Application of Dual-Source-Computed Tomography in Pediatric Cardiology in Children Within the First Year of Life.

PURPOSE: To assess fields of application and value of dual source computed tomography (DSCT) for diagnostics and therapy in patients with congenital heart disease during their first year of life. Evaluation of image quality, surgical use and radiation exposure of 2nd and 3 rd generation DSCT. MATERIALS AND METHODS: DSCT was applied in 118 cases between January 2012 and October 2014 for diagnostics of congenital heart defects. 2nd generation was used in 91 cases until April 2014 and 3 rd generation in 27 cases during the period thereafter. 3 D reconstructions of the image data were created for clinical diagnostics and planning of interventions. Image quality was assessed using a 4-point-scale. The visibility of the mammary arteries was analyzed, and signal-to-noise-ratio (SNR) and contrast-to-noise-ratio (CNR) were calculated. The usefulness of 3D-reconstructions for surgical planning was rated using a 5-point-scale. Radiation exposure and contrast dye consumption were determined. All cases were analyzed retrospectively. RESULTS: DSCT was successfully used in 118 cases. All image data obtained were interpretable. More than 60 percent of cases did not show any artifacts. The mammary arteries were visible down to the diaphragmatic arch in more than 80 percent of cases. Diagnostic value and surgical benefit were evaluated as "useful" or as "essential" in all cases. Median radiation dose was 0.4 mSv and 0.27 mSv for the 2nd and 3 rd generation DSCT, respectively. Consumption of contrast dye was 2 ml/kg in all cases. CONCLUSION: DSCT is a modern and extremely helpful technique for diagnostics and planning of interventions in patients with complex congenital heart defects. Extracardiac vascular structures in particular can be depicted three-dimensionally at high resolution. The use of iterative reconstruction with 3 rd generation DSCT yielded image quality similar to that of 2nd generation DSCT at considerably reduced radiation exposure level compared to 2nd generation DSCT. 3 rd generation DSCT is a low risk, accurate and extremely fast technique for diagnosing unstable patients with CHD. KEY POINTS: Expanded scope of indications for DSCT in diagnosing critically ill infants. Effective radiation dose is considerably lower than 0.5 mSv. Extremely rapid image acquisitions with high image quality. Possibility of optimized 3D-based surgical planning

Cardiac MRI in Children and Adolescents Who Have Undergone Surgical Repair of Right-Sided Congenital Heart Disease: Automated Left Ventricular Volumes and Function Analysis and Effects of Different Manual Adjustments.

PURPOSE: To evaluate automated segmentation and the effects of different manual adjustments regarding left ventricular parameter quantification in cardiac magnetic resonance (MR) data on children and adolescents who have undergone surgical repair of right-sided congenital heart disease (CHD). MATERIALS AND METHODS: Dedicated software (syngo.via, Siemens AG) was used to automatically segment and/or manually adjust the end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), myocardial mass (MM) and ejection fraction (EF) before/after manual apex/base adjustment (ADJ-step 1) and after manual apex/base/myocardial contour adjustment (ADJ-step 2; reference standard). MR data of 40 patients (13.1 ± 3. y, 4 - 17 y) with repaired CHD with decreased pulmonary blood flow (CHD-DPBF) were evaluated. Intra- and inter-rater reliability was determined for 10 randomly selected patients. RESULTS: The software correctly detected the left ventricle in 38/40 (95%) patients. EDV after automated segmentation: 119.1 ± 44.0 ml; after ADJ-step 1: 115.8 ± 9.5 ml; after ADJ-step 2: 116.2 ± 39.4 ml. The corresponding results for ESV were 52.0 ± 18.5/49.6 ± 6.9/49.7 ± 16.4 ml; for SV 67.1 ± 28.5/66.2 ± 25.4/66.5 ± 25. ml; for EF 55.5 ± 7.3/56.7 ± 6.6/56.7 ± 6.3%; for MM 83.7 ± 35.9/76.2 ± 8.3/74.6 ± 27.2 g. Significant differences were found for ESV/MM/EF comparing the automated segmentation results with these after ADJ-step 1 and ADJ-step 2. No significant differences were found when comparing all results of ADJ-step 1 and ADJ-step 2 or when comparing EDV/SV results. Intra- and inter-rater reliability was excellent. The mean time effort was 63.4 ± 6.9 s for the automated segmentation, 74.2 ± 8.9 s for ADJ-step 1 and 269.5 ± 39.4 s for ADJ-step 2. CONCLUSION: Automated left ventricular volumes and function analysis in children and adolescents with surgically treated CHD proved to be feasible with excellent intra- and inter-rater reliability. Automated segmentation with manual apex/base adjustment provided clinically acceptable results. KEY POINTS: Automated left ventricular volume and function analysis in children and adolescents with surgically treated right-sided heart disease is feasible with excellent intra- and inter-rater reliability. Automated segmentation with manual apex/base adjustment provides clinically acceptable results. Additional manual myocardial contour adjustment does not significantly improve the results.

