{ "schemaVersion": 2, "eyebrow": "Cardiomyopathy", "title": "Cardiac Amyloidosis", "summary": "Suspect cardiac amyloidosis in otherwise unexplained increased ventricular wall thickness or restrictive heart failure, then urgently exclude AL disease before using bone-tracer imaging to diagnose ATTR noninvasively. Amyloid typing determines whether immediate plasma-cell therapy or transthyretin-directed treatment is indicated.", "seoDescription": "Physician guide to diagnosing and managing cardiac amyloidosis, with AL exclusion, PYP scintigraphy interpretation, biopsy indications, and ATTR therapy.", "clinicalQuestion": "How should clinicians rapidly confirm and subtype suspected cardiac amyloidosis to direct urgent AL or ATTR-specific treatment?", "specialty": "Cardiology", "audience": "U.S. physicians and medical trainees", "tags": [ "cardiac amyloidosis", "ATTR cardiomyopathy", "AL amyloidosis", "99mTc-PYP scintigraphy", "tafamidis", "monoclonal gammopathy", "endomyocardial biopsy" ], "keyTakeaways": [ "Obtain serum free light chains plus serum and urine immunofixation in every suspected cardiac amyloidosis case before assigning ATTR subtype from bone scintigraphy. [8][9]", "Grade 2 or 3 myocardial uptake on 99mTc-PYP/DPD/HMDP confirmed by SPECT, together with negative monoclonal-protein testing, establishes nonbiopsy ATTR cardiac amyloidosis. [8]", "A monoclonal gammopathy or abnormal free-light-chain result prevents a scintigraphy-only ATTR diagnosis; tissue confirmation and amyloid typing are required because AL cardiomyopathy can show intense tracer uptake. [7][8][17]", "For wild-type or variant ATTR cardiomyopathy with NYHA class I-III heart-failure symptoms, tafamidis reduces cardiovascular morbidity and mortality; FDA-approved doses are tafamidis 61 mg or tafamidis meglumine 80 mg daily. [9][10]", "Treat atrial arrhythmias as high-thromboembolic-risk states in cardiac amyloidosis; anticoagulation is recommended for any atrial arrhythmia, while bleeding risk requires particular attention in systemic AL disease. [4][18]" ], "sections": [ { "id": "when-to-suspect", "eyebrow": "Case finding", "heading": "Who warrants an amyloidosis workup?", "intro": "Test when imaging and clinical phenotype are disproportionate to conventional hypertensive or valvular disease.", "paragraphs": [ "Initiate evaluation in patients with heart failure and otherwise unexplained increased LV wall thickness, especially when echocardiography shows a restrictive filling pattern, biatrial enlargement, and symmetric LV thickening. A mean LV wall thickness greater than 12 mm without hypertension or another adequate cause of hypertrophy is a recognized trigger for amyloid cardiomyopathy assessment; LV thickness of at least 15 mm with restrictive Doppler findings is particularly suggestive. [6][16]", "Prioritize amyloid testing when increased wall thickness coexists with peripheral or autonomic neuropathy, renal dysfunction or proteinuria, gastrointestinal involvement, carpal-tunnel-spectrum symptoms, unexplained atrial arrhythmia, or a family history compatible with dominantly inherited ATTR. Hereditary ATTR is late-onset and may involve peripheral and autonomic nerves as well as myocardium; wild-type ATTR predominantly affects men older than 60 years and is principally cardiac. [5][6]", "Do not wait for reduced ejection fraction. Cardiac ATTR disease commonly presents with heart failure despite preserved EF, and cardiac involvement is a dominant determinant of morbidity and mortality in AL, wild-type ATTR, and hereditary ATTR amyloidosis. [4][16]" ], "bullets": [ "Order transthoracic echocardiography and assess diastolic filling, atrial size, wall thickness, and EF in newly suspected amyloid heart disease. [7][16]", "Use cardiac MRI when echocardiography is equivocal or to characterize suspected infiltration and quantify cardiac amyloid burden; echocardiography and CMR are appropriate for this purpose. [7]", "If systemic AL amyloidosis or monoclonal gammopathy with abnormal free-light-chain testing is already known, evaluate cardiac involvement with echocardiography and CMR rather than bone scintigraphy alone. [7]" ], "subsections": [], "table": { "caption": "Clinical patterns that should change the next diagnostic step. [5][6][7][16]", "columns": [ "Pattern", "Interpretation", "Next action" ], "rows": [ [ "HF with unexplained LV wall thickness >12 mm", "Raises suspicion for amyloid cardiomyopathy when hypertension or another hypertrophy cause is absent. [6]", "Obtain serum free light chains, serum immunofixation, urine immunofixation, echocardiography, and consider CMR. [7][8]" ], [ "Restrictive filling, symmetric LV thickness ≥15 mm, biatrial enlargement", "Echocardiographic constellation is highly suggestive of cardiac amyloidosis. [16]", "Proceed to monoclonal-protein testing; use bone scintigraphy only after the AL pathway is addressed. [7][8]" ], [ "Monoclonal gammopathy or abnormal free-light-chain profile", "AL cardiac