Cardiac Amyloidosis : when to suspect
- The presence of unexplained LVWT >12 mm is observed.
- This is accompanied by a discrepancy between LVWT and electrocardiogram (ECG) QRS voltage( LVH with low voltage complexes)
- There is evidence of LVH with thickened valves, interatrial septum, and right ventricular (RV) free wall
- The LV global longitudinal strain (GLS) is reduced, with sparing of the apical region( cherry on top sign)
- There are very low mitral E’/S’/A’ velocity of <5 cm/s on tissue Doppler imaging (TDI)
- In cases of wATTR‑CM, it is more common to observe lone atrial fibrillation (AF) or complete heart block (CHB), with CHB occurring in one‑third of cases and AF in 40%–60% of patients at the time of diagnosis
- Unexplained HFpEF( 13-17 % will have Cardiac Amyloidosis)
- LFLG AS( low flow low gradient AS)
- Hypotension with beta‑blockers, angiotensin converting enzyme inhibitors, and diuretics in the context of heart failure
- The “spontaneous natural cure” of existing hypertension
- There is an ongoing increase in the levels of troponin I (Trop I) and natriuretic peptides [B-type natriuretic peptide (BNP) or N-terminal pro-brain natriuretic peptide (NT-proBNP)]
- carpal tunnel syndrome or spinal canal stenosis (thickening of the ligamentum flavum) or spontaneous rupture of the biceps tendon (ATTR‑CM). Carpal tunnel syndrome is observed in roughly 50% of individuals with wATTR‑CM, typically occurring before the onset of CM by an average duration of 5–10 years. The incidence of biceps tendon rupture in patients with wATTR‑CM has been documented to be 33%
- The occurrence of an unexplained peripheral neuropathy, characterized by symmetrical and largely sensory symptoms, initiating in the lower limbs, and progressing in an ascending manner. This pattern is observed in individuals with AL‑CM and those with mutation‑specific hATTR‑CM, often accompanied by heart failure
- The presence of unexplained proteinuria (AL‑CM) in conjunction with heart failure
- HFpEF is accompanied by macroglossia, periorbital bruising, and dysfunction of the autonomic nervous system (observed in 10% of patients with AL‑CM)
- LVH is accompanied by autonomic dysfunction resulting in orthostatic hypotension, as well as gastrointestinal symptoms such as diarrhea or constipation or erectile dysfunction. These symptoms are more prevalent in hATTR‑CM compared to wATTR‑CM and less prevalent in AL‑CM
- LVH is accompanied by microvascular angina, as well as claudication in the jaw, buttocks, and legs. This is seen in the cases with perivascular amyloid deposition, particularly in AL amyloidosis
- LVH is associated with ocular conditions such as glaucoma, intravitreal deposits, and scalloped pupils (hATTR‑CM)
- In the presence of familial amyloidosis, laryngeal involvement leading to dysphonia and the presence of right‑sided cardiac involvement on imaging (AApo AI).