[Flat detector computed tomography in diagnostic and interventional pediatric cardiology]. / Flachdetektor-Computertomografie in der diagnostischen und interventionellen Kinderkardiologie.

PURPOSE: In this study the use of flat detector computed tomography (FD-CT) in the catheterization of patients with congenital heart disease was evaluated. Application reports were created for various issues based on the achieved image quality in diverse anatomical regions. MATERIALS AND METHODS: FD-CT was applied in 176 cases during catheterization between January 2010 and April 2012. A five-point Likert scale ("essential" to "misleading") was used to evaluate image quality. All cases were analyzed retrospectively and application reports for the visualization of the aorta, pulmonary arteries, pulmonary veins, semilunar valves, cavopulmonary connections and atrial baffles were generated. Contrast dye consumption and radiation dose were evaluated. RESULTS: During the observation period FD-CT was applied in all 176 cases. The mean patient age was 7.0 years (0.01 - 42.53 years). The clinical value of FD-CT was rated superior to conventional angiography in 96.6 % of the cases and was never rated as "misleading". FD-CT was rated "essential" in 3.4 % of all cases, "very useful" in 77.3 % of all cases, "useful" in 15.9 % of all cases and "not useful" in 3.4 % of all cases. The mean dose-area product was 99 µGym2 (19.3 - 1276.6 µGym2), and the used contrast dye was 1.76 ml/kg (0.9 - 5 ml/kg). Application reports for the visualization of different anatomical regions are demonstrated. CONCLUSION: FD-CT is a new and auxiliary procedure in diagnostic and interventional catheterization of patients with congenital heart disease. Particularly extracardiac structures can be displayed in three-dimensional high resolution and be used for diagnosis, surgical planning and 3 D navigation.

Pericardial cyst or teratoma? Change of strategy during mediastinal tumor surgery.

This report describes the management of an 8 × 8-cm cystic mass that arose from the anterior mediastinum and prolapsed into the right pleural cavity adherent to the pericardium in an asymptomatic 16-year-old girl. The patient was scheduled for a video-assisted thoracoscopic operation with exposure, puncture and suction of a suspected pericardial cyst. However, during the procedure the strategy was changed due to the solid consistency of the mass, and the lesion was extirpated in toto by a short anterolateral thoracotomy. The complete histopathological investigation showed a highly fibrous, cystic, mature teratoma.

First description of three patients with multifocal lymphangiomatosis and protein-losing enteropathy following palliation of complex congenital heart disease with total cavo-pulmonary connection.

