SPC365

Echocardiographic Differentiation of Cardiac Amyloidosis Subtypes

Session: SESSÃO DE POSTERS 19 - IMAGEM NAS MIOCARDIOPATIAS
Speaker: João Mendes Cravo

Congress: CPC 2025
Topic: B. Imaging
Theme: 03. Imaging
Subtheme: 03.1 Echocardiography
Session Type: Cartazes
FP Number: ---
Authors: João Mendes Cravo; Ana Abrantes; Marta Vilela; Catarina Gregório; Catarina Campos; Isabel Conceição; Catarina Sousa; Fausto J.Pinto; Dulce Brito; João Agostinho
<p style="text-align:justify"><span style="font-size:18px"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong>Introduction:</strong></span></span></span></p>

<p style="text-align:justify"><span style="font-size:18px"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">Cardiac amyloidosis (CA) presents significant diagnostic challenges due to the overlapping echocardiographic (echo) features observed among its subtypes. Differentiating transthyretin wild-type (wtATTR), transthyretin hereditary (hATTR) and light chain (AL-CM) cardiac amyloidosis remains complex, especially when relying solely on echo criteria.</span></span></span></p>

<p style="text-align:justify"><span style="font-size:18px"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong>Purpose:&nbsp;</strong></span></span></span></p>

<p style="text-align:justify"><span style="font-size:18px"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">To compare echo features that can distinguish wtATTR, hATTR and AL-CM and to evaluate their progression over time.</span></span></span></p>

<p style="text-align:justify"><span style="font-size:18px"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong>Methods:&nbsp;</strong></span></span></span></p>

<p style="text-align:justify"><span style="font-size:18px"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">A retrospective, single-center study included patients with CA. Three groups of patients - wtATTR, hATTR and AL-CM - matched for age, gender and comorbidities were established. Echo parameters at diagnosis and 2-year follow-up were collected and analyzed.</span></span></span></p>

<p style="text-align:justify"><span style="font-size:18px"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong>Results:</strong></span></span></span></p>

<p style="text-align:justify"><span style="font-size:18px"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">A total of 120 patients with CA were initially screened and 45 were selected for the study: 14 (31%) with hATTR, 20 (44%) with wtATTR and 11 (25%) with AL-CM. Baseline characteristics were comparable across groups in terms of mean age: 82&plusmn;1.1 years for wtATTR, 77&plusmn;2.6 years for hATTR and 68&plusmn;3.1 years for AL-CM (p=0.06). Most patients were male: 13 (92%) vs 17 (85%) vs 9 (81%) respectively (p=0.2). At baseline, significant differences were observed between wtATTR and hATTR. Patients with wtATTR-CM had higher left ventricular (LV) mass (182&plusmn;47 g/m&sup2; vs. 138&plusmn;44 g/m&sup2;, p=0.01), reduced global longitudinal strain (GLS) and segmental strain (-10%&plusmn;0.6 vs. -13%&plusmn;0.9, p=0.01), higher left atrial (LA) volume (49&plusmn;10 mL/m&sup2; vs. 39&plusmn;11 mL/m&sup2;, p=0.15) and a higher right ventricle (RV) free wall strain to RV global strain ratio (1.4&plusmn;0.1 vs. 1.2&plusmn;0.1, p=0.01). Apical sparing was present in both groups, with higher prevalence in wtATTR, although the difference was not statistically significant (p=0.07). Comparatively, wtATTR and AL-CM were similar, except for septal thickness, greater in wtATTR (16&plusmn;0.5 mm vs. 13&plusmn;0.8 mm, p=0.02), and LA volume, which was higher in wtATTR (49&plusmn;10 mL/m&sup2; vs. 36&plusmn;9 mL/m&sup2;, p=0.001). The mean time to echo re-evaluation was 21 &plusmn; 1.6 months, during which all patients with ATTR were on tafamidis 61mg and all AL-CM patients were treated with standard of care. In the wtATTR group, re-evaluation demonstrated a significant reduction in LV mass (mean decrease of 26&plusmn;3 g/m&sup2;, p=0.023) and a worsening in GLS (mean reduction of 1.2% &plusmn; 1.3, p=0.01). There were no other statistically significant changes. Patients with hATTR, had significant improvement in the&nbsp;E/e&rsquo; ratios (mean reduction of 3.7&plusmn;1.3, p=0.01). Among AL-CM no significant changes were observed.&nbsp;&nbsp;</span></span></span></p>

<p style="text-align:justify"><span style="font-size:18px"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong>Conclusion:&nbsp;</strong></span></span></span></p>

<p style="text-align:justify"><span style="font-size:18px"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">Echo remains a challenge in differentiating cardiac amyloidosis subtypes. In this cohort, wtATTR showed more advanced features at diagnosis, likely due to the higher time from symptom onset to diagnosis when compared to the other CA types. At two years, treatment seems to lead to stabilization of most echo parameters but, strikingly, in patients with wtATTR LV mass reduction was observed.</span></span></span></p>

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