Cardiovascular Prognosis in Stable Patients with Cardiac Amyloidosis: A Novel and Simple Risk Score

TL;DR

This study creates a simple risk score to predict heart failure hospitalizations in patients with cardiac amyloidosis using common tests.

Contribution

A novel, non-invasive risk score (CAMY-HF) is developed for predicting heart failure hospitalization in cardiac amyloidosis patients.

Findings

Low QRS voltage, thickened heart wall, and reduced heart function independently predicted hospitalization.

The CAMY-HF score effectively stratified patients into low, intermediate, and high-risk groups for hospitalization.

Higher CAMY-HF scores correlated with increased mortality from heart failure and all causes.

Abstract

Background: Cardiac amyloidosis (CA) is frequently diagnosed in clinically stable patients, yet the risk of subsequent heart failure (HF) hospitalization remains difficult to predict using readily available tools. Early identification of high-risk outpatients is crucial to optimize follow-up and therapeutic strategies. Purpose: To develop a simple, non-invasive risk score to predict HF hospitalization in stable patients with cardiac amyloidosis using standard electrocardiographic and echocardiographic parameters. Methods: We prospectively enrolled 100 consecutive patients with confirmed cardiac amyloidosis from three tertiary centers. Baseline evaluation included clinical assessment, electrocardiography, and transthoracic echocardiography. The primary endpoint was HF hospitalization during follow-up; secondary endpoints were HF-related and all-cause mortality. Cox regression analysis was used to identify independent predictors of HF hospitalization and to derive a point-based risk (CAMY-HF) score. Results: During a median follow-up of 36 months, 55% of patients required HF hospitalization and 47% died. Low QRS voltage, interventricular septal thickness ≥14 mm, and left ventricular ejection fraction ≤40% independently predicted HF hospitalization and were incorporated into the CAMY-HF score (range, 0–4). HF hospitalization occurred in 0% of low-risk, 47.9% of intermediate-risk, and 80.6% of high-risk patients at 3 years. Higher CAMY-HF scores were also associated with increased HF-related and all-cause mortality. Conclusions: The CAMY-HF score is a simple, widely applicable tool for early risk stratification in clinically stable patients with cardiac amyloidosis. By relying on routine ECG and echocardiographic parameters, it may help identify outpatients at high risk of HF hospitalization and guide follow-up intensity and management strategies.

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