Cardiovascular prognosis in stable patients with amyloidosis: a novel and simple risk score.

Abstract Background Amyloidosis is a systemic disease characterized by deposition of amyloid fibrils in the intercellular space. Cardiac affectation is presented among 50%. The aim of this study was to provide the development and validation of a risk stratification score in order to identify patients with cardiac amyloidosis and a high risk of hospitalization to heart failure at intermediate follow-up. Methods We recruited 100 patients, diagnosed of cardiac amyloidosis between 2018 and 2021, prospectively recruited from three tertiary hospitals. We collected at start clinical condition and we performed an electrocardiogram and transthoracic echocardiogram. We followed up all patients prospectively (at 6,12, 24 and 36 months). Results Of all patients 31% were light chain amyloidosis (AL), 36% were wild-type transthyretin (ATTRwt) and 33% were genetic transthyretin (ATTRm). At the presentation, a significant proportion of patients were only mildly symptomatic but during the follow-up the 55% of patients have presented heart failure hospitalization, and the 30% died to heart failure decompensation. With the Univariate Cox regression analysis significant predictors for HF-hospitalization were the presence of a low voltage and pseudoinfarct pattern on the ECG and the thickness of interventricular septum and the posterior wall, the LVEF and the pseudonormal pattern of LV diastolic filling on the echocardiography. In the multiple Cox regression model remained significant the low voltage, the LVEF and the interventricular septum thickness. Were considered eligible for the final inclusion into the score: low voltage on the ECG, LVEF ≤40% and interventricular septum (SIV) ≥ 14 mm. All of these variables received a specified score ranging from 0 to 4. A range score 3-4 was related to 80% probability of HF-hospitalization, while a final score ranging between 1 and 2 was related to 50% of probability. Kaplan–Meier analysis showed a significantly higher probability of secondary endpoints (HF-related mortality and all-cause mortality) over time in the patients with a high score. At three years of follow-up the 84% of the patients with a high score goes to HF hospitalization, the 53%goes to HF death and the 68% goes to all-cause death. Conclusions The current score is a simple and rapid score that used in daily clinical practice, can really improve the prognostic information in patient with cardiac amyloidosis. Its importance depends on being able to predict, thanks to standard cardiological instrumental examinations, such as ECG and echocardiogram, the prognosis of these patients at intermediate term follow-up. It can be used in stable patients at the time of observation, and for this reason its role has a very important value for the clinician on the choice of the correct therapy and timing of follow-up in order to prevent the onset of cardiac events.Kaplan–Meier analysis for the HF Hospita Kaplan–Meier analysis for the HF death