The objective was to identify abdominal lymphatic malformations in pediatric patients with protein-losing enteropathy after palliation of complex congenital heart disease with total cavo-pulmonary connection (TCPC). In 2006, we performed complete hemodynamic and laboratory workup and thoracic and abdominal MRT screens in three patients who newly presented with symptoms of protein-losing enteropathy. All three patients, aged 3, 5, and 7 years, showed excellent TCPC hemodynamics with central venous pressures of 10-13 mm Hg. None of the patients had right-to-left overflow. All three patients showed extensive thoracic and mesenterial lymphangiomatosis. One patient died after 18 months of therapy, which included long-term parenteral nutrition, somatostatin, subcutaneous heparin injections, and frequent albumin and immunoglobulin substitution. The other two patients are in stable condition. Lymphangiomatosis might play an unknown role in the pathogenesis of protein-losing enteropathy after TCPC. It remains unclear whether lymphangiomatosis is a primary congenital disease related to the cardiac disease or if it is triggered by repeated surgery or venous congestion. The presence of lymphangiomatosis should be given diagnostic and therapeutic consideration in TCPC patients in the future.

[Effect of endoscopic hydrothermo- and laser coagulation on the human stomach wall in vivo]. / Die Auswirkung der endoskopisch angewandten Hydrothermo- und Lasercoagulation auf die humane Magenwand in vivo.

Effect of thermic and laser energy applied onto human in vivo gastric wall has not yet been reported in literature. In our study we evaluated the maximum amount of energy not harming the patient as well as principles for secure and sufficient therapy. In 8 patients hospitalized for gastric resection we applied vaporization by laser- and hydrothermosounds in this part of the stomach which should be resected. Endoscopic pictures were taken. We used a NdYAG laser (maximum performance 70 W, time of application 1-3 s) and hydrothermosounds (maximum performance 170 W, time of application 1-3 s). The stomach was resected 3-8 days following application. Comparing laser- and hydrothermosounds marks we observed a bigger area of necrosis at hydrothermosounds marks using the same amount of energy. In histological investigation correlation between depth and diameter of necrosis was found. After the same application time both depth and diameter of necrosis were bigger by hydrothermosounds than by laser. Lesions reached serosa at the maximum time of application of 3 s. Serosal lesion itself did not appear. Endoscopic treatment of tissue lesion by laser and thermic irradiation (vaporization of bleeding polyp pedicles, treatment of tumors) is secure using the maximum energy mentioned above. Serosal lesion did not appear. Bleeding lesions must be treated by higher energy because of absorption of energy by escaped blood.

[Therapy of esophageal varices using flexible endoscopes]. / Zur Therapie von Osophagusvarizen mit flexiblen Endoskopen.

Fibroendoscopic sclerotherapy of esophageal varices during or after a bleeding period is an effective method with low risks. From 1977 to June 1981, 60 patients underwent 125 sclerotherapy sessions. In 61,5% this palliative therapy had good results. 27% died during the first stay in hospital, 11,5% of them due to severe attacks of bleeding. Another 11,5% had rebleeding episodes later on. The complication rate of this method is 0,9%, the mortality rate 0%.

[Lung denervation. A study of therapy of shock lung]. / Die Lungendenervierung. Ein Versuch zur Therapie der Schocklunge.

It is the objective of this experiment to study the protective effect of pulmonary denervation in acute respiratory distress syndrome (shock lung). The experiments were performed by means of a model of pulmonary embolization with oleic acid in dogs. In the first series sequential tissue changes in the lung following oleic acid embolism are shown and the clinical changes are demonstrated. By a meticulous description of the anatomy of pulmonary innervation it is demonstrated that surgical denervation of the lung is only possible in the hilar area. A second series of experiments shows the effect of oleic acid embolism in animals after reimplantation of one lung. This means that the reimplanted lung is completely denervated. A description of the technique of pulmonary reimplantation is also given. It is stated that following oleic acid embolism, the reimplanted lung is less damaged than the contralateral innervated lung. In addition, animals with unilateral lung reimplantation have significantly better pulmonary function after oleic acid embolism than healthy animals with bilaterally innervated lungs. Finally, a technique of reversible bilateral denervation of the lung by transbronchial infiltration of both hili with a long acting local anaesthetic via bronchoscopy is described. Animals, whose lungs were denervated in this way were embolized with oleic acid. It could be shown that as long as the reversible denervation is effective there is no significant functional reaction following oleic acid embolism as compared to healthy and non-denervated animals.

