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With rising survival rates of patients with congenital heart disease (CHD), functional health variables have become the key aspect in treatment evaluation. The effectiveness of various treatment options on the health-related quality of life (HRQoL) and the objectively measured exercise capacity as peak oxygen uptake (VO2 peak) remains rather unclear and hence, its investigation is the primary aim of this study. Data from 1014 patients (≥14-years-old, various CHD) were retrospectively reviewed. The patients had completed at least twice the SF-36 questionnaire on HRQoL prior to a cardiopulmonary exercise test. Each patient was assigned to 1 of 4 treatment groups (i.e., surgery, catheter intervention, drug therapy, and surveillance) according to the received treatment between the baseline and the follow-up examination. After 4.0 ± 2.2 years of follow-up, patients with surgery and catheter intervention showed an increase in the physical summary score of HRQoL as compared to the other treatment groups (p <0.001). This effect remained also significant in a multivariable model accounting for anthropometric and baseline data. No significant differences in the mental summary score of HRQoL and the VO2 peak were evident between the different treatment groups in the multivariable model. No significant correlation was found between the changes in HRQoL and VO2 peak over time. In conclusion, despite insignificant changes in aerobic capacity, adolescents and adults with CHD report better physical HRQoL following surgery and catheter intervention compared to the other treatment options. HRQoL and exercise capacity need to be considered concurrently in the evaluation of adolescents and adults with CHD.

As thromboembolic events (TEE) are common in adults with congenital heart disease (ACHD), adequate oral anticoagulation for prophylaxis or treatment of TEE is important. Until now, mainly vitamin K antagonists have been used in these patients. The purpose of this study was to provide first data on the use of direct oral anticoagulants in ACHD. This prospective, observational, and longitudinal study included 102 consecutive ACHD, of whom 75 (37 women and 38 men; mean age 50 ± 13 years) could be analyzed. Most common CHD were pre-tricuspid shunts (n = 31; 41%), complex CHD (n = 16; 21%), left heart/aortic valve anomalies (n = 5; 6%), right-sided cardiac/pulmonary artery anomalies (n = 9; 13%), post-tricuspid shunts (n = 3; 4%), and others (n = 11; 15%). Five patients had cyanosis and 3 patients a Fontan circulation. Mean follow-up was 12 ± 11 months. Rivaroxaban was administered in 55 patients, apixaban in 13 and dabigatran in 7 patients for TEE prophylaxis in atrial arrhythmias (n = 57), stroke/transient ischemic attacks (n = 11), deep vein thrombosis (n = 4), pulmonary embolism (n = 1) and atrial thrombi (n = 3). Some patients had >1 indication for adequate oral anticoagulation. CHA2-DS2-VASc score was ≥2 in 23 (31%), and 9 (12%) had a HAS-BLED score ≥2. There were neither thrombotic or major bleeding events nor major side effects. In conclusion, direct oral anticoagulants appear to be safe and effective in ACHD. Long-term follow-up is needed to substantiate these findings.

The stressful and potentially traumatic perception of repeated hospitalization, outpatient check-ups, and medical interventions places a high stress burden on patients with congenital heart disease (CHD). These experiences can lead to post-traumatic stress symptoms (PTSSs). This study aimed to estimate the prevalence of PTSSs in adults with CHD (ACHDs) and to identify the associated risk factors. In this cross-sectional study, 234 ACHDs were recruited from November 2021 to August 2022 at a dedicated tertiary care center. Data were collected on general health, anxiety and depression, PTSSs, and on quality of life using validated and standardized questionnaires. In addition, the reasons for PTSSs were assessed using free-text responses. Overall, 17.1% to 20.5% (mean age: 35.2 ± 10.8 [18 to 66] years, 46.6% women) of the enrolled patients met the criteria for clinically relevant PTSSs related to their CHD or treatment. The associated risk factors (p <0.05) included preexisting mental distress (odds ratio [OR] 4.86), depression (OR 5.565) and anxiety (OR 3.36,), level of perceived mental distress during the traumatic event (OR 1.46), and number of medical procedures (OR 1.17). In addition, a worse clinical state was associated with more PTSSs (p = 0.018). Using free-text responses, the various reasons for PTSSs were identified, ranging from cardiac procedures to social stigma. In conclusion, the high prevalence of PTSSs calls for increased awareness of PTSSs in ACHDs in cardiovascular care. PTSSs and their associated disorder can adversely affect the manifestation and progression of cardiac disease. Thus, it is necessary to reflect upon psychocardiac prevention and intervention as an integral part of multidisciplinary cardiac care.

