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Background: Exercise capacity is a well-defined marker of outcome in congenital heart disease. We analyzed seventeen cardiovascular magnetic resonance (CMR) derived parameters and their correlation to exercise capacity in patients with Ebstein's anomaly (EA). Methods: Fifty-four surgery free patients, age 5 to 69 years (median 30 years) prospectively underwent CMR examination and cardiopulmonary exercise testing (CPET). The following volume/flow parameters were compared with peak oxygen uptake as the percentage of normal (peakVO2%) using univariate and multivariate analysis: right and left ventricular ejection fraction (RVEF and LVEF), the indexed end-diastolic and end-systolic volumes (RVEDVi, RVESVi, LVEDVi, and LVESVi), the indexed stroke volumes (RVSVi and LVSVi), the total normalized right and left heart volumes; the total right to left heart volume ratio (R/L-ratio). The indexed antegrade flow (ante), indexed net flow (net) as well as cardiac index (CI) in the aorta (Ao) and pulmonary artery (PA) were used. Results: RVEF (R2 0.2788), indexed flow PA net (R2 0.2330), and PA ante (R2 0.1912) showed the best correlation with peakVO2% (all p < 0.001) in the univariate model. Further significant correlation could also be demonstrated with CI-PA, LVEF, LVSVi, Aorta net, RVESVi, and Aorta ante. Multivariate analysis for RVEF and indexed net flow PA revealed a R2 of 0.4350. Conclusion: Functional CMR parameters as RVEF and LVEF and flow data of cardiac forward flow correlate to peakVO2%. Evaluation of the indexed net flow in the pulmonary artery and the overall function of the right ventricle best predicts the maximal exercise capacity in patients with EA. [ABSTRACT FROM AUTHOR]

Objective The purpose of this study was to assess the longitudinal follow-up of exercise performance in patients with native Ebstein anomaly and patients who had received previous tricuspid valve surgery. Patients and methods We identified 59 patients with Ebstein anomaly who had performed at least two cardiopulmonary exercise tests within a minimum of 6 months. 22 patients (15 female, 29.0 ± 13.4 years) with native conditions, 37 patients (21 female, 32.1 ± 15.3 years) had undergone surgery of their tricuspid valve before baseline testing. Results In the non-operated group peak workload (2.58 ± 0.7 to 2.38 ± 0.7 W/kg, p = .019) declined during a mean follow-up of 4.6 ± 2.6 years, whereas peak workload remained stable in the surgical group (2.02 ± 0.6 to 2.06 ± 0.7 W/kg, p = .229) throughout the follow-up of 3.4 ± 2.3 years. In the 18 patients who had their surgery less than one year prior to the first CPET the exercise performance even increased during the follow-up. Peak blood pressure as a marker of cardiac adaption to exercise increased in the follow-up examination only in the surgical group (159 ± 23 vs. 171 ± 30 mm Hg, p = .007). In the non-operated group blood pressure remained unchanged at follow-up (161 ± 26 vs. 166 ± 29, p = .358). Conclusion Exercise performance deteriorates in non-operated patients with Ebstein anomaly in contrast to patients after tricuspid valve surgery where exercise performance remains stable. These facts promote a surgical intervention latest at the time when exercise performance decreases. [ABSTRACT FROM AUTHOR]