amyloidosis remains possible regardless of bone-tracer uptake. [7][8]", "Pursue biopsy with definitive amyloid typing; do not diagnose ATTR from scintigraphy alone. [7][8]" ], [ "Late-onset cardiomyopathy with neuropathy or autonomic dysfunction", "Supports hereditary ATTR consideration, including disease with combined cardiac and neurologic involvement. [6]", "Confirm cardiac involvement and, after ATTR identification, determine whether disease is wild-type or hereditary. [5][6]" ] ] } }, { "id": "diagnostic-algorithm", "eyebrow": "Subtype before treatment", "heading": "Diagnostic algorithm: exclude AL first, then use bone scintigraphy correctly", "intro": "The critical decision is whether a plasma-cell disorder is present.", "paragraphs": [ "At first suspicion, order all three monoclonal-protein studies together: serum free-light-chain assay, serum immunofixation electrophoresis, and urine immunofixation electrophoresis. This panel is required before a noninvasive ATTR diagnosis because scintigraphy identifies myocardial tracer uptake but does not independently exclude AL amyloid. [8]", "If all monoclonal-protein studies are negative and echocardiography and/or CMR supports amyloid cardiomyopathy, obtain 99mTc-PYP scintigraphy in U.S. practice; DPD or HMDP are equivalent bone-avid tracers in other settings. Diagnose ATTR cardiac amyloidosis noninvasively only when myocardial uptake is grade 2 or 3 and SPECT confirms myocardial rather than blood-pool activity. [8]", "If serum or urine immunofixation identifies a monoclonal protein, or if free-light-chain testing is abnormal, do not use a positive PYP scan to assign ATTR. Bone scintigraphy alone is insufficient in suspected AL disease, and biopsy is required for amyloid typing. In one reported diagnostic error, grade 3 PYP uptake occurred with AL cardiomyopathy confirmed by endomyocardial biopsy and mass spectrometry; the false ATTR attribution delayed needed plasma-cell treatment. [7][8]", "Use biopsy when monoclonal protein is present, scintigraphy is not diagnostic, imaging and laboratory data conflict, or another amyloid type is plausible. Confirm tissue amyloid with Congo red staining and determine fibril type; mass spectrometry was used to establish AL type in the PYP-positive AL case. [5][8]" ], "bullets": [ "Interpret a grade 2 or 3 scan only after SPECT confirmation; planar uptake without SPECT can reflect cardiac blood-pool activity. [8][17]", "Consider recent myocardial infarction within 4 weeks, rib fracture, valvular or annular calcification, hypertrophic cardiomyopathy, hydroxychloroquine cardiotoxicity, AL amyloidosis, and blood-pool activity when PYP findings appear discordant. [17]", "Recognize that concurrent AL and ATTR cardiac amyloidosis is rare but documented; clinical discordance or a monoclonal process warrants definitive tissue typing rather than diagnostic closure. [17]" ], "subsections": [ { "heading": "What a negative or positive PYP scan changes", "paragraphs": [ "A grade 2 or 3 PYP/DPD/HMDP scan with SPECT-confirmed myocardial localization and negative serum free-light-chain, serum immunofixation, and urine immunofixation testing supports ATTR cardiac amyloidosis without endomyocardial biopsy. This pathway should lead to ATTR-directed evaluation and treatment planning. [8]", "A positive scan with any evidence of clonal plasma-cell disease is nondiagnostic for ATTR and should accelerate tissue confirmation because AL cardiac involvement has urgent therapeutic implications. A negative or low-grade scan does not establish an alternative diagnosis; reconcile the result with echocardiography, CMR, and the monoclonal-protein evaluation, then biopsy when the suspicion remains consequential. [7][8]" ], "bullets": [] } ], "table": { "caption": "Actionable interpretation of the ATTR noninvasive diagnostic pathway. [7][8][17]", "columns": [ "Monoclonal-protein panel", "Bone scintigraphy result", "Interpretation", "Required next step" ], "rows": [ [ "Negative serum free light chains, serum IFE, and urine IFE", "Grade 2-3 uptake confirmed by SPECT", "Nonbiopsy ATTR cardiac amyloidosis diagnosis. [8]", "Initiate ATTR management and determine wild-type versus hereditary ATTR. [5][8]" ], [ "Positive or abnormal", "Any uptake, including grade 3", "Cannot distinguish AL from ATTR; AL can produce substantial uptake. [7][8][17]", "Obtain biopsy and definitive amyloid typing; evaluate the plasma-cell disorder. [7][8]" ], [ "Negative", "Uptake not diagnostic or discordant with clinical imaging", "ATTR is not established by scintigraphy. [8]", "Use echocardiography/CMR correlation and biopsy when diagnosis will alter management. [7][8]" ] ] } }, { "id": "initial-management", "eyebrow": "Immediate care", "heading": "Manage congestion, hypotension, and rhythm risk while subtyping proceeds", "intro": "Supportive cardiac management must accommodate restrictive physiology and systemic amyloid complications.", "paragraphs": [ "Use diuretics and sodium restriction