Performance of cardiovascular magnetic resonance (CMR) in the planning phase of percutaneous pulmonary valve implantation (PPVI) is needed for the accurate delineation of the right ventricular outflow tract (RVOT), coronary anatomy and the quantification of right ventricular (RV) volume overload in patients with significant pulmonary regurgitation (PR). This helps to find the correct timings for the intervention and prevention of PPVI-related complications such as coronary artery compression, device embolization and stent fractures. A defined CMR study protocol should be set for all PPVI candidates to reduce acquisition times and acquire essential sequences that are determinants for PPVI success. For correct RVOT sizing, contrast-free whole-heart sequences, preferably at end-systole, should be adopted in the pediatric population thanks to their high reproducibility and concordance with invasive angiographic data. When CMR is not feasible or contraindicated, cardiac computed tomography (CCT) may be performed for high-resolution cardiac imaging and eventually the acquisition of complementary functional data. The aim of this review is to underline the role of CMR and advanced multimodality imaging in the context of pre-procedural planning of PPVI concerning its current and potential future applications.

ObjectiveThe present cross-sectional study investigated quality of life (QOL) in a large cohort of German adults with congenital heart disease (ACHDs) in association with patient-related and clinical variables.DesignCross-sectional survey.ParticipantsBetween 2016 and 2019, a representative sample of 4014 adults with various forms of congenital heart defect (CHD) was retrospectively analysed. Inclusion criteria were confirmed diagnosis of CHD; participant aged 18 years and older; and necessary physical, cognitive and language capabilities to complete self-report questionnaires.Primary and secondary outcome measuresQOL was assessed using the 5-level EQ-5D version (EQ-5D-5L). Sociodemographic and medical information was obtained by a self-devised questionnaire. Associations of QOL with patient-reported clinical and sociodemographic variables were quantified using multiple regression analysis and multiple ordinal logit models.ResultsOverall, ACHDs (41.8±17.2 years, 46.5% female) reported a good QOL comparable to German population norms. The most frequently reported complaints occurred in the dimensions pain/discomfort (mean: 16.3, SD: p<0.001) and anxiety/depression (mean: 14.3, p<0.001). QOL differed significantly within ACHD subgroups, with patients affected by pretricuspid shunt lesions indicating the most significant impairments (p<0.001). Older age, female sex, medication intake and the presence of comorbidities were associated with significant reductions in QOL (p<0.001). CHD severity was positively associated with QOL within the dimensions of self-care (OR 0.148, 95% CI 0.04 to 0.58) and mobility (OR 0.384, 95% CI 0.19 to 0.76).ConclusionCurrent findings temper widely held assumptions among clinicians and confirm that ACHDs experience a generally good QOL. However, specific subgroups may require additional support to cope with disease-related challenges. The negative correlation of QOL with age is especially alarming as the population of ACHDs is expected to grow older in the future.Trial registration numberDRKS00017699; Results.

