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Dieses Kapitel gibt einen Uberblick uber die kritische Aortenisthmusstenose und den unterbrochenen Aortenbogen. Hierbei werden die Anatomie, die klinische Situation der betroffenen Patienten, die diagnostischen und therapeutischen Moglichkeiten erortert. Zudem gibt es Informationen zu Assoziationen mit weiteren Fehlbildungen und Empfehlungen zur Endokarditisprophylaxe.

Dieses Kapitel gibt einen Uberblick uber deb Double Outlet Right Ventricle. Hierbei werden die Anatomie, die klinische Situation der betroffenen Patienten, die diagnostischen und therapeutischen Moglichkeiten erortert. Zudem gibt es Informationen zu Assoziationen mit weiteren Fehlbildungen und Empfehlungen zur Endokarditisprophylaxe.

The combination of optical detection techniques like photometry (UV) or laser-induced fluorescence (LIF) with mass spectrometry for capillary electrophoresis offers advantages, both for later use of stand-alone CE-UV or CE-LIF systems and for combined CE-UV-MS or CE-LIF-MS analysis. Faster method development is enabled, the identification of analytes is facilitated, and it allows christian the optical detection scheme to be used for more precise quantification. However, shortcomings of current methodology and equipment hindered the broader use of such detection combinations mainly due to the long distance between the detection points (at least 20 cm). Large shifts in migration times and changes in resolution are visible between the detection traces hindering their straightforward comparison. We present here novel equipment for a robust coupling of CE-LIF-MS with the shortest possible distance between detection points (12 cm) determined by the length of the electrospray needle. In addition, we encourage the use of a normalization of detection traces using a scale of effective electrophoretic mobility to obtain the same x-scale for both detection traces. As an example, the proposed methodology is applied to a mixture of labeled as well as non-labeled N-glycans.

The aim of the present study was to explore developmental outcome, psychosocial adjustment and self‐regulation of children who underwent corrective surgery for congenital heart disease (CHD) in early infancy and a possible association with type and procedural characteristics of the intervention. Two groups of children with CHD aged 2 to 6 years operated in infancy for ventricular septal defect (n = 19) or as neonates for transposition of the great arteries (n = 25) and a reference group (n = 35) underwent developmental and psychological testing. Vagal tone as an index for emotional self‐regulation capabilities was measured during baseline and challenge conditions. The study shows that children who underwent surgery for CHD in early infancy were at a disadvantage for cognitive, emotional, and motor development compared to the reference group at preschool age. Behavioural outcome and psychosocial adjustment were generally positive in our sample and we conclude that surgical procedures leave no discernable t...

To evaluate the diagnostic benefit of time-resolved CENTRA-keyhole contrast-enhanced cardiovascular magnetic resonance angiography (CE-CMRA) for improving arterial-venous separation of pulmonary vessels.Twenty-three patients (18 males; age = 58 +/- 11y) after radiofrequency pulmonary vein isolation to treat atrial fibrillation were examined using CENTRA-keyhole based multi-phase 3D CE-CMRA yielding 6 near-isotropic 3D datasets every 1.6 s (50-60 coronal partitions, 1.4 x 1.4 x 1.3 mm, SENSE-factor 3). Results were compared with conventional non-keyhole CE-CMRA (identical parameters, SENSE-factor 2).Data acquisition was accelerated by a speedup factor of approximately 9 compared with the reference CE-CMRA (SENSE 1.5*, keyhole 6*). No pulmonary venous stenoses were detected by either method, overall pulmonary venous diameters were 17.1 +/- 3.6 mm. Applying Bland-Altman analysis, vessel diameters differed by a mean of 0.1 mm + 2.1 mm/-2.0 mm (mean +/- 2 SD), indicating close agreement between both techniques. Interobserver variability was higher for CENTRA-keyhole (mean = 0.1 mm; mean +/- 2 SD: +2.5 mm/-2.3 mm) compared to conventional technique (0.0 mm; +1.6 mm/-1.5 mm), corresponding to a percentual deviation (mean +/- 2 SD) of the mean diameter of approximately +/- 15% (keyhole CE-CMRA) and +/- 10% (conventional CE-CMRA), respectively. Using keyhole-based time-resolved CE-CMRA, the contrast between pulmonary veins versus aorta/pulmonary artery was significantly increased (p0.05), which improved vessel depiction. In 12 cases, the contrast bolus arrival was delayed in one of the pulmonary veins by 1 dynamic frame (= 1.6 seconds); in 7 cases by 2 frames (= 3.2 seconds) and in 1 subject by 3 frames (= 4.8 seconds). The bolus usually appeared first in the upper right pulmonary vein whereas a delay occurred most often in the lower left pulmonary vein.Conventional CE-CMRA may be advantageous for accurate vessel size measures as evidenced by superior interobserver reproducibility in this study. Multi-dynamic CE-CMRA using CENTRA-keyhole with SENSE, however, allows for improved arterio-venous separation of pulmonary vessels and additional dynamical information on pulmonary venous perfusion, while maintaining high spatial resolution. Exact bolus timing is no longer needed.

