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Background: Stenting of stenotic right ventricular outflow tract is a palliative measure for severely impaired small babies with Tetralogy of Fallot or similar pathologies. Little is known about the histopathological fate of the stents in the right ventricular outflow tract. Methods: Eight samples of surgically removed right ventricular outflow tract stents were histologically analysed according to a predefined protocol. Results: The most frequent diagnosis was Tetralogy of Fallot in four patients, pulmonary atresia with ventricular septal defect in two patients, double outlet right ventricle with pulmonary obstruction in one patient, and muscular obstruction of the right ventricular outflow tract in one patient with a syndromic disease with hypertrophic cardiomyopathy. Stents mean implantation duration was 444 days ranging from 105 to 1117 days (median 305.5 days). Histology revealed a variable degree of pseudointima formation consisting of fibromuscular cells surrounded by extracellular matrix. Four of the specimen contained adjacent myocardial tissue fragments, which showed regressive changes. Neither myocardium nor pseudointima tissue or tissue parts locally related to stent struts were infiltrated by inflammatory cells. Conclusions: Histological analysis after explantation of early-in-life implanted right ventricular outflow tract stents revealed predominantly pronounced neo-intimal proliferation with a visible endothelial layer, no signs of inflammation, and no prolapse of muscular tissue through the stent struts. Thus, implantation of stents in early life seems to interfere little with the hosts’ immune system and might help to open up the right ventricular outflow tract by mechanical forces and regressive changes in adjacent muscular tissue.

Background Hoarseness due to laryngeal nerve injury is a known complication after cardiothoracic surgery involving the aortic arch. However, this complication is only rarely reported after catheter interventions. Results In this article we present the unusual case of a left-sided vocal cord paralysis in four patients after primary stenting of a re-coarctation, re-dilatation of a stented coarctation, a primary stenting of the left pulmonary artery (LPA), and prestenting for percutaneous pulmonary valve implantation with dilation of the LPA. After implanting bare metal stents, it is common practice, whilst contemplating the diameters of the adjacent structures, to optimize the stent diameter in a two-step procedure and dilate the stent until a maximum diameter is achieved and there is no residual gradient after applying this technique. Four of our patients experienced hoarseness after the intervention and a vocal cord paralysis was diagnosed. Angiography revealed no signs of extravasation or dissection. Clinical symptoms improved over the course of the following 6 months; patients with interventions at the aortic arch showed a complete remission, patients with procedures involving the LPA showed only mild regression of the symptoms. Conclusion To our knowledge, this complication (Ortner's syndrome, cardiovocal syndrome) after such interventions has rarely been reported before. Although a rare complication, the recognition of these symptoms may support colleagues in managing affected patients. In addition, awareness for hoarseness after interventional therapies and systematic screening for this complication might help to identify patients at risk in the future.

Persistent air leak (PAL) is a severe complication of secondary spontaneous pneumothorax (SSP). Surgical interventions are usually successful when medical treatment fails, but can be associated with significant complications and loss of potentially recoverable lung parenchyma.Retrospective analysis of efficacy and safety of interventional bronchus occlusions (IBO) using Amplatzer devices (ADs) in children with PAL secondary to SSP.Six patients (four males, 4-15 years of age) underwent IBO using ADs as treatment for PAL. Necrotizing pneumonia (NP) was the most common cause (n=4) of PAL. Three patients were previously healthy and three suffered from chronic lung disease. All patients required at least two chest tubes prior to the intervention for a duration of 15-43 days and all required oxygen or higher level of ventilatory support. In three cases, previous surgical interventions had been performed without success. All children improved after endobronchial intervention and we observed no associated complications. All chest tubes were removed within 5-25 days post IBO. In patients with PAL related to NP (n=4), occluders were removed bronchoscopically without re-occurrence of pneumothorax after a mean of 70 days (IQR: 46.5-94).IBO using ADs is a safe and valuable treatment option in children with PAL independent of disease severity and underlying cause. A major advantage of this procedure is its less invasiveness compared to surgery and the parenchyma- preserving approach.Hintergrund: Die persistierende Luftleckage stellt eine schwere Komplikation eines sekundären Spontanpneumothorax (SSP) dar. Chirurgische Interventionen sind bei erfolgloser konservativer Therapie oft erfolgreich aber können mit schweren Komplikationen und dem Verlust von potentiell erholungsfähigem Lungengewebe behaftet sein. Ziel: Retrospektive Analyse über die Effektivität und Sicherheit von interventionellen Bronchus-Okklusionen (IBO) mit Amplatzer Devices (ADs) bei Kindern mit persistierender Luftleckage. Ergebnise: Bei 6 Patienten (4 männlich, Alter 4–15 Jahre) wurde eine IBO zur Therapie einer persistierenden Luftleckage durchgeführt. Die nekrotisierende Pneumonie (NP) war die häufigste zugrundeliegende Ursache einer persistierenden Leckage (n=4). 3 Kinder waren zuvor gesund, bei 3 Kindern lag eine chronische Lungenerkrankung vor. Alle Patienten benötigten vor Intervention mindestens 2 Thoraxdrainagen für 15–43 Tage und alle Kinder benötigten Sauerstoff oder eine zusätzliche Atemunterstützung. Bei 3 Patienten erfolgten zuvor erfolglose chirurgische Interventionen. Es traten keine Komplikationen im Zusammenhang mit der IBO auf. Alle Patienten profitierten von der Prozedur, die Thoraxdrainagen konnten nach 5–25 Tagen gezogen werden. Bei allen Kindern mit persistierender Luftleckage aufgrund einer NP (n=4) wurden die ADs nach durchschnittlich 70 Tagen (IQR:46.5–94) entfernt ohne dass ein Pneumothorax-Rezidiv auftrat. Schlussfolgerung: Die IBO mithilfe von ADs stellt unabhängig von der Ursache und Krankheitsschwere ein vielversprechendes und sicheres Therapieverfahren zur minimal-invasiven Behandlung von Kindern mit persistierender Luftleckage dar. Vorteile dieser Prozedur im Vergleich zu operativen Verfahren sind die geringe Invasivität und der parenchymerhaltende Ansatz.

