D. Kececioglu

New aspects of the ventricular septum and its function: an echocardiographic study

Objectives: To examine whether the line dividing the septum into two layers is found consistently by conventional echocardiography and to evaluate functional differences in the right and left side of the septum in terms of wall thickening, strain rate, and strain imaging. Design: In a systematic study in 30 normal subjects, M mode and Doppler myocardial imaging data from the interventricular septum (IVS) were recorded. Velocity curves, regional strain rate, and strain profiles were obtained. Systolic deformation (wall thickening, radial and longitudinal strain rate, and strain) of both sides were assessed. Furthermore, three patients with one sided abnormalities were studied. Results: A bright echo consistently segmented the IVS into a left and right part. In this normal population radial deformation was different for the left and right side of the septum (mean (SD) wall thickening on the left, 49 (46)%, and on the right, 17 (38)%; strain rate on the left, 3.8 (0.6) 1/s, and on the right, 2.1 (1.9) 1/s; strain on the left, 41 (17)%, and on the right, 22 (14)%), whereas longitudinal deformation was found to be similar (strain rate on the left, −2.2 (0.7) 1/s, and on the right, −2.0 (0.6) 1/s; strain on the left, −28 (12)%, and on the right, −25 (12)%). The presented clinical examples show that abnormalities can be strictly limited to one layer. Conclusions: Differential radial deformation and knowledge of fibre architecture showing an abrupt change in the middle of the septum, together with the clinical cases, suggest the septum to be a morphologically and functionally bilayered structure potentially supplied by different coronary arteries.

Cause of death in adults with congenital heart disease — An analysis of the German National Register for Congenital Heart Defects

BACKGROUNDnDue to the great advances in the care of patients with congenital heart disease (CHD), mortality has decreased significantly over the last decades. Nonetheless, mortality for adults with congenital heart disease (ACHD) is still higher than for the general population. An analysis regarding causes of death in a nationwide contemporary cohort of ACHD is lacking.nnnMETHODSnA well-characterized cohort of the German National Register for Congenital Heart Defects was screened for patients over the age of 18 years who died between January 2001 and January 2015. Data relating to the cardiac diagnosis, symptoms, operations, interventions, comorbidities, and causes of death were analyzed.nnnRESULTSnDuring a median follow-up of 3.67 years (IQR 1.32-9.41), 239 (9.2%) out of 2596 patients died during the study period (110 female (46%), mean age at death 39.8 ± 17.8 years). The majority of these deaths was CHD-related (171 patients (71.5%)). Leading causes of death were heart failure (n=66, 27.6%), and sudden cardiac death (n=55, 23.0%). Deceased patients had a more complex CHD and more extracardiac comorbidities compared with living patients.nnnCONCLUSIONSnCauses of death of ACHD patients in a large contemporary cohort from a national register are in the majority still CHD-related, with heart failure being the leading cause of death. Additionally, extracardiac comorbidities gain increasing importance.

Interventional Closure of Atrial Septal Defects in Adult Patients with Ebstein's Anomaly

Ebsteins anomaly is frequently associated with interatrial communications. In patients with severe tricuspid regurgitation standard treatment is the surgical repair or replacement of the tricuspid valve and patch closure of the atrial septal defect. We sought to evaluate the feasibility and short-term outcome of interventional device closure of interatrial communications in Ebstein patients with mild to moderate tricuspid regurgitation and various degrees of clinical symptoms. In this case series of 9 patients the device closure could be performed safely and 8 of 9 patients improved in their exercise capacity or clinical condition. However, the patients need to be selected carefully and appropriately for this palliative method. In those with predominant left-to-right shunting, ASD-closure reduces the volume load of the right ventricle and can be performed according to routine procedures. In those patients with cyanosis and right-to-left shunting however, test occlusion of the interatrial communication with adequate balloon size followed by careful examination of the hemodynamics at rest and under catecholamine stimulation is compulsatory to evaluate the feasibility of device closure. The tricuspid regurgitation should not exceed moderate level, the right atrial and ventricular pressure should be within normal range for an adequate time during test occlusion and the systemic blood pressure and cardiac output maintained safely.

