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

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.