to control peripheral edema, ascites, and congestion, while reassessing blood pressure, renal function, and orthostatic symptoms with each titration. Amyloid cardiomyopathy often has restrictive physiology, and peripheral edema or ascites may be amplified by hypoalbuminemia in systemic disease. [23]", "Avoid automatically applying conventional neurohormonal heart-failure regimens. Beta-blockers, nondihydropyridine calcium-channel blockers, vasodilators including ACE inhibitors or ARBs, and digoxin are identified as agents often to avoid in cardiac amyloidosis because hemodynamic intolerance and restrictive physiology may limit use; individualize decisions when another compelling indication exists. [23]", "For neurogenic orthostatic hypotension or autonomic dysfunction, midodrine or droxidopa may be useful. This decision is most relevant in hereditary ATTR with autonomic involvement and in systemic amyloidosis with symptomatic hypotension that constrains diuresis or other cardiac management. [6][23]" ], "bullets": [ "Anticoagulate patients with any atrial arrhythmia unless bleeding risk outweighs expected thromboembolic benefit. [18]", "Consider thromboembolic risk even in sinus rhythm when atrial enlargement and atrial mechanical dysfunction are present; atrial thrombus can occur before atrial fibrillation, particularly in AL amyloidosis. [4][18]", "Before anticoagulation in extensive systemic AL disease, assess bleeding risk because factor X deficiency and gastrointestinal involvement can increase hemorrhagic risk. [4]" ], "subsections": [ { "heading": "When advanced heart-failure referral is appropriate", "paragraphs": [ "Refer patients with progressive advanced heart failure for transplant-center assessment after amyloid subtype and extracardiac disease burden are defined. In AL and ATTR cardiac amyloidosis, transplant candidacy is constrained by multiorgan infiltration; frailty, including Fried frailty phenotype of three or more criteria, is an important adverse selection factor. [9]" ], "bullets": [] } ], "table": { "caption": "Supportive management decisions in cardiac amyloidosis. [4][18][23]", "columns": [ "Clinical problem", "Action", "Key exception or monitoring issue" ], "rows": [ [ "Volume overload", "Use diuretics and sodium restriction. [23]", "Reassess renal function, blood pressure, and orthostatic tolerance during decongestion." ], [ "Orthostatic hypotension from autonomic dysfunction", "Consider midodrine or droxidopa. [23]", "Balance pressor therapy against congestion and supine hypertension." ], [ "Atrial fibrillation or another atrial arrhythmia", "Anticoagulate unless contraindicated. [18]", "Evaluate AL-related factor X deficiency or gastrointestinal involvement when bleeding risk is high. [4]" ], [ "Routine HF pharmacotherapy consideration", "Avoid reflex use of beta-blockers, calcium-channel blockers, ACE inhibitors/ARBs, vasodilators, or digoxin. [23]", "Use only after individualized assessment of hemodynamic tolerance and competing indications." ] ] } }, { "id": "subtype-directed-treatment", "eyebrow": "Disease modification", "heading": "ATTR and AL require different definitive treatment pathways", "intro": "Subtype confirmation is the treatment-defining event.", "paragraphs": [ "For confirmed wild-type or variant ATTR cardiac amyloidosis with NYHA class I-III heart-failure symptoms, prescribe tafamidis to reduce cardiovascular morbidity and mortality. The FDA-approved regimens are tafamidis 61 mg orally once daily or tafamidis meglumine 80 mg orally once daily; tafamidis stabilizes the TTR tetramer, slowing dissociation, fibril formation, and cardiac deposition. [9][10]", "Set expectations that tafamidis slows progression rather than reliably regressing established disease; initiate it early after ATTR confirmation rather than delaying until advanced functional decline. Economic burden is a meaningful treatment tradeoff: at 2020 list prices, the AHA/ACC/HFSA guideline classified tafamidis as low value at more than $180,000 per quality-adjusted life-year gained. [9][10]", "If AL cardiac amyloidosis is confirmed or remains plausible because monoclonal studies are abnormal, direct care urgently toward plasma-cell–targeted therapy rather than ATTR therapy alone. The ACC pathway depicts AL regimens built around cyclophosphamide, bortezomib, dexamethasone, and daratumumab, while contemporary review evidence describes daratumumab as an addition to standard therapy, particularly in relapsed or refractory AL disease. [9][20]", "After ATTR is identified, distinguish hereditary from wild-type disease because hereditary ATTR is a dominantly inherited systemic disorder with frequent neurologic and autonomic involvement, whereas wild-type disease is primarily cardiac. This distinction changes family implications and the scope of neurologic and systemic assessment. [5][6]" ], "bullets": [ "Do not start tafamidis on the basis of PYP uptake if monoclonal testing is abnormal; biopsy and