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Ποια από τις παρακάτω μορφές αμυλοείδωσης σχετίζεται με τα συμπτώματα της αυτοάνοσης νόσου;
Ποια από τις παρακάτω μορφές αμυλοείδωσης σχετίζεται με τα συμπτώματα της αυτοάνοσης νόσου;
Ποια ηλικιακή ομάδα είναι περισσότερο επιρρεπής στην ATTRwt αμυλοείδωση;
Ποια ηλικιακή ομάδα είναι περισσότερο επιρρεπής στην ATTRwt αμυλοείδωση;
Ποιο παράγοντα κινδύνου σχετίζεται με την ATTRv αμυλοείδωση;
Ποιο παράγοντα κινδύνου σχετίζεται με την ATTRv αμυλοείδωση;
Ποια μορφή αμυλοείδωσης είναι γνωστή για τη σχέση της με τις λυσίνες;
Ποια μορφή αμυλοείδωσης είναι γνωστή για τη σχέση της με τις λυσίνες;
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Ποιο είναι το αναγνωρίσιμο χαρακτηριστικό της AL αμυλοείδωσης;
Ποιο είναι το αναγνωρίσιμο χαρακτηριστικό της AL αμυλοείδωσης;
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Ποιες είναι οι λιγότερο συχνές μορφές αμυλοείδωσης σύμφωνα με τα δεδομένα;
Ποιες είναι οι λιγότερο συχνές μορφές αμυλοείδωσης σύμφωνα με τα δεδομένα;
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Ποια από τις παρακάτω είναι μια σημαντική τάση στην αντιμετώπιση της αμυλοείδωσης;
Ποια από τις παρακάτω είναι μια σημαντική τάση στην αντιμετώπιση της αμυλοείδωσης;
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Ποίο είναι το κύριο χαρακτηριστικό των τριών τύπων αμυλοείδωσης που περιγράφονται;
Ποίο είναι το κύριο χαρακτηριστικό των τριών τύπων αμυλοείδωσης που περιγράφονται;
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Ποια βιολογική διαδικασία σχετίζεται με την AL αμυλοείδωση;
Ποια βιολογική διαδικασία σχετίζεται με την AL αμυλοείδωση;
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Σύμφωνα με τα δεδομένα, ποια από τις παρακάτω ομάδες ασθενών έχει την υψηλότερη συχνότητα εμφάνισης αμυλοείδωσης;
Σύμφωνα με τα δεδομένα, ποια από τις παρακάτω ομάδες ασθενών έχει την υψηλότερη συχνότητα εμφάνισης αμυλοείδωσης;
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Σύμφωνα με το περιεχόμενο, ποιες από τις παρακάτω συμπτωματολογίες δείχνουν πιθανή ύπαρξη αμυλοείδωσης;
Σύμφωνα με το περιεχόμενο, ποιες από τις παρακάτω συμπτωματολογίες δείχνουν πιθανή ύπαρξη αμυλοείδωσης;
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Ποια από τις παρακάτω ομάδες ασθενών έχει την υψηλότερη συχνότητα εμφάνισης αμυλοείδωσης, σύμφωνα με τα δεδομένα, μετά από ασθενείς με HFpEF και αριστερή υπερτροφία;
Ποια από τις παρακάτω ομάδες ασθενών έχει την υψηλότερη συχνότητα εμφάνισης αμυλοείδωσης, σύμφωνα με τα δεδομένα, μετά από ασθενείς με HFpEF και αριστερή υπερτροφία;
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Σύμφωνα με το περιεχόμενο, ποια από τις παρακάτω προσεγγίσεις είναι λάθος όσον αφορά την διάγνωση της ATTR-CM;
Σύμφωνα με το περιεχόμενο, ποια από τις παρακάτω προσεγγίσεις είναι λάθος όσον αφορά την διάγνωση της ATTR-CM;
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Ποια από τις παρακάτω ομάδες ασθενών με υπερτροφία αριστερού κοιλιακού τοιχώματος (LVH) δείχνει μεγαλύτερη πιθανότητα να πάσχει από αμυλοείδωση;
Ποια από τις παρακάτω ομάδες ασθενών με υπερτροφία αριστερού κοιλιακού τοιχώματος (LVH) δείχνει μεγαλύτερη πιθανότητα να πάσχει από αμυλοείδωση;
