Echocardiographic and Biohumoral Characteristics in Patients With AL and TTR Amyloidosis at Diagnosis

Abstract

Background: Few studies have analyzed the clinical and echocardiographic differences between light-chain (AL) and transthyretin (TTR) amyloidosis. Hypothesis: The aim of the present research was to compare, in a real-world setting, the clinical and echocardiographic profiles of these kinds of amyloidosis, at the time of diagnosis, using new-generation echocardiography. Methods: Seventy-nine patients with AL and 48 patients with TTR amyloidosis were studied. Results: According to the criterion of mean left ventricular (LV) thickness >12 mm, 45 AL (C-AL) and all TTR patients had cardiac amyloidotic involvement, whereas 34 AL patients did not. TTR patients had increased right ventricular (RV) and LV chambers with increased RV and LV wall thickness and reduced LV ejection fraction and fractional shortening. Furthermore, TTR patients showed lower N-terminal pro Brain Natriuretic Peptide concentrations and New York Heart Association functional class when compared with C-AL. Conclusions: Our data show that at time of first diagnosis, TTR patients have a more advanced amyloidotic involvement of the heart, despite less severe symptoms and biohumoral signs of heart failure. We can hypothesize that we observed different diseases at different stages. In fact, AL amyloidosis is a multiorgan disease with quick progression rate, that becomes rapidly symptomatic, whereas TTR amyloidosis might have a slow progression rate and might remain poorly symptomatic for a greater amount of time.

Prognostic implication of relative regional strain ratio in cardiac amyloidosis

“…Earlier studies have shown certain clinical and echocardiographic parameters to be associated with mortality in CA including NYHA class,11 early mitral inflow velocity, deceleration time16 and LV wall thickness 12. Echocardiographic assessment of systolic global LS has also been used to evaluate prognosis in CA 13–15.…”

Wild-Type Transthyretin Cardiac Amyloidosis: Novel Insights From Advanced Imaging

“…hypertrophic cardiomyopathy, Fabry's disease) 1,3,5,6,12 . While morphologic features of AL and ATTRwt can overlap, prior studies have reported patients with ATTRwt to manifest greater LV wall thickness than those with AL 2,25,26,27 – mean wall thickness of 17mm±2 versus 15mm±2 in a recent study 27 . Irrespective of diagnostic subtype, increased wall thickness in patients with amyloid is due to protein (AL or TTR) deposition rather than actual myocyte hypertrophy, thereby explaining concomitant “low voltage” on ECG 1,5 .…”

“…Longitudinal strain (LS) can be analyzed using tissue-Doppler imaging (TDI) and 2D speckle-tracking. Furthermore, 3D speckle-tracking can be used to differentiate between ATTRwt-CA and other hypertrophic diseases by potentially yielding improved assessment of global radial, circumferential, and longitudinal strain 1,3,5,6,15,25,28 . Radial and longitudinal strain have each been shown to be reduced in ATTRwt-CA as well as other conditions, although the pattern of strain decrement can be used to identify CA.…”

“…A novel insight from strain imaging is that patients with ATTRwt-CA typically demonstrate marked decrease in LS in basal and mid-wall areas with relative apical sparing, referred to as “cherry on top” or apical preservation 17 ( Figure 1 ). Conversely, patients with HCM or aortic stenosis related LVH typically demonstrate reduced LS in regions of maximal hypertrophy 5,6,17,25,29 . Generally, abnormal LS portends a worse survival.…”

Transthyretin Cardiac Amyloidosis in Older Adults: Optimizing Cardiac Imaging to the Corresponding Diagnostic and Management Goal

“…Typical echocardiographic features of CA include left ventricular (LV) and right ventricular (RV) wall thickening without an explanatory stimulus (i.e. hypertension or valvular disease, specifically aortic stenosis) [10], bi-atrial enlargement, thickening of the interatrial septum, and the presence of a pericardial effusion [11–14]. Diastolic dysfunction is common [13, 15] and often advanced, typically with a restrictive filling pattern with high early (E) and relatively low atrial (A) wave with an E/A ratio >2, and a short deceleration time [10, 13, 15].…”