A5078387258- Amyloidosis: Diagnosis, Treatment, Outcomes
- Parathyroid Disorders and Treatments
- Cardiovascular Function and Risk Factors
- Pulmonary Hypertension Research and Treatments
- Cardiomyopathy and Myosin Studies
- Medical Imaging and Pathology Studies
- Heart Failure Treatment and Management
- Advanced MRI Techniques and Applications
- Sarcoidosis and Beryllium Toxicity Research
- Cardiovascular and exercise physiology
- Peptidase Inhibition and Analysis
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Liver Disease Diagnosis and Treatment
- Liver Disease and Transplantation
- Cardiovascular Disease and Adiposity
- Eosinophilic Disorders and Syndromes
- Cardiac Valve Diseases and Treatments
- Blood Pressure and Hypertension Studies
- Hepatocellular Carcinoma Treatment and Prognosis
- Cardiovascular Issues in Pregnancy
- Dialysis and Renal Disease Management
- Infective Endocarditis Diagnosis and Management
- Takotsubo Cardiomyopathy and Associated Phenomena
- Long-Term Effects of COVID-19
- Cardiac Imaging and Diagnostics
Medical University of Vienna
2020-2025
University Clinic of Traumatology
2023
The 2021 European Society of Cardiology (ESC) screening recommendations for individuals carrying a pathogenic transthyretin amyloidosis variant (ATTRv) are based on expert opinion. We aimed to (i) determine the penetrance ATTRv cardiomyopathy (ATTRv-CM) at baseline; (ii) examine value serial evaluation; and (iii) establish yield first-line diagnostic tests (i.e. electrocardiogram, echocardiogram, laboratory tests) as per ESC position statement.
Background: Extracellular volume (ECV) by cardiovascular magnetic resonance (CMR) imaging is associated with disease burden and clinical outcomes. Recent studies in patients valvular heart (VHD) have suggested that the indexed total ECV (iECV) = ECVx(LVmass/1.05)/body surface area may supersede terms of prognostication. In this study, we aimed to compare prognostic capability conventional iECV an all-comer CMR cohort. Methods: From January 2012 2023, were measured consecutive patients....
Background: This study assessed the prognostic value of submaximal cardiopulmonary exercise testing (CPET) in cardiac amyloidosis and explored CPET as an alternative to 6-min walk test (6MWT).
Abstract Aims We aimed to establish one-minute sit-to-stand test (1-min STST) cut-off values that align with the guideline-recommended six-minute walk (6MWT) thresholds (165m and 440m) for one-year mortality risk stratification in pulmonary hypertension (PH) patients. Furthermore, we compare clinical characteristics long-term among patients stratified by these proposed 1-min STST cut-offs. Methods All performed 6MWT. Receiver operating analysis was determine cut-offs corresponding 6MWT...
Background: Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy (HCM) remains challenging. Late gadolinium enhancement (LGE) on MRI signifies myocardial fibrosis and is linked to adverse outcomes HCM. However, the threshold of LGE that clinically significant a subject debate. We hypothesized even small amounts (≥ 5%) or history syncope are associated with worse outcomes. Methods: Between May 2018 June 2023, HCM patients were prospectively enrolled at Medical...
Introduction. Several randomised double-blind placebo-controlled phase III trials (RCT) explore disease-modifying therapeutics in transthyretin amyloid cardiomyopathy (ATTR-CM). However, it is currently unclear whether patients eligible to participation the RCT are representative of real-world patients. Methods. ATTR-CM presenting a tertiary referral centre for cardiac amyloidosis at Medical University Vienna between March 2012 and May 2024 were included prospective registry. Inclusion...
Sodium-glucose cotransporter 2 inhibitors (SGLT2i) have been found to potential hematopoietic effects in patients with heart failure (HF). However, these benefits not studied cardiac amyloidosis (CA). CA present HF symptoms and often suffer from iron deficiency, which has a negative impact on erythropoiesis leads lower hemoglobin hematocrit levels. We sought determine the of SGLT2i hematological parameters functional capacity (FC) patients. A prospective analysis was conducted compare who...
Background: Inflammatory indices have been proposed as simple and routinely obtainable markers of systemic inflammation in cardiac disease. This study investigated whether the neutrophil-to-lymphocyte ratio (NLR), monocyte-to-lymphocyte (MLR), pan-immune value (PIV) serve biomarkers for risk stratification outcomes measures patients with severe aortic stenosis (AS) following valve replacement (AVR). Methods: In this retrospective analysis (January 2017–June 2022), AS underwent pre-procedural...
Background The one-minute sit-to-stand-test (1-min STST) is a quick, space saving test to evaluate functional capacity. Exercise testing plays an important role in the long-term follow-up of pulmonary hypertension (PH) patients and currently evaluated using six-minute-walk-test (6MWT). aim study was assess convergent validity 1-min STST with PH its association markers severity. Methods We 106 1-min-STST 6MWT measured cardiorespiratory parameters (heart rate, blood pressure, oxygen...
