology, coexisting anomalies, and their impact on short-term neonatal outcome. We also assessed changing trends in the prenatal diagnostic workup of TOF. METHODS: A retrospective cohort study including fetuses diagnosed with TOF between 2002 and 2019 was conducted in a tertiary Fetal Cardiology Center. Medical records and echocardiographic examinations were reviewed to collect demographic, sonographic, and genetic data. RESULTS: Among 326 TOF fetuses, 237 (73%) had pulmonary stenosis (TOF-PS), 72 (22%) pulmonary atresia (TOF-PA), and 17 (5%) absent pulmonary valve (TOF-APV). The yearly number of diagnoses increased during the study period, with decreasing fetal age at the time of diagnosis. Extracardiac malformations were found in 172 (53%) fetuses, cardiovascular malformations in 159 (49%), and genetic anomalies in 99 (39% of the tested group). Hypoplastic thymus, right aortic arch, and polyhydramnios were sonographic markers of microdeletion 22q11. Left-to-right ductal flow was predictive of postnatal ductal dependency. The perinatal outcome was dependent on the presence of associated anomalies and disease subtype, with TOF-APV having the worst prognosis. CONCLUSIONS: Extracardiac and genetic anomalies are common in fetuses with TOF, and, together with disease subtype and ductal flow assessment, they impact the perinatal management and outcomes. Genetic testing with array comparative genomic hybridization should be offered in all cases.

Virtual simulations in planning intravascular treatment of aortic coarctation - a retrospective analysis.

Introduction: A number of studies on using both three-dimensional printing and virtual models in assessment of aortic coarctation have been published, yet none of them uses virtual modelling as a planning tool in a blind retrospective analysis. Aim: Assessment of virtual modelling and virtual reality in planning interventional treatment of aortic coarctation. Material and methods: The study involved computed tomography scans of 20 patients performed prior to interventional treatment of aortic coarctation, which were used to create a virtual three-dimensional model of the aorta in Materialise Mimics. A group of potential stents was modelled in Materialise 3-Matic and complete simulations were assessed in Mimics Viewer using a virtual reality headset in order to choose an optimal stent, which was later compared with the implanted one. Results: In 5 cases identical or very similar stents were proposed, in 12 cases simulations had slight, potentially avoidable misestimations either in stent length or diameter, and in 3 cases differences were more considerable. Overall, in 14 cases the location of the stent was concordant between the simulation and reality and in the remaining 6 cases the simulated stent was located lower than the actual one. Conclusions: The method of computer modelling provided a satisfactory success rate of predicting the possible stents to use during the procedure. Differences in chosen stents may have been caused by individual experience in interventional cardiology, the lack of availability of certain stents in the heart catheterization laboratory, the lack of information about the diameter of the vascular access and differences in dimensions measured on the model, tomography and angiography.

Fetal Cardiac Interventions-Are They Safe for the Mothers?

The aim of fetal cardiac interventions (FCI), as other prenatal therapeutic procedures, is to bring benefit to the fetus. However, the safety of the mother is of utmost importance. The objective of our study was to evaluate the impact of FCI on maternal condition, course of pregnancy, and delivery. 113 mothers underwent intrauterine treatment of their fetuses with critical heart defects. 128 percutaneous ultrasound-guided FCI were performed and analyzed. The patients were divided into four groups according to the type of FCI: balloon aortic valvuloplasty (fBAV), balloon pulmonary valvuloplasty (fBPV), interatrial stent placement (IAS), and balloon atrioseptoplasty (BAS). Various factors: maternal parameters, perioperative data, and pregnancy complications, were analyzed. There was only one major complication-procedure-related placental abruption (without need for blood products transfusion). There were no cases of: procedure-related preterm prelabor rupture of membranes (pPROM), chorioamnionitis, wound infection, and anesthesia associated complications. Tocolysis was only necessary only in two cases, and it was effective in both. None of the patients required intensive care unit admission. The procedure was effective in treating polyhydramnios associated with fetal heart failure in six out of nine cases. Deliveries occurred at term in 89%, 54% were vaginal. The results showed that FCI had a negligible impact on a further course of pregnancy and delivery.

Fetal Cardiac Interventions-Polish Experience from "Zero" to the Third World Largest Program.

