ライフサイエンスコーパス: ATTR

1 ATTR amyloidosis causes cardiomyopathy in up to approxim

2 ATTR amyloidosis genotypes and phenotypes are highly het

3 ATTR amyloidosis is caused by the deposition of transthy

4 ATTR amyloidosis is debilitating and associated with poo

5 ATTR amyloidosis results from misfolded TTR protein depo

6 ATTR are caused by aggregation of transthyretin (TTR), a

7 ATTR can occur in association with normal TTR genetic se

8 ATTR can result from substitution of valine for isoleuci

9 ATTR is a progressive disease resulting from the deposit

10 ATTR was diagnosed by DPD and absence of monoclonal prot

11 (ATTR-ACT; ATTRIBUTE-CM [Efficacy and Safety of AG10 in S

12 ATTR-CA was determined by radionuclide imaging, with blo

13 ATTR-CM and non-ATTR-CM cohorts were compared.

14 ATTR-CM is an inexorably progressive and eventually fata

15 ATTR-CM testing positivity was compared with historical

16 ATTR-CM, New York Heart Association functional class II

17 ATTR-specific medication was independently associated wi

18 ATTR-specific treatment and AVR both result in significa

19 subjects with autopsy-documented AL (n=10), ATTR (n=10), and nonamyloid controls (n=10) using (18)F-

20 hypertrophic cardiomyopathy, 95 AL, and 116 ATTR) from 56 institutions were included (269 men aged 5

21 Forty-five subjects (12 AL, 16 ATTR wild type, and 17 ATTR mutants) underwent (99m)Tc-P

22 e subjects (12 AL, 16 ATTR wild type, and 17 ATTR mutants) underwent (99m)Tc-PYP planar and single-ph

23 ign and rationale behind the ongoing phase 3 ATTR-ACT study (Tafamidis in Transthyretin Cardiomyopath

24 olume of 0.40 to 0.43 (AL) and 0.39 to 0.40 (ATTR) and to transmural at 0.48 to 0.55 (AL) and 0.47 to

25 mural at 0.48 to 0.55 (AL) and 0.47 to 0.59 (ATTR).

26 hs (20-67), systemic AL: 23.5 months (0-95), ATTR amyloidosis: 17 months, and AA, 15 months (0-77).

27 Enthusiasm about ATTR-CM has grown as a result of 3 simultaneous areas of

28 pite available therapeutic options, advanced ATTR amyloidosis still presents unmet medical needs.

29 nd associations between any and incident AF, ATTR-CA, and all-cause mortality.

30 Adjusting for age, ATTR-CM prevalence differed by sex, with 15 of 149 men (

31 iers with HF had ATTR-CA, while 55.8% of all ATTR-CA cases had normal TTR genotype.

32 Hereditary transthyretin amyloid (ATTR) amyloidosis is a rare but fatal autosomal dominant

33 ular pattern, whereas transthyretin amyloid (ATTR) more commonly showed patchy deposits.

34 m transthyretin-related cardiac amyloidoses (ATTR) is imperative given implications for prognosis, th

35 The transthyretin amyloidoses (ATTR) are invariably fatal diseases characterized by pro

36 Transthyretin cardiac amyloidosis (ATTR) is an underrecognized cause of heart failure (HF)

37 (AS) and transthyretin cardiac amyloidosis (ATTR) is increasingly recognized.

38 ransthyretin-associated cardiac amyloidosis (ATTR-CA) is an increasingly recognized cause of structur

39 reness of transthyretin cardiac amyloidosis (ATTR-CA).

40 criminate transthyretin cardiac amyloidosis (ATTR-CM) from other causes of increased left ventricular

41 , 10%), wild-type transthyretin amyloidosis (ATTR) (N = 1, 3.3%), and amyloid of uncertain type (N =

42 Transthyretin amyloidosis (ATTR) is a heterogeneous disorder with multiorgan involv

43 ve suggested that transthyretin amyloidosis (ATTR) is a more common cause of heart failure (HF) than

44 Hereditary transthyretin amyloidosis (ATTR) is an autosomal dominant disease characterized by

45 Cardiac transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failu

46 Familial transthyretin amyloidosis (ATTR) is associated with extracellular deposition of wil

47 Hereditary transthyretin amyloidosis (ATTR) is usually characterised by a progressive peripher

48 ed for hereditary transthyretin amyloidosis (ATTR) since 1990.

49 ted with familial transthyretin amyloidosis (ATTR), a hereditary degenerative disease.

50 Lkappa, ALlambda, transthyretin amyloidosis (ATTR), and Abeta amyloid deposits in tissue sections.

51 manifestation of transthyretin amyloidosis (ATTR), which is an underrecognized systemic disease wher

52 loidosis (AL) and transthyretin amyloidosis (ATTR).