fibril typing are needed to avoid missing treatment-urgent AL disease. [7][8]", "Use tafamidis only within its supported ATTR cardiomyopathy population; the guideline recommendation applies to NYHA class I-III symptoms. [10]", "Assess extracardiac involvement and frailty early when considering advanced therapies, including transplantation. [9]" ], "subsections": [ { "heading": "Monitoring that changes management", "paragraphs": [ "Follow symptoms, congestion, blood pressure and orthostatic tolerance, rhythm status, renal function during diuretic treatment, and serial structural or functional assessment with echocardiography or CMR when disease burden or treatment response is being assessed. Expert imaging consensus rates echocardiography and CMR as appropriate for quantifying cardiac amyloid burden and assessing cardiac response to therapy. [7]" ], "bullets": [ "Escalate reassessment for new atrial arrhythmia, embolic neurologic symptoms, worsening congestion, or progressive hypotension because each may require anticoagulation review, thrombus evaluation, diuretic adjustment, or autonomic-directed treatment. [4][18][23]" ] } ], "table": { "caption": "Subtype-directed treatment decisions after cardiac amyloid typing. [9][10][20]", "columns": [ "Confirmed subtype", "Disease-modifying direction", "Selection boundary" ], "rows": [ [ "ATTR cardiac amyloidosis, wild-type or variant", "Tafamidis 61 mg orally daily or tafamidis meglumine 80 mg orally daily. [9]", "Guideline-supported for NYHA class I-III HF symptoms to reduce cardiovascular morbidity and mortality. [10]" ], [ "AL cardiac amyloidosis", "Urgent plasma-cell–directed treatment pathway, including cyclophosphamide, bortezomib, dexamethasone, and daratumumab-based approaches. [9][20]", "Do not substitute an ATTR diagnosis from scintigraphy when monoclonal-protein testing is abnormal. [7][8]" ], [ "Possible mixed or indeterminate amyloid type", "Tissue biopsy with definitive fibril typing. [5][8][17]", "Concurrent AL and ATTR disease is rare but documented; treatment must match the typed deposits. [17]" ] ] } }, { "id": "prognosis-and-escalation", "eyebrow": "Risk framing", "heading": "Use stage and extracardiac burden to accelerate referral", "intro": "Delayed subtype diagnosis has direct consequences because prognosis and treatment urgency differ by amyloid type.", "paragraphs": [ "Cardiac ATTR amyloidosis carries substantial mortality, with reported median survival ranging from 2 to 6 years depending on disease stage at diagnosis. This supports early diagnostic action in patients with a compatible phenotype rather than serial observation of unexplained wall thickening or restrictive heart failure. [2]", "For ATTR prognostic staging, NT-proBNP greater than 3,000 ng/L and estimated GFR below 45 mL/min/1.73 m² are proposed cutoffs: neither abnormality corresponds to stage I, whereas both correspond to stage III. Use these measures to communicate risk and to prioritize timely advanced-heart-failure and amyloidosis-center involvement. [23]", "For AL staging, the cited framework assigns one point each for troponin T greater than 0.025, NT-proBNP greater than 1,800, and free light chain greater than 18 mg/dL. Biomarker elevation should increase urgency for comprehensive cardiac evaluation, but staging should not delay biopsy-based typing and hematology-directed treatment when a clonal process is suspected. [23]" ], "bullets": [ "Escalate to an amyloidosis-experienced multidisciplinary team when a monoclonal protein is present, tissue typing is required, extracardiac disease is evident, or advanced heart failure develops. [7][9]", "Consider transplant evaluation selectively; extensive extracardiac involvement and frailty can preclude acceptable post-transplant outcomes. [9]" ], "subsections": [], "table": { "caption": "Biomarker thresholds used for prognostic staging. [23]", "columns": [ "Amyloid context", "Biomarker threshold", "Clinical use" ], "rows": [ [ "ATTR cardiac amyloidosis", "NT-proBNP >3,000 ng/L; eGFR <45 mL/min/1.73 m². [23]", "Neither criterion: stage I; both criteria: stage III. [23]" ], [ "AL amyloidosis", "Troponin T >0.025; NT-proBNP >1,800; free light chain >18 mg/dL. [23]", "Assign one point for each cutoff in the cited staging framework. [23]" ] ] } } ], "faq": [ { "question": "Can a strongly positive 99mTc-PYP scan rule out AL cardiac amyloidosis?", "answer": "No. Grade 2 or 3 PYP uptake supports nonbiopsy ATTR diagnosis only when serum free-light-chain testing and serum and urine immunofixation show no clonal plasma-cell process. AL cardiomyopathy can produce grade 3 uptake; abnormal monoclonal testing requires biopsy and amyloid typing. [7][8][17]" }, { "question": "When is endomyocardial biopsy necessary in suspected cardiac amyloidosis?", "answer": "Perform biopsy when monoclonal-protein testing is abnormal, scintigraphy is nondiagnostic or discordant, or an alternative or mixed amyloid type is plausible. Confirm amyloid histologically