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. 2021 Jun 25;8:644508. doi: 10.3389/fcvm.2021.644508

Table 1.

Summary of commonly encountered “red flag” symptoms associated with ATTR-CM from the HIDDEN mnemonic present in each illustrative case.

“Red flag” symptoms associated with ATTR-CM Case 1 ATTR-CM + MGUS Case 2 AL amyloidosis Case 3 ATTRwt-CM Case 4 ATTRm-CM
HFpEF and other cardiac conditions, including atrial fibrillation, arrhythmia and atrioventricular block (10)
Intolerance to standard heart failure therapies (e.g., angiotensin converting enzyme inhibitors/angiotensin receptor blockers and beta-blockers) (19)
Discordance of QRS voltage and left ventricular wall thickness seen on echocardiography (24, 37)
Diagnosis of carpal tunnel syndrome, biceps tendon rupture or lumbar spinal stenosis (38, 39)
Echocardiography showing increased left ventricular wall thickness and/or low-flow gradient aortic stenosis and additional echocardiography parameters# (24)
Nervous system—autonomic nervous system dysfunction, including gastrointestinal complaints or unexplained weight loss (40)
#

Echocardiographic, speckle-strain and tissue doppler “red flags”: Heart failure normal or mid-range ejection fraction; increased wall thickness; left atrial enlargement; low stroke volume index; low-flow, low-gradient aortic stenosis (Stage D2); low myocardial contraction fraction; advanced diastolic dysfunction; impaired global longitudinal strain with apical sparing; low mitral annular tissue Doppler S' (average septal and lateral annulus) (24).

AL, light-chain amyloidosis; ATTR-CM, transthyretin amyloid cardiomyopathy; ATTRm-CM, hereditary (mutant) transthyretin amyloid cardiomyopathy; ATTRwt-CM, wild-type transthyretin amyloidosis cardiomyopathy; HFpEF, heart failure with preserved ejection fraction; MGUS, monoclonal gammopathy of undetermined significance; QRS, Q wave, R wave, and S wave.