Abstract:

Background: The introduction of a non-invasive diagnostic algorithm in 2016 led to increased awareness and recognition of cardiac amyloidosis (CA).

Objectives: To analyze the impact of the introduction of the non-invasive diagnostic algorithm on diagnosis and prognosis in a multicenter Italian CA cohort.

Methods: This was a retrospective analysis of 887 CA patients from 5 Italian Cardiomyopathies Referral Centres: 311 light-chain CA (AL), 87 variant transthyretin (TTR)-related CA (ATTRv), 489 wild type TTR-related CA (ATTRwt). Clinical characteristics and outcomes (all-cause mortality and heart failure [HF] hospitalizations) were compared overall and for each CA subtype between patients diagnosed before versus after 2016. Outcomes were further compared by propensity score weighted (PSW) Kaplan-Meier analysis and Cox regression analysis.

Results: CA diagnoses increased after 2016, in particular for ATTRwt. Patients diagnosed after versus before 2016 were older, had less frequently a history of HF prior to diagnosis and NYHA class III-IV at diagnosis. Over a median follow-up of 18 months, 172 (86%) patients diagnosed before 2016 died or had an HF hospitalization, versus 300 (44%) diagnosed after 2016. PSW Kaplan-Meier analysis showed worse outcomes (p<0.001) for patients diagnosed before 2016. At Cox regression analysis, CA diagnosis after 2016 was an independent protective factor for the composite outcome (HR: 0.69, p=0.001), with interaction by CA subtype (significant in TTR-related CA and null in AL).

Conclusion: CA patients diagnosed after 2016 showed a less severe phenotype and a better prognosis. The impact of the non-invasive diagnostic algorithm on outcomes was particularly relevant in TTR-related CA.

JACC: Advances Editor-in-Chief 

Candice K. Silversides, MD, FACC

JACC: Advances CME Editor

Kenneth A. Ellenbogen, MD

Author

Stephen Clarkson, MD

Important Dates

Date of Release: October 23, 2024 

Term of Approval/Date of CME/MOC Expiration: October 22, 2025