Premise of research. The geographical distribution of ploidy levels provides insights into evolutionary pathways. For the subtropical tree line species Polylepis australis (Rosaceae), we tested the hypotheses that (1a) incidence of polyploidy is higher in the northern parts than in the southern parts of the species range due to the presence of related species that might favor hybridization (allopolyploidy), (1b) incidence of polyploidy is higher in the southern part of the range because the species here presumably reaches the limit of its environmental tolerance (autopolyploidy), and (2) ploidy levels increase with elevation, as polyploids are believed to perform better in stressful environments. Methodology. We used flow cytometry to assess the ploidy levels of 361 individuals from 27 populations across most of the distribution range of the species in two disjunct Argentinean high mountain regions. Pivotal results. The northern stands had lower ploidy levels (diploid) than the southern populations, in which we found mainly tetraploids intermixed with diploids, triploids, and a single hexaploid. We did not find any environmental correlates to the geographical distribution of ploidy levels. Conclusions. Polyploidy appears to have arisen in P. australis via autopolyploidy, either twice in different parts of the range or, more likely, once followed by long-distance dispersal. This is also supported by amplified fragment length polymorphism (AFLP) data from a previous study that confirmed higher numbers of AFLP fragments and private bands in the southern populations. The checkerboard distribution of ploidy levels in the southern Sierras de Córdoba may represent a time capture of the spread of the polyploid condition. We propose that polyploidy represents a key factor to understanding the high morphological variation in P. australis and should be taken into account in ongoing reforestation activities. [ABSTRACT FROM AUTHOR]

Abstract: Background: Clinical, hemodynamic and functional effects of tricuspid valve surgery in patients with Ebstein''s anomaly are not well understood. Methods: Sixteen patients (median age of 27.7years) were examined before and eight months after surgery by means of echocardiography, cardiovascular magnetic resonance (CMR) and cardiopulmonary exercise testing. Results: Peak work load (1.87 to 2.0W/kg; p=0.026), maximum oxygen uptake (21 to 22ml/kg/min; p=0.034) as well as cardiac output (2.7 to 2.9l/min/m2; p=0.035) increased postoperatively. The reduction of tricuspid regurgitation led to a higher pulmonary stroke volume (29 to 42ml/m2, p=0.005) and augmented the left ventricular (LV) volume (55 to 63ml/min/m2; p=0.001) with a trend to better ejection fraction (61 to 64%; p=0.083). Right ventricular (RV) volume index (124 to 108ml/m2; p=0.034) and ejection fraction (50 to 42%; p=0.036) decreased on CMR. Echocardiographic measurements of RV function also decreased (tricuspid annular plane systolic excursion 2.3 to 1.7; p=0.002; isovolumic acceleration 0.98 to 0.65; p=0.004; and 2-d longitudinal global strain −19.3 to −16.25; p=0.006). Conclusion: Tricuspid valve surgery improves exercise capacity in patients with Ebstein''s anomaly. The reduction of tricuspid regurgitation decreases the volume of the right ventricle and increases pulmonary antegrade flow. As a result LV volume and cardiac output increase. This hemodynamic benefit occurs despite the preload dependent reduction in RV volume and ejection fraction. [Copyright &y& Elsevier]

Abstract: Background: Atrial septal defects (ASD) and persistent foramen ovale (PFO) are managed in increasing numbers by catheter interventions as an attractive alternative to surgery. Early complications have been described in clinical series whereas late complications are rare. No series are reported with clinical, echocardiographic and histological data. Methods and results: We collected clinical, echocardiographic, and histolological data of nine patients with surgically explanted devices. Occlusion devices were explanted after a mean interval of 3.4±2.4years (range 0.9–8.3). Indications were recurrent thromboembolic events in five, residual shunt/dislocation in three, and growing mass on echocardiography despite oral anticoagulation in one patient. Two patients suffered potentially live threatening events due to coronary embolism. One of them had to be resuscitated due to ventricular fibrillation. Histologically, residues of superficial thrombus formation could be demonstrated in two of the devices. In another patient, hyperplastic tissue formation was related to a local inflammatory process but not to a thrombus as suspected by echocardiography. Conclusion: Late complications after device implantation may occur up to 8years after device implantation and may be potentially live threatening. Echocardiographic controls should be prolonged beyond the first year after implantation and every explanted device should be histologically worked up in an experienced center. Up to now, the mechanisms of late thrombogenesis are not fully understood. [Copyright &y& Elsevier]

