Lo scompenso cardiaco nella cardiomiopatia amiloidotica: uno studio emodinamico all'interno delle tre principali forme eziologiche.

Introduction. In amyloidotic cardiomyopathy (AC), heart failure (HF) is one of the main clinical manifestations. However, a precise pathophysiological and prognostic characterization of HF in this condition is not available. We assessed the clinical and instrumental profile and outcome of patients with advanced HF (i.e. NYHA class III-IV) at the time of first evaluation in light-chain (AL), hereditary transthyretin-related (m-ATTR) and non-mutant transthyretin-related (wt-ATTR) AC. Methods. We analysed the 340 patients diagnosed with AC (145 AL, 119 m-ATTR, 76 wt-ATTR) at our Centre between 1990 and 2015. We evaluated clinical, ECG, echocardiographic and hemodynamic profiles as well as survival data of those with advanced HF at time of diagnosis. Results. 96 (28%) patients presented advanced HF at first evaluation (52 AL, 22 m-ATTR, 22 wt-ATTR). Left ventricle ejection fraction (LVEF) ranged between 22 and 67% and was <50% in 54 patients (52%). Increased pulmonary capillary wedge pressure (PCWP) and reduced cardiac index (CI) were documented in more than 70%. 45% of patients showed reduced LVEF with increased PCWP, while in about 1/3 of cases a normal LVEF and increased PCWP were present. 10 patients with HF at presentation had normal LVEF and PCWP. During follow-up 66 (27%) developed HF: 29 AL, 23 wt-ATTR, 14 m-ATTR with incidence rate of 6.2% person/years. Survival was reduced in patients with HF both in overall population and in three main subgroups. AL AC and reduced CI were independent predictors of mortality in patients in NYHA class III-IV at presentation. Conclusion. In 30% of AC patients, HF is one of main manifestation at first evaluation. The pathophysiological substrate of HF in these patients is systolic and/or diastolic dysfunction. Survival was reduced in AC patients with HF at presentation. AL etiology and CI were indipendent predictors of outcome.