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

Functional null mutations in the gonosomal homologue gene TBL1Y are associated with non-syndromic coarctation of the aorta.

In patients with congenital heart defects, chromosomal anomalies are 100 times more frequent than in control subjects. Coarctation of the aorta can be detected in 15-20% of patients with Ullrich-Turner syndrome. By extensively reviewing literature involving breakpoint analysis of gonosomal deletions in Ullrich- Turner syndrome patients with and without coarctation of the aorta, we identified several gonosomal homolgous gene pairs of interest. Four of these homologous gene pairs were investigated by standard DNA sequencing in a cohort of 83 patients with non-syndromic coarctation of the aorta. Subsequently stability of mutant RNA and protein was analyzed to verify functional relevance of detected mutations. We identified two unreported missense mutations in Exon 8 (p.D69H) and 9 (p.R176W) of TBL1Y. Bioinformatic analysis and 3D modelling predicted that both mutations lead to TBL1Y loss of function. In RT-PCR and Western blot analyses of HEK293 cells transfected with a vector carrying the full-length TBL1Y (wild-type and mutant), we documented the predicted protein instability by showing protein decay for both mutant proteins. TBL1Y is similar to its gonosomal homologue, TBL1X, and its autosomal homologue, TBLR1, on chromosome 3. Both genes are part of co-repressor machineries and required for transcriptional activation by transcription factors that involve CtBP1/2, which contributes to Notch signaling. Several studies have shown that Notch signalling is important for proper development of the left ventricular outflow tract. Our findings suggest that TBL1Y is involved in the genesis of non-syndromic coarctation of the aorta.

Aortic arch repair: let it beat!

Objective aortic arch repair (AAR) on the beating heart may reduce cross-clamping times and offer improved postoperative cardiac function.Methods A single-center review of all patients (n = 24) who underwent surgical AAR during biventricular repair between 01/2006 and 01/2008 was done. All patients were operated on under cardiopulmonary bypass (CPB) with antegrade cerebral perfusion (ACP). During AAR, 13 patients (group 1) received cardioplegic arrest, and were compared to 11 patients (group 2) who underwent a beating-heart modification with selective myocardial perfusion. Seventeen patients had additional intracardiac lesions and underwent simultaneous correction during the procedure.Results Durations of CPB, AAR and ACP did not differ statistically between groups. Cardioplegic arrest time was significantly lower in group 1 (34 ± 13 vs. 76 ± 11 min, p = 0.02) and resulted in a subsequent reduction of myocardial ischemic damage as borne out by lower postoperative levels of troponin T and CK-MB (2.5 ± 0.7 vs. 7.1 ± 1.4 ng/mL, p = 0.02; 68.7 ± 11.5 vs. 149.1 ± 27.2 U/l, p = 0.03). We observed an enhanced patient recovery with shorter inotropic and ventilatory support times (p < 0.05).Conclusion Pediatric aortic arch correction on a CPB beating heart with selective myocardial perfusion is technically feasible and safe. The reduction of the myocardial ischemic time is effective and results in less myocardial damage.

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.

The Norwood procedure - does the type of shunt determine outcome?

BACKGROUND: Stage I palliation of hypoplastic left heart syndrome (HLHS) and its variants is usually performed by a Norwood operation. The management of pulmonary blood flow during this procedure remains controversial. The RV-to-PA conduit (RVPAC) has been proposed as the better alternative compared to a systemic-to-pulmonary shunt (SPS). METHODS: A retrospective single center chart review of consecutive patients who underwent a Norwood I procedure between 01/1997 and 09/2006 was performed. All patients were operated in deep hypothermia, with or without circulatory arrest, using different shunt modifications according to surgeon's preference. Patients were divided into two groups depending on surgical management for pulmonary blood flow (modified BT shunt [BT] and non-valved RVPAC [Sano]). RESULTS: Fifty-four patients were included in the study (BT: 31 patients vs. Sano: 23 patients). Diastolic blood pressure during the first 24 hours postoperatively was significantly lower in the BT group (BT: 38.6 +/- 6.9 mmHg vs. Sano: 42.4 +/- 7.2 mmHg; P < 0.01) with a trend towards a higher systolic blood pressure (BT: 74.1 +/- 13.5 mmHg vs. Sano: 69.8 +/- 12.1 mmHg; P = 0.08). Mean circulatory arrest time in the BT group was significantly longer compared to the Sano patients (BT: 41 +/- 21 min vs. Sano: 25 +/- 23 min; P < 0.01). The mean hospital stay was 18.5 days for BT patients and 20 days for Sano patients ( P = 0.45). Early mortality for the total cohort was 14.8 % (n = 8) (BT 19.4 % [n = 6] vs. Sano 8.7 % [n = 2]; P = 0.12). There was no significant difference in inter-stage mortality between the two groups (BT: 18.2 % vs. Sano: 21.1 %; P = 0.47). CONCLUSION: The results for both established surgical methods (BT and Sano) for the palliation of HLHS and its variants have improved over time and are reaching acceptable early mortality rates. There was a trend towards a favorable early outcome for Sano patients, which did not reach statistical significance in this study due to the low patient numbers.