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Ποια από τις παρακάτω προσεγγίσεις φαίνεται να συμφωνεί με τα δεδομένα του κειμένου όσον αφορά την διάγνωση της ATTR-CM;
Ποια από τις παρακάτω προσεγγίσεις φαίνεται να συμφωνεί με τα δεδομένα του κειμένου όσον αφορά την διάγνωση της ATTR-CM;
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Σύμφωνα με το κείμενο, ποια από τις παρακάτω καταστάσεις δεν δείχνει πιθανή ύπαρξη αμυλοείδωσης;
Σύμφωνα με το κείμενο, ποια από τις παρακάτω καταστάσεις δεν δείχνει πιθανή ύπαρξη αμυλοείδωσης;
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Ποια από τις παρακάτω καταστάσεις ΔΕΝ περιγράφεται στο κείμενο ως πιθανή αιτία για λανθασμένη διάγνωση της ATTR-CM;
Ποια από τις παρακάτω καταστάσεις ΔΕΝ περιγράφεται στο κείμενο ως πιθανή αιτία για λανθασμένη διάγνωση της ATTR-CM;
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Ποιο από τα παρακάτω ενδεικτικά στοιχεία δεν σχετίζεται με την αμυλοείδωση;
Ποιο από τα παρακάτω ενδεικτικά στοιχεία δεν σχετίζεται με την αμυλοείδωση;
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Study Notes
Amyloidosis Subtypes
- Systemic amyloidosis subtypes include AL, arising from plasma dyscrasia and increased immunoglobulin light chains, and AA, associated with autoimmunity or infections.
- TTR amyloidosis involves transthyretin, further categorized into ATTRwt, predominantly affecting the elderly (>75 years), and ATTRv, which is linked to mutations. ATTRv also shows a prevalence among black patients (approximately 3%).
- Rarer forms exist, such as ApoAI and ALECT2.
Amyloidosis Incidence Over Time
- The cumulative incidence of neurologic, mixed, and cardiac amyloidosis, as well as Systemic Senile Amyloidosis (SSA), varies with age at onset.
Increasing Awareness of Cardiac Amyloidosis
- Awareness, as measured by referrals, has been increasing in the UK from 2002-2021.
- In Greece, an increase in the number of diagnosed cardiac amyloidosis patients is evident from 2020-2023.
Prognosis and Survival in Cardiac Amyloidosis
- Survival is impacted by the specific type of cardiac amyloidosis. The survival difference between various forms (e.g., HCM, VHD, HHD, dCMP, IHD, ATTRwt-CM, AL-CM) is significant in both the survival curves.
- Survival is negatively impacted for ATTR-CM on a global level.
Global Prevalence of ATTR-CM
- The survival rate for 2-year period varies globally; with some areas showing higher survival indicators over others.
Subgroups to Suspect for Cardiac Amyloidosis
- Patients with various cardiovascular diseases or symptoms, such as unexplained LVH, HCM phenotype, or aortic stenosis, should be considered for cardiac amyloidosis screening.
Cardiac Amyloidosis Prevalence in Different Populations
- The prevalence of cardiac amyloidosis varies across various subgroups, including bone scintigraphy, carpal tunnel syndrome, unexplained LVH, and post-mortem evaluation.
Common Manifestations of Amyloidosis
- Cardiac manifestations involve heart failure, atrial fibrillation, and conduction disturbances.
- Musculoskeletal issues include carpal tunnel syndrome, back pain, and distal biceps ruptures.
- Polyneuropathy presents with painful sensations in the hands and feet.
