Wire-frame integrity of patch-like Gore devices following atrial septal defect closure.

OBJECTIVES: To assess the structural integrity of the patch-like Gore Septal Occluder (GSO) used for device closure of secundum-type atrial septal defects (ASD II) in pediatric patients. BACKGROUND: GSO has shown to be effective and safe for ASD device closure in children and adolescents. METHODS: Single-center, retrospective mid- to long-term follow-up of all children and adolescents with a GSO in situ (≥12 months). Periprocedural data and follow-up data were evaluated, including chest X-rays to assess the GSO's wire-frame morphology. RESULTS: Ninety-one consecutive patients were enrolled with a median age and weight of 5 years (range 2-18) and 20 kg (range 11-95) at implantation. ASD anatomy included 64 single and 27 multi-fenestrated defects, with 39 patients having small retro-aortic rims (≤4 mm). Median follow-up period was 42.5 months (range 12-74). Chest X-rays were available in 80 children: in 74 of them, the GSO's visualization on X-ray enabled us to reliably assess the wire-frame structure. Wire-frame fracture (WFF) was ultimately detected in five of the 74 patients (6.8%); however, those occluders appeared stably anchored and well aligned to both sides of the septum, and no free wire fragments had escaped the GSO matrix. Thus, no further treatment was required. CONCLUSIONS: Our data confirm that the GSO is safe and effective for ASD closure. Despite its lightweight construction, the GSO seems to offer reliable mechanical durability. Wire-frame fractures occur, but the free wire-ends appear to have remained stable within the GSO matrix without any clinical sequelae so far.

Transcatheter device occlusion of the left ventricular outflow tract as treatment for severe aortic regurgitation in hypoplastic left heart syndrome.

Aortic regurgitation is a rare but dangerous condition in patients with hypoplastic left heart syndrome (HLHS). We report the case of a 2-year-old girl with HLHS with aortic/mitral stenosis (AS-MS) subtype after stage II palliation via the bilateral bidirectional Glenn (BBDG) procedure, when aortic regurgitation presented with the clinical manifestation of a paroxysmal coronary steal phenomenon provoked by exertion. Transcatheter closure of the left ventricular outflow tract (LVOT) with an Amplatzer(TM) Vascular Plug 4 minimized and finally abolished aortic reflux without compromising either coronary perfusion or atrioventricular (AV) conduction. Midterm follow-up over 3 years revealed complete disappearance of her symptoms, and follow-up catheterization displayed complete LVOT closure. This case illustrates the promising potential of modern cardiac catherization techniques in certain cases and emphasizes that precise assessment of the native aorta and coronary system is critical in HLHS patients.

Transcatheter closure of atrial septal defects in children and adolescents: single-center experience with the GORE® septal occluder.

OBJECTIVES: Device closure of atrial septal defects (ASD II) is an alternative to surgery. ASD morphology and intracardiac relationships may influence device selection. Biocompatibility, techniques for closing large or multiple defects, and the risk of erosion are main issues in children BACKGROUND: The GORE® Septal Occluder (GSO) is a non-self-centering device with a flat and conformable nitinol-frame covered with expanded polytetrafluoroethylene. We hereby evaluate our initial experience in children to demonstrate feasibility, efficacy, and safety. METHODS: Single-center retrospective intention-to-treat analysis of GSO procedures in children and adolescents with hemodynamically significant ASD. All procedures were conducted under general anesthesia, fluoroscopy and transesophageal echocardiography. All defects were sized using stop-flow-technique. GSOs of 20-30 mm disc-diameter were implanted. RESULTS: GSO implantation was attempted in 45 with technical success in 41 patients, median age 6 (range 3-17) years, bodyweight 19 (12-95) kg. Median balloon-sized diameter was 13 (8-19) mm resulting in a GSO-to-ASD-ratio of 2 (1.7-2.5). Deficient retro-aortic rims (<3 mm) were found in 12 patients, and 10 of these 12 defects could be closed. We observed no complications. Four extensive or multiple ASDs were not treated successfully with GSO. After retrieval, one was closed with a different device; three patients needed surgical therapy. The median follow-up of four (range 0.2-22) months was uneventful in all patients CONCLUSIONS: We found the GSO device closure of ASDs in children to be effective. However, our initial experience is limited to short- and mid-term-follow-up of a low number of patients.

