Die erste Leitlinie der European Society of Cardiology (ESC) zum Management der Myokarditis und Perikarditis führt das inflammatorische myoperikardiale Syndrom (IMPS) als übergeordnete Erkrankung für das gesamte Spektrum entzündlicher myokardialer und perikardialer Erkrankungen ein. Sie betont gemeinsame Ätiologien, klinische Überlappungen und die Notwendigkeit erhöhter Aufmerksamkeit für Risikokonstellationen („red flags“). Ein zentrales Novum ist der symptombasierte Algorithmus (Thoraxschmerz, Arrhythmie, Herzinsuffizienz) mit klaren diagnostischen und therapeutischen Pfaden. Die multimodale, primär nicht invasive Diagnostik, insbesondere mittels kardiovaskulärer Magnetresonanztomographie, ermöglicht eine definitive Myokarditisdiagnose. Die Endomyokardbiopsie sollte bei Hochrisikokonstellationen, wie z. B. bei instabiler Kreislaufsituation, oder bei Verdacht auf spezifische Myokarderkrankungen mit schnellem Therapiebedarf wie der Riesenzellmyokarditis angewendet werden. Genetische, autoimmunologische und autoinflammatorische Mechanismen werden stärker berücksichtigt inklusive selektiver genetischer Testung. Therapeutisch werden eine risikoadaptierte, personalisierte Strategie von antiinflammatorischer Behandlung über Herzinsuffizienz- und Device-Therapie sowie eine individualisierte Sport- und Arbeitskarenz angeboten. Für die rezidivierende Perikarditis werden Anti-Interleukin-1-Therapien integriert. Multidisziplinäre Betreuung ist bei komplizierten Verläufen ein zentraler Bestandteil des Managements.

BACKGROUND AND AIMS:The prediction of the first major arrhythmic event (MAE) is still an unmet need in the recently defined scenario of non-dilated left ventricular cardiomyopathy (NDLVC). METHODS:A cohort of 337 patients with NDLVC and no history of MAE was retrospectively identified at two large centres. Patient-tailored diagnostic workup included cardiac magnetic resonance (CMR), endomyocardial biopsy, and genetic testing. The primary endpoint was the occurrence of the first MAE, including sustained ventricular tachycardia (VT), ventricular fibrillation, or appropriate implantable cardioverter-defibrillator therapy, by 60-month follow-up. A pool of 216 NDLVC patients from 11 European centres served as a validation cohort. RESULTS:In the study cohort (mean age 37 ± 15 years, 62% male), the mean left ventricular ejection fraction (LVEF) was 52 ± 8%, and 79% of patients had late gadolinium enhancement (LGE) at baseline CMR. By 60-month follow-up, 51 patients (15%) experienced a MAE. The primary endpoint was predicted by male sex [hazard ratio (HR) 2.4, 95% confidence interval (CI) 1.3-4.4, P = .007], baseline non-sustained VT (HR 3.1, 95% CI 1.7-5.6, P < .001), LVEF < 45% (HR 5.5, 95% CI 2.7-11.0, P < .001), septal (HR 2.0, 95% CI 1.0-4.0, P = .046) and ring-like pattern of LGE (HR 1.3, 95% CI .6-2.6, P = .54), pathogenic/likely pathogenic variants in guideline-defined high-risk genes (HR 4.6, 95% CI 2.3-9.1, P < .001), and biopsy/CMR-proven myocardial inflammation (HR 15.7, 95% CI 6.1-40.3, P < .001). The results were confirmed in the validation cohort (Uno's C-index 0.81, 95% CI .75-.88). A novel risk score was subsequently derived. CONCLUSIONS:In NDLVC, male sex, non-sustained VT, LVEF < 45%, septal and ring-like LGE, high-risk genotypes, and myocardial inflammation predicted the first episode of MAE by 60 months.