This study aims to evaluate the long-term efficacy and reintervention rate after primary percutaneous portal vein stent angioplasty for portal vein stenosis (PVS) in pediatric liver transplantation (LT) recipients. From 2004 to 2020, a total of 470 pediatric LTs were performed in our center. All cases were screened for interventional PVS treatment and analyzed retrospectively. We identified 44 patients with 46 percutaneous angioplasties for posttransplantation PVS. The median interval from LT to percutaneous catheter intervention was 5 months (16 days-104 months) with a median follow-up (f/u) period after catheter intervention of 5.7 years (2-156 months). In 40 patients, an endovascular stent was placed as primary (n = 38) or secondary (n = 2) intervention. The median age at stent placement was 23 (6-179) months with a median weight of 10 kg (6-46 kg). Technical success and relief of PVS were achieved in all patients irrespective of age or weight. Adverse events occurred peri-interventionally in two patients and were resolved with standard care. All primary portal vein (PV) stents remained patent until the end of f/u. Reinterventions have been successfully performed in 10 patients for suspected or proven restenosis, resulting in a primary patency rate of 75% and an assisted patency rate of 25%. The median time to reintervention was 6.2 years (range 1-10 years). The need for reintervention was independent of age or weight at both transplantation and initial angioplasty as well as of additional risk factors due to portal hypertension. Percutaneous transhepatic PV stent angioplasty in children is safe and effective in all age groups, with excellent long-term patency. Primary stent angioplasty should be considered as first-line treatment for PVS after pediatric LT.

IntroductionAlthough the right jugular vein approach for percutaneous pulmonary valve implantation is well described, there are no reports that describe a percutaneous pulmonary valve implantation through a left superior caval vein to coronary sinus pathway.CaseA 14-year-old female with tetralogy of Fallot, mesocardia, left superior caval vein draining into the coronary sinus, and hemiazygos continuation of the inferior caval vein underwent ventricular septal defect closure, with homograft insertion from the right ventricle to the pulmonary artery, patch augmentation of the left pulmonary artery, and creation of an atrial communication. Thereafter followed numerous catheterisations and interventions with stent implantation for stenosis of the left pulmonary artery and the homograft, as did device closure of the atrial communication. When she was a 12-year-old, the indications for a percutaneous pulmonary valve implantation were fulfilled and she underwent implantation of a 22 mm Melody® valve through the left superior caval vein. The extra-stiff exchange wire was pre-formed into a “U-spiral”-type configuration, according to the underlying anatomy, in order to provide a smooth route for the delivery of stents, to create the landing zone, and for the implantation of the Melody “ensemble”. The procedure was performed under deep sedation according to our standard protocol. The duration of the procedure was 172 min and the radiation time was 24.9 min.ConclusionOn the basis of this unique experience, percutaneous pulmonary valve implantation is safe and feasible even in patients with unusual anatomy. Crucial is the “U-spiral” shaped configuration of the guide wire.

Objective: We report our recent experience with the new Occlutech (R) PDA Occluder, which seems suitable especially for closure of large patent ductus arteriosus (PDA) sizes with elevated pulmonary artery pressure (PAH). Background: Although considered a standard procedure in pediatric cath labs, closure of very large PDA (PDA/Aorta ratio>0.5) and PAH remains a challenge. Methods/Results: The Occlutech (R) PDA occluder was used in eight children and adolescents (age 4-16, median 10.75 years), body weight from 14 to 54 kg (median 21 kg) with very large PDA and PAH: PDA diameter was 5-13 mm (median 10 mm);PAP/Aortic pressure ratio was 0.58-0.96. Sufficient occlusion of the PDA was documented by angiography and/or echocardiography in all cases. Four different device sizes were selected: Minimal diameter 6-14 mm (median 12 mm), length 6.3-16 mm (median 14 mm), aortic disc 13-24 mm (median 20 mm), size at the pulmonary end 8-18 mm (median 15 mm). A balloon test occlusion of the PDA was performed in five patients in order to evaulate the decline of pulmonary artery pressure or to delineate the exact anatomy of the PDA. Before release of the device, a careful "wiggle manoevre" was performed to assess the stability of the implanted device and to proof the inability to embolize to the aorta. Conclusions: With the new Occlutech (R) PDA Occluder closure of very large PDAs and PAH is feasible and efficient. The wider pulmonary artery end of the Occlutech (R) PDA Occluder offers enhanced stability and reduces the risk of embolization. (C) 2016 Wiley Periodicals, Inc.