[Modern functional and physiological techniques in abdominal surgery (author's transl)]. / Moderne funktionsgerechte physiologischen abdominalchirurgische Verfahren.

The increasing importance of physiological and functional surgical procedures in the surgical therapy of benign abdominal disease is implied. Positive results were achieved at the 2nd Department of Surgery of the University of Vienna following parietal cell vagotomy in hypersecretory gastroduodenal ulcer, latero-lateral pancreatico-jejunostomy according to Puestov-Mercadier in chronic relapsing pancreatitis, distal splenorenal shunt according to Warren in portal hypertension and following peritoneo-venous shunt according to Warren in portal hypertension and following peritoneo-venous shunt according to Le Veen in ascites and cirrhosis of the liver.

In vitro evidence of cellular and humoral immune responses following lung allotransplantation in canine recipients with and without immunosuppressive treatment.

Using donor lung antigens as targets, two test methods, a rosette-forming cell test (RFC) and indirect immunofluorescence test (indir. IF), were applied to focus on the first set lung allograft rejection in canine recipients with and without immunosuppressive treatment. In both groups the first sign of humoral or cellular sensitization became evident on the 4th postoperative day, which might answer some unsolved problems in early lung graft damage, recently discussed as a manifestation of certain immunologic reactions. The tests used, indir. IF and RFC, revealed a steadily climbing tendency of immunization in untreated recipients and more variable and reversible positive results in the immunosuppressively treated group. No correlation between cellular and humoral immunity could be proven by our tests. The fact that we found no incorrect positive results in RFC suggests that this test provides important hints for the diagnosis of lung allograft rejection.

Donor- and organ-specific evaluation of antibodies eluted from canine lung allografts rejected by immunosuppressively treated and untreated recipients.

Using elution techniques, the humoral lung allograft rejection in immunosuppressively treated versus untreated recipients is analyzed at the donor organ specific level. The antibodies were evaluated quantitatively and qualitatively by elution of lung graft bound immunoglobulins and by testing the eluates against donor lung antigen using passive hemagglutination and indirect immunofluorescence. By correlating the results in those assays, a considerable amount of humoral anti-donor lung antibodies could be proved only in dogs not treated immunosuppressively, and there was no accordance with the results of direct immunofluorescence by quantification. The difference in the organ-bound antibodies between immunosuppressively treated and untreated lung grafts seems to be remarkable because of a similar mononuclear infiltration. Thus, enabling a specific improvement of some previous speculations about the lung-specific humoral alloimmune reaction, this type of rejection seems to be similar to rather stereotypical allograft rejection, but may be modified by a standard immunosuppression with methylprednisolone and azathioprine.

[The problem of anti-lung basal membrane immunity in the framework of allogenic lung transplant rejection]. / Zur Problematik der Antilungenbasalmenbran-Immunität im Rahmen der allogenen Lungentransplantatabstossung.

This study is concerned with the significance of autoimmune processes caused by the damage of lung tissue after lungallo- and autotransplantation. In 15 mongrel dogs a left side lung-allotranplantation was performed, 5 of them were treated with immunsuppressive therapy in the posttransplantation period. A group of 5 dogs with autotransplantation of the left lung served as a control-group. In the posttransplantation period the development of humoral antibodies responding with lung basal membrane antigens was examined by daily taken sera as well as by elution of immuneglobulins from the rejected grafts in the passive hemagglutination and compared with the development of alloantibodies against donor-lung's tissue. Besides of regularly traceable humoral alloantibodies of lung allograft recipients in the posttransplantation period, in no group antilung basal membrane antibodies could be found.

[Bronchospirometric results after single lung homotransplantation in dogs with experimentally induced pulmonary emphysema (author's transl)]. / Bronchospirometrische Ergebnisse nach einseitiger Lungenhomotransplantation an Hunden mit experimentell erzeugtem Lungenemphysem

Pulmonary emphysema was induced in 8 bastard dogs by intratracheal instillation of Papain. Development of emphysema was documented by pulmonary function tests. The emphysematous animals underwent single lung homotransplantation. Postoperative bronchospirometric measurements revealed that up to the 6th week after transplantation there is no evidence of serious ventilation perfusion inbalance.