The first guidelines of the European Society of Cardiology (ESC) on the management of myocarditis and pericarditis introduce the inflammatory myopericardial syndrome (IMPS) as an overarching disease encompassing the full spectrum of inflammatory myocardial and pericardial diseases. It emphasizes shared etiologies, clinical overlaps and the need for heightened awareness of high-risk features (red flags). A key innovation is the symptom-based algorithm (chest pain, arrhythmia, heart failure) providing structured diagnostic and treatment pathways. Multimodal imaging, primarily noninvasive and particularly using cardiovascular magnetic resonance imaging, enables a definitive diagnosis of myocarditis. An endomyocardial biopsy should be included in high-risk constellations, such as hemodynamic instability or suspected specific myocardial diseases requiring rapid treatment, such as giant cell myocarditis. Greater emphasis is placed on genetic, autoimmune and autoinflammatory mechanisms, including selective genetic testing. Treatment management follows a risk-adapted, personalized strategy ranging from anti-inflammatory treatment to heart failure and device treatment as well as individualized abstention from sports and work. Anti-interleukin-1 therapy is incorporated for recurrent pericarditis. Multidisciplinary care is a central component of the management in complicated cases.

BACKGROUND:Rapid diagnostic screening is an unmet need in patients with suspected cardiomyopathy (CM). The authors recently demonstrated in a retrospective study that magnetocardiography (MCG) may be a suitable tool to detect CM. OBJECTIVES:This study aims to prospectively evaluate the accuracy of MCG as a screening tool in patients with suspected CM. METHODS:MCG uses a superconducting quantum interference device to detect the magnetic field of the heart. In another recent retrospective study by the same investigators, a T-beg-Tmax (MCG score) ≥0.051 identified patients with CM (dilated, hypertrophic, nondilated left ventricular, and restrictive CM) after exclusion of coronary artery disease. The authors assessed the diagnostic accuracy of MCG compared with advanced imaging and/or endomyocardial biopsy. The authors enrolled 110 adult patients with angina-like symptoms after exclusion of coronary artery disease from the emergency department, inpatient wards, and outpatient clinics. A total of 220 healthy individuals were incorporated into the study as control subjects by using 1:2 direct matching (age decade and sex) for a total of 330 participants. RESULTS:MCG detected CM with 94.74% sensitivity (95% CI: 85%-99%), 98.54% specificity (95% CI: 96%-100%), 93.1% negative predictive value (95% CI: 84%-98%), and 94.34% positive predictive value (95% CI: 84%-98%). In 3 patients, cardiac magnetic resonance failed to detect CM, whereas MCG showed pathologic results consistent with results of endomyocardial biopsy. Results were highly reproducible (statistical uncertainty: 2.5%, T-beg-Tmax [interval and electrical vector from the beginning of a T-wave to its peak (MCG score)] ±0.004). CONCLUSIONS:MCG offers a noninvasive, reproducible methodology for rapid and accurate detection of CM after exclusion of ischemic heart disease. Our data indicate that MCG may be an efficient tool for allocation of advanced imaging to those patients with a high likelihood of disease. (Magnetocardiography as a Diagnostic Screening Tool for Myocarditis and Other Types of Cardiomyopathy [MagMa]; NCT06689098).

INTRODUCTION:Myocarditis and pericarditis represent a broad spectrum of inflammatory heart diseases and are important differential diagnoses in patients presenting with chest pain, heart failure, or arrhythmias. Diagnosis is often challenging due to non-specific clinical presentation and biomarkers. Growing recognition of the clinical relevance of myocardial inflammation, together with accumulating evidence, has led to increased scientific and clinical attention. This is reflected in emerging concepts such as the introduction of the new term "inflammatory myocardial syndrome (IMPS)" in the recently published guidelines of the European Society of Cardiology (ESC). Notably, these are the first ESC guidelines to cover the full spectrum of inflammatory diseases of the myocardium and pericardium - marking an important paradigm shift in the field. Current ESC guidelines highlight the central role of multimodality cardiac imaging in the evaluation of myocardial inflammation. While echocardiography serves as a first-line tool, cardiac magnetic resonance imaging (CMR) is considered the non-invasive gold standard for tissue characterization. Nuclear imaging techniques such as positron emission tomography (PET) are increasingly relevant, particularly in specific entities such as cardiac sarcoidosis. This review highlights the practical role of multimodality imaging in the diagnostic work-up of myocardial inflammation, with emphasis on clinically relevant decision-making.