Analysis of the bone metabolism by quantitative computer tomography and clinical chemistry in a primate model (Callithrix jacchus).

BACKGROUND: Common marmosets are widely used as experimental primates; however, little is still known about their bone physiology. Therefore, the aim of our study was to analyse body weight, age and bone-specific blood parameters in relation to morphological bone parameters. METHODS: Fifty-eight common marmosets were analysed for blood calcium (Ca), inorganic phosphor (P(i) ), alkaline phosphatase (AP) and 17-ß-estradiol (E2). The examination of bone parameters was undertaken in the lumbar spine by computer tomography. RESULTS: There was a correlation between bone mineral density (BMD) and body weight, trabecular area ratio and polar moment as well as between BMD and AP or Ca (only males), whereas there were no correlations between BMD and age, P(i) or E2 in all analysed genders. CONCLUSIONS: Our data support the assumption that the common marmoset is a reliable primate model to study changes in bone metabolism because of the similarity of our results to humans.

The conclusiveness of less-invasive imaging techniques (computer tomography, X-ray) with regard to their identification of bone diseases in a primate model (Callithrix jacchus).

BACKGROUND: Although common marmosets seem to be appropriate animal models to examine bone diseases, no data about the conclusiveness of less-invasive techniques are available. Therefore, the aim was to combine different techniques to analyse changes in bone metabolism of common marmosets with bone diseases. METHODS: Five monkeys were examined by X-ray, computer tomography (CT), histology and immunohistochemistry (IHC). RESULTS: Monkeys with lowest bone mineral density (BMD) showed increased bone marrow, decreased cancellous bone and decreased contrast in X-ray. Highest alkaline phosphatase (AP)-levels were detected in bones with low elastic modulus. Expression of osteopontin (OPN), osteocalcin (OC) and runt-related transcriptions factor 2 (RUNX 2) was detected in bones with high modulus. No expression was present in bones with lower modulus. Collagen type I and V were found in every bone. CONCLUSIONS: In conclusion, CT, X-ray and AP are useful techniques to detect bone diseases in common marmosets. These observations could be confirmed by IHC.

Parameters of haematology, clinical chemistry and lipid metabolism in the common marmoset and alterations under stress conditions.

Common marmosets are suitable non-human primate models for many human diseases. Standard values for blood parameters are required to evaluate physiological and pathological situations. Two studies were conducted: study I to determine standard values and study II to examine these under changed housing conditions. In study I, all parameters for clinical chemistry were similar in range for both genders with these specifics: male marmosets had significantly higher total and LDL cholesterol levels than females, whereas the mean corpuscular volume and the mean corpuscular haemoglobin were significantly lower than in females. In study II, glucose, lymphocytes and salivary cortisol were significantly lower, and faecal cortisol was increased during the change of housing conditions. In conclusion, standard values for haematology and clinical chemistry for the common marmoset were determined. Further on, parameters that are influenced by relocation stress and its importance for experimental results are described.

Prophylactic use of clopidogrel in paediatric cardiac patients.

Thromboembolic complications in infants with congenital heart defects are common despite inhibition of platelet function with acetylsalicylic acid (ASS). Yet there is still insufficient pharmacologic data on the use of clopidogrel in infants. The adult dose of 75 mg/d is significantly higher than the dose lately recommended in infants (0.2 mg/kg/d). Moreover, we know of nonresponders to both acetylsalicylic acid and clopidogrel. Normal coagulation tests fail to identify those patients.Prospective monocentric study on 14 children (median age 5, range 0.7-84 months, 9 male, 5 female). Shunt thrombosis had occurred in 4 infants on ASS therapy. Seven days after starting clopidogrel (0.2 mg/kg/d), platelet function was tested by stimulation with ADP (4 and 10 µmol/l). We considered the range for the clopidogrel effect to be optimal if the maximum aggregation on ADP 4 µmol/l was between 30-50%.Clopidogrel 0.18-0.24 mg/kg/d in addition to ASS 2-4 mg/kg/d resulted in effective inhibition of platelet function in 93% (ADP 4 µmol/l: median 38%, range 30-63). All patients were responders. We observed neither any thromboembolic events nor severe bleeding episodes during the median 11-month follow-up period (range 1-30 mo).Testing platelet function makes clopidogrel dosing safer, and simplifies therapy adjustments in long-term treatment. A clopidogrel dose of 0.2 mg/kg/d was safe and effective in combination with ASS in this small patient cohort.

