Distribution and prognostic implications of right and left ventricular systolic dysfunction in wild-type transthyretin amyloid cardiomyopathy
We aimed to characterize the prevalence and severity of left ventricular (LV) and right ventricular (RV) systolic dysfunction and their prognostic implications in wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM). We enrolled 256 consecutive contemporary ATTRwt-CM patients. LV systolic function was assessed by LV ejection fraction (LVEF), global longitudinal strain (LV-GLS), and stroke volume index (SVi), while RV systolic function was assessed using Tricuspid Annular Plane Systolic Excursion (TAPSE). At ATTRwt-CM diagnosis time, median LVEF was 47.5% (Q1-Q3: 40.7%-54.3%). Preserved LVEF (≥ 50%) was observed in 43.4% of patients, mildly-reduced LVEF (40–49%) in 34.4%, and reduced LVEF (< 40%) in 22.3%. LV-GLS (absolute value: 11.9 ± 3.3%) and SVi (28.6 mL/m²; Q1–Q3: 23.3–34.4) were moderately reduced. Among patients with preserved LVEF, 90% had impaired LV-GLS and 57% reduced SVi. Impaired LVEF (< 50%) was associated with higher all-cause mortality compared with preserved LVEF (adjusted hazard ratio [aHR] 2.0; 95% CI: 1.26–3.17). TAPSE was 17.7 ± 5.1 mm and demonstrated an independent association with mortality (aHR 0.92; 95% CI: 0.88–0.96). One-third of patients had preserved LVEF and TAPSE (≥ 18 mm), showing the most favorable prognosis compared with patients with impaired LVEF, TAPSE, or both. LV and RV systolic dysfunction are common at the time of ATTRwt-CM diagnosis and span a broad spectrum of severity. LV-GLS and SVi are frequently impaired despite preserved LVEF, highlighting the need for a multiparametric assessment of LV function. Incorporating TAPSE for RV evaluation may further refine risk stratification, particularly when LVEF is preserved.
Authors
- Henrik Vase
- Tor Skibsted Clemmensen (ORCID: https://orcid.org/0000-0002-6042-6404)
- Mads J. Andersen (ORCID: https://orcid.org/0000-0002-9320-8227)
- Bertil Ladefoged (ORCID: https://orcid.org/0000-0002-3749-6789)
- Sie Kronborg Fensman (ORCID: https://orcid.org/0000-0003-1726-7652)
- Anders Lehmann Dahl Pedersen (ORCID: https://orcid.org/0000-0002-3258-5192)
- Ali Hussein Jaber Mejren (ORCID: https://orcid.org/0000-0001-7727-0373)
- Steen Hvitfeldt Poulsen
- Marish I. F. J. Oerlemans
Institutions
- Aarhus University (DK)
- Aarhus University Hospital (DK)
- University Medical Center Utrecht (NL)
- ERN GUARD-Heart (NL)
Publication Details
- Journal
- The International Journal of Cardiovascular Imaging
- Published
- 2026-01-19
- DOI
- https://doi.org/10.1007/s10554-026-03614-y
- Primary Topic
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- Aarhus Universitet