- Patients experience various symptoms related to autonomic dysfunction, such as orthostatic hypotension, chronic diarrheal/constipation issues, and erectile dysfunction.
Diagnostic Modalities for ATTR-CM
- Diagnostic modalities for ATTR-CM include native T1 and extracellular volume (ECV) imaging, Cine-CMR and T1 mapping, and LGE-CMR and ECV-mapping.
Clinical Profile of ATTR-CM Patients (Undergoing TAVI)
- Patients with ATTR undergoing TAVI tend to be older and more likely to be male.
- Various comorbidities are frequently co-occurring in these patients.
- The presence of ATTR-CM is associated with a higher risk of mortality.
Links Between Cardiac Amyloidosis and AS/TAVI
- Older patients undergo TAVI.
- Amyloid deposition in the atrioventricular (AV) node may contribute to calcification, and aortic stenosis (AS).
Estimated Prevalence of Cardiac Amyloidosis in TAVI Patients
- The estimated prevalence of cardiac amyloidosis in TAVI patients (as indicated by studies) is approximately 11.2% (with a 95% confidence interval ranging from 9.4% to 13.3%).
Latent Cardiac Amyloidosis in TAVI: Real-World Data
- Results from a US national inpatient sample of approximately 30 million hospitalizations suggest that patients with cardiac amyloidosis (ATTR-CM) show a higher risk for in-hospital mortality in TAVR
Clinical Profile of CA Patients Undergoing TAVI
- Specific characteristics, such as age, gender, treatment approach, and hospital costs, are noted to differ between TAVR patients with and without clinically diagnosed cardiac amyloidosis.
ATTR in TAVI Patients
- Cardiac amyloidosis (CA) remains underdiagnosed in patients undergoing TAVI for aortic stenosis (AS).
- Patients with CA present with more comorbidities in comparison to patients with no CA undergoing TAVI.
- The presence of CA leads to an increase in the mortality rate of TAVI patients.
TAVI for AS Patients With ATTR-CM
- Patients with both Aortic Stenosis (AS) and Cardiac Amyloidosis (ATTR-CM) have a poorer prognosis compared to patients with AS alone.
- AVR/TAVR procedures, in this instance, have provided better long-term outcomes, but a full understanding of their efficacy is still sought in the field.
How to Screen for Cardiac Amyloidosis
- Invasive options are available for diagnosing all types of cardiac amyloidosis, including looking for a positive cardiac biopsy result and/or an extracardiac biopsy of an amyloid positive specimen, which can be supported by echocardiographic/CMR criteria.
Diagnostic Algorithm for Cardiac Amyloidosis
- A diagnostic algorithm for cardiac amyloidosis considers patient symptoms, ECG, echocardiography or CMR findings, blood tests (free light chains, immune fixation), bone scintigraphy with or without SPECT, and potential genetic testing to investigate for various forms.
Multimodality Imaging of Cardiac Amyloidosis
- Multimodality imaging modalities, including echocardiography, CMR, nuclear, and cardiac CT imaging, are utilized in evaluating cardiac amyloidosis. Varied characteristics of the heart may vary for each form of amyloidosis under different conditions.
CMR for Diagnostic Assessment of Cardiac Amyloidosis
- Accurate and effective diagnostic techniques include Native T1, Cine, LGE, and ECV techniques.
Differentiation of AL vs. ATTR by CMR/Echo
- Accurate differentiation is required to establish the right treatment strategy. Clinical evaluation, imaging (chiefly CMR), and/or comprehensive laboratory tests can provide a differential diagnosis.
Bone Scintigraphy for Cardiac Amyloidosis (ATTR) Diagnosis
- A bone scintigraphy scan with technetium-99m (Tc99m-DPD or similar) can contribute to the diagnosis. This is especially important when there is a high suspicion of ATTR. Different screening grades, such as the Perugini scale, may be used by medical professionals.
Tissue Biopsy for Cardiac Amyloidosis
- Direct tissue sampling by biopsy is used to isolate and analyze amyloid deposits for accurate subtype identification.