Heart involvement in eosinophilia represents a rare entity with substantial cardiac morbidity and mortality. This disorder is often overlooked and diagnosed in its advanced stages. In a subgroup of patients with eosinophilic heart disease, peripheral blood eosinophilia may not be present, and the biopsy of the affected organ or tissue is essential to establish the correct diagnosis. In this clinical consensus statement, an expert consensus group reviews the epidemiology, aetiology, diagnosis, and management of eosinophilic heart disease. The aim of the document is to increase awareness about the cardiac involvement associated with eosinophilia in order to diagnose the disease in early phases and prevent deleterious heart damage and adverse outcomes of affected individuals. The spectrum of physiological and immunological derangements requires a multidisciplinary approach for optimal management of affected individuals.

Myocarditis is an inflammatory disease involving the heart muscle and potentially the pericardium. While there are many potential causative agents, commonly grouped into infectious (viral, bacterial, parasitic) or noninfectious (autoimmune, systemic disorders, drugs, cancer related), the main pathological pathways ultimately lead to an inflammatory process of the myocardium resulting in necrosis and edema. As there are specific therapies available for patients with myocarditis, reliable and early diagnosis is crucial. Multimodality imaging, especially cardiovascular magnetic resonance, has made a noninvasive diagnosis feasible. Cardiovascular magnetic resonance can not only provide a diagnosis based on the updated Lake Louise criteria, but it also functions as a diagnostic gateway, leading to other imaging modalities, for example, positron emission tomography or computed tomography. Finally, imaging results can help to initiate treatment options as well as determine when a patient can return to work or exercise. This review will cover multimodal imaging in patients with myocarditis with a focus on cardiovascular magnetic resonance, providing case examples of how imaging can guide care and treatment in these patients. In addition, the review focuses on the recent European Society for Cardiology guideline on the management of myocarditis and pericarditis comparing the recommendation to the American College of Cardiology expert consensus statements and the Japanese Circulation Society guidelines.

AIMS:Concomitant aortic stenosis (AS) and cardiac amyloidosis (CA) result in heart failure and reduced life expectancy. Early detection of CA in AS is an unmet clinical need to prevent disease progression. The SAVER study aims to establish a simple CA screening for AS patients. METHODS AND RESULTS:SAVER is a prospective cohort study enrolling AS patients planned for transcatheter aortic valve implantation (TAVI) or surgical aortic valve replacement (SAVR). Firstly, patients were assessed for CA-specific symptoms and history on top of AS evaluation. Patients with suspected CA underwent DPD scintigraphy or magnetic resonance imaging. Secondly, we performed multiple-regression analysis to identify optimal parameters for selecting patients at risk of CA. From 2021 to 2023, 1001 patients were enrolled, with 405 (40%) flagged for potential CA. Two hundred six (21%) patients received further diagnostics due to the screening and five (0.5%) patients due to physician discretion, leading to 17 (2%) confirmed CA cases. Key predictors of CA included male sex [OR 23.8 (95% CI 2.6; 216.9)], carpal tunnel syndrome [OR 5.5 (95% CI 1.4; 22.0)], spinal stenosis [OR 4.1 (95% CI 1.1; 14.7)], heaviness or numbness of arms or legs [OR 3.8 (95% CI 1.1; 13.3)], NT-proBNP [OR 6.7 (95% CI 1.8; 25.3)], and sparkling myocardium [OR 4.8 (95% CI 1.3; 17.3)]. The optimized approach reached an AUC of 0.88 (95% CI 0.81-0.96). CONCLUSION:The SAVER approach is the first holistic screening method focusing on CA's multiorgan manifestations in AS. This approach can be implemented in clinical settings to prevent futile outcomes of combined disease.

BackgroundSystemic amyloidosis comprises a heterogeneous group of rare diseases characterised by extracellular deposition of misfolded protein fibrils, leading to progressive organ dysfunction. Due to the variability in clinical presentation and course, collection of system-specific and longitudinal data is essential for understanding disease progression, treatment response and patient outcomes. At the Amyloidosis Center Charité Berlin (ACCB), a prospective amyloidosis registry has been established to systematically collect clinical, laboratory, imaging and patient-reported data with the aim of improving the characterization of the diseases and facilitating translational research.MethodsThis is a single-center prospective registry study that enrols patients diagnosed with systemic amyloidosis. The registry includes demographic data, multidisciplinary clinical phenotyping, biomarkers, biobanking, genetic information, imaging studies, and patient reported outcomes. Here, we describe the standardised protocol for diagnostic workup, baseline and longitudinal data collection, and disease-specific follow-up algorithms. Data will be collected digitally in interoperable data formats to ensure shareability in accordance with GDPR-policies.DiscussionThis registry will serve as a resource for characterizing amyloidosis as a rare disease model in a real-world setting and identifying patterns in disease progression and treatment efficacy. By prospectively collecting high-quality longitudinal data, the study aims to generate insights that can inform clinical decision-making, improve risk stratification and support future intervention studies. In addition, the registry enables collaboration in the discovery of biomarkers and new therapeutic approaches. Ongoing analysis of this cohort will provide a basis for the further development of personalised treatment strategies and the improvement of patient care.Ethics and disseminationEthical approval was given by the local ethic committee. Dissemination of data in publications with different scientific observational and correlational questions is planned. Clinical trial registration: DRKS00032002.