Aims:Transcatheter implantation of pulmonary balloon-expandable stent-valves requires pre-stenting of the right ventricular outflow tract with large calibre stents. To increase awareness of the associated risks of this part of transcatheter pulmonary valve replacement therapy, we report potential fatal complications during the implantation of AndraStents® in the right ventricular outflow tract in six cases from five different European institutions and their management.Method and result:We present a retrospective case series analysis looking at the time period from 2013 to 2018. Of 127 AndraStents® implanted in the right ventricular outflow tract, in six patients, age from 13 to 71 years, a misconfiguration of the AndraStent® occurred forming a “diabolo”-configuration. During inflation of the balloon, the stent showed extreme “dog-boning”, an expansion of the stent at both ends with the middle part remaining unexpanded. This led to rupture of the balloon and loss of manoeuvrability in four patients. Out of the total six cases, in four patients the stent was eventually expanded with high-pressure balloons, and in one case the stent was surgically retrieved. In one patient, in whom a percutaneous retrieval of the embolised stent was attempted, a fatal bleeding occurred.Conclusions:Pre-stenting of the right ventricular outflow tract by AndraStents® can lead to misconfiguration of the stent with potentially fatal complications. Rescue strategies of misconfigured stents include stent inflation and placement with high pressure non-compliant balloons or surgical backup. Interventional retrieval measures of AndraStents® cannot be advised.

Aims: Endomyocardial biopsies (EMBs) are performed infrequently in children owing to significant past complication rates and controversial discussions about the therapeutic value of results. The objective of this study was to investigate the safety and feasibility of EMBs for suspected myocardial disease in relation to their clinical value. Methods and results: We performed a retrospective multicentre review of the Working Group for Interventional Cardiology of the German Society for Paediatric Cardiology. During three consecutive years, 206 EMBs (84 female/mean age 8.95 +/- 6.62 years) were performed and analysed at 15 heart centres. In the majority of cases, biopsies were taken from the right ventricle (RV/89.8%;p

BackgroundThe Occlutech Figulla ASD device series (OFSO) shows an improved device design for interventional ASD closure, larger follow-up series are missing. MethodsWe retrospectively reviewed the feasibility, safety, implantation properties, results, and follow-up of ASD closure using Occlutech devices over a 5 year period by establishing a multi-institutional collaborative result registry with 16 contributing centers from 11 countries (IRFACODE). ResultsIn 1315 patients of all age groups (female 66.9%), successful (98%) ASD closure was performed (mean age 28.9 years, weight 52 kg, height 148.6 cm). Of the defects, 47.9% showed no or only a deficient aortic rim;in 11.9%, there was more than one defect;a septum aneurysm was present in 21.5%;and the mean implanted device size was 20.5 mm. Immediate closure was achieved in 78.6%, at discharge in 83.1%, and 96.4% and 97.3% at 6 and 12 months follow-up, respectively. During a mean follow-up of 2.7 years (in total 3597 patient years), significant complications were minimal (total=8

Background Interventional closure of patent ductus arteriosus (PDA) has become a common and safe procedure in most pediatric cath labs. Interventional treatment of PDAs still remains a challenge in those children with low body weight and a large PDA. The Nit-Occlud PDA-R® device was developed and especially designed for large PDAs. We report our most recent experience in children with a body weight lower than 10 kg. Materials and Methods The PDA-R® device was used in seven children (age 1–10, median 6 months) with a body weight from 4.1 to 9.7 kg (median 5.9 kg): ductal length was 12 mm (median), with a large ampulla (median 9 mm) which exceeded the diameter of the aorta (median 6 mm) and large diameter (median minimal diameter 4 mm). In six cases, the Nit-Occlud PDA-R was selected with an aortic disc of 12 mm and in one case an occluder with an aortic disc of 14 mm. Results Occlusion of the PDA was documented by angiography and/or echocardiography in all cases. At a mean follow-up of 21.4 months, no flow obstruction to the left or right pulmonary artery or new onset coarctation of the aorta was noted. Conclusions The Nit-Occlud PDA-R® device is suitable in children with a body weight below 10 kg when a relative large PDA is present. © 2015 Wiley Periodicals, Inc.

Introduction In the search for a biodegradable device that leaves nothing but the tissue of the patient after complete endotheliazation and absorption, the BioSTAR® device was introduced in 2007 (CE Mark in European community and HPB in Canada) for ASD and PFO closure. It consists of a metal framework covered by a biodegradable membrane generated from a layer of porcine collagen that is broken down and absorbed over time. In a sheep model, the results were promising, showing complete closure of the defect with degradation of approximately 90% of the implanted membrane material after two years. Methods We report a retrospective analysis of implantation failures, early and late complications in a series of 34 patients with 30 implanted BioStar® devices in a single center with a total follow-up of more than 75 patient years. Results We report 12% of implantation failures, 9% of early and 12% of late complications. Implantation failures include one embolized device, which was interventionally retrieved. Early complications were exclusively rhythm disturbances, one patient needed electrical and pharmacological therapy. Four relevant late complications occurred. One device required explantation after 61 days because of recurrent severe fever episodes, severe headache, and malaise that subsequently subsided after device removal. One patient presented with Dressler's syndrome with pericardial effusion 5 month after implantation requiring pericardiocentesis and steroid treatment. One device showed a central residual shunt that was not clearly seen initially. Finally, one device was explanted after hemorrhagic pericardial effusion due to perforation of an arm of the frame through the right atrial roof into the pericardial fold after 19 months. Conclusion We conclude that implantation of the Biostar® device is difficult in patients with deficient aortic rims and early complications are similar to those seen in other devices. Of importance, the late complications seen with the Biostar® device might be attributable to specific material and immunological properties of the partially biodegradable device. Although a biodegradable device might theoretically be more favorable more efforts for optimization of these devices have to be taken. © 2015 Wiley Periodicals, Inc.