4D-MR flow analysis in patients after repair for tetralogy of Fallot.

OBJECTIVES: Comprehensive analysis of haemodynamics by 3D flow visualisation and retrospective flow quantification in patients after repair of tetralogy of Fallot (TOF). METHODS: Time-resolved flow-sensitive 4D MRI (spatial resolution ~ 2.5 mm, temporal resolution = 38.4 ms) was acquired in ten patients after repair of TOF and in four healthy controls. Data analysis included the evaluation of haemodynamics in the aorta, the pulmonary trunk (TP) and left (lPA) and right (rPA) pulmonary arteries by 3D blood flow visualisation using particle traces, and quantitative measurements of flow velocity. RESULTS: 3D visualisation of whole heart haemodynamics provided a comprehensive overview on flow pattern changes in TOF patients, mainly alterations in flow velocity, retrograde flow and pathological vortices. There was consistently higher blood flow in the rPA of the patients (rPA/lPA flow ratio: 2.6 ± 2.5 vs. 1.1 ± 0.1 in controls). Systolic peak velocity in the TP was higher in patients (1.9 m/s ± 0.7 m/s) than controls (0.9 m/s ± 0.1 m/s). CONCLUSIONS: 4D flow-sensitive MRI permits the comprehensive evaluation of blood flow characteristics in patients after repair of TOF. Altered flow patterns for different surgical techniques in the small patient cohort may indicate its value for patient monitoring and potentially identifying optimal surgical strategies.

Preoperative selective embolization with vascular coiling of giant sacrococcygeal teratoma.

Sacrococcygeal teratoma is one of the most common congenital tumors. Its optimal management requires interdisciplinary care by obstetricians, radiologists, pediatric surgeons, and neonatologists. Early surgery entailing complete tumor excision is the main therapy aim, but a substantial risk of life-threatening complications remains, especially uncontrollable intraoperative hemorrhage. To reduce the risk of bleeding in a female neonate with a giant sacrococcygeal teratoma, we successfully coil-embolized the tumor's main feeding arteries. Her subsequent complete surgical resection was uneventful, and the child is well with favorable reconstructive and functional status of all involved and adjacent organ systems.

Bronchial compression following pulmonary artery stenting in single ventricle lesions: how to prevent, and how to decompress.

OBJECTIVES: To assess airway compression during pulmonary artery (PA) intervention in single ventricle (SV) palliation. BACKGROUND: SV lesions with a prominent neo-aortic root are considered a high risk for branch PA and/or bronchial stenosis. PA stenting is well established, but may result in ipsilateral bronchial compression. METHODS: Single-centre retrospective analysis of 19 palliated SV patients with branch PA stenosis and close proximity to the ipsilateral main bronchus who underwent cardiac catheterisation at a median age and weight of 8.5 years (0.5-25) and 16.5 kg (6-82) between 12/2011 and 05/2015. RESULTS: Two of the 19 patients suffered an almost-closed left-main bronchus (LMB) following PA stenting. Fortunately, LMB decompression succeeded in both those patients by re-shaping the PA stents by compressing the chest while splinting the LMB with an inflated balloon. To prevent the other 17 patients from suffering this serious complication, we adopted a thorough preparation strategy: 13 patients underwent safe simultaneous bronchoscopy and cardiac catheterisation; in the remaining 4 patients CT-angiography enabled accurate risk evaluation prior to re-catheterisation. CONCLUSIONS: In SV lesions accompanied by branch PA stenosis, thorough preparation via cross-sectional imaging is mandatory, including simultaneous bronchoscopy and cardiac catheterisation in selected cases, to rule out any airway compression before considering endovascular stent implantation. If a PA stent's compression has already caused severe bronchial obstruction, our balloon-splinted decompression technique should be considered.