Cardiac sarcoidosis (CS) is frequently diagnosed late due to its heterogeneous clinical presentation and limitations of cardiac magnetic resonance imaging (CMR). Early diagnosis is crucial to prevent irreversible myocardial damage and to improve outcomes. This study evaluates the added diagnostic value of positron emission tomography–computed tomography with 18 F-fluorodeoxyglucose (FDG) PET-CT following inconclusive CMR in a tertiary care setting. We retrospectively analyzed 29 patients with CS who underwent sequential CMR and FDG PET-CT between 2014 and 2024. Diagnosis of CS was based on European Society of Cardiology criteria, requiring compatible clinical features and histologic confirmation of either endomyocardial or systemic sarcoidosis. We assessed imaging findings, diagnostic timelines, and clinical outcomes. Of the 29 patients, 19 (65.5%) had endomyocardial biopsy-confirmed CS, and 10 (34.5%) had histologically confirmed systemic sarcoidosis with cardiac imaging findings suggestive of CS. CMR correctly identified CS in 14 patients (48.3%) of the total cohort, while FDG PET-CT identified CS correctly in the remaining 15 patients (51.7%). Among these, 11 had unremarkable CMR findings, and 4 had been initially misdiagnosed with dilated or nondilated left ventricular cardiomyopathy by CMR. In our cohort, FDG PET-CT uncovered a notable proportion of cardiac sarcoidosis cases that were not detected on CMR (51.7%). In addition our study revealed that diagnostic delays were substantial with up to 615 days from symptom onset to clinical suspicion of sarcoidosis. Taken together, our findings underscore the clinical importance of heightened awareness of sarcoidosis symptoms and the use of a complementary multimodal imaging strategy.

BACKGROUND:Biomarker-based prognostic staging systems, including the National Amyloidosis Centre (NAC) and the Mayo staging systems, are widely-used but have only been validated for treatment-naive patients with cardiac transthyretin amyloidosis (ATTR-CA). OBJECTIVES:The purpose of this study was to assess the accuracy of the NAC and Mayo staging systems in patients with ATTR-CA treated with tafamidis. METHODS:A retrospective observational study following patients with ATTR-CA from initiation of tafamidis (baseline) to time of all-cause death was conducted. Patients were stratified according to the NAC and an adapted Mayo staging system incorporating high-sensitivity cardiac troponin T. Agreement was assessed using weighted Cohen's kappa. The staging systems' ability to identify subgroups with distinct overall survival was assessed using Kaplan-Meier analyses and pairwise log-rank tests. RESULTS:A total of 251 patients with wild-type ATTR-CA treated with tafamidis were followed for a median of 521 (IQR: 262-842) days. There was substantial agreement (weighted kappa = 0.661; P < 0.001) between the NAC and the adapted Mayo staging system. Significant differences in estimated overall survival were observed across all disease stages of the adapted Mayo staging system (I vs II: P = 0.032; I vs III: P < 0.001; II vs III: P = 0.036). Accordingly, estimated overall survival was significantly lower in NAC III compared to NAC I (P < 0.001) and NAC II (P = 0.015). However, there was no significant difference between NAC I and NAC II (P = 0.340). CONCLUSIONS:Both staging systems identified a distinct group of patients with ATTR-CA at the highest risk of death but only the adapted Mayo staging system could accurately distinguish between low- and intermediate-risk patients in the setting of disease-modifying treatment with tafamidis.