Stenting of vascular, extracardiac or lately intracardiac stenosis has become an established interventional treatment for a variety of problems in congenital or acquired heart disease. Most stent procedures are completed successfully and the long-term outcome is favorable in the majority of cases. Stent collapse or deformation is a well recognized entity in peripheral stents and can be attributed to insufficient radial force; it can also be attributed to excessive external forces, like deformation of stents in the right ventricular outflow tract, where external compression is combined with continuous movement caused by the beating heart. The protection of the thoracic cage may prove to be insufficient in extraordinary circumstances, such as chest compression in trauma or cardiopulmonary resuscitation (CPR). In this case series, we describe three patients in whom large endovascular stents were placed to treat significant stenosis of the aorta, the aortic arch or the venous system of the inferior vena cava close to the atrium. In all patients, CPR was necessary during their clinical course for various reasons; after adequate CPR, including appropriate chest compression all patients survived the initial resuscitation phase. Clinical, echocardiographic as well as radiologic re-evaluation after resuscitation revealed significant stent distortion, compression, displacement or additional vascular injury. The possibility of mechanical deformation of large endovascular stents needs to be considered and recognized when performing CPR; if CPR is successful, immediate re-evaluation of the implanted stents--if possible by biplane fluoroscopy--seems mandatory.

Arterial thrombosis in neonates and children is a rare event and is often associated with external risk factors such as asphyxia or sepsis. We report our experiences with two neonates with spontaneous aortic arch thrombosis mimicking aortic coarctation. Despite single case reports until now, no data exist for the underlying thrombophilic risk factors and prognosis of this rare event. Both patients were carriers of a heterozygous factor V Leiden mutation, which has been reported once before as a risk factor for aortic arch thrombosis. One of our patients was operated upon successfully and is alive. The second patient suffered a large infarction of the right medial cerebral artery and had a thrombotic occlusion of the inferior caval vein. The patient obtained palliative care and died at the age of 6 days. In the literature, we identified 19 patients with neonatal aortic arch thrombosis. Of the 19 patients, 11 (58%) died. Including the two reported patients, the mortality rate of patients with multiple thromboses was 80% (8/10) compared with 18% (2/11) for patients with isolated aortic arch thrombosis; this difference reached statistical significance (p = 0.009). The analysis of thrombophilic disorders revealed that factor V Leiden mutation and protein C deficiency seem to be the most common risk factors for aortic arch thrombosis.Conclusion:Neonatal aortic arch thrombosis is a very rare but life-threatening event, with a high rate of mortality, especially if additional thrombotic complications are present. Factor V Leiden mutation seems to be one important risk factor in the pathogenesis of this fatal disease.

We report our recent experience with the new OcclutechAlthough considered a standard procedure in pediatric cath labs, closure of very large PDA (PDA/Aorta ratio 0.5) and PAH remains a challenge.The OcclutechWith the new Occlutech

In August 2010, the Nit-Occlud

In August 2010, the Nit-Occlud® Le (EUREVECO) became available for transcatheter coil occlusion of ventricular septal defects (VSDs). Retrospective European Registry for VSD Closure using the Nit-Occlud® Le-VSD-Coil; analysis of the feasibility, results, safety and follow-up of VSD-closure over a 3-year period in 18 European centers. In 102 of 111 patients (female 66), successful VSD closure was performed (mean age 8.2 years, mean weight 28.82 kg), 81 perimembranous VSDs (48 with aneurysm), 30 muscular VSDs, mean procedure time was 121.1 min, and mean fluoroscopy time was 26.3 min. Short- and midterm term follow-up was possible in 100/102 patients, there was 1 embolization and 1 explantation after 24 months. Immediate complete closure occurred in 49 of 101 patients (48.5%), trivial residual shunt was present in 51 (50.0%), closure rate was 95% after 6 months and 97% after 1 year. Out of the 102 patients, there were 2 severe complications (1.8%) (1 severe hemolysis, 1 embolization) and 8 moderate/transient (=7.2%) including 1 transient AV block. During a mean follow-up period of 31.3 months (range 24–48) and a total follow-up time of 224.75 patient years, no further problems occurred. VSD closure with the Nit-Occlud® Le VSD coil is feasible and safe with a minimal risk of severe side effects. The long-term effects and safety require further clinical follow-up studies.