Um besser zu verstehen, was ein angeborener Herzfehler bedeutet, sind die Aufgaben von Herz und Lunge, die Lage und der Aufbau des Herzens sowie seine Leistung dargestellt. Das Kapitel beschreibt zudem die Uberforderung des Herzens und der Organe, die durch einen Herzfehler in Mitleidenschaft gezogen werden.

Background— Operator-independent isotropic 3D MRI may greatly simplify the assessment of complex morphology in congenital heart disease. We sought to evaluate the reliability of this new approach. Methods and Results— In 31 adolescent and adult patients (age, 6 to 42 years; median, 16 years) with congenital heart disease, cardiac morphology was determined with free-breathing (navigator-gated), isotropic, 3D steady-state free-precession (3D SSFP) MRI and independently evaluated by 2 observers. Cardiac diagnoses and multiple distance measurements were compared with conventional MR reference sequences (ie, spin-echo, cine gradient-echo, contrast-enhanced MR angiography) and with echocardiography/cine cardioangiography or surgery. Of the 31 patients, 24 had native congenital heart defects or residual defects after repair that warranted immediate treatment. None of these defects was missed by 3D SSFP. Novel diagnostic issues were discovered in 4 of 31 patients (coronary anomalies, n=3; left juxtaposition of the right atrial appendage in double-outlet right ventricle and transposition of the great arteries, 1). For sizes of valves and vessels, we found minor mean differences of −1.1 to 1.6 mm, with SD ranging from 1.2 to 2.9 mm, demonstrating overall good agreement with standard MRI (Bland-Altman analysis). Interobserver variability of 3D SSFP distance measures was low; mean differences ranged from −1.5 to 1.0 mm, and SD ranged from 0.8 to 2.5 mm. Scatter was lower for extracardiac than intracardiac measures. Conclusions— In adolescents and adults, isotropic 3D SSFP MRI allows reliable assessment of complex cardiac morphology. Distance measurements are accurate and reproducible. Thus, a single operator-independent acquisition may substitute for conventional 2D MRI sequences to accelerate and simplify MR scanning in congenital heart disease.

Background:We reviewed our 12-year experience with staged reconstruction for hypoplasia of the left heart, examining the results of each surgical step and the impact of the year of the Norwood operation on survival. We compared survival of patients with hypoplasia of the left heart subsequent to completion of the Fontan circulation to survival of patients with a dominant left ventricle undergoing a Fontan procedure.Patients:Between 1989 and 2001, we performed a first stage procedure in 89 patients. Their median age was 9 days, with a range from 2 to 140 days, and the median weight was 3.4 kg, with a range from 2.4 to 5.4 kg.Results:Survival at 1, 4, and 10 years was 55%, 49%, and 49%, respectively. We experienced 23 early deaths (26%), and 12 deaths between the stages of the Norwood cascade. Of our patients, 42 underwent the second stage, and 30 the third stage. Prior to the first stage, symptoms of necrotising enterocolitis, and of obstructed pulmonary venous return, influenced survival significantly. The latter was eliminated as risk factor when surgery was performed within the first week of life. During the later part of our experience, survival at the first stage operation improved significantly, with survival at 3 years increasing from 42% to 75% for the patients at standard-risk (p = 0.017), and from 17% to 42% for those deemed to be at high-risk (p = 0.1). No deaths occurred in 23 patients older than 3 years of age, all of whom had proceeded through the third stage. After completion of the Fontan circulation, the survival of the patients with hypoplasia of the left heart at 4 years was comparable to the survival of patients undergoing the Fontan procedure with a dominant left ventricle (88% versus 90%, p = 0.8).Conclusions:Early and intermediate survival has improved significantly over the period of 12 years. Late death has been uncommon, and none of our patients are listed for cardiac transplantation.

