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SUSPECT TRANSTHYRETIN AMYLOID CARDIOMYOPATHY (ATTR-CM)

懷疑是運甲狀腺素蛋白類澱粉沉著症造成之心肌病變 (ATTR-CM)

ATTR-CM presents with heart failure symptoms. Learn how to recognize the
clues to make a diagnosis.1-3

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HIDDEN IN PLAIN SIGHT
藏在眼前的疾病

SUSPECT ATTR-CM
懷疑可能是 ATTR-CM

The diagnosis of ATTR-CM is often delayed or missed. Routine heart failure assessments such as echo and electrocardiogram (ECG), along with advanced imaging techniques, can help identify clues on the diagnostic pathway. By increasing your suspicion of ATTR-CM, you can identify patients who may require further testing to make a diagnosis.1-4

grit-magnify-glass

CONSIDER THE FOLLOWING CLINICAL CLUES, ESPECIALLY IN COMBINATION, TO RAISE SUSPICION FOR ATTR-CM AND THE NEED FOR FURTHER TESTING

hfpef

正常收縮分率心衰竭 (HFpEF) 患者，通常為 60 歲以上4

  • In ATTR-CM, diastolic function is impaired due to amyloid fibril deposition in the myocardium, resulting in thicker and inelastic ventricles, thereby decreasing stroke volume. It is not until the later stages of ATTR-CM disease that ejection fraction drops8,9
  • Prevalence among older HFpEF patients:
    • ~10% of patients referred to a dedicated center had ATTR-CM confirmed by EMB10†
    • ~13% of hospitalized patients with HFpEF and increased LV wall thickness had wtATTR-CM confirmed by scintigraphy5‡
  • In patients undergoing transcatheter aortic valve replacement for severe calcific AS, prevalence of ATTR-CM was 16% overall and 22% among men6

Reduced longitudinal strain with apical sparing should raise suspicion of ATTR-CM1,9

Strain

Strain_mobile

Illustrative representation.

intolerance-grad

無法耐受標準心衰竭藥物治療 (如：ACEi、ARBs、β-blockers 等)9

  • Intolerance to standard heart failure medications is common in patients with cardiac amyloidosis; such agents exacerbate symptomatic hypotension in the setting of concomitant autonomic dysfunction or by lowering heart rate and reducing cardiac output11
discordance-grad

心電圖的 QRS 電位與心臟超音波影像的左心室壁厚度呈現矛盾的結果10,11

  • The classic ECG feature of ATTR-CM is a discordance between QRS voltage and LV mass ratio7
  • The amplitude of the QRS voltage is not reflective of the increased LV wall thickness, because the increase is due to extracellular amyloid protein deposition rather than myocyte hypertrophy1
    • Absence of a low QRS voltage does not, however, rule out amyloidosis, as low voltage can vary among cardiac amyloidosis etiologies12,14-17

Discordance between LV wall thickness and QRS voltage

discordance

discordance mobile

Illustrative representation.

diagnosis-grad

診斷出腕隧道症候群或腰椎管狹窄症12,13

Bilateral carpal tunnel syndrome and lumbar stenosis

  • Often seen in ATTR-CM due to amyloid deposition in these areas; lumbar spinal stenosis is principally seen in wtATTR-CM7,18,19
  • Bilateral carpal tunnel syndrome and lumbar spinal stenosis are known clinical predictors of ATTR-CM and may precede heart failure symptoms by several years7,19
  • Among patients undergoing carpal tunnel release surgery, 10.2% had amyloid deposits22

Biceps tendon rupture

  • Among patients with wtATTR-CM, biceps tendon rupture has been observed in 33% of patients, occurring in the dominant arm in 95% and bilaterally in 24% of patients20

Hip and knee arthroplasty

  • In a study of 313 patients (172 with ATTR-CM), hip and knee arthroplasty surgeries were more frequent than in the general population, and on average, arthroplasty occurred 7.2 years before ATTR-CM diagnosis21
echo-grad

心臟超音波結果顯示左心室壁增厚10

  • Unexplained increased LV wall thickness (eg, hypertension) should raise suspicion for cardiac amyloidosis3

Transthoracic echocardiograms showing increased LV wall thickness

Parasternal long-axis view

Parasternal short-axis view

ATTR amyloidosis heart

Amyloid Heart Long view_Vid

Video courtesy of Filiale d'Imagerie Cardiovasculaire.