Background Congenital heart defects (CHD) affect about 8 out of 1000 births worldwide. Most of the patients reach adulthood and are exposed to an increased risk of endocarditis. Since bacteria already enter the bloodstream during everyday activities, oral hygiene is given special importance in the prevention of endocarditis. Methods In this study 81 boys (55.1%) and 66 (44.9%) girls with CHD received a dental exam and additionally an assessment using the DIAGNOdent® pen. This study group consisting of patients with CHD was matched with a healthy epidemiological control group in Germany. Results Eighty-one boys (55.1%) and 66 (44.9%) girls were examined. The mean age was 11 ± 4 years. 38.8% showed at least one untreated carious lesions. 37.4% had a dmft/DMFT ≥2 and thus represented a group with an increased caries risk. The dmft value was 2.12 ± 1.25 in the age group 3-6 year olds. In the group of the 7-12 year old patients the DMFT/dmft was 2.06 ± 2.27, whereas DMFT in 13-17 year olds was at 2.12 ± 1.58. However, children and adolescents with CHD had a higher DMF index than healthy children in the same age group. Conclusions The present study reveals that more than one third of those examined have a dental condition in need of rehabilitation. In future, close interdisciplinary cooperation between pediatric cardiologists and dentists should ensure regular dental check-ups.

Background and Objectives: Congenital heart defects (CHD) affect around 1% of the population, making them the most common congenital disease worldwide. Thanks to advances in treatment, over 90% of affected children are able to reach adulthood, shifting focus to long-term outcomes such as disease-specific quality of life (DsQoL). To date, there has been no validated, standardized instrument for assessing DsQoL in young German CHD patients. This study introduces the Congenital Heart Disease Specific Inventory (CHDSI), the first freely available German-language instrument for measuring DsQoL in children and adolescents with CHD. Materials and Methods: The CHDSI was developed at the German Heart Center Munich in collaboration with affected children and adolescents and validated nationwide via the National Register for Congenital Heart Defects (NRCHD) with 1201 participants (46 kindergarten children, 530 children, 625 adolescents). Two age-specific versions (36/37 items) and a 31-item preschool version were created, alongside a 6-item short form (CHDSI-SF) for rapid screening. Reliability was assessed using Cronbach’s alpha and split-half methods; construct validity via confirmatory factor analysis (CFA) using DWLS; and score interpretation through standardized stanine scales. The small sample size of kindergarten children precluded a model test for this group. The standard values given for this subsample should therefore be interpreted with caution. Results: The CHDSI showed excellent internal consistency (Cronbach’s α = 0.856 to 0.900) and high split-half reliability (>0.95). CFA confirmed a robust six-factor structure with excellent model fit (CFI and TLI ≥ 0.991, RMSEA ≤ 0.05). Subscales showed strong discriminant validity, and significant differences were found by CHD severity and sex. Conclusions: The CHDSI is a psychometrically valid, age-appropriate, and freely available instrument for assessing DsQoL in children and adolescents with CHD. It provides valuable support for clinical decision-making and research. Further studies should explore international validation and cultural adaptation.

Background: Congenital heart disease (CHD) is associated with an increased risk of anxiety and depression in adults. However, little is known about the mental health of children and adolescents with CHD. The aim of this study was to assess differences in anxiety and depression symptoms between children and adolescents with CHD and healthy controls. Methods: A total of 232 children and adolescents (age 7–18 years; mean age 13.5 ± 2.7 years, 50.9% female) were enrolled, consisting of 116 patients with CHD and 116 age- and sex-matched healthy controls. Participants were recruited during routine medical examinations at the German Heart Center and Munich schools, respectively. The Beck Anxiety Inventory (BAI) and the Depression Inventory for Youth (BDI-Y) were used to assess anxiety and depression symptoms. Results: The CHD cohort included patients with right heart obstruction (11.2%), left heart obstruction (19.8%), isolated shunts (15.5%), transposition of the great arteries (14.7%), univentricular heart (14.7%), and other defects (24.1%). According to published cut-off values, at least a mild form of anxiety was present in 46.5% CHD patients. However, no significant differences were observed between the CHD group and healthy controls in either the BDI-Y score (CHD: 7.9 ± 7.7 vs. controls: 8.6 ± 8.5; p = 0.569) or the BAI score (CHD: 9.3 ± 8.6 vs. controls: 9.3 ± 10.3; p = 0.429). The complexity of the heart defect was not associated with BAI scores (simple: 5.9 ± 5.7; moderate: 11.1 ± 8.1; complex: 9.3 ± 9.0; p = 0.073) or BDI-Y scores (simple: 7.4 ± 7.5; moderate: 9.0 ± 7.1; complex: 7.0 ± 7.7; p = 0.453). No significant differences in BAI (p = 0.141) or BDI-Y (p = 0.326) scores were found by type of heart defect. Conclusions: Children and adolescents with CHD did not exhibit significantly higher levels of depression or anxiety symptoms compared to healthy controls. Nevertheless, given the increased psychological risk observed in adults with CHD, ongoing mental health monitoring remains important to enable early identification and timely intervention. Further research, particularly through longitudinal studies, is needed to monitor mental health trajectories over time and to identify early predictors of psychological vulnerability in this population.