First Report of Cardiac TTR/LECT2 Double Amyloidosis
- A reported case demonstrates the coexistence of Transthyretin (TTR) and Leukocyte Chemotactic Factor 2 (LECT2) amyloidosis in multiple organs.
False Positive Referrals for Cardiac Amyloidosis
- A study shows a trend in the percentage of false positive referrals to specialist centres for diagnosis, which can vary according to the factors involved (e.g., equivocal Tc99m-PYP, abnormal echocardiogram or CMR data).
Treating Cardiac Amyloidosis
- Therapies focused on the underlying variety are used for therapeutic treatment.
Treatment Options for ATTR
- Specific treatments such as tafamidis, crispr-cas9 (Vutrisiran or similar), and NI301A are available for different subtypes of ATTR-CM, based on the presence or absence of comorbidities.
Treating ATTR Cardiomyopathy
- New treatments are available to treat ATTR cardiomyopathy.
Critical Steps in the Management of hATTR Patients
- Confirm cardiac amyloidosis, establish the ATTR diagnosis, perform genetic testing, and treat the proband.
ATTR: Disease Staging
- This presents a staging system to guide therapeutic approaches based on disease progression, as measured through clinical criteria and biomarker status.
Bone Scan: Regression of Myocardial Infiltration by Treatment
- Biomarker changes, such as serum TTR levels and Congo red grade, are tracked over time following treatments to monitor efficacy.
ECHO & CMR to Monitor Treatment
- ECHO and CMR imaging are used to monitor the effects and efficacy of treatment. The measures used in these evaluations may differ by condition.
Practical Clinical Algorithms: How to Assess Risk
- Clinical algorithms are used to evaluate risk factors. Diagnostic criteria and algorithms for risk assessment are varied and potentially complex within the medical field.
Trends in Cardiac Amyloidosis Outcomes
- Analysis of data shows that survival rates of patients with cardiac amyloidosis have improved over various time periods, suggesting potential progress in diagnosis and/or treatment.
Clinical Staging of ATTR-CM
- A clinical staging system for ATTR-CM, potentially relying on NYHA categories, can be utilized to assess disease severity and predict outcomes.
ATTR-CM: Prognosis by NYHA Class
- The presence and severity of symptoms, in combination with the NYHA class, significantly influence survival in patients with ATTR-CM.
ATTR-CM: Biomarker-Based Disease Staging Systems
- Systems using biomarkers like NT-proBNP and hsTn-l can provide valuable insight for prognosis and guiding treatment strategies.
Looking Outside the Heart in ATTR-CM to Assess Risk
- Evaluating non-cardiac factors, such as CPET and PFT, can be used to predict outcomes in ATTR-CM.
Imaging for Risk Stratification of ATTR-CM
- Imaging offers diverse methods, like echocardiography and bone scintigraphy, to support assessing the risk in ATTR-CM.
ECHO for Risk Stratification in Cardiac Amyloidosis
- This assesses global longitudinal strain (GLS) of the left ventricle (LV) to aid in risk stratification for cardiac amyloidosis outcomes.
RV Strain ECHO in Cardiac Amyloidosis
- Echocardiography based on RV strain can be utilized as a crucial tool for the assessment of risk, by utilizing a method that accounts for RVFW strain and LVEF.
Bone Scintigraphy for Risk Stratification in ATTR-CM
- Whole-body bone scintigraphy images can demonstrate the presence of focal versus diffuse uptake of the tracer molecule (particularly with Tc99m-DPD/SPECT). Diffuse tracer uptake may infer an increased risk of mortality compared with focal uptake.
CMR for Risk Stratification in AL Amyloidosis
- CMR techniques (particularly ECV measures) can help follow responses to treatment for AL amyloidosis patients, facilitating prognostic stratification.
CMR for Risk Stratification in ATTR Amyloidosis
- Native T1 and ECV data correlate tightly with DPD grade. Both markers predict survival, supporting their use in diagnosis and risk stratification.
