Prognostic value of different echocardiographic and cardiac magnetic resonance parameters in patients with AL amyloidosis

Abstract

Abstract Introduction Light chain amyloidosis (AL) is a systemic disease, highlighting its infiltration at the myocardium. Recent advances in imaging have increased the suspicion of cardiac involvement. Current risk stratification models are based exclusively on serum biomarkers. We studied the prognostic value of echocardiographic and cardiac magnetic resonance (cMRI) parameters in a cohort of patients with a confirmed diagnosis of AL amyloidosis. Methods Retrospective observational study of consecutive patients diagnosed with AL amyloidosis by the Hematology Department in our center between 1999-2022. All had to have quantification of biomarkers (free light chains -dFLC-, NTpro-BNP and troponin T -TnT-) and transthoracic echocardiography (TTE) at diagnosis, collecting clinical and epidemiological characteristics and complementary tests. Results Of a total of 71 diagnoses of AL amyloidosis, 39 patients who met the inclusion criteria were selected, most diagnosed in the last 5 years. The mean age was 65.2 ± 12.9 years old, 56.4% male. The sample was divided according to the Mayo Clinic risk stratification model, into low (I-II, 33%) and high (III-IV, 67%) risk stages. Cardiac imaging variables were analyzed using Bootstrap statistics increasing the database to 150 patients (Table 1). Among echocardiographic variables, patients in the high-risk groups had greater systolic dysfunction of both ventricles, diastolic dysfunction, myocardial thickness, and pericardial effusion was observed more frequently. Among patients with cMRI (53.8%), we highlight the presence of an interventricular septal thickness ≥12 mm (47.6%) and a late gadolinium enhancement (LGE) in the left ventricle (66.6%, LV), most with a diffuse subendocardial pattern. In our cohort, the presence of LGE, greater maximum LV thickness, and increased LV extracellular volume (ECV) were significantly associated with the Mayo Clinic high-risk stages groups. Finally, a global longitudinal strain > -11.5% by TTE, and a T1 mapping > 1042 ms or ECV > 49% by cMRI, were significantly associated with a reduction in overall survival (Figure 1). Conclusions There are cardiac imaging variables that have prognostic implications and could have a role in risk stratification of patients with AL amyloidosis. Its inclusion in new risk stratification models along with serum biomarkers should be considered.Table 1Figure 1