Hypertrophic cardiomyopathy (HCM) caused by autosomal-dominant mutations in genes coding for structural sarcomeric proteins, is the most common inherited heart disease. HCM is associated with myocardial hypertrophy, fibrosis and ventricular dysfunction. Hypoxia-inducible transcription factor-1α (Hif-1α) is the central master regulators of cellular hypoxia response and associated with HCM. Yet its exact role remains to be elucidated. Therefore, the effect of a cardiomyocyte-specific Hif-1a knockout (cHif1aKO) was studied in an established α-MHC 719/+ HCM mouse model that exhibits the classical features of human HCM. The results show that Hif-1α protein and HIF targets were upregulated in left ventricular tissue of α-MHC 719/+ mice. Cardiomyocyte-specific abolishment of Hif-1a blunted the disease phenotype, as evidenced by decreased left ventricular wall thickness, reduced myocardial fibrosis, disordered SRX/DRX state and ROS production. cHif1aKO induced normalization of pro-hypertrophic and pro-fibrotic left ventricular remodeling signaling evidenced on whole transcriptome and proteomics analysis in α-MHC 719/+ mice. Proteomics of serum samples from patients with early onset HCM revealed significant modulation of HIF. These results demonstrate that HIF signaling is involved in mouse and human HCM pathogenesis. Cardiomyocyte-specific knockout of Hif-1a attenuates disease phenotype in the mouse model. Targeting Hif-1α might serve as a therapeutic option to mitigate HCM disease progression.

Background: Cardiovascular magnetic resonance Feature Tracking (CMR-FT) is a well-established method to assess myocardial contraction with diagnostic and prognostic value in many diseases. We aimed to evaluate the role of right ventricular (RV) CMR-FT in the perioperative assessment of Ebstein patients undergoing Cone repair. Methods: We analyzed the CMR data of 18 Ebstein patients before and after Cone repair including CMR-FT-derived global radial (GRS), global circumferential (GCS) and global longitudinal strain (GLS). Results: Following Cone repair, tricuspid regurgitation decreased from 48% to 6%, p = 0.0001. RV ejection fraction (51% to 33%, p = 0.0002), indexed RV stroke volumes (74 mL/m2 to 43 mL/m2, p = 0.0013) and GLS (−15.01% to −14.53%, p = 0.0155) decreased postoperatively. Conversely, GRS (15.00% to 17.83%, p = 0.0202) and GCS (−8.82% to −13.02%, p = 0.0026) improved. Indexed RV end-diastolic volumes (RVEDVis) decreased, although not significantly, from 161 mL/m2 to 122 mL/m2, p = 0.3465. Eight patients exhibited a higher RVEDVi after surgery. Pulmonary artery and aortic flow and left ventricular (LV) functional parameters remained unchanged. Conclusions: RV GLS appears to be affected by the hemodynamic alterations caused by Cone repair. RV GCS and GRS might serve as more independent parameters of myocardial function.