Abnormal elastic properties of the ascending aorta in patients with coarctation of the aorta (CoA) are already present in neonates before and after successful repair. To prove the midterm outcome for vascular pathology, a cohort of 15 patients was prospectively reevaluated. In this study, 15 patients after neonatal CoA repair (study group) and 15 healthy normal children (control group) were examined (mean age, 3.0 ± 1.0 years). The aortic wall stiffness index and distensibility were calculated using ascending and abdominal aortic M-mode diameters and noninvasive estimation of pulse pressure. The study group was compared with the control group and with its own previously obtained pre- and postoperative measurements. After a mean follow-up period of 2.9 ± 0.9 years, the elastic properties of the ascending aorta remained impaired in the study group compared with the control group (distensibility: 62 ± 28 vs 94 ± 34; p = 0.01; stiffness index: 4.4 ± 1.9 vs 2.7 ± 1.0; p = 0.008). At follow-up evaluation, the elasticity of the ascending aorta in the study group showed no change from the initial neonatal findings (distensibility: 63 ± 24 vs 62 ± 28; p = 0.82; stiffness index: 4.0 ± 1.6 vs 4.4 ± 1.9; p = 0.65). In both groups, descending aortic elasticity increased during the follow-up period. Our data suggest that the diminished elastic properties of the prestenotic arteries remain unchanged years after successful CoA repair. Comparisons with the neonatal data showed no evidence for remodeling of the ascending aorta in this group. [ABSTRACT FROM AUTHOR]

The C–C*–CO bending mode in camphor (C* denotes a chiral carbon) as originally investigated for parity violation effects by Arimondo et al. [Opt. Commun. 23 (1977) 369] lies in the CO2 laser frequency range with the most intense transition at 1045 cm−1. The vibrational spectrum of camphor is, therefore, reinvestigated by ab initio and density functional calculations and compared with experimental results. This provides the basis for a local mode analysis which reveals that the C–C*–CO bending mode has only minute parity violation contributions (<10−5 Hz) to vibrational transitions far below the current detection limit of high resolution spectroscopy. [Copyright &y& Elsevier]

Good quality of life correlates with a good exercise capacity in daily life in patients with tetralogy of Fallot (ToF). Patients after correction of ToF usually develop residual defects such as pulmonary regurgitation or stenosis of variable severity. However, the importance of different hemodynamic parameters and their impact on exercise capacity is unclear. We investigated several hemodynamic parameters measured by cardiovascular magnetic resonance (CMR) and echocardiography and evaluated which parameter has the most pronounced effect on maximal exercise capacity determined by cardiopulmonary exercise testing (CPET). 132 patients with ToF-like hemodynamics were tested during routine follow-up with CMR, echocardiography and CPET. Right and left ventricular volume data, ventricular ejection fraction and pulmonary regurgitation were evaluated by CMR. Echocardiographic pressure gradients in the right ventricular outflow tract and through the tricuspid valve were measured. All data were classified and correlated with the results of CPET evaluations of these patients. The analysis was performed using the Random Forest model. In this way, we calculated the importance of the different hemodynamic variables related to the maximal oxygen uptake in CPET (VO%predicted). Right ventricular pressure showed the most important influence on maximal oxygen uptake, whereas pulmonary regurgitation and right ventricular enddiastolic volume were not important hemodynamic variables to predict maximal oxygen uptake in CPET. Maximal exercise capacity was only very weakly influenced by right ventricular enddiastolic volume and not at all by pulmonary regurgitation in patients with ToF. The variable with the most pronounced influence was the right ventricular pressure. [ABSTRACT FROM AUTHOR]

Abstract: Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a very rare congenital abnormality. Echocardiographic findings are specific but may be easily misinterpreted. We therefore present the case of a 5-year old boy with emphasis on the echocardiographic findings. [Copyright &y& Elsevier]

Resources from sewage sludge ashes can be re-utilized for the recycling economy and pollutants can be removed by using a thermochemical process. An online analysis method has been developed for the determination of element mass fractions in the ashes and their products originating from the process. The applied analysis system is a method combination of laser-induced breakdown spectroscopy (LIBS) and X-ray fluorescence (XRF) spectroscopy. Robustness and a modular construction are important demands for successful future applications. [ABSTRACT FROM AUTHOR]

