PATHFINDER-CHD: prospective registry on adults with congenital heart disease, abnormal ventricular function, and/or heart failure as a foundation for establishing rehabilitative, prehabilitative, preventive, and health-promoting measures: rationale, aims, design and methods.

BACKGROUND: Adults with congenital heart defects (ACHD) globally constitute a notably medically underserved patient population. Despite therapeutic advancements, these individuals often confront substantial physical and psychosocial residua or sequelae, requiring specialized, integrative cardiological care throughout their lifespan. Heart failure (HF) is a critical challenge in this population, markedly impacting morbidity and mortality. AIMS: The primary aim of this study is to establish a comprehensive, prospective registry to enhance understanding and management of HF in ACHD. Named PATHFINDER-CHD, this registry aims to establish foundational data for treatment strategies as well as the development of rehabilitative, prehabilitative, preventive, and health-promoting interventions, ultimately aiming to mitigate the elevated morbidity and mortality rates associated with congenital heart defects (CHD). METHODS: This multicenter survey will be conducted across various German university facilities with expertise in ACHD. Data collection will encompass real-world treatment scenarios and clinical trajectories in ACHD with manifest HF or at risk for its development, including those undergoing medical or interventional cardiac therapies, cardiac surgery, inclusive of pacemaker or ICD implantation, resynchronization therapy, assist devices, and those on solid organ transplantation. DESIGN: The study adopts an observational, exploratory design, prospectively gathering data from participating centers, with a focus on patient management and outcomes. The study is non-confirmatory, aiming to accumulate a broad spectrum of data to inform future hypotheses and studies. PROCESSES: Regular follow-ups will be conducted, systematically collecting data during routine clinical visits or hospital admissions, encompassing alterations in therapy or CHD-related complications, with visit schedules tailored to individual clinical needs. ASSESSMENTS: Baseline assessments and regular follow-ups will entail comprehensive assessments of medical history, ongoing treatments, and outcomes, with a focus on HF symptoms, cardiac function, and overall health status. DISCUSSION OF THE DESIGN: The design of the PATHFINDER-CHD Registry is tailored to capture a wide range of data, prioritizing real-world HF management in ACHD. Its prospective nature facilitates longitudinal data acquisition, pivotal for comprehending for disease progression and treatment impacts. CONCLUSION: The PATHFINDER-CHD Registry is poised to offer valuable insights into HF management in ACHD, bridging current knowledge gaps, enhancing patient care, and shaping future research endeavors in this domain.

Quality of life in patients with Marfan syndrome: a cross-sectional study of 102 adult patients.

BACKGROUND: Marfan syndrome (MFS) is a genetically determined multiorgan disease that leads to severe physiological and psychological impairments in adult life. Little consensus exists regarding quality of life (QOL) in individuals with MFS. The present study sought to investigate QOL in a representative cohort of adults with MFS. METHODS: Patient-reported outcome measures from a representative sample of 102 adults with MFS (39.3±13.1 years of age; 40.2% female) were retrospectively analyzed and compared with those from adults with different congenital heart defects (CHD), at the German Heart Center Munich. QOL was assessed using the updated five-level version of the EQ-5D. RESULTS: Differences between both populations were analyzed. Subjects affected by MFS reported an overall reduced QOL. Compared to CHD patients, individuals with MFS scored significantly lower in the dimensions of pain/discomfort, anxiety/depression, mobility and usual activities (P<0.05). CONCLUSIONS: Patients with MFS are at high risk for impaired QOL, especially in mental and physical domains. Psychosocial consequences of MFS cost resources for both, patients and professionals. Current findings highlight the great importance of additional psychological support to cope with disease-related challenges. Increased attention should be directed towards enhancing their subjective wellbeing to potentially improve their QOL and long-term health outcomes.

Prognostic value of different biomarkers for cardiovascular death in unselected older patients in the emergency department.

BACKGROUND: Risk stratification of elderly patients presenting with heart failure (HF) to an emergency department (ED) is an unmet challenge. We prospectively investigated the prognostic performance of different biomarkers in unselected older patients in the ED. METHODS: We consecutively enrolled 302 non-surgical patients ⩾70 years presenting to the ED with a wide range of cardiovascular and non-cardiovascular comorbid conditions. N-terminal pro-B-type natriuretic peptide (NT-proBNP), mid-regional pro-adrenomedullin (MR-proADM), mid-regional pro-atrial natriuretic peptide (MR-proANP), C-terminal pro-endothelin-1 (CT-proET-1), ultrasensitive C-terminal pro-arginine-vasopressin (Copeptin-us) and high-sensitivity cardiac troponin T (hs-cTnT) were measured at admission. Two cardiologists independently adjudicated the final diagnosis of HF after reviewing all available baseline data using circulating NT-proBNP levels. A final diagnosis of HF was found in 120 (40%) of the 302 patients. All patients were followed up for cardiovascular death within the following 12 months. In order to test the prognostic performance of the investigated biomarkers we used boosting models with age and sex as mandatory covariates. Boosting is a statistical learning technique with built-in variable selection developed to obtain sparse and interpretable prediction models. RESULTS: Follow-up was 100% complete. During a median follow-up time of 225 days (interquartile range (IQR) 156-319 days), 30 (9.9%) of 302 patients (aged 81±6 years) had cardiovascular deaths. Of these 30 patients, 21 had HF and nine had no HF diagnosed prior to admission. The boosting model selected MR-proADM and hs-cTNT as predictors of cardiovascular deaths. The median values of MR-proADM and hs-cTnT at presentation were significantly higher in patients with cardiovascular deaths compared to surviving patients during follow-up (2.56 nmol/L (IQR 1.62-4.48) vs. 1.11 nmol/L (IQR 0.83-1.80), P<0.001 and 81 ng/L (IQR 38-340) vs. 17 ng/L (IQR 0.9-38), P=0.004). One unit increase in the log-transformed MR-proADM levels was associated with a 1.99-fold risk of death (95% confidence interval (CI) 1.61-2.45, P<0.001). The second marker, hs-cTnT, showed an increased predicted risk but was not significantly correlated to event-free survival (hazard ratio 3.22, 95% CI 0.97-10.68, P=0.056). CONCLUSION: Within different biomarkers, MR-proADM was the only predictor of cardiovascular deaths in unselected older patients presenting to the ED.