and type fibrils, ideally with definitive methods such as mass spectrometry. [5][7][8][17]" } ], "references": [ { "number": 1, "title": "Tafamidis Treatment for Patients with Transthyretin Amyloid ...", "detail": "www.nejm.org", "url": "https://www.nejm.org/doi/full/10.1056/NEJMoa1805689", "authors": "www.nejm.org", "host": "www.nejm.org" }, { "number": 2, "title": "Patisiran Treatment in Patients with Transthyretin Cardiac Amyloidosis", "detail": "www.nejm.org", "url": "https://www.nejm.org/doi/full/10.1056/NEJMoa2300757", "authors": "www.nejm.org", "host": "www.nejm.org" }, { "number": 3, "title": "Phase 1 Trial of Antibody NI006 for Depletion of Cardiac ...", "detail": "www.nejm.org", "url": "https://www.nejm.org/doi/full/10.1056/NEJMoa2303765", "authors": "www.nejm.org", "host": "www.nejm.org" }, { "number": 4, "title": "Updates in Cardiac Amyloidosis: A Review", "detail": "www.ahajournals.org", "url": "https://www.ahajournals.org/doi/10.1161/jaha.111.000364", "authors": "www.ahajournals.org", "host": "www.ahajournals.org" }, { "number": 5, "title": "Amyloidosis Diagnosis and Treatment Strategies | Pathology | Clinical Sciences | Health sciences | Topics | Nature Index", "detail": "www.nature.com", "url": "https://www.nature.com/nature-index/topics/l4/amyloidosis-diagnosis-and-treatment-strategies", "authors": "www.nature.com", "host": "www.nature.com" }, { "number": 6, "title": "Early Identification of Transthyretin-Related Hereditary Cardiac Amyloidosis∗", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jcmg.2014.03.007", "authors": "www.jacc.org", "host": "www.jacc.org" }, { "number": 7, "title": "ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI Expert Consensus Recommendations for Multimodality Imaging in Cardiac Amyloidosis: Part 2 of 2—Diagnostic Criteria and Appropriate Utilization", "detail": "www.ahajournals.org", "url": "https://www.ahajournals.org/doi/10.1161/HCI.0000000000000030", "authors": "www.ahajournals.org", "host": "www.ahajournals.org" }, { "number": 8, "title": "Cardiac Scintigraphy With Technetium-99m-Labeled Bone-Seeking Tracers for Suspected Amyloidosis: JACC Review Topic of the Week", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jacc.2020.04.022", "authors": "www.jacc.org", "host": "www.jacc.org" }, { "number": 9, "title": "2023 ACC Expert Consensus Decision Pathway on ... - JACC", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jacc.2022.11.022", "authors": "www.jacc.org", "host": "www.jacc.org" }, { "number": 10, "title": "2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jacc.2021.12.012", "authors": "www.jacc.org", "host": "www.jacc.org" }, { "number": 11, "title": "Update in ‘wild-type’ transthyretin cardiac amyloidosis: Clinical guide for its diagnosis and treatment - ScienceDirect", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/pii/S2387020624003152", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com" }, { "number": 12, "title": "Review of Cardiac Amyloidosis : Journal of the Practice of Cardiovascular Sciences", "detail": "journals.lww.com", "url": "https://journals.lww.com/jpcs/fulltext/2018/04030/review_of_cardiac_amyloidosis.5.aspx", "authors": "journals.lww.com", "host": "journals.lww.com" }, { "number": 13, "title": "A Review of Cardiac Amyloidosis: Presentation, Diagnosis, and Treatment - ScienceDirect", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/abs/pii/S0146280622002638", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com" }, { "number": 14, "title": "Optimizing drug therapies in cardiac amyloidosis - ScienceDirect", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/abs/pii/S0163725824001785", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com" }, { "number": 15, "title": "Therapy of ATTR Cardiac Amyloidosis: Current Indications - ScienceDirect", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/abs/pii/S014628062200384X", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com" }, { "number": 16, "title": "The role of echocardiography and 99mTc-HDP scintigraphy in... : Medicine", "detail": "journals.lww.com", "url": "https://journals.lww.com/md-journal/fulltext/2019/09200/the_role_of_echocardiography_and_99mtc_hdp.68.aspx", "authors": "journals.lww.com", "host": "journals.lww.com" }, { "number": 17, "title": "Concurrent light chain and transthyretin cardiac... : Medicine", "detail": "journals.lww.com", "url": "https://journals.lww.com/10.1097/MD.0000000000046900", "authors": "journals.lww.com", "host": "journals.lww.com" }, { "number": 18, "title": "Management of complications of cardiac amyloidosis: 10 questions ...", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurjpc/article/28/9/1000/6353467", "authors": "academic.oup.com", "host": "academic.oup.com" }, { "number": 19, "title": "Treatment of amyloidosis: present and future - Oxford Academic", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurheartjsupp/article/25/Supplement_B/B99/7135777", "authors": "academic.oup.com", "host": "academic.oup.com" }, { "number": 