Intense exercise has been shown to have negative effects on systolic and diastolic ventricular function in adults. Very little is known about the normal reaction of the growing heart to endurance stress. For this study, 26 healthy children (18 males) with a mean age of 12.61 years (range, 7.92-16.42 years) took part in an age-adapted triathlon circuit. The athletes were investigated by two-dimensional (2D) echocardiographic/speckle tracking, M-mode, pulse-wave Doppler, color Doppler, and color-coded tissue Doppler at 2-4 weeks before and immediately after the race. After the competition, cardiac output increased, mediated by an increase in heart rate and not by an elevated preload, according the Frank-Starling mechanism. Two-dimensional speckle tracking showed a reduced longitudinal strain in the right and left ventricles and additionally reduced circumferential strain in the left ventricle. The late diastolic inflow velocities were increased in both ventricles, indicating reduced diastolic function due to an impairment of myocardial relaxation. Immediately after endurance exercise, systolic and diastolic functions were attenuated in children and adolescents. In contrast to adult studies, this study could show a heart rate-mediated increase in cardiac output. The sequelae of these alterations are unclear, and the growing heart especially may be more susceptible to myocardial damage caused by intense endurance stress. [ABSTRACT FROM AUTHOR]

Ventricular septal defects (VSDs) are a common congenital heart disease. Usually, surgical repair with cardiopulmonary bypass (CPB) is the treatment of choice, whereas percutaneous techniques have technical limitations, predominantly a mismatch of catheter size and body weight. A 7-year-old girl underwent periventricular closure of a perimembranous VSD on the beating heart. Echocardiography guided implantation through a minimally invasive sternotomy was uneventful. The described approach adds favorably to the current practice avoiding the use of CPB. Cosmetic aspect and rapid early postoperative recovery are convincing. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Surgical septal myectomy is performed to relieve left ventricular outflow tract narrowing in severe drug-refractory obstructive hypertrophic cardiomyopathy. The objective of this study was to assess the perioperative and long-term clinical outcome of this procedure performed during infancy. METHODS: Clinical, transthoracic echocardiographic, electrocardiographic, 24-h Holter, cardiopulmonary exercise test and genetic data were extracted by medical record review. A subset of patients underwent additional prospective detailed clinical evaluation including cardiac magnetic resonance imaging with contrast. RESULTS: Surgery was performed in 23 paediatric patients between 1978 and 2015 at the German Heart Centre Munich. Twelve patients had undergone surgery during infancy (≤1 year) (Group A), 11 between 1 and 18 years of age (Group B). The underlying genetic diagnosis was Noonan syndrome spectrum and non-syndromic hypertrophic cardiomyopathy. As compared to Group B, patients in Group A showed more concomitant cardiac procedures and received more homologous transfusions. One perioperative death occurred in Group A, and none in Group B. Two patients in Group A but no patient in Group B required redo septal myectomy. The long-term clinical outcome was similar between the 2 groups. One patient in Group B required cardioverter-defibrillator/pacemaker implantation for higher degree atrioventricular block and none in Group A. There was no evidence of differences in myocardial fibrosis between groups on longterm follow-up magnetic resonance imaging. CONCLUSIONS: Surgical septal myectomy can be performed safely during infancy with favourable perioperative and long-term clinical outcome but with a trend towards a higher reoperation rate later in life. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Only little data exist on the durability of bioprostheses in the tricuspid position in patients with congenital heart disease (CHD). The aim of the study was to determine the reoperation rate and the valve function after primary implantation. METHODS: Between 1990 and 2013, 51 patients with CHD underwent tricuspid valve (TV) replacement with a bioprosthesis. The median age at operation was 32 years (range: 8-69). The underlying morphology was Ebstein’s anomaly in 62% of the patients. Implanted valves included 38 pericardial and 13 porcine valves. All available echocardiographic examinations (n = 714) and clinical data were retrospectively reviewed. Dysfunction was defined as an at least moderate regurgitation or a mean diastolic gradient ≥9 mmHg. Freedom from death, reoperation and prosthetic valve dysfunction was estimated using the Kaplan-Meier method. RESULTS: The 30-day mortality rate was 9%. The estimated survival rate was 86% at one and 80% at ten years. The freedom from reoperation at 1, 5 and 10 years was 100, 86 and 81%, and that from prosthesis dysfunction detected by echocardiography at 1, 5 and 10 years was 89, 66 and 58%, respectively. The main reason for dysfunction was insufficiency (89%). Valve implantation at an age below 16 years was associated with earlier reoperation and dysfunction (the 5-year freedom rate from reoperation/dysfunction was 70%/30% compared with 89%/78% in the rest of the patients, P = 0.016/0.0009). CONCLUSIONS: Serial echocardiography shows a high rate of dysfunction of TV bioprosthesis in patients with CHD, which already occurred a few years after implantation. In patients below 16 years of age, most prostheses are dysfunctional within 5 years. [ABSTRACT FROM AUTHOR]