Abstract Aims The pathophysiological hallmark of cardiac amyloidosis (CA) is the deposition amyloid within myocardium. Consequently, extracellular volume (ECV) affected patients increases. However, studies on ECV progression over time are lacking. We aimed to investigate and its prognostic impact in CA patients. Methods results Serial magnetic resonance (CMR) examinations, including quantification, were performed consecutive Between 2012 2021, 103 underwent baseline follow-up CMR,...
Novel ribonucleic acid interference (RNAi) therapeutics such as patisiran and inotersen have been shown to benefit neurologic disease course quality of life in patients with hereditary transthyretin amyloidosis (ATTRv). We aimed determine the impact RNAi on myocardial amyloid load using quantitative single photon emission computed tomography/computed tomography (SPECT/CT) imaging ATTRv-related cardiomyopathy (ATTRv-CM). furthermore compared them wild-type ATTR-CM (ATTRwt-CM) treated tafamidis.
Cardiac amyloidosis (CA) is associated with several distinct electrocardiographic (ECG) changes. However, the impact of amyloid depositions on ECG parameters not well investigated. We therefore aimed to assess correlation burden and test prognostic power findings outcomes in patients CA. Consecutive CA underwent assessment cardiac magnetic resonance imaging (CMR), including quantification extracellular volume (ECV) T1 mapping. Moreover, seven additional using immunohistochemistry staining...
Aims Patients with transthyretin amyloid cardiomyopathy (ATTR-CM) experience reduced functional capacity. We evaluated changes in capacity over extensive follow-up using cardiopulmonary exercise testing (CPX). Methods ATTR-CM patients underwent CPX and blood at baseline, first [V1, 8 (6–10) months] second (V2) 35 (26–41) months after start of disease-specific therapy. Results included 34 patients, aged 77 (±6) years (88.2% men). showed two patterns improvement V1 deterioration V2. Peak work...
The pathophysiological hallmark of wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) is the deposition within myocardium.
Heart failure with preserved ejection fraction (HFpEF) is associated heart (HF) hospitalizations and death. Previous studies have shown that altered muscle composition higher risk of adverse outcome in HFpEF patients.
Transthyretin amyloid cardiomyopathy (ATTR-CM) is associated with severely impaired health-related quality of life (HRQL). HRQL an independent predictor outcome in heart failure (HF), but data on patients ATTR-CM scarce. This study therefore aims to evaluate the association ATTR-CM.
Abstract Aims Quantification of cardiac [99mTc]-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) uptake enhances diagnostic capabilities and may facilitate prognostic stratification in patients with transthyretin amyloidosis (ATTR-CA). This study aimed to evaluate the association quantitative left ventricular (LV) DPD myocardial structure function, their implications on outcome ATTR-CA. Methods Results Consecutive ATTR-CA (n=100) undergoing planar scintigraphy Perugini grade 2 or 3,...
Abstract Aims Amyloid cardiomyopathy is caused by the deposition of light chain (AL) or transthyretin amyloid (ATTR) fibrils, that leads to a restrictive cardiomyopathy, often resulting in heart failure (HF) with preserved reduced ejection fraction. This study aimed determine whether cardiac output reduction ventilation inefficiency plays predominant role limiting exercise patients cardiomyopathy. Methods We conducted multicentre prospective AL ATTR who underwent cardiopulmonary testing...
Background: Liver damage is frequently observed in patients with cardiovascular disease but infrequently quantified. We hypothesized that undergoing cardiac magnetic resonance, liver T1-times indicate and are associated outcome. Methods: measured hepatic T1-times, displayed on standard T1-maps, an all-comer resonance-cohort. At the time of we assessed validated general fibrosis scores. Kaplan-Meier estimates Cox-regression models were used to investigate association between a composite...
Growing interest has accrued in the co-existence of cardiac amyloidosis and valvular heart disease. Amyloid infiltration from either transthyretin (ATTR) or light chain (AL) origin may affect any structure heart, including valves. Recent literature mainly focused on aortic stenosis amyloidosis, improving our understanding epidemiology, diagnosis, treatment prognosis this dual pathology. Even though high clinical relevance, data mitral/tricuspid regurgitation is rather scarce mostly limited...
The PARAGON-HF clinical trial suggested that sacubitril/valsartan may become a treatment option for particular subgroups of patients with heart failure and preserved ejection fraction (HFpEF). However, the proportion real-world HFpEF who are theoretically superimposable population is yet unknown. present study was performed to define PARAGON-HF-like describe their characteristics long-term prognosis in comparison those would not meet criteria. We systematically applied inclusion exclusion...