Cardiac amyloidosis across the spectrum of left ventricular function: multimodal functional and prognostic insights.

BACKGROUND

Although cardiac amyloidosis (CA) is often considered to be a cause of heart failure with preserved ejection fraction (HFpEF), many patients present with mildly reduced (HFmrEF) or reduced ejection fraction (HFrEF). Recognising CA across this spectrum is essential for diagnosis and risk stratification.

METHODS

We studied 2244 patients with CA (557 light chain amyloidosis, 392 hereditary transthyretin amyloidosis, 1137 wild-type transthyretin amyloidosis) at the French national reference centre. Left ventricular ejection fraction (LVEF) was classified according to European Society of Cardiology guidelines. We evaluated the prognostic relevance of LVEF and its interaction with global longitudinal strain (GLS) and cardiac index (CIx). Survival was assessed with a Kaplan-Meier analysis, and a decision tree combined LVEF, GLS and CIx. Our findings were confirmed externally in an independent, French validation cohort.

RESULTS

Although HFpEF was the most common phenotype, 39% of patients presented with HFmrEF or HFrEF. The survival time varied with the phenotype; the median was 30 months in HFrEF, 40 months in HFmrEF and was not reached in HFpEF. LVEF correlated moderately with GLS and weakly with CIx. A decision tree integrating LVEF, GLS and CIx identified four prognostic groups with HRs for 4-year mortality ranging from 1.6 to 3.7.

CONCLUSIONS

CA affects the full spectrum of LVEF phenotypes. The integration of LVEF, GLS and CIx improves prognostic stratification and argues in favour of a multimodal imaging approach for early diagnosis and individualised management.