20, "title": "Cardiac amyloidosis: Innovations in diagnosis and treatment", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurheartjsupp/article/27/Supplement_1/i88/8023290", "authors": "academic.oup.com", "host": "academic.oup.com" }, { "number": 21, "title": "2024 European Heart Rhythm Association/Heart Rhythm Society ...", "detail": "academic.oup.com", "url": "https://academic.oup.com/europace/article/26/4/euae043/7639428", "authors": "academic.oup.com", "host": "academic.oup.com" }, { "number": 22, "title": "Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC Working Group on Myocardial and Pericardial Diseases - PubMed", "detail": "www.ncbi.nlm.nih.gov", "url": "http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=33825853&dopt=Abstract", "authors": "www.ncbi.nlm.nih.gov", "host": "www.ncbi.nlm.nih.gov" }, { "number": 23, "title": "[PDF] Apparent Natural History of Cardiac Amyloidosis - ACC.org", "detail": "www.acc.org", "url": "https://www.acc.org/~/media/Non-Clinical/Files-PDFs-Excel-MS-Word-etc/Meetings/2019/Course%20PDFs/Cardio-Oncology/Sun/Sun%201015%20Lenihan.pdf", "authors": "www.acc.org", "host": "www.acc.org" }, { "number": 24, "title": "Cardiac Amyloidosis", "detail": "www.escardio.org", "url": "https://www.escardio.org/Education/ESC-Education-by-Topic/focus/FOCUS-on-Cardiac-Amyloidosis", "authors": "www.escardio.org", "host": "www.escardio.org" } ], "editorialNote": "Prepared from cited clinical literature using Astra's research workflow. Verify recommendations against current guidance and patient-specific factors.", "citations": [ { "number": 1, "title": "Tafamidis Treatment for Patients with Transthyretin Amyloid ...", "detail": "www.nejm.org", "url": "https://www.nejm.org/doi/full/10.1056/NEJMoa1805689", "authors": "www.nejm.org", "host": "www.nejm.org", "snippet": "A single-center, nonrandomized study showed an association between tafamidis and improved survival. The Transthyretin Amyloidosis Cardiomyopathy", "score": 0.45711306 }, { "number": 2, "title": "Patisiran Treatment in Patients with Transthyretin Cardiac Amyloidosis", "detail": "www.nejm.org", "url": "https://www.nejm.org/doi/full/10.1056/NEJMoa2300757", "authors": "www.nejm.org", "host": "www.nejm.org", "snippet": "The median survival among patients with ATTR cardiac amyloidosis ranges from 2 to 6 years, depending on the disease stage at diagnosis.", "score": 0.370522 }, { "number": 3, "title": "Phase 1 Trial of Antibody NI006 for Depletion of Cardiac ...", "detail": "www.nejm.org", "url": "https://www.nejm.org/doi/full/10.1056/NEJMoa2303765", "authors": "www.nejm.org", "host": "www.nejm.org", "snippet": "When ATTR amyloidosis leads to cardiomyopathy, ATTR deposits accumulate primarily in the cardiac extracellular space. ATTR deposits stiffen the myocardium and", "score": 0.20339128 }, { "number": 4, "title": "Updates in Cardiac Amyloidosis: A Review", "detail": "www.ahajournals.org", "url": "https://www.ahajournals.org/doi/10.1161/jaha.111.000364", "authors": "www.ahajournals.org", "host": "www.ahajournals.org", "snippet": "Title: Updates in Cardiac Amyloidosis: A Review\nImage 3: Journal of the American Heart Association logo. It may present to almost any specialty, and diagnosis is frequently delayed.1 Cardiac involvement is a leading cause of morbidity and mortality, especially in primary light chain (AL) amyloidosis", "score": 0.78294516 }, { "number": 5, "title": "Amyloidosis Diagnosis and Treatment Strategies | Pathology | Clinical Sciences | Health sciences | Topics | Nature Index", "detail": "www.nature.com", "url": "https://www.nature.com/nature-index/topics/l4/amyloidosis-diagnosis-and-treatment-strategies", "authors": "www.nature.com", "host": "www.nature.com", "snippet": "Amyloidosis denotes a heterogeneous group of protein misfolding disorders characterised by the extracellular deposition of insoluble amyloid fibrils in multiple organs and tissues, resulting in progressive dysfunction. The predominant subtypes are immunoglobulin light-chain amyloidosis (AL) and tran", "score": 0.75723875 }, { "number": 6, "title": "Early Identification of Transthyretin-Related Hereditary Cardiac Amyloidosis∗", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jcmg.2014.03.007", "authors": "www.jacc.org", "host": "www.jacc.org", "snippet": "Title: Early Identification of Transthyretin-Related Hereditary Cardiac Amyloidosis∗\nNoninvasive Risk Stratification of Patients With Transthyretin Amyloidosis. Authors: Eloisa Arbustini, MDe.arbustini@smatteo.pv.it, and Giampaolo Merlini, MDAuthors Info & Affiliations. Transthyretin-related heredit", "score": 0.71496695 }, { "number": 7, "title": "ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI Expert Consensus Recommendations for Multimodality Imaging in Cardiac Amyloidosis: Part 2 of 2—Diagnostic Criteria and Appropriate Utilization", "detail": "www.ahajournals.org", "url": "https://www.ahajournals.org/doi/10.1161/HCI.0000000000000030", "authors": "www.ahajournals.org", "host": "www.ahajournals.org", "snippet": "In this paper, Part 2 of a series, a panel of international experts from multiple societies define the diagnostic criteria for cardiac amyloidosis and appropriate utilization of echocardiography, cardiovascular magnetic resonance imaging, and radionuclide imaging in the evaluation of patients with k", "score": 0.8282873 }, { "number": 8, "title": "Cardiac Scintigraphy With Technetium-99m-Labeled Bone-Seeking Tracers for Suspected Amyloidosis: JACC Review Topic of the Week", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jacc.2020.04.022", "authors": "www.jacc.org", "host": "www.jacc.org", "snippet": "2.