ATTR amyloidosis heart

attr-amyloid-heart

Increased LV end diastolic interventricular wall thickness

Normal heart

parasternal-heart

Illustrative representation.

nervoussystem-grad

自主神經系統功能異常，包括腸胃道不適或無法解釋的體重減輕14

  • Gastrointestinal complaints due to autonomic dysfunction include chronic diarrhea, constipation, or both23
  • Orthostatic hypotension due to autonomic dysfunction is another symptom that may occur with ATTR-CM23

*Notably those with a low-flow, low-gradient AS pattern.6

†A prospective analysis in 108 patients (61% women, age range: 57-74 years) seen at the Johns Hopkins University HFpEF Clinic who underwent EMB to evaluate myocardial tissue histopathology.10

‡A prospective, cross-sectional, single-center study at a tertiary university hospital in Madrid, Spain. Included 120 patients ≥60 years of age (59% women, mean age: 82 ± 8 years) admitted for HFpEF, with LV ejection fraction ≥50% and LV hypertrophy ≥12 mm. 99mtechnetium-labeled 3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) scintigraphy used to confirm ATTR-CM.5

Echo, echocardiography.

DOWNLOAD HIDDEN MNEMONIC

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USE THE estimATTR TO LEARN ABOUT CLINICAL CONDITIONS COMMONLY ASSOCIATED WITH wtATTR-CM

利用 estimATTR，了解 wtATTR-CM 常見的相關臨床症狀

A PROBABILITY ESTIMATOR FOR wtATTR-CM

This tool is only to be used by Taiwan health care professionals. It is for educational purposes only, and is not to be used for the suspicion or diagnosis of wtATTR-CM in individual patients in a clinical setting.

estimattr

TRY IT NOW

The wtATTR-CM estimATTR was developed based on an AI/ML algorithm using diagnosis codes from a large, real-world claims dataset comprising 1678 patients with wtATTR-CM.

Using hypothetical patient cases presenting with heart failure, the estimATTR can highlight the types of combinations of cardiac and noncardiac conditions that should raise suspicion for wtATTR-CM by:

  • Estimating the probability of wtATTR-CM based on combinations of clinical features known to be associated with the disease
  • Illustrating important features that help distinguish between wtATTR-CM and heart failure from other causes

THE HIDDEN CLINICAL CLUES OF ATTR-CM

Discover how a cardiologist suspects ATTR-CM.

In the first video, a colleague asked Dr. Detective for help with a tough case. Watch to see how looking for certain signs and symptoms can point to ATTR-CM.

