Wild-type TTR amyloidosis among patients with unexplained heart failure and systolic LV dysfunction

Abstract

Aim Transthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized cause of heart failure (HF) with preserved left ventricular ejection fraction (LVEF), typically presenting as restrictive cardiomyopathy. The potential co-existence of ATTR-CA with systolic heart failure has not been studied. The aim of this study is to describe the prevalence of ATTR-CA and its clinical characteristics in HF patients with reduced LVEF. Methods Patients with an unexplained cause of LV systolic dysfunction were screened for ATTR-CA by a 99mTc-PYP planar scintigraphy. Patients in whom presence of ≥ 2 uptake was confirmed by SPECT imaging were included. Their clinical, laboratory and echocardiographic data were collected. Results Out of 75 patients (mean age 65±12 years, LVEF 35.8±7.9%) included in this study, 7 (9.3%) patients (mean age 75±6 years, LVEF 32.0±8.3%) had ATTR-CA. Patients with ATTR-CA were more symptomatic at diagnosis (NYHA FC 3–4 (86% vs 35% (p = 0.03)) and had a more severe clinical course evident by recurrent hospitalizations for HF, and a need for intravenous diuretic treatment (p = 0.04 and p<0.01, respectively) at follow-up, compared with patients with no ATTR-CA. Patients with ATTR-CA had similar LVEF but a clear trend for larger LV mass index (157.1±60.6 g/m2 vs. 121.0±39.5 g/m2, p = 0.07) and a larger proportions of ATTR-CA patients had IVS thickness >13 mm (57.1% vs 13.1%, p = 0.02) as compared to HF patients with no ATTR-CA. Conclusion In our study, a meaningful percentage of patients with unexplained LV dysfunction had a co-existing ATTR-CA indicating that the clinical heterogeneity of ATTR-CA is much broader than previously thought.

Prevalence and clinical outcomes of transthyretin amyloidosis: a systematic review and meta‐analysis

“…is provided in Table 1 8–25,34–77 . A graphical representation of the prevalence of cardiac amyloidosis across various populations subgroups is provided in Figure (and online supplementary Figure – focused version).…”

Re-Definition of the Epidemiology of Cardiac Amyloidosis

“…Specific clinical settings have been recognized as strongly associated with CA but further studies are needed to better characterize these populations, mainly to stratify patient risk and evaluate which patients are ideal candidates for the newly available disease-modifying therapies. Acknowledgments: We would like to thank all the nuclear medicine doctors, hematologists, neurologists, pathologists and nephrologists of the participating centres for providing their essential [8,[13][14][15][16]20,27,28,33,[36][37][38][39]41,42,[46][47][48][49][50][51][54][55][56].…”

“…Prevalence of ATTR-CA was also evaluated in patients with HFrEF/HFmrEF; Lopez-Sainz et al reported a prevalence of approximately 11% in HFrEF/HFmrEF patients [ 41 ], while Goland et al found ATTR-CA in 9.3% of 75 HF patients with reduced LVEF [ 42 ] ( Table 1 ).…”

“… Prevalence of CA in different screening studies [ 8 , 13 , 14 , 15 , 16 , 20 , 27 , 28 , 33 , 36 , 37 , 38 , 39 , 41 , 42 , 46 , 47 , 48 , 49 , 50 , 51 , 54 , 55 , 56 ]. …”

“…Figure 1. Prevalence of CA in different screening studies[8,[13][14][15][16]20,27,28,33,[36][37][38][39]41,42,[46][47][48][49][50][51][54][55][56].…”