Evaluation of the short- and long-term safety and therapy outcomes of the everolimus-eluting bioresorbable vascular scaffold system in patients with coronary artery stenosis: Rationale and design of the German-Austrian ABSORB RegIstRy (GABI-R).

BACKGROUND: Third-generation drug-eluting metal stents are the gold standard for treatment of coronary artery disease. The permanent metallic caging of the vessel, however, can result in limited vasomotion, chronic inflammation, and late expansive remodeling, conditions that can lead to late and very late stent thrombosis. The development of bioresorbable scaffolds (BRSs) promises advantages over metal stents due to complete biodegradation within 2-4years. Theoretically, since vessel scaffolding is temporary and no permanent implant remains in the vessel, BRSs, as opposed to metal stents, once degraded would no longer be potential triggers for stent-related adverse events or side effects. METHODS/DESIGN: The short- and long-term outcome after implantation of an everolimus-eluting, poly-L-lactic acid-based bioresorbable scaffold system (ABSORB, Abbott Vascular, Santa Clara, CA, USA) in the world-wide greatest all-comers cohort will be evaluated in the prospective, non-interventional, multicenter German-Austrian ABSORB RegIstRy (GABI-R). GABI-R will include over 5000 patients from about 100 study sites in Austria and Germany. Safety endpoints such as cardiac death, myocardial infarction, and clinically driven percutaneous or surgical target lesion and vessel revascularization will be evaluated during hospitalization and in the follow-up period (minimum of 5years). CONCLUSION: Although two randomized controlled trials and several registries have documented safety and efficacy as well as non-inferiority of this everolimus-eluting ABSORB device compared with drug-eluting metal stents, the current knowledge regarding clinical application, treatment success, and long-term safety of using this BRS in daily routine is limited. Thus, the goal of GABI-R is to address this lack of information.

Impact of coronary artery calcium characteristics on accuracy of CT angiography.

OBJECTIVES: This study sought to evaluate which specific calcium characteristics impact diagnostic accuracy of coronary computed tomography angiography (CTA). BACKGROUND: Coronary calcifications comprise one of the most significant factors interfering with diagnostic accuracy of coronary CTA. Despite this fact, there is paucity of data regarding this phenomenon. METHODS: A total of 525 coronary lesions (252 calcified and 273 reference [noncalcified] lesions) within 97 arteries of 60 patients (19 women, age 63 ± 10 years) underwent assessment with both 2 × 64-slice computed tomography and intravascular ultrasound (IVUS). Nineteen calcium characteristics were determined. The main outcome was coronary CTA inaccuracy defined as the deviation of minimum lumen area within the calcification measured with coronary CTA from that measured with IVUS, in both absolute (mm(2)) and relative (%) terms. RESULTS: Presence of calcification was found to be independently correlated to coronary CTA inaccuracy in both absolute and relative terms (p < 0.001 for both). The relative (%) inaccuracy of coronary CTA was independently correlated to total calcium length (p = 0.004), total calcium volume (p = 0.008), cross section calcium thickness (p = 0.023), cross section calcium area (p = 0.023), and cross section lumen area (p = 0.001). The absolute inaccuracy of CTA was correlated to calcium length (p = 0.010), calcium volume (p = 0.017), and cross section calcium area (p < 0.001). The presence of both total calcium arc ≥47° and mean lumen diameter of ≤2.8 mm provided the best predictive accuracy for detection of excessive lumen underestimation by CTA. The best accuracy for prediction of excessive lumen overestimation provided combination of 2 of 3 features: maximum calcium density <869 HU, OR whole calcium length <2.4 mm, OR total calcium volume <6.4 mm(3). CONCLUSIONS: Our results indicate which specific calcium characteristics impact accuracy of coronary CTA in lumen assessment within calcified lesions. This may provide practical assistance in predicting coronary lumen underestimation or overestimation by coronary CTA, therefore mitigating risk of diagnostic errors in clinical practice.

First performance evaluation of a dual-source CT (DSCT) system.

We present a performance evaluation of a recently introduced dual-source computed tomography (DSCT) system equipped with two X-ray tubes and two corresponding detectors, mounted onto the rotating gantry with an angular offset of 90 degrees . We introduce the system concept and derive its consequences and potential benefits for electrocardiograph [corrected] (ECG)-controlled cardiac CT and for general radiology applications. We evaluate both temporal and spatial resolution by means of phantom scans. We present first patient scans to illustrate the performance of DSCT for ECG-gated cardiac imaging, and we demonstrate first results using a dual-energy acquisition mode. Using ECG-gated single-segment reconstruction, the DSCT system provides 83 ms temporal resolution independent of the patient's heart rate for coronary CT angiography (CTA) and evaluation of basic functional parameters. With dual-segment reconstruction, the mean temporal resolution is 60 ms (minimum temporal resolution 42 ms) for advanced functional evaluation. The z-flying focal spot technique implemented in the evaluated DSCT system allows 0.4 mm cylinders to be resolved at all heart rates. First clinical experience shows a considerably increased robustness for the imaging of patients with high heart rates. As a potential application of the dual-energy acquisition mode, the automatic separation of bones and iodine-filled vessels is demonstrated.