Grey Zones in Risk Assessment of ATTR-CM
- Grey zones in the prognostication criteria for ATTR-CM include arrhythmic risk stratification. Various factors, such as low thresholds for PPM deployment in patients with conduction disease, and use of ICD for primary prevention in patients with CA, create challenges.
Grey-Zones: Arrhythmic Risk Stratification
- Screening and analysis criteria are presented which highlight specific grey zones for cardiac amyloidosis, primarily as it relates to arrhythmia risk stratification.
Taking Care of the Family
- Management protocols for hereditary ATTR are presented.
Genetic Testing for hATTR: Cascade Screening
- Genetic testing in hereditary ATTR is detailed.
Cardiovascular Screening of Asymptomatic hATTR Patients
- A structured screening program for asymptomatic hATTR patients is detailed.
Take-Home Messages
- Key messages regarding ATTR-CM are outlined.
Typical Cases of ATTR Cardiomyopathy
- Diagnostic criteria based on history, physical findings, and findings from various imaging/laboratory tests are described, including examples.
Typical Cases of wtATTR Cardiomyopathy
- Diagnostic criteria based on history, physical findings, and imaging/laboratory tests are provided while distinguishing this from other forms.
A Typical Case of Hereditary ATTR
- Examples of hereditary ATTR presentation are provided as case studies.
A Typical Case of Cardiac Amyloidosis
- Examples are provided for diagnosing typical cases of cardiac amyloidosis.
Awareness for Atypical Cases of CA
- The importance is highlighted for being aware of atypical cases of cardiac amyloidosis
Atypical Cardiac Amyloidosis: Case A
- The presentation of a case in which atypical cardiac amyloidosis was diagnosed.
Left Ventricular Hypertrophy in Amyloid Cardiomyopathy
- Asymmetric hypertrophy and symmetric hypertrophy are contrasted in the context of LVH and ATTR-CM.
Clinical Case Presentation
- A clinical case that highlights the clinical assessment of patients presenting with cardiac signs, as well as a history and details of the symptoms
Diagnostic Work-Up
- A diagnostic discussion to determine whether a patient warrants screening for cardiac amyloidosis is presented based on the patient's symptoms and medical history
Diagnostic Work-Up for Amyloidosis
- Criteria and a diagnostic approach are provided for assessing patients regarding cardiac amyloidosis (especially with regards to AV conduction disease, aortic stenosis and/or HF).
How To Screen for Cardiac Amyloidosis
- The process for screening for cardiac amyloidosis is presented.
Diagnostic Algorithm for Cardiac Amyloidosis
- An algorithm for diagnosing cardiac amyloidosis is described.
Screening for Cardiac Amyloidosis
- The diagnostic process involved in screening for cardiac amyloidosis is presented.
Is this wtATTR-CM?
- An example of a patient's diagnosis, to illustrate the screening process and diagnostic issues.
Not every cardiac amyloidosis is ATTR
- The importance of considering other forms of cardiac amyloidosis along with ATTR is highlighted.
Not all cardiac amyloidosis is ATTR-CM
- Specific aspects of a specific patient presentation that deviate from typical ATTR-CM scenarios are presented, along with imaging findings that may provide a differential diagnosis.
Serum/Urine Electrophoresis - Genetic Testing
- Information on additional diagnostic tools and their applications is presented.
Conclusions
- Based on available data, diagnoses and conclusions about cardiac amyloidosis cases are described, in the context of unknown etiology, and common clinical features.
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Description
Ανακαλύψτε τους διαφόρους υποτύπους της αμυλοείδωσης, συμπεριλαμβανομένων των AL και AA. Εξερευνήστε την επίπτωση της καρδιακής αμυλοείδωσης και τη σημασία της ευαισθητοποίησης στις διαγνώσεις. Μάθετε για τις πρόγνωση και επιβίωση στην καρδιακή αμυλοείδωση.
