Evolución a mediano plazo de pacientes con diagnóstico de cardiopatía amiloidótica por transtiretina / Mid-term Outcome of Patients with Diagnosis of Transthyretin Cardiac Amyloidosis
El compromiso cardíaco es la principal causa de morbimortalidad en la amiloidosis, independientemente de la patogenia productora del amiloide subyacente. La amiloidosis por transtiretina (TTR) es una de las variantes más frecuentes, por lo cual el objetivo de este trabajo fue evaluar las características de una cohorte de pacientes con diagnóstico de cardiopatía amiloidótica por TTR (CA-TTR) Material y
El seguimiento de nuestros pacientes con CA-TTR expresa los cambios que ha sufrido el proceso diagnóstico, con una reducción de estudios invasivos y tiempo para la caracterización. El diagnóstico de pacientes en etapas "tempranas" de la enfermedad parece impactar en los resultados a mediano plazo.
The aim of this study was thus to assess the characteristics of a cohort of patients with diagnosis of TTR cardiac amyloidosis (ATTR-CA).
Methods:
Baseline data and diagnostic and follow-up methodology were collected from 49 patients treated at the cardiomy-opathy 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-CA 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.