Background— Parallel imaging by sensitivity encoding (SENSE) may considerably reduce scan time in MRI. For rapid flow quantification in children with congenital heart disease, we evaluated phase-contrast MRI (PC-MRI) techniques combined with SENSE. Methods and Results— In 22 pediatric patients (mean age, 7.2±6.2 years) with cardiac left-to-right shunt, blood flow rate in the pulmonary artery (Q p ) and ascending aorta (Q s ) and flow ratio Q p /Q s were determined by PC-MRI with SENSE reduction-factor 2 and 3 (SF-2 and SF-3). Additionally, we used PC-MRI with higher spatial in-plane resolution (1.6×2.1 versus 2.3×3.1 mm) with and without SF-3. Results were compared with a recently validated standard PC-MRI protocol and tested in vitro using a pulsatile flow phantom. Reduction of signal averages from 2 to 1 and application of SENSE accelerated flow measurements by a factor of 3.5 (5.2) using PC-MRI with SF-2 (SF-3) compared with standard PC-MRI. For blood flow rate through the pulmonary artery and aorta, as well as for the Q p /Q s ratio we found negligible differences of ±3%, lower limits of agreement (mean±2 SD) of −7% to −18%, and upper limits of agreement (mean±2 SD) of +3 to +24%, demonstrating good agreement with standard PC-MRI. Mean Q p /Q s ratio by standard PC-MRI was 1.69±0.45 (range, 1.27 to 2.79). Interobserver variability was low, and high accuracy was confirmed in vitro for all protocols. Conclusions— PC-MRI for flow quantitation may be combined with SENSE to achieve a substantive reduction of scanning time. In children with left-to-right shunt, Q p /Q s quantification is possible by PC-MRI+SF-3 in

The ground beetles and spiders of two salt marshes at the German Northand Baltic Sea coast were investigated by pitfall traps in 1997 and 1998. Whilethe sites at the North Sea coast are tidal salt marshes, the salt marshes at theBaltic Sea are not influenced by tides. Pitfall traps were installed in agradient from 20 to 150 cm above MHT (mean high tide: 157cm + NN, NN: 500 cm above 0 at Amsterdam gauge) atthe North Sea coast or NN at the Baltic Sea coast at six or sevensampling elevations, each with five replicates. Conductivity, water content, organic substance,frequency or duration of floodings, sand content and pH of the soil weredetermined. The flooding regime is the major factor controlling the zonation ofinvertebrates. Two and three invertebrate assemblages at the North and BalticSea, respectively, were distinguished. These corresponded well with thevegetational zones. The border between the two zones was at 60–80cm above MHT at the North Sea. The three zones at the Baltic Seaextended between 20 and 30 cm, 40 to 80 cm and 100 to150 cm above NN. The elevation of the mean abundance of speciesabove MHT or NN was calculated. A tide simulation experiment resulted in ashifting population and in an increasing activity under a tidal regime aspredicted for the global climate change conditions in 2050. From the actualelevation of the mean abundance, the habitat size of salt marsh species wascalculated for a moderate and worse scenario of global climate change. Habitatreduction becomes highest for species of the lower salt marsh zone. Under worseconditions the gradiental length of habitat will only amount to a maximum of 20m at the slopes of the dikes.

The influence of sheep grazing on the invertebrate fauna was investigated in the framework of the project Ecosystem Research Wadden Sea in Schleswig-Holstein. Two mainland salt marshes of five different grazing intensities each had been considered. During those investigations many biological indicators of the invertebrate fauna (e.g., Araneae, Rhynchota, Coleoptera and selected families of Diptera) were analysed. With these indicator species most of the influences caused by sheep grazing in salt marshes can be described. On the base of the results attained in this study recommendations on using important indicator taxa and species salt marsh monitoring are given. Furthermore, information on monitoring are given for representative salt marsh sites, adequate sampling methods, respectively spatial and temporal sampling frequencies.