Eisenmenger syndrome and long-term survival in patients with Down syndrome and congenital heart disease

Objective To characterise patients with trisomy 21 (Down syndrome, DS) based on the data of the German National Register for Congenital Heart Defects, to identify changes in the availability of surgical therapy over time and to analyse the impact of these changes on developing Eisenmenger syndrome (ES) as well as survival. Methods Out of 1549 patients with DS with congenital heart disease in the National Register for Congenital Heart Defects, 894 patients (55% female, mean age 17.5u2005years) had a post-tricuspid shunt lesion (atrioventricular septal defect 69.5%, ventricular septal defect 27.7%, patent arterial duct 2.6%) and were included in the current study. Results The likelihood of being treated interventionally or surgically before the age of 1u2005year increased significantly over time. In parallel, the likelihood of developing ES decreased over time (53% birth cohort during 1950s/1960s vs 0.5% birth cohort during 2000–2009, p<0.0001). Overall survival after 1, 10, 20 and 40u2005years was 96.8%, 94.1%, 92.6% and 75.5%, respectively. Patients with ES had a significantly worse survival compared with those without ES (HR 18.1; 95% CI 7.2 to 45.4; p<0.0001). Conclusions The availability of surgical correction was associated with a decrease in the likelihood of developing ES. Patients with DS still have reduced survival prospects compared with the general population, but this effect is largely driven by patients developing ES who still have a very poor prognosis.

Bovine Jugular Veins versus Homografts in the Pulmonary Position: An Analysis across Two Centers and 711 Patients-Conventional Comparisons and Time Status Graphs as a New Approach.

BACKGROUNDnVarious diseases and diversity in implantation ages, together with evolving diagnostic and therapeutic options, hinder comparative evaluations of long-term outcomes for valved conduits used for reconstruction of the right ventricular outflow tract (RVOT). We combined two common evaluation methods to optimally use information obtained by pooling the raw data from two high volume centers, each with very regular follow-up procedures, with the aim of analyzing durability differences between conventional homografts and bovine jugular veins.nnnPATIENTS AND METHODSnIn the period 1985 to 2012, a total of 444 bovine jugular veins and 267 homografts were implanted, and 6,738 postoperative examinations took place. Evaluations included age-stratified Kaplan-Meier analyses, Cox regression models, and time status graphs, the third showing age-group stratified, time-related frequencies of intact, insufficient, stenotic, both insufficient and stenotic, and postinterventional conduits below the freedom from explantation curve. They take into account interventions, explantations, and the nonterminal character of echocardiographic findings.nnnRESULTSnThe durability of intact bovine jugular veins in children and young adults is not inferior to that of homografts. Averaged over the first 12 years after implantation, the age groupsu2009<u200925 years in fact showed advantages for bovine jugular vein recipients. The average fraction of patients younger than 25 years whose conduits were not explanted, postinterventional, stenotic, insufficient, or stenotic and insufficient was at least 10% higher in recipients of bovine jugular veins than in homograft recipients.nnnCONCLUSIONnAccording to the time status graphs, the use of bovine jugular veins for RVOT in patients younger than 25 years appears to lead to superior results when compared with cryopreserved homografts.

Three parties, one direction: Research priorities in adults with congenital heart disease. What do professionals, patients and relatives want to know?

BACKGROUNDnPatients, their relatives, as well as medical specialists from the fields of paediatric cardiology, cardiology and cardiac surgery were surveyed to investigate the current research needs in the field of congenital heart disease (CHD) focussing specifically on the needs of those affected.nnnMATERIAL AND METHODSnAn online survey including four groups of patients with CHD (Fontan circulation, transposition of the great arteries [TGA] after atrial switch [AS] and after arterial switch operation [ASO], tetralogy of Fallot [TOF]) was performed. Each questionnaire comprised twelve topics. Persons affected (patients and relatives) were surveyed by means of CHD group specific questionnaires. Participants were recruited through the German National Register for Congenital Heart Defects.nnnRESULTSnN=596 affected persons (Fontan circulation: n=189; TGA after AS: n=64; TGA after ASO: n=90; TOF: n=253) and 75 physicians (57.3% paediatric cardiologists, 28.0% cardiologists, 10.7% cardiac surgeons, 4.0% other) participated. In general, those affected assume a greater need for research than physicians. Regarding the CHD related topics to be the focus of future research, those affected largely agreed with the participating physicians, although with a different ranking of research topics.nnnCONCLUSIONSnBased on the results of our study the challenges immanent in routine care for the CHD patient groups investigated can be identified. Accordingly, these topics should be prioritized in the research of the coming years.

