5 The echocardiograph granular or “sparkling” ventricular wall appearance, a reported classic feature, is primarily associated with low sensitivity to detect cardiac amyloidosis, can occur in other causes of LV hypertrophy, and is less specific with the application of tissue harmonics (increases myocardial echogenicity in general) in contrast to standard 2D echocardiography imaging

alone.5 “Left ventricular hypertrophy” is a misnomer given that the histologic pathological hallmark of cardiac amyloid disease is extracellular infiltration and not myocyte hypertrophy (Figure 2). While there are many echocardiographic features common in amyloid disease, none are highly specific Inhibitors,research,lifescience,medical and a combination of several is Inhibitors,research,lifescience,medical often needed to make a diagnosis. Figure 2 Endomyocardial biopsy

findings of cardiac amyloid deposition.(A) Light microscopy of cardiac tissue with intervening stroma (extracellular) with hyalinized appearance (appears pink to red with Congo red staining, see red arrow) secondary to amyloid deposits. … Recently, gadolinium-enhanced cardiac magnetic resonance (CMR) has been PD0332991 concentration clinically utilized to demonstrate Inhibitors,research,lifescience,medical late gadolinium enhancement (LGE) in cardiac disease states characterized by cardiac interstitial or extracellular expansion.9 This extracellular cardiac expansion represents fibrosis replacement or scar in the context of coronary artery disease, Inhibitors,research,lifescience,medical lymphocytic infiltration in the

context of myocarditis, and amyloid deposition in the context of cardiac amyloidosis. In the largest series to date (120 patients with amyloidosis), of the 35 patients with histologically verified cardiac amyloidosis, abnormal LGE was present in 34 (97%). A smaller case series of patients with histologically proven AL amyloidosis showed LGE by CMR in 76% of patients.10 Interestingly, in these cohorts of patients, increased echocardiographic Inhibitors,research,lifescience,medical left ventricular wall thickness was less frequently present as an imaging surrogate of cardiac amyloid deposition (32, or 91% of patients).7 The most common pattern of LGE seen by CMR is Terminal deoxynucleotidyl transferase transmural or subendocardial global enhancement (Figure 3). While global LGE is most common in patients with cardiac amyloidosis, focal patchy LGE has also been observed.7 Figure 3 Delayed enhancement cardiovascular magnetic resonance imaging detection of cardiac amyloidosis.Short axis mid-ventricular view of the left (LV) and right ventricles (RV). Yellow arrows indicates global late gadolinium enhancement (transmural bright white … Cardiac biomarkers including troponin T and I and brain natriuretic peptide (BNP) and N-terminal (NT)-proBNP can be elevated in cardiac amyloidosis.2 The presumed mechanism to account for myocardial necrosis in cardiac amyloidosis is small-vessel ischemia due to amyloid deposition.