Das akute Rechtsherzversagen (RHV) ist eine haufige und ernste Komplikation in der perioperativen und intensivmedizinischen Behandlungsphase. Die haufigsten Ursachen sind pulmonale Hypertension, Folgen eines Linksherzversagens, Lungenembolie, Sepsis, acute lung injury (ALI) sowie kardiochirurgische Eingriffe. Ein akutes RHV selbst tragt nicht nur per se zu Morbiditat und Mortalitat bei, es beeinflusst auch masgeblich die Einsatzmoglichkeiten und den Erfolg routinemasig durchgefuhrter intensivmedizinischer Therapiemasnahmen, wie beispielsweise die Gabe von Vasopressoren. Im Gegensatz zum linken Ventrikel ist uber die Physiologie und Pathophysiologie des rechten Ventrikels (RV) jedoch relativ wenig bekannt und die Diagnostik des RHV ist eine grose Herausforderung. Zur Therapie des RHV stehen zwar einerseits viele Medikamente zur Verfugung, andererseits fehlen jedoch noch haufig kontrollierte, randomisierte Studien (RCT). Der Artikel gibt eine Ubersicht uber Atiologie und Pathophysiologie des akuten Rechtsherzversagens und zeigt aktuelle diagnostische und therapeutische Moglichkeiten nicht nur fur Anasthesisten und Intensivmediziner auf.

The Occlutech Figulla ASD device series (OFSO) shows an improved device design for interventional ASD closure, larger follow-up series are missing.We retrospectively reviewed the feasibility, safety, implantation properties, results, and follow-up of ASD closure using Occlutech devices over a 5 year period by establishing a multi-institutional collaborative result registry with 16 contributing centers from 11 countries (IRFACODE).In 1315 patients of all age groups (female 66.9%), successful (98%) ASD closure was performed (mean age 28.9 years, weight 52 kg, height 148.6 cm). Of the defects, 47.9% showed no or only a deficient aortic rim; in 11.9%, there was more than one defect; a septum aneurysm was present in 21.5%; and the mean implanted device size was 20.5 mm. Immediate closure was achieved in 78.6%, at discharge in 83.1%, and 96.4% and 97.3% at 6 and 12 months follow-up, respectively. During a mean follow-up of 2.7 years (in total 3597 patient years), significant complications were minimal (total = 8,1%) with secondary device embolizations in five and AV-blocks in three patients. No erosion or death was reported.ASD closure using OFSO is feasible in a large variety of patients, safe with only a minimal risk of severe side effects and especially without any aortic erosions despite a large percentage of large and complicated defects. © 2016 Wiley Periodicals, Inc.

Here we report on a male infant presenting the typical pattern of Jacobsen syndrome including trigonocephaly, thrombocytopenia, congenital heart defect, urethral stenosis, and partial agenesis of the corpus callosum. Conventional karyotyping, FISH, SKY and CGH analyses showed that the region distal to the MLL locus on 11q23 was lost and replaced by the distal region of 11p, leading to a partial trisomy of 11p and a partial monosomy of 11q. According to ISCN (1995) the karyotype can be described as 46,XY,add(11)(q2?3). ish 11ptel(D11S2071x3),11qtel(VIJyRM2072x1). Array-CGH analysis allowed us to narrow down the breakpoints to 11p15.1 and 11q24.1. Methylation analyses of genes located on 11p showed an increased level of the non-methylated paternal allele of the KCNQ1OT1 gene, confirming the concomitant presence of Beckwith-Wiedemann syndrome (BWS). The phenotype resulting from the 11q deletion seems to dominate the phenotype due to the distal 11p trisomy. Investigation of the parents revealed that this chromosomal rearrangement was caused by a paternal pericentric inversion inv(11)(p15q24). Since chromosomal aberrations like the one described here can easily be overlooked during routine chromosome analysis, combined FISH analysis using subtelomeric and possibly additional probes should be applied if there is any doubt about the integrity of telomeric regions.

Objective Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency disorder characterized by recurrent infections, autoimmunity, microthrombocytopenia, and susceptibility to malignant tumors. Compared with the conventional treatment using allogeneic bone marrow transplantation, hematopoietic stem cell gene therapy might offer more specific and less toxic therapeutic options. Methods We investigated retroviral WAS protein (WASP) gene transfer to assess functional correction and potential toxicities in human CD34 + cells from WAS patients and healthy individuals, respectively. Results WASP mRNA and protein levels were restored in CD14 + cells derived from WASP-transduced hematopoietic stem cells. Functional reconstitution in WASP-transduced myeloid cells was documented by podosome formation and FcγR-mediated phagocytosis. Importantly, overexpression of WASP in CD34 + cells from healthy donors did not cause any discernible toxic effects. Conclusions Our studies document the feasibility of WASP gene transfer into human CD34 + cells and suggest that the phenotype of WASP-deficient myeloid cells can be restored upon retroviral gene transfer.

Aims To investigate the implantation safety, anatomic performance and septal alignment of the Occlutech Figulla Flex occluder (FFO) device, an atrial septal defect (ASD) closure device with specific left-sided deployment characteristics and right-sided septal alignment properties. Methods and results Between January 2011 and December 2013 we prospectively collected the change of orientation of the device to the septum during the release process and the feasibility of implantation of the FFO in 122 patients. The mean age was 10.7 years (±10.2), weight 32.9 kg (±20.3), and height 129.4 cm (±30). Devices used were 9 (n=13), 10.5 (n=16), 12 (n=16), 15 (n=39), 18 (n=17), 21 (n=8), 24 (n=5), 27 (n=7) and 30 mm (n=3) in size. No additional implantation techniques were required. Before release, the mean angles of the left and right-sided discs were 29.2° (±9.9°) and 43.4° (±9.2°) to the body axis, and 18.7° (±8.7°) and 27.0° (±10°) immediately thereafter. Thus, there was only a slight change in orientation of the left-sided (10.6°±7.5°) and right-sided (16.3°±7.9°) discs. Conclusions The design of this occluder system results in an ideal septum alignment which increases its feasibility as well as patient safety during implantation.