BACKGROUND: Despite previous histopathologic evidence for its presence, the role of myocardial inflammation in the development and progression of cardiac transthyretin amyloidosis (ATTR-CA) remains insufficiently understood. Thus, this study sought to characterize the prevalence and potential prognostic implications of myocardial inflammation in ATTR-CA. METHODS: A retrospective observational study including patients with ATTR-CA diagnosed by endomyocardial biopsy was conducted. Myocardial inflammation was diagnosed through a review of routine endomyocardial biopsy reports. Baseline characteristics were compared using the Mann-Whitney U test and the Pearson χ 2 test. Clinical outcomes were monitored via follow-up visits or telephone calls. Primary outcomes were all-cause death and a composite end point of all-cause death or heart failure hospitalization. Kaplan-Meier analyses, as well as univariable and age- and sex-adjusted multivariable Cox regression analyses, were used to assess differences in overall and composite end point-free survival between patients with ATTR-CA with and without myocardial inflammation. RESULTS: A total of 103 patients with ATTR-CA (100 wild type; 3 variant) were enrolled. Median follow-up was 18.2 (8.0–31.1) months. Myocardial inflammation was prevalent in 32% (n=33/103) of patients with ATTR-CA. Among evaluable patients with myocardial inflammation, 96% (n=26/27) and 31% (n=9/29) had elevated CD68 (clusters of differentiation 68)–positive macrophage and CD3 (clusters of differentiation 3)–positive T-cell counts, respectively. Overall survival ( P =0.017) and composite end point-free survival ( P =0.014) were significantly impaired in patients with ATTR-CA with myocardial inflammation (n=33) compared with those without (n=70). Statistical significance for both associations was sustained after adjustment for age and sex, yielding adjusted hazard ratios of 4.72 (95% CI, 1.33–16.71; P =0.016) and 2.30 (95% CI, 1.04–5.11; P =0.041) for all-cause death and the composite end point, respectively. CONCLUSIONS: Our findings affirm previous evidence that myocardial inflammation is present in approximately one-third of all patients with ATTR-CA. Moreover, we provide first data indicating that myocardial inflammation may be associated with a higher risk of death and heart failure hospitalizations in ATTR-CA.

Background: Rapid diagnostic screening is an unmet need in patients with suspected cardiomyopathy. We recently demonstrated that magnetocardiography (MCG) might be a suitable tool to detect cardiomyopathies. We now tested diagnostic accuracy of MCG prospectively. Methods: MCG uses a superconducting quantum interference device (SQUID) to detect the electromagnetic field of the heart. In our recent retrospective study, a T-beg-Tmax (MCG Score) > 0.051 identified patients with non-ischemic cardiomyopathy. We assessed the diagnostic accuracy of MCG compared to advanced imaging and/or endomyocardial biopsy (EMB). Results from EMB took precedence over advanced imaging. We enrolled 110 patients with angina-like symptoms after exclusion of coronary artery disease (Power calculation: Expected 80% power at a 5% significance level) from the emergency department, in-patient wards and outpatient clinics. Exclusion criteria included presence of intracardiac metal devices and treatment with immunosuppressants. We incorporated 220 healthy individuals using 1:2 propensity score matching (age, sex) for a total of 330 participants. Results: MCG detected cardiomyopathy with 94.74% sensitivity (95% CI: 85-99%), 98.54% specificity (95% CI: 96-100%), 93.1% negative predictive value (NPV, 95% CI: 84-98%) and 94.34% positive predictive value (PPV, 95% CI: 84-98%). In 3 patients, CMR failed to detect non-ischemic cardiomyopathy, whereas MCG showed pathological results consistent with results of EMB. Results were highly reproducible (statistical uncertainty: 2.5%, T-beg-Tmax (MCG Score) +/− 0.004). Conclusion: MCG offers a non-invasive, reproducible methodology for rapid and accurate detection of non-ischemic cardiomyopathy. Our data indicate that MCG may be an efficient tool for allocation of advanced imaging to those in greatest need. ### Competing Interest Statement BH is inventor on patents that use RNA for diagnosis of myocarditis. BH is an inventor on a patent protection that is in process for MCG for diagnosis and measurement of therapy response in inflammatory cardiomyopathy. To avoid any potential conflict of interest, BH was not involved in interpreting MCG data related to the TbegTmax metric (MCG Score > 0.051) used for determining diagnostic accuracy. ### Clinical Trial NCT06689098 ### Funding Statement This work was funded by a project grant from the Swiss National Science Foundation issued to Bettina Heidecker, MD (money follows researcher grant). Phillip Suwalski, MD and Bettina Heidecker, MD are supported by the Deutsch Herzstiftung e.V. Bettina Heidecker is participant in the BIH-Charite Advanced Clinician Scientist Pilotprogram funded by the Charite Universitaetsmedizin Berlin and the Berlin Institute of Health. ### Author Declarations I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained. Yes The details of the IRB/oversight body that provided approval or exemption for the research described are given below: Ethics Committee of DHZC Universitaetsmedizin Berlin approved the project. I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals. Yes I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance). Yes I have followed all appropriate research reporting guidelines, such as any relevant EQUATOR Network research reporting checklist(s) and other pertinent material, if applicable. Yes An anonymized dataset and code can be made available upon request. Access is only granted to academic institutions and after signing a data sharing agreement.

