Clinical Clues | Additional Key Considerations
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HEART FAILURE WITH PRESERVED EJECTION FRACTION in patients typically over 605-7
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INTOLERANCE to standard heart failure therapies, such as angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, and beta blockers1,4,11
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DISCORDANCE between QRS voltage on ECG and left ventricular (LV) wall thickness seen on echocardiography12-14
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ECG and echocardiography images showing discordance of limb lead QRS voltages and the degree of LV wall thickness in a patient with cardiac amyloidosis |
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DIAGNOSIS of carpal tunnel syndrome or lumbar spinal stenosis4,16,18-24
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ECHOCARDIOGRAPHY showing increased LV wall thickness6,12,20,27,28
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NERVOUS SYSTEM: Autonomic nervous system dysfunction, including gastrointestinal complaints or unexplained weight loss6,20,29,30
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ADDITIONAL KEY CONSIDERATIONSThere are several additional signs/symptoms that could be clues for cardiac amyloidosis and ATTR-CM, which include: Strain imaging showing apical sparing or apical preservation1,3,8,12,28 Longitudinal strain seen on echocardiography is reduced in the basal and midwall area; however, the apical strain is spared or preserved1,3,8,12,28 A history of bicep tendon rupture31,32 A diagnosis of hypertrophic cardiomyopathy1,3,33 Arrhythmias such as atrial fibrillation (most common) or other conduction abnormalities, which may require a pacemaker5,6,14,16,33,34 Aortic stenosis (AS) and ATTR-CM may occur in elderly patients, notably those with a low-flow, low-gradient AS pattern35-37 Hip and knee arthroplasty38 Echocardiology in cardiac amyloidosis. a-b Granular echogenic appearance of the ventricular wall with the clear hypertrophy and some pericardial effusion. Left ventricular ejection fraction is preserved (c), septial tissue Doppler longitudinal movement is reduced (d). Longitudinal strain analysis from the 3 apical views showing characteristic apical sparing (bull's eye) with reduced strain at the mid and basal level (e).Reproduced from Oerlemans MIFJ, Rutten KHG, Minnema MC, Raymakers RAP, Asselbergs FW, de Jonge N. Cardiac amyloidosis: the need for early diagnosis. Neth Heart J. 2019;27(11):525-536. doi: 10.1007/s12471-019-1299-1, under Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/).
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PREVIOUS SECTION: OVERVIEW UNDERSTANDING—ATTR-CM NEXT SECTION: BE EMPOWERED WITH INFORMATION—DETECT ATTR-CM
References:
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19. 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.
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21. 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.
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26. Pinney JH, Whelan CJ, Petrie A, et al. Senile systemic amyloidosis: clinical features at presentation and outcome. J Am Heart Assoc. 2013;2(2):e000098.
27. Phelan D, Collier P, Thavendiranathan P, et al. Relative apical sparing of longitudinal strain using two-dimensional speckle-tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis. Heart. 2012;98(19):1442-1448.
28. Ternacle J, Bodez D, Guellich A, et al. Causes and consequences of longitudinal LV dysfunction assessed by 2D strain echocardiography in cardiac amyloidosis. JACC Cardiovasc Imaging. 2016;9(2):126-138.
29. 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.
30. Swiecicki PL, Zhen DB, Mauermann ML, et al. Hereditary ATTR amyloidosis: a single-institution experience with 266 patients. Amyloid. 2015;22(2):123-131.
31. Nativi-Nicolau J, Maurer MS. Amyloidosis cardiomyopathy: update in the diagnosis and treatment of the most common types. Curr Opin Cardiol. 2018;33(5):571-579.
32. 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.
33. González-López E, Gagliardi C, Dominguez F, et al. Clinical characteristics of wild-type transthyretin cardiac amyloidosis: disproving myths. Eur Heart J. 2017;38(24):1895-1904.
34. Givens RC, Russo C, Green P, Maurer MS. Comparison of cardiac amyloidosis due to wild-type and V122I transthyretin in older adults referred to an academic medical center. Aging Health. 2013;9(2):229-235.
35. Castaño A, Narotsky DL, 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.
36. Galat A, Guellich A, Bodez D, et al. Aortic stenosis and transthyretin cardiac amyloidosis: the chicken or the egg? Eur Heart J. 2016;37(47):3525-3531.
37. Treibel TA, Fontana M, Gilbertson JA, et al. Occult transthyretin cardiac amyloid in severe calcific aortic stenosis: prevalence and prognosis in patients undergoing surgical aortic valve replacement. Circ Cardiovasc Imaging. 2016;9(8). doi: 10.1161/CIRCIMAGING.116.005066.
38. 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.
PP-RDP-IRL-0111 Date of Preparation February 2021