[Antibiotic therapy with doxycycline in dogs with lung transplantations (author's transl)]. / Antibiotische Behandlung lungentransplantierter Hunde mit Doxycyclin.

In contrast with kidney, heart and liver transplantations, lung transplantation involves an organ which has contact with the environment and bears a special risk of infection. This circumstance and the diminished resistance during immunosuppressive therapy makes long-term treatment with an effective broad-spectrum antibiotic advisable for lung transplantations. Taking into consideration the different objectives and questions within the research project concerned the 182 lung allotransplantations carried out in dogs can be divided into two therapeutic groups in regard to postoperative treatment: lung allotransplantations with and without immunosuppressive therapy (methylprednisolone, azathioprine). Long-term doxycycline therapy was carried out in both groups. Those animals which were not treated with immunosuppressive drugs survived for an average of 9 days, those receiving immunosuppressive therapy survived for an average of 30 days. The longest survival period was 2.5 years. Clinically and histologically it could be demonstrated that doxycycline was successful both during the immediate postoperative period and in antibiotic long-term therapy after experimental lung transplantations.

[Complement system and lymphocytotoxic antibodies in presensitized dogs after homologous lung transplantation ]. / Komplementsystem und lymphozytotoxische Antikörper nach homologer Lungentransplantation an sensibilisierten Empfängerhunden

Left side orthotopic homotransplantation of the lung was performed in 8 mongrel dogs pre-sensitized by full-thickness skin transplants. The degree of sensitization was assessed by control of the lymphocytotoxic antibody titre. The behaviour of haemolytic complement activity, as well as the alterations in antibody titre were recorded up to 60 minutes after opening of the anastomoses. Evaluation was performed by analyses of significance. A significant decrease in lymphocytotoxic antibody titre (p less than 0.001), as well as a significant diminution in complement activity (p less than 0.01) was observed in blood samples from the pulmonary vein of the graft during the first five minutes following recirculation. This resulted from an interaction of both systems in the immune reaction. The further complement consumption by the graft in contrast to the constancy of the lymphocytotoxic antibody titre in the subsequent post-transplantation period can be explained by antibody-independent complement activation. Vena cava-- pulmonary vein differences in the lymphocytotoxic antibody titre and complement activity are correlated only five minutes after opening of the anastomoses (r = 0.75), which confirms the independence of both systems during later course of the reaction.

[Specific humoral immunity in the circulation of dogs with allotransplanted lungs]. / Spezifische humorale Immunität in der Zirkulation von lungenallotransplantierten Hunden.

A method appropriate for identification and quantification of circulating antibodies reacting with donorlungantigens is presented in allogenous lungtransplantation. Following incubation of native tissue-slides taken from the second removed but not grafted donor lung with the recipient's serum, the specific humoral antibodies are detected by indirect immunfluorescence. Quantifying evaluation is performed by serum-dilutions. The preexisting specific antibody-titer found in DLA-sensitized animals shows a significant correlation with the duration of graft function when compared with lymphocytotoxic antibody-titer. In unsensitized dogs a regular appearance of circulating specific antibodies can be seen beginning from the fourth or fifth postoperative day. The increasing antibody-titer during further course is a feature of a humoral graft rejection represented in the periphereal blood-circulation.

[Roentgenmorphologic findings in single homotransplantation of non immunosuppressive treated canine lungs (author's transl)]. / Röntgenmorphologie der unbehandelten homotransplantierten Hundellnge

According to plain chest roentgenography correlated to needle biopsies the following grades in the course of rejection after single lungtransplantation could be evaluated. 1) Begin of rejection: perihilar alveolar infiltrate. 2) Increase of the infiltrates (positive Airbronchogram). 3) Almost total consolidation of the graft. 4) Complete consolidation of the graft, mediastinal hernia caused by the own lung.