Ebstein's anomaly. The technique consists of extensive leaflet mobilization, longitudinal plication of the atrialized ventricle and coneshaped reconstruction of the tricuspid valve, allowing for leaflet-to-leaflet coaptation. We evaluated the influence of Da Silva's cone repair on tricuspid competency, right ventricular size and function. METHODS: From February 2010 until July 2013, 20 patients (median age 30.0 years, range 6.6-68.3 years) underwent Da Silva's cone repair. A 4- to 6-mm interatrial communication was left in all patients. Echocardiographic studies and magnetic resonance imaging (MRI) before and after the repair were evaluated. RESULTS: Median follow-up was 11 (0.5-36) months. There were 2 early deaths and no late death. Echocardiography at follow-up revealed mild or absent tricuspid regurgitation in 16 patients. Two patients showed moderate tricuspid insufficiency. MRI studies showed that the mean functional RV end-diastolic volume decreased after surgery (pre 334 [135-656] ml; post 175 [115-404] ml, P < 0.001). The mean RV ejection fraction decreased (pre 47 ± 10%; post 35 ± 13%, P = 0.001), and the mean antegrade net stroke volume of the RV increased (pre 65 ± 28 ml; post 75 ± 30 ml, P = 0.057). CONCLUSIONS: Da Silva's cone repair for Ebstein's anomaly creates excellent valve function in all patients. Consecutively, the size of the RV decreases and the antegrade net stroke volume increases 6 months after the operation. [ABSTRACT FROM AUTHOR]

Abstract: Background: The size of the functional right ventricle of patients with Ebstein's anomaly is, according to contemporary opinion, small. However, the in vivo size of the functional right ventricle in patients with Ebstein's anomaly is unknown. Furthermore, it is unknown how the apical displacement and regurgitant fraction of the tricuspid valve in Ebstein's anomaly affects the size of the functional right ventricle. Therefore the primary aim of this study was to determine the volume of the functional right ventricle in patients with Ebstein's anomaly. The secondary aim of this study was to assess correlation of the volume of the functional right ventricle to the apical displacement and regurgitant fraction of the tricuspid valve. Methods: Thirty-two consecutive patients with Ebstein's anomaly without previous cardiac surgery or shunts were studied prospectively by cardiovascular magnetic resonance. Functional right ventricle, left ventricle, and atrialized right ventricle volumes and tricuspid valve regurgitation were measured. Results: Functional right ventricle end diastolic volumes were median 127ml/m² (range: 76–339ml/m²) and were median 2.5 (range: 1.3–8.8) times larger than the left ventricle volumes. Furthermore, functional right ventricle volumes correlated in a strong positive fashion with tricuspid valve regurgitation (p <0.001, R 2 =0.65) and modestly with the atrialized right ventricle volumes (p =0.027, R 2 =0.16). Conclusion: Patients with untreated Ebstein's anomaly have large functional right ventricles. The size of the enlarged functional right ventricle seems to depend on the degree of tricuspid valve regurgitation and not on the size of the atrialized right ventricle or the age of the patient. [Copyright &y& Elsevier]