From 1986 to 1997, 41 newborn infants and 19 children (1–6 years) with pulmonary atresia and ventricular septal defect were treated at the Heart Center North Rhine-Westphalia. The infants' birth weights ranged from 1140 g to 4100 g (mean 2990 g). Eighteen of these 60 patients had significant additional cardiovascular malformations and they were excluded from this study. The results of surgical treatment in the remaining 42 patients are presented and discussed here. Twenty patients had a main pulmonary arterial trunk with confluent branch pulmonary arteries and nine had an atretic trunk with confluent pulmonary arteries. In nine patients without confluence, one branch pulmonary artery originated normally from the main pulmonary trunk and the other originated abnormally. In four patients the intrapericardial vessels were completely missing. The Nakata index measured by cineangiography ranged from 50 to 240 mm 2 /m 2 (mean 150 mm 2 /m 2 ). All newborns with a patent ductus arteriosus received prostaglandin intravenously. In eight of them oral prostaglandin was used to provide long-term stability before surgery. A total of 65 surgical aortopulmonary anastomoses were created, primary repair was carried out in six patients, and secondary corrective surgery was performed in 21 patients. After the first anastomosis, oxygen saturations increased by 5–15% (mean 8%), depending on the pulmonary vascular pathology. When a second shunt was created in the same patient, the increase in oxygen saturations ranged from none to 7%. After a mean interval of four years following the last operation, inspiratory vital capacity and intrathoracic gas volume were measured in 22 patients. Values of both studies decreased progressively as more thoracotomies were performed. All 42 patients were treated surgically and 13 died (31%). A primary or secondary repair was performed in 27 with 23 survivors (85%), and 15 had palliative procedures with six survivors (40%). Twenty of the 29 survivors (69%) are in a good clinical condition.

Fatty acid influx into human myocardium was studied in 15 patients during the cooling phase of cardiopulmonary bypass at myocardial temperatures of 37 degrees to 25 degrees C. The fitting of the data to a functional relation, developed in this study, revealed fatty acid influx to be a temperature-dependent saturable process corresponding to a Michaelis-Menten constant (Km) at 37 degrees C of 0.26 +/- 0.084 mumol/g, a maximal fatty acid influx velocity (Vmax) at 37 degrees C of 0.28 +/- 0.045 mumol/g per minute, activation energy for fatty acid binding to the putative carrier (E) of 23.8 +/- 5.6 kcal/mol, and a free energy for conformational change of the carrier (U) of 10.9 +/- 8.0 kcal/mol. In short-term cultured hepatocytes, Km increased in the absence of Na+ from 171 +/- 48 to 301 +/- 71 nmol/L, and Vmax of [3H]oleate decreased from 1063 +/- 69 to 847 +/- 68 pmol/min per milligram protein. The fitting of these data to a functional relation revealed a transmembrane potential-dependent component of parameters E and U to be -0.479 and -0.374 kcal/mol, respectively. It is proposed that for fatty acid influx a protonated fatty acid form is preferred that consists of a Na+ complex with the mesomeric form of nondissociated fatty acid from which Na+ and H+ are released during collision with the carrier.

The use of cartilage allografts is being discussed because of the possible transmission of the human immunodeficiency virus (HIV). To further delineate the possibility for an HIV infection of cartilage cells the susceptibility and permissivity of normal human chondrocytes to HIV-1 was assessed in culture. Isolated cartilage cells were incubated during 30 days with the HIV, testing the production of viral p24 antigens and the formation of particle-bound reverse transcriptase (RT) in the supernatant. The H9 cell line was used as positive control. The production of p24 antigen did not occur on chondrocytes during the whole culture time. Furthermore, no RT activity was detected throughout the entire experimental period. Our results indicate clearly that HIV was not able to infect cartilage cells under these in vitro conditions, and, conclusively, HIV infection of cartilage in vivo is improbable.