Interventional closure of patent ductus arteriosus (PDA) has become a common and safe procedure in most pediatric cath labs. Interventional treatment of PDAs still remains a challenge in those children with low body weight and a large PDA. The Nit-Occlud PDA-R® device was developed and especially designed for large PDAs. We report our most recent experience in children with a body weight lower than 10 kg.The PDA-R® device was used in seven children (age 1-10, median 6 months) with a body weight from 4.1 to 9.7 kg (median 5.9 kg): ductal length was 12 mm (median), with a large ampulla (median 9 mm) which exceeded the diameter of the aorta (median 6 mm) and large diameter (median minimal diameter 4 mm). In six cases, the Nit-Occlud PDA-R was selected with an aortic disc of 12 mm and in one case an occluder with an aortic disc of 14 mm.Occlusion of the PDA was documented by angiography and/or echocardiography in all cases. At a mean follow-up of 21.4 months, no flow obstruction to the left or right pulmonary artery or new onset coarctation of the aorta was noted.The Nit-Occlud PDA-R® device is suitable in children with a body weight below 10 kg when a relative large PDA is present.

Members of the Wnt/wingless family of secreted proteins act as short-range inducers and long-range organizers during axis formation, organogenesis and tumorigenesis in many developing tissues. Wnt signalling pathways are conserved in nematodes, insects and vertebrates. Despite its developmental significance, the evolutionary origin of Wnt signalling is unclear. Here we describe the molecular characterization of members of the Wnt signalling pathway--Wnt, Dishevelled, GSK3, beta-Catenin and Tcf/Lef--in Hydra, a member of the evolutionarily old metazoan phylum Cnidaria. Wnt and Tcf are expressed in the putative Hydra head organizer, the upper part of the hypostome. Wnt, beta-Catenin and Tcf are transcriptionally upregulated when head organizers are established early in bud formation and head regeneration. Wnt and Tcf expression domains also define head organizers created by de novo pattern formation in aggregates. Our results indicate that Wnt signalling may be involved in axis formation in Hydra and support the idea that it was central in the evolution of axial differentiation in early multicellular animals.

This study describes a modified Seldinger technique for 2- and 3-French peripherally inserted central venous catheters: A device similar to that used in heart catherisation with a standard micro-introducer serving as sheath and an arterial catheter serving as inner dilator was pushed forward over a wire guide that had before been inserted via a peripheral venous catheter. With this method 2-and 3-French catheters could be safely inserted into peripheral veins of 14 paediatric patients. In conclusion successful insertion of a small peripheral venous catheter offers in most cases a possibility for the placement of a central venous line.

Various model organisms such as mouse, xenopus, or zebrafish embryos have been studied in the past to gain insight into the complex processes driving normal and abnormal development of the vertebrate heart. Despite the fact that the chicken embryo has been a favored classic model system used by embryologists and cardiovascular scientists for centuries to illustrate the principles of basic vertebrate embryology and cardiovascular development, so far, no one has provided a thorough documentation of heart development in this model from early visual stages to the stage of a completely formed heart with (a) images and (b) video recordings of beating hearts. However, in vivo documentation of heart development stages is indispensable because the initially tubular embryonic heart not only undergoes dramatic morphological changes, but also intriguing functional changes during cardiogenesis, which, only if they follow and remain within the normal developmental pathway, lead to the establishment of the normal four-chambered heart. In this work we present the first reference catalogue of cardiac development in vivo with (1) 25 plates of high resolution colour images in different views from Hamburger-Hamilton (HH)-stage 12 (day 2, relatively straight heart tube, early myocardial contractions) through HH-stage 35 (day 9, four-chambered heart) in end-diastole and end-systole, including a plate with an overview of all these stages; (2) collection of 82 video recordings of beating hearts in different views corresponding to the stages shown in the plates.

Acute right heart failure (RHF) is a frequent and severe complication during perioperative and intensive care treatment in intensive care units (ICUs). The most common causes are pulmonary hypertension, left heart failure, pulmonary embolism, sepsis, acute lung injury (ALI) and thoracosurgical procedures. Acute RHF is not only a major contributor to morbidity and mortality; it also influences efficacy and outcome of routinely performed procedures, such as vasopressors, in critically ill patients. In contrast to the left ventricle, the right ventricle's physiology and pathophysiology are understudied, and the diagnosis of acute RHF is frequently challenging. Although many drugs are available for the treatment of RHF, randomized trials for this setting are still missing. This article gives an overview of aetiology and pathogenesis of RHF and reviews the diagnostic and therapeutic interventions currently available for providers in anaesthesiology and critical care.