This article presents the technical aspects of the Polish fetal cardiac interventions (FCI) program, including preparation of the team and modifications in the technique of the procedure that aim to increase its safety for the mother and the fetus. Over 9 years, 128 FCI in 113 fetuses have been performed: 94 balloon aortic valvuloplasties (fBAV), 14 balloon atrioseptoplasties (fBAS) with stent (BAS+), 5 balloon atrioseptoplasties without stent placement (BAS-), and 15 fetal pulmonary valvuloplasties (fBPS). The technical success rate ranged from 80% (BAS-) to 89% (fBAV), while the procedure-related death rate (defined as death within 72 hours following the procedure) ranged from 7% (fBAV and fBPV) to 20% (BAS). There were 98 live births after all FCI (3 pregnancies continue). Median gestational age at delivery was 39 weeks in our center and 38 weeks in other centers.

Complete closure of the ductus arteriosus in the foetus with transposition of the great arteries.

Prenatal restriction of the ductus arteriosus can manifest as persistent pulmonary hypertension in the newborn, especially dangerous with the transposition of the great arteries. Its aetiology has long been related to maternal intake of non-steroidal anti-inflammatory drugs; however, some other substances, including polyphenols, may have similar properties. We describe a case of complete prenatal closure of the ductus arteriosus in the foetus with transposition of the great arteries. The newborn presented with pulmonary hypertension unresponsive to pharmacotherapy and died of multi-organ failure.

Fetal echocardiography gives a clue for the maternal diagnosis of tuberous sclerosis complex.

The detection of multiple cardiac tumors during fetal echocardiography allowed us to make the diagnosis of tuberous sclerosis complex in the mother and establish the reason of her first epileptic seizures.

Ultrasound guided balloon catheterisation: a new method of fetal lower urinary tract obstruction management.

OBJECTIVES: Fetal lower urinary tract obstruction (LUTO), most often associated with presence of posterior urethral valves, poses high risk of perinatal mortality or postnatal renal failure. Looking for a method of causative treatment we have devel-oped a technique of fetal urethroplasty with a coronary angioplasty balloon catheter inserted under an ultrasonographic guidance via an 18-gauge needle introduced transabdominally to fetal bladder. MATERIAL AND METHODS: We have used this procedure in three women with singleton pregnancies (two primiparas and one multipara, 32-35 years of age), diagnosed with fetal megacystis at 12-16 weeks of gestation. Urethral catheterization was carried out at 16-18 weeks and an unobstructed urine flow was achieved in all three cases immediately after the procedure, followed by a resolution of megacystis and normalization of amniotic fluid volume. RESULTS: In all three cases, the post-procedure period was uneventful. In the first two fetuses, amniotic fluid volume re-mained normal until 30 weeks of gestation when a gradual development of oligohydramnios and some signs of renal cystic dysplasia were observed. Nevertheless, both pregnancies were continued till term (37 and 39 weeks, respectively) and two boys without signs of pulmonary hypoplasia were delivered. The third patient is currently 25 weeks pregnant; volume of amniotic fluid in her fetus is normal and no signs of urinary flow obstruction or renal dysplasia have been recorded thus far. CONCLUSIONS: Although some technical aspects of the procedure still need to be established, it seems worth consideration as a form of potentially least traumatic intrauterine intervention in fetuses with lower urinary tract obstruction.

How Normal is a 'Normal' Heart in Fetuses and Infants with Down Syndrome?

BACKGROUND: Congenital heart disease is present in 44-56% of fetuses with Down syndrome (DS). There are, however, signs that hearts in DS without apparent structural heart defects also differ from those in the normal population. We aimed to compare the atrioventricular (AV) septum and valves in 3 groups: DS without AV septal defect (DS no-AVSD), DS with AVSD (DS AVSD) and control hearts. METHODS: The ventricular septum, membranous septum and AV valves were examined and measured in histological sections of 15 DS no-AVSD, 8 DS AVSD and 34 control hearts. In addition, the ventricular septum length was measured on ultrasound images of fetal (6 DS AVSD, 9 controls) and infant (10 DS no-AVSD, 10 DS AVSD, 10 controls) hearts. RESULTS: The membranous septum was 3 times larger in DS no-AVSD fetuses compared to control fetuses, and valve dysplasia was frequently (64%) observed. The ventricular septum was shorter in patients with DS both with and without AVSD, as compared to the control group. CONCLUSION: DS no-AVSD hearts are not normal as they have a larger membranous septum, shorter ventricular septum and dysplasia of the AV valves as compared to control hearts.

[Fetal cardiac interventions - are we ready for them?]. / Interwencyjne zabiegi kardiologiczne u plodów - czy jestesmy do nich przygotowani?