Aims: Myocardial inflammation in cardiac amyloidosis is associated with poor clinical outcomes. This study aimed to (a) investigate the relationship between peripheral blood cytokine levels and the presence of inflammatory cells within the myocardium, and to (b) evaluate the potential of cytokines as predictors of major adverse cardiovascular events (MACE) in transthyretin (ATTR) and immunoglobulin light chain (AL) cardiac amyloidosis. Methods: Peripheral blood samples were collected from 50 patients with cardiac ATTR or AL amyloidosis between 2018 and 2023 at baseline and every three months during follow-up visits. Cytokine analysis was performed using Olink’s Proximity Extension Assay. For MACE prediction analysis, only patients with MACE occurring within ±14 days of a study visit were included (n = 16). Associations were evaluated using linear models. Results: No significant associations were identified between the EMB-confirmed myocardial presence of inflammatory cells and cytokine levels. There was a trend of weak-to-moderate associations between serial blood cytokine levels and MACE, albeit this was non-significant after adjustment for multiple testing (FDR): r2 = 0.28 for PON3 (p = 0.00075, FDR = 0.28), SIGLEC1 (p = 0.00077, FDR = 0.28), and IL-6 (p = 0.00086, FDR = 0.31). Conclusions: Peripheral blood cytokine levels were not reliable biomarkers for the myocardial presence of inflammatory cells. PON3, SIGLEC1, and IL-6 demonstrated a statistically non-significant trend of a weak-to-moderate association with MACE in cardiac amyloidosis. Since we recently demonstrated that amyloidosis with an inflammatory component is associated with poor outcomes, these additional findings underscore the need for alternative approaches to identify and manage inflammation in this patient population.

Background Bio-ADM (bioactive adrenomedullin) is a vasoactive peptide hormone that predicts clinical outcomes in heart failure-the main driver of adverse outcomes in cardiac amyloidosis (CA). This prospective observational study sought to assess the prognostic role of bio-ADM in CA. Methods Patients with CA were enrolled from amyloid centers in Germany (observation cohort), Japan, and the United States (combined validation cohort). Bio-ADM was quantified using the sphingotest bio-ADM assay. Associations of bio-ADM with all-cause death and major adverse cardiovascular events over 2 years were assessed using Kaplan-Meier and Cox regression analyses. Likelihood ratio chi-square tests for nested models evaluated whether adding bio-ADM improves validated prognostic staging systems. Results In both the German observation cohort (n=86) and the combined validation cohort from Japan and the United States (n=124), elevated bio-ADM (>29 pg/mL) was associated with more frequent all-cause death and major adverse cardiovascular events. Bio-ADM remained independently associated with impaired overall (P<0.001) and major adverse cardiovascular events-free survival (P<0.001) after adjustment for age, sex, and established prognostic biomarkers in the entire cohort. Adding categorized bio-ADM (>29 pg/mL) significantly improved the prognostic accuracy of the National Amyloidosis Centre (C-index 0.674 to 0.787; P=0.002) and MayoATTR (C-index 0.662 to 0.757; P<0.001) staging systems for cardiac transthyretin amyloidosis. Adding bio-ADM to staging systems for cardiac immunoglobulin light chain amyloidosis yielded no significant changes. Conclusions Bio-ADM is a promising prognostic biomarker, especially in cardiac transthyretin amyloidosis, where it improved risk stratification when added to established staging systems. Further research is needed to clarify its role as part of staging systems for cardiac immunoglobulin light chain amyloidosis.