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BE EMPOWERED WITH INFORMATION—DETECT ATTR-CM

CONTENT DIRECTORY

References: 1. Narotsky DL, Castaño A, Weinsaft JW, Bokhari S, Maurer MS. Wild-type transthyretin cardiac amyloidosis: novel insights from advanced imaging. Can J Cardiol. 2016;32(9):1166.e1-1166.e10. 2. AlJaroudi WA, Desai MY, Tang WH, Phelan D, Cerqueira MD, Jaber WA. Role of imaging in the diagnosis and management of patients with cardiac amyloidosis: state of the art review and focus on emerging nuclear techniques. J Nucl Cardiol. 2014;21(2):271-283. 3. Rapezzi C, Lorenzini M, Longhi S, et al. Cardiac amyloidosis: the great pretender. Heart Fail Rev. 2015;20(2):117-124. 4. Brunjes DL, Castano A, Clemons A, Rubin J, Maurer MS. Transthyretin cardiac amyloidosis in older Americans. J Card Fail. 2016;22(12):996-1003. 5. González-López E, Gallego-Delgado M, Guzzo-Merello G, et al. Wild-type transthyretin amyloidosis as a cause of heart failure with preserved ejection fraction. Eur Heart J. 2015;36(38):2585-2594. 6. Castaño A, Narotsky D, Hamid N, et al. Unveiling transthyretin cardiac amyloidosis and its predictors among elderly patients with severe aortic stenosis undergoing transcatheter aortic valve replacement. Eur Heart J. 2017;38(38):2879-2887. 7. Ruberg FL, Grogan M, Hanna M, Kelly JW, Maurer MS. Transthyretin amyloid cardiomyopathy: JACC state-of-the-art review. J Am Coll Cardiol. 2019;73(22):2872-2891. 8. Borlaug BA, Paulus WJ. Heart failure with preserved ejection fraction: pathophysiology, diagnosis, and treatment. Eur Heart J. 2011;2(6):670-679. 9. Siddiqi OK, Ruberg FL. Cardiac amyloidosis: an update on pathophysiology, diagnosis, and treatment. Trends Cardiovasc Med. 2018;28(1):10-21. 10. Hahn VS, Yanek LR, Vaishnav J, et al. Endomyocardial biopsy characterization of heart failure with preserved ejection fraction and prevalence of cardiac amyloidosis. JACC Heart Fail. 2020;8(9):712-724. doi:10.1016/j.jchf.2020.04.007 11. Castaño A, Drachman BM, Judge D, Maurer MS. Natural history and therapy of TTR-cardiac amyloidosis: emerging disease-modifying therapies from organ transplantation to stabilizer and silencer drugs. Heart Fail Rev. 2015;20(2):163-178. 12. Quarta CC, Solomon SD, Uraizee I, et al. Left ventricular structure and function in transthyretin-related versus light-chain cardiac amyloidosis. Circulation. 2014;129(18):1840-1849. 13. Carroll JD, Gaasch WH, McAdam KP. Amyloid cardiomyopathy: characterization by a distinctive voltage/mass relation. Am J Cardiol. 1982;49:9-13. 14. Maurer MS, Hanna M, Grogan M, et al. Genotype and phenotype of transthyretin cardiac amyloidosis: THAOS (Transthyretin Amyloid Outcome Survey). J Am Coll Cardiol. 2016;68(2):161-172. 15. Connors LH, Sam F, Skinner M, et al. Heart failure resulting from age-related cardiac amyloid disease associated with wild-type transthyretin: a prospective, observational cohort study. Circulation. 2016;133(3):282-290. 16. Cyrille NB, Goldsmith J, Alvarez J, Maurer MS. Prevalence and prognostic significance of low QRS voltage among the three main types of cardiac amyloidosis. Am J Cardiol. 2014;114(7):1089-1093. 17. Ng B, Connors LH, Davidoff R, Skinner M, Falk RH. Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis. Arch Intern Med. 2005;165(12):1425-1429. 18. Nakagawa M, Sekijima Y, Yazaki M, et al. Carpal tunnel syndrome: a common initial symptom of systemic wild-type ATTR (ATTRwt) amyloidosis. Amyloid. 2016;23(1):58-63. 19. Westermark P, Westermark GT, Suhr OB, Berg S. Transthyretin-derived amyloidosis: probably a common cause of lumbar spinal stenosis. Ups J Med Sci. 2014;119(3):223-228. 20. Geller HI, Singh A, Alexander KM, et al. Association between ruptured distal biceps tendon and wild-type transthyretin cardiac amyloidosis. JAMA. 2017;318(10):962-963. 21. Rubin J, Alvarez J, Teruya S, et al. Hip and knee arthroplasty are common among patients with transthyretin cardiac amyloidosis, occurring years before cardiac amyloid diagnosis: can we identify affected patients earlier? Amyloid. 2017;24(4):226-230. doi:10.1080/13506129.2017.1375908 22. Sperry BW, Reyes BA, Ikram A, et al. Tenosynovial and cardiac amyloidosis in patients undergoing carpal tunnel release. J Am Coll Cardiol. 2018;72(17):2040-2050. 23. Coelho T, Maurer MS, Suhr OB. THAOS - The Transthyretin Amyloidosis Outcomes Survey: initial report on clinical manifestations in patients with hereditary and wild-type transthyretin amyloidosis. Curr Med Res Opin. 2013;29(1):63-76.