Effective valve opening area in the detection of dysfunctional aortic valve prostheses: a differentiated statistical analysis of this parameter including the introduction of minimal expected normal values as borderline to dysfunctional stenotic prostheses.

Background: Dysfunction of heart valve prostheses (VP) is a life‐threatening complication and the diagnosis remains difficult. The motivation for this study was to improve the detection of dysfunctional VP by optimizing application of the prosthetic effective orifice area (VA). For this reason the minimal expected normal VA (VAexpected) was introduced. Methods: We investigated echocardiographically 1,369 normally functioning aortic valve prostheses (AVP). Mean VA, transprosthetic peak (PPG) and mean pressure gradients (MPG) were evaluated to gain reference values depending on prosthetic size and construction principle. Mean VAexpected was calculated by applying a simple formula that was developed empirically using statistical analyses. The results were compared with those of 65 dysfunctional AVPs. Results: VAexpected can be applied as a threshold between normal and dysfunctional stenotic AVP and showed a correct estimation in 87% of all normally functioning and 100% of dysfunctional stenotic VPs. The sensitivity for all prosthetic sizes is 1.0, independently of the constructional principle of the VP. Specificity ranged between 0.8 and 1.0, dependent on VP size. The formula representing VAexpected is simple and can be executed easily. Conclusion: As nearly independent of stroke volume and in consideration of VAexpected, VA seems to have become one of the preferable parameters for detecting pathological stenotic AVPs echocardiographically. The additional application of PPG/MPG and other parameters permits prostheses with relevant isolated regurgitation and patient‐prosthesis‐mismatch to be distinguished. (Echocardiography 2012;29:713‐719)

Validation and Reference Values for Three-Dimensional Echocardiographic Right Ventricular Volumetry in Children: A Multicenter Study

Background: Functional assessment of the right ventricle using real‐time three‐dimensional echocardiography (RT3DE) has fundamental relevance in young patients with congenital heart disease. Reference values for the pediatric population are scarce. This multicenter study was designed to (1) validate new evaluation software for RT3DE and (2) establish pediatric reference values. Methods: For validation, right ventricular (RV) end‐diastolic volume (EDV) and end‐systolic volume (ESV) were determined from real‐time three‐dimensional echocardiographic data sets of 38 subjects (n = 17 healthy individuals and n = 21 patients with congenital heart disease) using new dedicated evaluation software (RV‐Function 2.0) and compared with cardiac magnetic resonance investigations of the same patient cohort. In a prospective multicenter design, 360 real‐time three‐dimensional echocardiographic data sets of healthy children (172 girls) were analyzed. To create reference centiles, the cohort was subdivided into group I (children <7 years of age, n = 136 [female and male]), group II (girls 7–18 years of age, n = 106), and group III (boys 7–18 years of age, n = 118). Results: Using RT3DE, RV volumes were slightly higher than using cardiac magnetic resonance (EDV, 0.8 ± 5.8% [limits of agreement, −10.8% to 12.5%; r = 0.993]; ESV, 2.0 ± 13.1% [limits of agreement, −24.2% to 28.2%; r = 0.989). Reproducibility was promising (intraobserver variability, 3.9 ± 11.4% for EDV and −1.7 ± 13.4% for ESV [intraclass correlation coefficient range, 0.94–0.98]; interobserver variability, 1.9 ± 11.8% for EDV and −0.3 ± 22.8% for ESV [intraclass correlation coefficient range, 0.85–0.96]). Regarding functional parameters, no significant gender differences were found among children in group I. In contrast, children in groups II and III differed in RV volumes, dimensional parameters, and tricuspid annular plane systolic excursion (P < .005); the children did not differ in deformation parameters. Feasibility was 90%. Conclusions: RT3DE yields accurate and reproducible RV volumes. The calculated percentile curves may facilitate the clinical use of RT3DE to analyze RV function in children. HIGHLIGHTSValidation of right ventricular 3D Echocardiography in congenital heart disease.Reference values with high accuracy and reproducibility.Gender differences for RV volumes, EF, linear dimensions and TAPSE.