OBJECTIVE: The aim of the study was to analyze types and methods of intrauterine fetal cardiac interventions performed between June 2011 and December 2013, and to assess the perinatal management of the neonates. METHODS: The program was developed after analysis of the available literature, practical individual training in Linz, Austria, and simulation of the procedure in a dissecting-room. The rules for anesthesia in pregnant women and their fetuses were developed. The interventions were performed in fetuses with critical cardiac defects, in the operating room, under ultrasonographic control. The protocol was approved by the Local Bioethics Committee at the Centre of Postgraduate Medical Education. MATERIAL: We included fetuses with critical aortic stenosis (n=29), critical pulmonary stenosis (n=2), and closed or extremely restricted foramen ovale (n=7). Between June 2011 and December 2013, the team comprised of JD, MD and AK conducted 42 interventions in 35 fetuses, including 32 balloon aortic valvuloplasties (in 29 fetuses), 2 pulmonary valvuloplasties, 4 balloon atrial septostomies and 4 atrial septal stent placement. Three fetuses required both, aortic valvuloplasty and fenestration of the atrial septum. RESULTS: Out of the 42 procedures, 41 (97%) were technically successful. We recorded 3 cases of fetal demise associated with the intervention. We modulated the protocol of anesthesia given to pregnant women, switching from general to local anesthesia with intravenous sedation. We always provided additional fetal anesthesia with fentanyl and atracurium via the umbilical vein. CONCLUSIONS: Based on our 2.5-year experience, it seems safe to conclude that all types of fetal cardiac interventions may be successfully conducted at Polish centers. The procedures are safe for the pregnant women and improve fetal status. Most of the neonates treated prenatally were referred in good general condition to a tertiary pediatric cardiology and cardiac surgery center

[The first successful fetal aortic balloon valvuloplasty in Poland]. / Pierwsza w Polsce skuteczna przezskórna plastyka balonowa zastawki aortalnej u plodu.

Prenatal aortic valvuloplasty is performed only in few perinatal centers in the world. Critical aortic stenosis which can lead to hypoplastic left heart syndrome or severe fetal heart failure with nonimmune hydrops is an indication for this procedure. Prenatal intervention can change the natural course of the disease. Authors present the first successful fetal balloon aortic valvuloplasty in Poland. It was performed in a 29-week fetus with critical aortic stenosis, severe impairment of left ventricular function, heart failure and fetal hydrops. After successful intervention, without any complications, left ventricular function and fetal condition improved gradually Effective fetal intervention was possible after few months of preparation and building a team of specialists. This is the first successful fetal cardiac intervention in Poland, which opens the way to the new era of fetal cardiology and hopefully will lead to improve results in children with this critical heart defect.

[Post-mortem stereomicroscopic examination of embryonic and fetal hearts in the 1st trimester of pregnancy]. / Badanie autopsyjne serc zarodków i plodów w I trymestrze ciazy metoda stereomikroskopowa.

UNLABELLED: Post-mortem examination of embryos and fetuses becomes increasingly important due to the development of prenatal diagnostic methods. Possibilities of visualization of embryonic and fetal heart in echocardiography and autopsy develops rapidly and due to learning curve there is a need for confirmation of results. There are different methods for visualization of cardiac anatomy. Some morphologists prefer classical histology while others use stereomicroscopy OBJECTIVES: To develop our own autopsy routine for embryonic and small fetal hearts. MATERIAL AND METHODS: Twenty five normal and malformed fetal hearts aged from 7 to 12 weeks, fixed and unfixed, were submerged in saline solution and dissected using microsurgical instruments. nikon SMZ 1500 microscope, Nikon DS-Fi1 camera and NIS-Elements 3.00 software were used for observation and documentation. The chest was opened by median sternotomy the heart was dissected in situ and sequential segmental analysis was applied. RESULTS: Detailed anatomy of atriums, interatrial septum, pulmonary and systemic veins drainage were examined. In all cases it was possible to assess morphology of chambers, atrioventricular and ventriculoarterial connections. Congenital heart defect was diagnosed in only 3 out of 25 cases: one in the 10th week of gestation (complete atrioventricular septal defect--CAVSD) and two in the 12th week of gestation (1 case of CAVSD with trisomy 21 confirmed cytogenetically and one of tetralogy of Fallot). CONCLUSION: Microautopsy of fetal heart in the 1st trimester of pregnancy is possible using stereomicroscopy and allows us to diagnose congenital heart diseases. Detailed structures are best visible when specimen is unfixed and submerged in saline solution.