MULTIMODALITY IMAGING IN CARDIAC AMYLOIDOSIS: FROM HISTOPATHOLOGICAL BASIS TO RISK STRATIFICATION

1. The present clinicopathological study demonstrated that amyloid is variably distributed in the heart of patients affected by CA, with vascular involvement being more frequent in AL-CA. The amyloid burden at histology was significantly greater in the basal and mid-ventricular levels compared with the apex and in the subendocardial layers compared with the subepicardial ones (i.e. longitudinal and transmural gradients). A direct correlation between segmental amyloid burden and LS in each segment is evident. The RELAPS phenomenon at echocardiography is present in nearly half of cases and is not always explained by a base-to-apex gradient of amyloid burden at histopathology, suggesting that RELAPS might be an epiphenomenon of complex interactions among amyloid infiltration, myocardial structure, and adaptation. 2. In patients with AL-CA and ATTR-CA, IABs are common and together with advanced age and reduced LAEF on CMR are independent predictors of incident AF. Patients with these features might benefit from closer AF screening strategies during follow-up. 3. In a modern cohort of patients with wtATTR-CM, RV-PA uncoupling emerged as an early and strong predictor of outcome, being independently associated with the risk of HF hospitalisation or all – cause death. No differences in risk prediction were observed among M-mode and strain-based RV function parameters. The evaluation of RV-PA uncoupling should be considered in the clinical practice for risk stratification and prognosis assessment of patients with wtATTR-CM, with potential implications treatment strategies definition. 4. In summary, within 12 months of starting tafamidis, 9% of patients experienced HF hospitalization or death, and up to one-third exhibited markers of disease progression. Baseline NAC/Mondor stage III and a high daily loop diuretic dose were independently associated with adverse outcomes. Using a 12-month landmark analysis, we demonstrated that clinical and biochemical worsening over time was associated with subsequent events. Two progression-based models effectively identified high-risk patients may help guide treatment decisions in clinical practice as well as serve as potential endpoints for future clinical trials.