\n\nEchocardiographic and/or CMR findings suggestive of cardiac amyloidosis (Table 1);\n\n3.\n\nThe presence of ≥grade 2 uptake (by 99m Tc-PYP, 99m Tc-DPD, 99m Tc-HMDP) that is confirmed by SPECT imaging; and\n\n4.\n\nThe absence of a monoclonal gammopathy by serum free light chain (sFLC) assay and serum an", "score": 0.7760977 }, { "number": 9, "title": "2023 ACC Expert Consensus Decision Pathway on ... - JACC", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jacc.2022.11.022", "authors": "www.jacc.org", "host": "www.jacc.org", "snippet": "### 6.2 Treatment\n\n#### 6.2.1 The role of the general cardiologist in the management of cardiac amyloidosis\n\n##### 6.2.1.1 Tafamidis\n\nAs of 2022, tafamidis is the only medication approved by the U.S. Food and Drug Administration (FDA) for treatment of ATTR cardiac amyloidosis. It acts as a TTR stabi", "score": 0.6912478 }, { "number": 10, "title": "2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jacc.2021.12.012", "authors": "www.jacc.org", "host": "www.jacc.org", "snippet": "| COR | LOE | Recommendations |\n --- \n| 1 | B-R | 1. In select patients with wild-type or variant transthyretin cardiac amyloidosis and NYHA class I to III HF symptoms, transthyretin tetramer stabilizer therapy (tafamidis) is indicated to reduce cardiovascular morbidity and mortality (1). |\n| Value ", "score": 0.5608546 }, { "number": 11, "title": "Update in ‘wild-type’ transthyretin cardiac amyloidosis: Clinical guide for its diagnosis and treatment - ScienceDirect", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/pii/S2387020624003152", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com", "snippet": "Click to open first page preview Special issue articles Recommended articles * F.L. Ruberg _et al._ ### Transthyretin amyloid cardiomyopathy: JACC state-of-the-art review ### J Am Coll Cardiol (2019) * D.S. Knight _et al._ ### Cardiac structural and functional consequences of amyloid deposition by c", "score": 0.8088086 }, { "number": 12, "title": "Review of Cardiac Amyloidosis : Journal of the Practice of Cardiovascular Sciences", "detail": "journals.lww.com", "url": "https://journals.lww.com/jpcs/fulltext/2018/04030/review_of_cardiac_amyloidosis.5.aspx", "authors": "journals.lww.com", "host": "journals.lww.com", "snippet": "Title: Review of Cardiac Amyloidosis : Journal of the Practice of Cardiovascular Sciences\n# Review of Cardiac Amyloidosis. Cardiac amyloidosis is characterized by clinically significant extracellular amyloid infiltration of the heart that is commonly associated with multiorgan involvement depending ", "score": 0.75268626 }, { "number": 13, "title": "A Review of Cardiac Amyloidosis: Presentation, Diagnosis, and Treatment - ScienceDirect", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/abs/pii/S0146280622002638", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com", "snippet": "Elsevier logo\nCurrent Problems in Cardiology\n\n## Current Problems in Cardiology\n\n## Published by: Elsevier\n\n### Published by\n\nElsevier\n\n# A Review of Cardiac Amyloidosis: Presentation, Diagnosis, and Treatment\n\n## Article preview\n\n## Abstract\n\n## Introduction\n\n## Organizational access\n\n### Other acc", "score": 0.7283819 }, { "number": 14, "title": "Optimizing drug therapies in cardiac amyloidosis - ScienceDirect", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/abs/pii/S0163725824001785", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com", "snippet": "### Surveillance for disease progression of transthyretin amyloidosis after heart transplantation in the era of novel disease modifying therapies\n\n### The Journal of Heart and Lung Transplantation: the Official Publication of the International Society for Heart Transplantation\n\n### ATTR Amyloidosis:", "score": 0.65914243 }, { "number": 15, "title": "Therapy of ATTR Cardiac Amyloidosis: Current Indications - ScienceDirect", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/abs/pii/S014628062200384X", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com", "snippet": "### JACC Cardiovasc Imaging\n\n### Diagnosis and treatment of cardiac amyloidosis. A position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases\n\n### Eur J Heart Fail\n\n### RNA-targeting and gene editing therapies for transthyretin