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Page 2 of 10 Licordari et al. Vessel Plus 2022;6:12 https://dx.doi.org/10.20517/2574-1209.2021.86

Results: A higher value of LV thickness, E/E’, and RRSR and lower LV volumes, EF, GLS, MBLS were observed in
patients with CA than the ones with only TTRv. During follow-up (median of 7.7 years), 25 major events (23
cardiac death and 2 appropriate ICD shocks) occurred. The logistic univariate analyses showed that LV EF, LV
septal thickness, E/E’, GLS, and MBLS were all predictors of cardiovascular death. In multivariate analysis, MBLS
was the only independent predictor of major events. A cut off of -14 of MBLS was selected as the best value to
discriminate a worse prognosis on Kaplan-Meyer analysis.

Conclusion: Longitudinal dysfunction is observed in the early stages of ATTRv amyloidosis with cardiomyopathy.
Medio-basal LV longitudinal dysfunction is a strong independent echocardiographic predictor of cardiac death.

Keywords: Cardiac amyloidosis, myocardial strain imaging, prognosis

INTRODUCTION
Familial transthyretin amyloidosis due to a mutation of the gene coding for transthyretin (TTRv) is one of
the three most frequent subtypes, together with light chain (AL) and wild-type TTR amyloidosis
[1-3]
(wtTTR) . Among these, mutated TTR represents the most uncommon form of cardiac amyloidosis (CA).

In TTRv patients, penetrance and disease phenotype is variable according to the different well-known TTR
gene mutations . In the clinical management of carriers, the identification of early cardiac involvement,
[4]
ideally in a presymptomatic stage, is of paramount importance to achieve correct management and early
treatment.

Non-invasive diagnosis of CA is performed using an integrated evaluation of biomarkers (troponins and
natriuretic peptides), echocardiography, cardiac magnetic resonance, and Tc-3,3-diphosphono-1,2
99m
propanodicarboxylic acid (DPD) scintigraphy. DPD scintigraphy, identifying with high accuracy TTR-
related amyloidosis, is considered the non-invasive gold standard diagnostic tool for ATTR amyloidosis
with cardiomyopathy .
[5-7]

In clinical practice, echocardiography is the imaging tool most used during both initial evaluation and
follow-up of CA patients. A relative “apical sparing” pattern of longitudinal deformation is an easily
recognizable, accurate, and reproducible method of differentiating CA from other causes of left ventricular
(LV) hypertrophy .
[8]
Echocardiographic parameters such as increased LV wall thickness, global longitudinal strain (GLS),
reduced ejection fraction (EF), and a high relative regional strain ratio are independent predictors of cardiac
mortality in AL amyloidosis .
[9]
On the contrary, no large studies have evaluated the prognostic role of an integrated echocardiographic
approach in patients with ATTR amyloidosis with cardiomyopathy especially in the early stage of the
disease.

Our study aimed to assess the best conventional and strain-derived echocardiographic prognostic
parameters in the early clinical stage of ATTRv amyloidosis with cardiomyopathy.