Einleitung: Fur peripher verlegte zentrale Katheter (=Schwemmkatheter) werden erst ab einem Durchmesser von 4-Fr Einfuhrhilfen angeboten, die mittels modifizierter Seldinger Technik verlegt werden konnen. Dunnere Katheter mussen bisher mittels Einfuhrhilfen, die wie peripher venose Zugange verlegt werden eingeschwemmt werden. Wir mochten hier eine modifizierte Seldinger Technik fur 2- und 3-Fr Schwemmkatheter sowie erste klinische Erfahrungen mit dieser neuen Methode vorstellen. Methode: Hierfur wurde ein mittels Seldinger Technik verlegbarer Arterienkatheter mit einer Einfuhrkanule fur Schwemmkatheter kombiniert. Zuerst wird die Stahlnadel aus der Einfuhrkanule entfernt und an deren Stelle der Arterienkatheter eingefuhrt (Abb. A). Danach wird ein peripherer Venenzugang in eine passende Vene verlegt (24-G fur 2-F Ketheter, 22-G fur 3-F Katheter). Uber diese wird die Seldingerspirale des Arterienkatheters eingefuhrt (Abb. B). Nach Entfernung des periphervenosen Zuganges und Vergroserung der Hauteintrittsstelle mittels einer minimalen Stichinzision werden uber die Spirale Arterienkanule und Einfuhrkanule in die Vene vorgeschoben (Abb. C). Spirale und Arterienkatheter konnen dann entfernt und der Katheter eingeschwemmt werden (Abb. D). Die Methode entspricht somit dem Verlegen einer Katheterschleuse, wie sie fur Herzkatheteruntersuchungen verwendet wird. Die Einfuhrhilfe entspricht hierbei der Schleuse, der Arterienkatheter dem innenliegenden Dilatator. Ergebnisse: Mit dieser Methode wurden bei 14padiatrischen Patienten im Alter zwischen dem ersten Lebenstag und sieben Jahren 13 zweilumige 2-F, ein einlumiger 2-F sowie ein 3-F Katheter erfolgreich verlegt. Die Katheter lagen zwischen 1 und 44 Tagen in situ. Bei 2 weiteren Patienten konnte zwar die Einfuhrhilfe mittels Seldinger Methode eingebracht werden, der Katheter konnte jedoch nicht nach zentral vorgeschoben werden, weshalb er wieder entfernt werden musste. Komplikationen traten nur bei Versuchen mit der Einfuhrhilfe NEOCATH SPLITH der Firma Vygon auf. Es war grosteils nicht moglich, diese Einfuhrhilfe bis in die Vene vorzuschieben obwohl Spirale und Arterienkatheter richtig lagen. Es zeigte sich, dass sich die Spitze der Einfuhrhilfe beim vorschieben vom Arterienkatheter abgehoben hat und deshalb subcutan lag. Ursache dafur war die vorbestehende Schlitzung der NEOCATH SPLITH Kanule an ihrer Unterseite. Danach wurde nur noch die erst beim Entfernen zu spaltende „Peelable Sheath“ Kanule von Smith Medical verwendet. Mit dieser Einfuhrhilfe trat diese Komplikation nicht mehr auf. Schlussfolgerung: Durch die Verwendung einer modifizierten Seldinger Methode zum Verlegen von 2- und 3-Fr Katheter konnen diese mit sehr hoher Sicherheit verlegt werden. Dies bedeutet, dass mit dem erfolgreichen Verlegen eines periphervenosen Zuganges auch mit dem erfolgreichen Verlegen des Katheters gerechnet werden kann.

Complexes of a N,N-bis(2-picolyl)amine (bpa) derivative with a pendant ethoxyethanol side chain (bpa(CH2)2O(CH2)2OH) (1) with late divalent transition metal ions Co(II), Ni(II), Cu(II) and Zn(II) have been studied. All complexes, [[bpa(CH2)2O(CH2)2OH]Co(NO3)](NO3) (1Co), [[bpa(CH2)2O(CH2)2OH]Ni(NO3)](NO3) (1Ni), [[bpa(CH2)2O(CH2)2OH]Cu(H2O)(NO3)](NO3) (1Cu) and [[bpa(CH2)2O(CH2)2OH]Zn(NO3)](NO3) (1Zn), were comprehensively characterized and their X-ray single crystal structures have been determined. The complexes show hexacoordinated geometries, in which 1 acts as a tetradentate (1Cu) or pentadentate (1Co, 1Ni and 1Zn) ligand. DNA cleavage experiments have been performed on supercoiled double stranded DNA plasmids in order to compare the cleavage efficiency of all four metals in the same ligand environment of 1. In this assay, 1Co and 1Cu showed the highest cleavage efficiency, whereas 1Ni and 1Zn were virtually inactive. Quantification of the gel electrophoresis bands showed that more than 80% of the plasmid has suffered at least one single strand cut in the case of 1Cu, and about 50% of the plasmid was nicked by 1Co. The differential cleavage activity is discussed in relation to the structural findings and a mechanism is proposed for 1Cu.