[Intestinal microecology in human and veterinary medicine--a short excursion through the human and animal gut]. / Intestinale Mikroökologie in der Human- und Veterinärmedizin--Eine kurze Exkursion durch menschliches und tierisches Gedärm.

In honour to a great gut microbiologist the authors descend into the abdomen of humans and animals. By the way through the gut and excrements they illustrate the medical, culture-historical and literary importance of the gut and the gut flora.

[Progression of intestinal secretory immunoglobulin A and the condition of the patients during naturopathic therapy and fasting therapy]. / Verläufe des sekretorischen Immunglobulins A des Darms und Befindlichkeit von Patienten unter naturheilkundlicher Therapie und Heilfasten.

BACKGROUND: In clinical observations it was noticed that patients who have been treated with naturopathic methods - especially with the treatment of heal fasting - experience a stabilization of their immune system. The immune system stands in close relation to the intestinal flora and influences the condition of the patient. QUESTION: For this reason the influence of a 3-week in-hospital naturopathic therapy - among others a heal-fasting therapy -, a corresponding follow-up on the intestine-associated immune system as well as the condition of the patients have been examined. PATIENTS AND METHODS: Classical naturopathic treatments based on standardized therapy concepts were applied. The patients (n = 55) showed different kinds of basic diseases, whereof in 56% of the cases the patients suffered of diseases of the skeleton, the muscles, and the connective tissue. The patients got a 3-week standardized high-quality nutrition or they took part in a juice-modified fasting therapy. The state of the local endogenous defense system within the intestine was measured by the concentration of the secretory immunoglobulin A (sIgA) in the feces of the patients. A positive criterion was a sIgA concentration in the stool of under 0.5 mg/g. Additionally the judgement of the patients concerning their own condition ('quality of life') was determined by the condition scale. RESULTS: It was shown that the content of the sIgA within the stool was increased during the course of the in-hospital stay, in the complete population of patients as well as in the different subgroups 'with heal-fasting' and 'without heal-fasting'. Even in the follow-up analysis it could be observed that 3 months after the treatment the content of secretory IgA in the stool of all groups was between 0.78 and 0.89 mg/g. This value was significantly higher than the starting values. The most intensive effects concerning the change of sIgA were measured within the group of the patients who performed heal fasting (epsilon > 1.0). Aside from the increase of the secretory IgA, a mediocre improvement of the condition of the patients within the pre-post comparison could be noticed, but no correlation between the secretory IgA values and the values concerning the condition was found. CONCLUSION: A 3-week in-hospital therapy with classical naturopathic treatments (but especially the application of the heal-fasting therapy) leads to a significant improvement of the immune condition of the intestine and consequently of the whole organism. The improving effect lasts beyond the stay in hospital.

[Demonstration of pneumonia in swine as a constant problem: culture and immunofluorescence microscopic studies of bronchioalveolar lavage (BAL) and serological findings]. / Nachweis von Pneumonieerregern bei Schweinen aus Problembeständen: Kulturelle und immunfluoreszenzmikroskopische Untersuchungen der bronchoalveolären Lavage (BAL) und serologische Befunde.

Bronchoalveolar lavage was performed in 128 pigs from five fattening units showing acute pneumonia (48 animals), subclinical purulent pneumonia (17 animals), and chronic purulent pneumonia (63 animals). These samples were investigated for bacteria. Additionally immunofluorescence microscopy as well as serological investigations were performed to detect antibodies against several bacteria and viruses. Pasteurella multocida could be detected in more than a half of the samples of pigs with acute pneumonia. Bordetella bronchiseptica and mycoplasmas were isolated in a lower amount. Probably these bacteria infected the pigs of at least one herd after a primary infection with influenza virus because (i) influenza virus could be detected in three of four animals investigated for influenza virus by culture methods, (ii) the virus could be detected in one third of the animals investigated for by immunofluorescence microscopy, and (iii) antibodies against influenza virus could be detected in almost all animals. From pigs with subclinical purulent pneumonia Bordetella bronchiseptica as the only bacterial lung pathogen could be isolated exclusively from nearly each sample. From the samples of pig suffering from chronic purulent pneumonia first of all Bordetella bronchiseptica, Pasteurella multocida and different mycoplasma species could be detected. Using cultural methods Actinobacillus pleuropneumoniae could be isolated from six samples only, in contrast to frequent positive reactions against Actinobacillus pleuropneumoniae antigens obtained by immunofluorescence microscopy and CFT.