Wild-type TTR amyloidosis among patients with unexplained heart failure and systolic LV dysfunction.
<h4>Aim</h4>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 failur...
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2021
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oai:doaj.org-article:70ec845e9cab478ea1c7e82ef99c724a2021-12-02T20:09:22ZWild-type TTR amyloidosis among patients with unexplained heart failure and systolic LV dysfunction.1932-620310.1371/journal.pone.0254104https://doaj.org/article/70ec845e9cab478ea1c7e82ef99c724a2021-01-01T00:00:00Zhttps://doi.org/10.1371/journal.pone.0254104https://doaj.org/toc/1932-6203<h4>Aim</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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.<h4>Conclusion</h4>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.Sorel GolandIgor VolodarskyYacov FabricantShay LivschitzSagi TshoriValeri CuciucLiaz ZilbermanIrena FugenfirovValeri MeledinSara ShimoniSagie JosfbergJacob GeorgePublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 16, Iss 7, p e0254104 (2021) |
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| topic |
Medicine R Science Q |
| spellingShingle |
Medicine R Science Q Sorel Goland Igor Volodarsky Yacov Fabricant Shay Livschitz Sagi Tshori Valeri Cuciuc Liaz Zilberman Irena Fugenfirov Valeri Meledin Sara Shimoni Sagie Josfberg Jacob George Wild-type TTR amyloidosis among patients with unexplained heart failure and systolic LV dysfunction. |
| description |
<h4>Aim</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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.<h4>Conclusion</h4>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. |
| format |
article |
| author |
Sorel Goland Igor Volodarsky Yacov Fabricant Shay Livschitz Sagi Tshori Valeri Cuciuc Liaz Zilberman Irena Fugenfirov Valeri Meledin Sara Shimoni Sagie Josfberg Jacob George |
| author_facet |
Sorel Goland Igor Volodarsky Yacov Fabricant Shay Livschitz Sagi Tshori Valeri Cuciuc Liaz Zilberman Irena Fugenfirov Valeri Meledin Sara Shimoni Sagie Josfberg Jacob George |
| author_sort |
Sorel Goland |
| title |
Wild-type TTR amyloidosis among patients with unexplained heart failure and systolic LV dysfunction. |
| title_short |
Wild-type TTR amyloidosis among patients with unexplained heart failure and systolic LV dysfunction. |
| title_full |
Wild-type TTR amyloidosis among patients with unexplained heart failure and systolic LV dysfunction. |
| title_fullStr |
Wild-type TTR amyloidosis among patients with unexplained heart failure and systolic LV dysfunction. |
| title_full_unstemmed |
Wild-type TTR amyloidosis among patients with unexplained heart failure and systolic LV dysfunction. |
| title_sort |
wild-type ttr amyloidosis among patients with unexplained heart failure and systolic lv dysfunction. |
| publisher |
Public Library of Science (PLoS) |
| publishDate |
2021 |
| url |
https://doaj.org/article/70ec845e9cab478ea1c7e82ef99c724a |
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