amyloidosis\n\n### Nat ", "score": 0.62934244 }, { "number": 16, "title": "The role of echocardiography and 99mTc-HDP scintigraphy in... : Medicine", "detail": "journals.lww.com", "url": "https://journals.lww.com/md-journal/fulltext/2019/09200/the_role_of_echocardiography_and_99mtc_hdp.68.aspx", "authors": "journals.lww.com", "host": "journals.lww.com", "snippet": "Title: The role of echocardiography and 99mTc-HDP scintigraphy in... : Medicine\n# The role of echocardiography and 99m Tc-HDP scintigraphy in non-invasive diagnosis of cardiac amyloidosis. Abbreviations: 99m Tc-DPD = technetium-99m 3,3-diphosphono-1,2-propanodicarboxylic acid, 99m Tc-HDP = technetiu", "score": 0.71758664 }, { "number": 17, "title": "Concurrent light chain and transthyretin cardiac... : Medicine", "detail": "journals.lww.com", "url": "https://journals.lww.com/10.1097/MD.0000000000046900", "authors": "journals.lww.com", "host": "journals.lww.com", "snippet": "Title: Concurrent light chain and transthyretin cardiac... : Medicine\nThis study presents an exceedingly rare case of concurrent light chain cardiac amyloidosis (AL-CA) and ATTR cardiac amyloidosis. The diagnosis of coexisting AL-CA and wild-type transthyretin cardiac amyloidosis was confirmed throu", "score": 0.68496037 }, { "number": 18, "title": "Management of complications of cardiac amyloidosis: 10 questions ...", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurjpc/article/28/9/1000/6353467", "authors": "academic.oup.com", "host": "academic.oup.com", "snippet": "Anticoagulation should be given for any atrial arrhythmia, and might be considered in patients in sinus rhythm but an enlarged and", "score": 0.59824234 }, { "number": 19, "title": "Treatment of amyloidosis: present and future - Oxford Academic", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurheartjsupp/article/25/Supplement_B/B99/7135777", "authors": "academic.oup.com", "host": "academic.oup.com", "snippet": "The trial demonstrated that treatment with tafamidis, especially at a dose of 80 mg/day, compared with placebo, was associated with", "score": 0.44941738 }, { "number": 20, "title": "Cardiac amyloidosis: Innovations in diagnosis and treatment", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurheartjsupp/article/27/Supplement_1/i88/8023290", "authors": "academic.oup.com", "host": "academic.oup.com", "snippet": "Recently, daratumumab, an anti-CD38 monoclonal antibody, has been added to standard therapy, particularly for relapsed or refractory AL", "score": 0.43323857 }, { "number": 21, "title": "2024 European Heart Rhythm Association/Heart Rhythm Society ...", "detail": "academic.oup.com", "url": "https://academic.oup.com/europace/article/26/4/euae043/7639428", "authors": "academic.oup.com", "host": "academic.oup.com", "snippet": "... with cardiac amyloidosis referred for electrical cardioversion, despite prior anticoagulation, mainly with DOACs.379. Based on the", "score": 0.29808816 }, { "number": 22, "title": "Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC Working Group on Myocardial and Pericardial Diseases - PubMed", "detail": "www.ncbi.nlm.nih.gov", "url": "http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=33825853&dopt=Abstract", "authors": "www.ncbi.nlm.nih.gov", "host": "www.ncbi.nlm.nih.gov", "snippet": "Title: Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC Working Group on Myocardial and Pericardial Diseases - PubMed\nAn official website of the United States government. **The .gov means it’s official.**. Federal government websites often end in .gov or .mil. sharing ", "score": 0.7443038 }, { "number": 23, "title": "[PDF] Apparent Natural History of Cardiac Amyloidosis - ACC.org", "detail": "www.acc.org", "url": "https://www.acc.org/~/media/Non-Clinical/Files-PDFs-Excel-MS-Word-etc/Meetings/2019/Course%20PDFs/Cardio-Oncology/Sun/Sun%201015%20Lenihan.pdf", "authors": "www.acc.org", "host": "www.acc.org", "snippet": "Cutoffs: NTproBNP >3000 ng/L EGFR <45ml/min/1.73m2 If both negative= Stage I If both present= Stage III European Heart Journal (2018) 39, 2799–2806 doi:10.1093/eurheartj/ehx589 Disease modifying therapeutic opportunities for TTR amyloidosis Liver TTR Tetramer TTR Monomer Misfolded State Amyloid Fibr", "score": 0.73299384 }, { "number": 24, "title": "Cardiac Amyloidosis", "detail": "www.escardio.org", "url": "https://www.escardio.org/Education/ESC-Education-by-Topic/focus/FOCUS-on-Cardiac-Amyloidosis", "authors": "www.escardio.org", "host": "www.escardio.org", "snippet": "Title: Cardiac Amyloidosis\n* All ESC Clinical Practice Guidelines. + Patient versions of ESC Guidelines. + ESC CVD Risk Calculation App. + ESC Handbook of Cardiovascular Rehabilitation. + ESC Handbook of Preventive Cardiology. + The ESC Handbook on Cardiovascular Pharmacotherapy. + ESC Textbook of C", "score": 0.73138535 } ], "publishedAt": "2026-09-15T22:38:26.285887+00:00", "updatedAt": "2026-09-15T22:38:26.285887+00:00", "readingMinutes": 7, "slug": "cardiac-amyloidosis" }