Mid-term Outcome of Patients with Diagnosis of Transthyretin Cardiac Amyloidosis

pp. 410-414

Abstract

Background: Cardiac involvement is the main cause of morbidity and mortality in amyloidosis, regardless of the underlying pathogenesis of amyloid production, and transthyretin (TTR) amyloidosis is one of the most frequent variants.

Objective: The aim of this study was thus to assess the characteristics of a cohort of patients with diagnosis of TTR cardiac amyloidosis (ATTR-CM).

Methods: Baseline data and diagnostic and follow-up methodology were collected from 49 patients treated at the cardiomyopathy clinic of our institution.

Results: Median follow-up was 1,258 days (410-2004). Mean age was 79±9 years, and 57% of patients were in functional class (FC) I, 26% in FC II and 16% in FC III-IV at follow-up onset. Diagnosis was made with diphosphonate scintigraphy in 92% of patients and 24% required a biopsy. Overall mortality was 19%, with 15% of cardiovascular death. The rate of hospitalization for heart failure was 29% and 63% of patients worsened their FC.

Conclusions: Follow-up of patients with ATTR-CM expresses the changes undergone by the diagnostic process, with a reduction of invasive studies and time to characterization. The diagnosis of patients at “early stages of the disease” seems to have an impact on mid-term outcomes.