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Peer-reviewedCC BY-NCHeart

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

Abstract

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.

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BibTeX
@article{belaidi2026cardiac,
  title = {{Cardiac amyloidosis across the spectrum of left ventricular function: multimodal functional and prognostic insights}},
  author = {Sabrina Belaidi and Mounira Kharoubi and Olivier Lairez and Erwan Donal and Silvia Oghina and Safaa Mehenni and Shirley Odouard and Denis Tixier and Emmanuel Teiger and Amaury Broussier and Thibaud Damy and Amira Zaroui},
  journal = {Heart},
  year = {2026},
  month = oct,
  doi = {10.1136/heartjnl-2025-327260},
  url = {https://hal.science/hal-05830083v1},
  note = {CC BY-NC},
}
RIS
TY  - JOUR
TI  - Cardiac amyloidosis across the spectrum of left ventricular function: multimodal functional and prognostic insights
AU  - Sabrina Belaidi
AU  - Mounira Kharoubi
AU  - Olivier Lairez
AU  - Erwan Donal
AU  - Silvia Oghina
AU  - Safaa Mehenni
AU  - Shirley Odouard
AU  - Denis Tixier
AU  - Emmanuel Teiger
AU  - Amaury Broussier
AU  - Thibaud Damy
AU  - Amira Zaroui
PY  - 2026
DA  - 2026/10/21
JO  - Heart
DO  - 10.1136/heartjnl-2025-327260
UR  - https://hal.science/hal-05830083v1
AB  - 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.
SN  - 1355-6037
SN  - 1468-201X
ER  -