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

Institutions

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

Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Distribution and prognostic implications of right and left ventricular systolic dysfunction in wild-type transthyretin amyloid cardiomyopathy

Henrik Vase, Tor Skibsted Clemmensen, Mads J. Andersen, Bertil Ladefoged et al.
The International Journal of Cardiovascular Imaging
Amyloidosis: Diagnosis, Treatment, Outcomes
article

Distribution and prognostic implications of right and left ventricular systolic dysfunction in wild-type transthyretin amyloid cardiomyopathy

Henrik Vase, Tor Skibsted Clemmensen, Mads J. Andersen, Bertil Ladefoged, Sie Kronborg Fensman, Anders Lehmann Dahl Pedersen, Ali Hussein Jaber Mejren, Steen Hvitfeldt Poulsen, Marish I. F. J. Oerlemans
article en

Abstract

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.

The International Journal of Cardiovascular Imaging
Aarhus University (DK), Aarhus University Hospital (DK), University Medical Center Utrecht (NL), ERN GUARD-Heart (NL)
Aarhus Universitet
Good health and well-being
Openalex Percentile: Top 85%
Amyloidosis: Diagnosis, Treatment, Outcomes
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.