Objective Calcification is a principal problem in noninvasive coronary angiography (CA) performed with multislice computed tomography (MSCT). Magnetic resonance imaging (MRI) supposedly neglects calcium. We compared both modalities. Methods Sixty-eight patients admitted for CA underwent MSCT- and MRI-based noninvasive CA the day before. Stenosis detection (>or=50%) was visually performed and validated by quantitative CA. Results The patient-based overall sensitivity, specificity, positive predictive value, and negative predictive value were 96.2%, 95.2%, 92.6%, and 97.6% for MSCT versus 69.2%, 64.3, 54.5%, 64.3%, and 77.1% for MRI, respectively. The segment-based overall results were 74.7%, 97.8%, 75.7%, and 97.6% for MSCT versus 40%, 93.8%, 37.5%, and 94.4% for MRI, respectively. Investigating the impact of coronary calcifications in the patient group with a volume score of 300 microL or greater resulted in 100%, 80%, 92.3%, and 100% for MSCT versus 70.8%, 86.7%, 89.5%, and 65% for MRI, respectively. The segment group with considerable hardening artifacts resulted in 85.3%, 83.9%, 76.3%, and 90.4% for MSCT versus 52.9%, 92.9%, 81.8%, and 76.5% for MRI, respectively. Conclusions There are no major advantages of MRI in cases of advanced coronary calcification.

Fatty acid binding protein has been found in rat aortic endothelial cell membrane. It has been identified to be a 40-kDa protein that corresponds to a 40-kDa fatty acid binding protein with high affinity for a variety of long chain fatty acids isolated from rat heart myocytes. It is proposed that this endothelial membrane fatty acid binding protein might mediate the myocardial uptake of fatty acids. For evaluation of this hypothesis in vivo, influx kinetics of tracer-labeled fatty acids was examined in 15 normal subjects by scintigraphic techniques. Variation of the plasma fatty acid concentration and plasma perfusion rate has been achieved by modulation of nutrition state and exercise conditions. The clinical results suggest that the myocardial fatty acid influx rate is saturable by increasing fatty acid plasma concentration as well as by increasing plasma flow. For analysis of these data, functional relations describing fatty acid transport from plasma into myocardial tissue in the presence and absence of an "unstirred layer" were developed. The fitting of these relations to experimental data indicate that the free fatty acid influx into myocardial tissue reveals the criteria of a reaction on a capillary surface in the vicinity of flowing plasma but not of a reaction in extravascular space or in an unstirred layer and that the fatty acid influx into normal myocardium is a saturable process that is characterized by the quantity corresponding to the Michaelis-Menten constant, Km, and the maximal velocity, Vmax, 0.24 +/- 0.024 mumol/g and 0.37 +/- 0.013 mumol/g(g.min), respectively. These data are compatible with a nondiffusional uptake process mediated by the initial interaction of fatty acids with the 40-kDa membrane fatty acid binding protein of cardiac endothelial cells.

Forty infants with complete atrioventricular septal defect have undergone primary repair within their 1st year of life. The mean age at time of surgery was 4 months (range 1-12 months) and the mean weight 4.2 kg (range 2.9-7.0 kg). Either the one- or the two-patch method was used. Four patients died (10%). There were no late deaths. Three patients needed reoperation due to mitral valve insufficiency. Postoperative complications were: 1 total atrioventricular block in an infant with an absent ventricular septum 3 weeks after surgery, 9 complete right bundle branch blocks, 3 small residual ventricular septal defects. Mean follow-up of the 36 survivors is 22 months (3-46 months). Thirty (83%) are in NYHA functional class I, 4 (11%) in NYHA class II and 2 (6%) in NYHA class III. No atrioventricular valve regurgitation is detectable by the color Doppler technique in 19 (53%) patients. Mitral incompetence is mild in 13 cases (36%) and moderate in 4 (11%). In ten recatheterized patients the average systolic pulmonary to systemic artery pressure ratio dropped from 1.0 to 0.42 and the average pulmonary arteriolar resistance was reduced from 5.5 to 3.9 U/m2. All but one patient gained weight and underwent adequate statomotoric development. Primary repair should be performed at the age of 5-6 months or even earlier, if pulmonary arteriolar resistance exceeds 5 U/m2.