Comparison of two methods of goniometry.

This investigation was conducted to determine if there are significant differences between the lateral and the over-the-joint methods of goniometry for measurements of the elbow joint. Forty physical therapy students with previous goniometric training were divided randomly into four goniometric measurement groups: 1) lateral method for an obtuse angle, 2) lateral method for an acute angle, 3) over-the-joint method for an obtuse angle, and 4) over-the-joint method for an acute angle. All students in each group participated in four sessions during which a proctor's elbow joint was measured according to the method of the assigned goniometric measurement group. The joint was held in a fixed position by a plywood stabilizing device. The results of a three-factor analysis of variance with repeated measures on one factor showed that there was no difference between the lateral and the over-the-joint methods of goniometric measurement of the elbow joint.

Experimental model for heart failure in rats--induction and diagnosis.

BACKGROUND: Clinical heart failure is generally preceded by hypertrophy. Many animal models (e. g. toxic heart failure models) do not consider this hypertrophy. We set out to develop a heart failure model in rats by inducing pressure-overload hypertrophy. METHODS: We induced coarctation of the aortic arch with a tantalum clip (0.35 mm internal diameter) In 3-week-old rats (n=17). Starting at seven weeks postoperatively, we measured ejection fraction (EF), fractional shortening (FS), end-systolic (LVESD) and end-diastolic (LVEDD) left ventricular dimensions by echocardiography each week. Heart, lung, and liver specimens were analyzed histopathologically at least eleven weeks after the operation. RESULTS: Contractile function was significantly decreased in hearts from animals with aortic banding (EF: 45+/-5% vs. 73+/-5%, p<0.01; FS: 20+/-3% vs. 35+/-5%, p<0.01). At the same time, left ventricles were dilated (LVEDD: 9.1+/-0.6 mm vs. 7.4+/-0.5 mm; LVESD: 7.3+/-0.6 mm vs. 4.8+/-0.4 mm, p<0.01). These observations were associated with clinical and histopathological changes characteristic for chronic left heart failure. CONCLUSION: Placing a tantalum clip around the aortic arch in 3-week-old rats consistently induces left ventricular decrease in contractile function and dilatation after eleven weeks.

Arginine506 to glutamin mutation in the factor V gene in infancy and childhood: evidence of fibrinolytic impairment.

UNLABELLED: Resistance to activated protein C (APCR), in the majority of cases due to arginine506 (Arg506) to glutamine (Gln) mutation in the factor V gene, has emerged as the most important hereditary cause of venous thrombo-embolism. To determine to what extent this relatively common gene mutation influences the fibrinolytic system we investigated a population of APC resistant children (n = 65) in comparison with a control group of sex- and age-matched healthy children (n = 100). Compared to the controls, plasma levels of tissue-type plasminogen activator (t-PA), urokinase-type plasminogen activator (u-PA) and plasminogen activator inhibitor (PAI) 1 antigen, D-Dimer and enhanced thrombin generation were significantly (P < 0.0001) increased in children with the common factor V mutation. No difference was found between symptomatic and non-symptomatic children. Whether high concentrations of t-PA, u-PA and PAI 1 antigen can predict future vascular occlusion in children with APCR requires a more extensive multicentre study. CONCLUSION: Our data indicate that hypercoagulability in children with the Arg506 to Gln mutation in the factor V gene is mainly attributed to the genetic aetiology of the disease.

[Abuse of artificial sweetener as differential diagnosis of lactose intolerance]. / Süssstoffabusus als Differentialdiagnose der Laktoseintoleranz.

A two year old dystrophic boy with chronic diarrhea was described. At the age of 6 month the boy developed a severe gastroenteritis with a secundary lactase deficiency. Dietary treatment was however not successful and consequently the boy became dystrophic. At last, an abuse of sweetener was diagnosed.