Real-Time Three-Dimensional Echocardiography of the Left Ventricle—Pediatric Percentiles and Head-to-Head Comparison of Different Contour-Finding Algorithms: A Multicenter Study

Background Real‐time three‐dimensional echocardiography (RT3DE) is a promising method for accurate assessment of left ventricular (LV) volumes and function, however, pediatric reference values are scarce. The aim of the study was to establish pediatric percentiles in a large population and to compare the inherent influence of different evaluation software on the resulting measurements. Methods In a multicenter prospective‐design study, 497 healthy children (ages 1 day to 219 months) underwent RT3DE imaging of the LV (ie33, Philips, Andover, MA). Volume analysis was performed using QLab 9.0 (Philips) and TomTec 4DLV2.7 (vendor‐independent; testing high (TomTec75) and low (TomTec30) contour‐finding activity). Reference percentiles were computed using Coles LMS method. In 22 subjects, cardiovascular magnetic resonance imaging (CMR) was used as the reference. Results A total of 370/497 (74.4%) of the subjects provided adequate data sets. LV volumes had a significant association with age, body size, and gender; therefore, sex‐specific percentiles were indexed to body surface area. Intra‐ and interobserver variability for both workstations was good (relative bias ± SD for end‐diastolic volume [EDV] in %: intraobserver: QLab = −0.8 ± 2.4; TomTec30 = −0.7 ± 7.2; TomTec75 = −1.9 ± 6.7; interobserver: QLab = 2.4 ± 7.5; TomTec30 = 1.2 ± 5.1; TomTec75 = 1.3 ± 4.5). Intervendor agreement between QLab and TomTec30 showed larger bias and wider limits of agreement (bias: QLab vs TomTec30: end‐systolic volume [ESV] = 0.8% ± 23.6%; EDV = −2.2% ± 17.0%) with notable individual differences in small children. QLab and TomTec underestimated CMR values, with the highest agreement between CMR and QLab. Conclusions RT3DE allows reproducible noninvasive assessment of LV volumes and function. However, intertechnique variability is relevant. Therefore, our software‐specific percentiles, based on a large pediatric population, serve as a reference for both commonly used quantification programs. HighlightsPediatric reference percentiles for 3D‐RTE of the left ventricle are provided.Reference values are gender‐specific and correlate with BSA, weight, and height.LV 3D volumes are dependent on the software algorithm used for 3D data analysis.

Synkopen nach Belastung

ZusammenfassungGefäßbedingte Stenosen der Luftwege gehören zur Differenzialdiagnose des Asthma bronchiale. Wir berichten über ein 11-jähriges Mädchen mit seit der Kindheit therapiertem Asthma bronchiale, welches uns zur Abklärung rezidivierender Synkopen nach Belastung vorgestellt wurde. Es zeigte sich eine hochgradige Trachealstenose durch einen doppelten Aortenbogen mit reflektorischer Hypoventilation und CO2-Retention in der Spiroergometrie. Nach Durchtrennung des die Stenose verursachenden rechten Aortenbogens kam es zur vollständigen Normalisierung der spirometrischen Befunde und Beschwerdefreiheit.AbstractVascular airway compression syndromes have to be differentiated from bronchial asthma. We report the case of an 11-year-old girl who had been treated for bronchial asthma since childhood and presented with recurrent syncope after exercise. A double aortic ring caused severe tracheal stenosis with reflex ventilation depression and CO2 retention documented by spiroergometry. After transsection of the right dorsal arch the symptoms resolved and flow-volume charts and gas exchange were normal.