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.

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.