Several publications report strokes early after embolotherapy for pulmonary arteriovenous malformations (pAVM) ([1–3][1]). Various mechanisms are discussed for this event, including embolization of newly formed thrombotic material originating from the aneurysmal sac after occlusion of the arterial

Hydra, a member of the diploblastic phylum Cnidaria, exhibits the most basic type of organized metazoan tissues. Two unicellular sheets of polarized epithelial cells - ectoderm and endoderm - form a double layer throughout the body column. The double layer can be reestablished from single-cell suspensions by tissue-specific cell-sorting processes. However, the underlying pattern of interactions between ectodermal and endodermal epithelial cells responsible for double-layer formation is unclear. By analyzing cell interactions in a quantitative adhesion assay using mechanically dissociated Hydra epithelial cells, we show that aggregation proceeds in two steps. First, homotypic interactions within ectodermal epithelial cells (ecto-ecto) and within endodermal epithelial cells (endo-endo) form homotypic cell clusters. Second, at an aggregate size of about ten epithelial cells/cluster, ectodermal and endodermal clusters start to form heterotypic aggregates. Homotypic ecto-ecto interactions are inhibited by a polyclonal anti-Hydra membrane antiserum, and under these conditions homotypic endo-endo interactions do not proceed beyond a size of about ten epithelial cells/cluster. These data suggest that homotypic cell clusters reduce their initial homotypic affinity and acquire a new heterotypic affinity. A link between cell adhesion and cell signaling in early Hydra aggregates is discussed.

We introduce a new method of rotational image acquisition for four-dimensional (4D) optical coherence tomography (OCT) of beating embryonic chick hearts. The rotational axis and the central A-scan of the OCT are identical. An out-of-phase image sequence covering multiple heartbeats is acquired at every angle of an incremental rotation of the deflection mirrors of the OCT system. Image acquisition is accomplished after a rotation of 180◦. Comparison of a displayed live M-mode of the central A-scan with a reference M-mode allows instant detection of translational movements of the embryo. For calculation of 4D data sets, we apply an imagebased retrospective gating algorithm using the phase information of the common central A-scan present in all acquired images. This leads to cylindrical three-dimensional data sets for every time step of the cardiac cycle that can be used for 4D visualization.We demonstrate this approach and provide a video of a beating Hamburger and Hamilton stage 16 embryonic chick heart generated from a 4D OCT data set using rotational image acquisition. peerReviewed

Objectives: Recently introduced paediatric cardiac output monitor (COstatus, Transonic Systems Inc. Ithaca, NY) also measures blood volumes and detects cardiac shunts. The current study assessed its ability to identify left-to-right shunts and measured associated blood volumes during cardiac catheterisation in children. Patients and Methods: Cardiac catheterisation was performed in 20 children and adolescents (3.5 kg- 102 kg). Shunts and pulmonary (Qp) versus systemic blood flow (Qs) ratio were determined by routine oximetry. Subsequently three measurements with COstatus were performed while the monitor software announces the recognition of left-to-right shunt. Dilutions curves were recorded and validated. Results: Left-to-right shunts due to various types of cardiac defects were detected in 6 patients by oximetry. Applying COstatus diagnostic accuracy was very convincing, with a sensitivity of 100% (6/6) and a specificity of 92.8% (13/14). The threshold for determination a left-to-right shunt was an Qp/Qs ratio of 1.3. In one case of a hypertrophic obstructive cardiomyopathy the distortion of the dilution curve led to a false positive result. A significant difference was detected for means of central blood volume index between patients with left-to-right shunts and controls with no cardiac shunts (95% CI; 24.9 versus 18.2 ml/kg; P< 0.05). Conclusion: Novel ultrasound dilution technique identifies left-to-right shunts in children with a high sensitivity and detects even small shunts with an Qp/Qs ratio of 1.3. Raised central blood volume index corresponds well with clinical features in patients with left-to-right shunts and may become helpful a diagnostic parameter in the future.

Neonatal thrombocytopenia occurs in about 1% of all newborns. Inherited forms like 11q- or Jacobsen syndrome are rare. However, they may remain undetected with karyotyping because the deleted regions in 11q often involve small subtelomeric regions. Here we report on the detection of deletions in 11q in two newborns with normal routine karyotypes who were shown to carry subtelomeric deletions in 11q by means of fluorescence in situ hybridization (FISH) using a subtelomeric 11q probe (Abbott, Diagnostics, Wiesbaden, Germany). Both children showed thrombocytopenia (18.000/μl and 26.000/μl, respectively) and dysmegakaryopoiesis (absence of normal megakaryocytes and presence of micromegakaryocytes) associated with facial dysmorphism, cardiac defects and psychomotoric retardation. In the second case, the mother and the grandmother also showed mild thrombocytopenia. In both patients, FISH analyses on peripheral blood and bone marrow showed the loss of the telomere-associated region of 11q distal of the MLL gene. In the first patient, the deletion of 11q resulted from an unbalanced complex rearrangement with duplication of 11p. As the source of this chromosomal aberration, a paternal pericentric inversion of chromosome 11 was identified. The partial monosomy 11q and the partial trisomy 11p in the first patient were confirmed by comparative genomic hybridization (CGH) analysis. Array/matrix CGH assisted in determining the breakpoints at 11p15.1 and 11q24.1. No structural aberrations of 11q were found in the mother of the second patient, but further investigations are under way. These findings give further evidence that small subtelomeric deletions of 11q and probably mutations of genes located therein cause thrombocytopenia. Since it can be very difficult to detect these deletions by karyotyping, FISH using a subtelomeric 11q probe seems to be an extremely useful new diagnostic tool. This new method should be applied in children with congenital thrombocytopenia, in particular if they have additional complex dysmorphic features.