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

Data on the inter-observer consistency for myocardial strain analysis are reported in
Supplementary Table 1.

Cardiac endpoint
The average duration of follow-up was 6.5 years (7.7 median, range 2-9.8 years). Major events occurred in
25 patients (54%), cardiac events in 23 patients (50%) and ICD shocks in 2. As reported in Table 2, both
systolic and diastolic functions were significantly worse in patients with major cardiac events. Left
ventricular posterior wall thickness and IVS were higher in patients with major cardiac events compared to
those without. Patients with major cardiac events also showed lower GLS, ALS, and MBLS compared to
survivors.

The logistic analysis [Table 3] showed that LV EF, LVST, E/E’, GLS, and MBLS were all predictors of
cardiovascular death in univariate analyses.

The multivariate analysis showed that, among eco-derived variables, MBLS was an independent predictor of
mortality [Table 3].

According to ROC analysis, a cut-off of -14 of MBLS was the best result in terms of combined sensitivity
and specificity in identifying major cardiac events on follow-up (sensitivity 100%, specificity 70%, with an
AUC 0.81).

The patients were divided into two groups based on the previous MBLS value found by ROC curve. Kaplan-
Meyer analysis across the TTRm CA population showed a significantly reduced survival in patients with a
MBLS lower than -14 (P < 0.001) [Figure 2].

DISCUSSION
The main results of the present study are: (1) longitudinal dysfunction is observed in TTRv patients with
CA in the early stages; (2) mediobasal LV longitudinal dysfunction is the best echocardiographic predictor
of cardiac death in ATTRv amyloidosis with cardiomyopathy; and (3) a relative apical sparing of strain is
not a predictor of cardiac death in TTRv CA in patients with non-severe heart failure (NYHA I-II) and no
other cardiac disease.

In the present study, we showed that strain imaging is not only useful for diagnosis, but it is a powerful
prognostic indicator of long-term survival even if applied in the subset of early stages ATTRv amyloidosis
with cardiomyopathy.

Previous studies demonstrated the value of tissue Doppler and strain imaging for diagnosis and staging of
[17]
cardiac dysfunction in patients with CA [14-16] as well in other systemic diseases with cardiac involvement .
Global longitudinal strain and apical sparing have been shown to be an accurate diagnostic marker of CA.

Particularly, apical sparing permits discriminating CA in the context of hypertrophic phenotypes
[18]
cardiomyopathies .
A relative apical sparing of strain has been reported as the only independent predictor of major adverse
[9]
cardiac events in AL CA [8,9,19] . Senapati et al. studied 97 patients with a definite diagnosis of CA (59 AL, 23
wtTTR, and 15 TTRv) and found that, among predictors (AL subtype, EF, and NYHA class ≥ III), the ratio
between apical and mid-basal segments remains independently predictive of all-cause mortality or heart