53 ession of cardiac transthyretin amyloidosis (ATTR-CA) remains insufficiently understood.

54 c monomers, which underlies TTR amyloidosis (ATTR) pathophysiology.

55 hereditary transthyretin (TTR) amyloidosis (ATTR).

56 An ATTR-directed therapy that inhibited misfolding of the T

57 .83-11.02), AT 1.04 (95% CI, 0.73-1.50), and ATTR 1.71 (95% CI, 1.21-2.41).

58 to study systemic amyloidosis of type AL and ATTR affecting the heart and should be investigated furt

59 apir binds specifically to myocardial AL and ATTR amyloid deposits.

60 um of cardiac involvement in systemic AL and ATTR amyloidosis.

61 apir specifically binds to myocardial AL and ATTR deposits in humans and offers the potential to scre

62 F-florbetapir uptake was noted in all AL and ATTR samples and in none of the control samples.

63 nt survival benefit in dual pathology AS and ATTR-CA.

64 Functional impairment in ATTR-CM and ATTR-mixed phenotypes (peak mean [SD] oxygen consumption

65 gnosed by transthoracic echocardiography and ATTR-CA by myocardial uptake on bone scintigraphy and/or

66 A total of 2,557 patients with HF and ATTR-CM were identified.

67 n time between initial loop prescription and ATTR-CM diagnosis was 835 days (Q1-Q3: 250-1850).

68 irmed as AL type (AL-CA) in 276 (97%) and as ATTR-CM in only one case with an extremely rare TTR vari

69 sthyretin cardiac amyloidosis (also known as ATTR cardiac amyloidosis) is an increasingly recognized

70 , biomarkers, and myocardial mechanics in AS-ATTR following valve replacement is unknown.

71 strain showed a base-to-apex gradient in AS-ATTR, whereas all but apical segments improved in lone A

72 After AS treatment, AS-ATTR transfers into a lone ATTR cardiomyopathy phenotype

73 This population-based cohort study assessed ATTR-CM prevalence in 1235 consecutive patients in south

74 Asymptomatic ATTR cardiac amyloid infiltration.

75 ans with a loop diuretic prescription before ATTR-CM diagnosis, the median time between initial loop

76 4x10(-17)) as well as an association between ATTR amyloidosis and RBP4 levels in community-dwelling i

77 4x10(-17)) as well as an association between ATTR amyloidosis and RBP4 levels in community-dwelling i

78 Recently, a relationship between ATTR-fibril composition and cardiomyopathy has been note

79 myopathy [ATTR-CM], polyneuropathy, or both [ATTR-mixed]), differences in CPET parameters based on de

80 Transthyretin-CA (ATTR) was diagnosed by DPD and absence of a clonal immun

81 rder of first presentation, they are CABANA, ATTR-ACT, COAPT, DECLARE, REDUCE-IT, and AUGUSTUS.

82 Cardiac ATTR amyloidosis, a serious but much under-diagnosed for

83 >99% sensitive and 86% specific for cardiac ATTR amyloid, with false positives almost exclusively fr

84 ty and positive predictive value for cardiac ATTR amyloidosis of 100% (positive predictive value conf

85 noninvasive diagnostic criteria for cardiac ATTR amyloidosis that are applicable to the majority of

86 maging modality for the diagnosis of cardiac ATTR amyloidosis has lately been revisited.

87 cintigraphy enables the diagnosis of cardiac ATTR amyloidosis to be made reliably without the need fo

88 t the generation of a mouse model of cardiac ATTR amyloidosis with transgenic expression of human TTR

89 d) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 (99m)Tc-DPD), and 12 asymptomatic individu

90 Subjects comprised 263 patients with cardiac ATTR corroborated by grade 2 to 3 (99m)Tc-DPD ((99m)Tc-3

91 ing was typical in all patients with cardiac ATTR.

92 Transthyretin amyloid cardiomyopathy (ATTR-CM) is an under-recognized cause of heart failure (

93 ground Transthyretin amyloid cardiomyopathy (ATTR-CM) often coexists with severe aortic stenosis (AS)

94 Transthyretin amyloid cardiomyopathy (ATTR-CM) results in a restrictive cardiomyopathy caused

95 sis of transthyretin amyloid cardiomyopathy (ATTR-CM).

96 Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is an increasingly recognized cause of heart fa

97 or transthyretin amyloidosis cardiomyopathy (ATTR-CM) that mimics a protective TTR mutation.

98 or transthyretin amyloidosis cardiomyopathy (ATTR-CM), N-terminal prohormone of B-type natriuretic pe

99 btype (transthyretin amyloid cardiomyopathy [ATTR-CM]), the role of heart failure medications remains

100 isease phenotypes (ATTR with cardiomyopathy [ATTR-CM], polyneuropathy, or both [ATTR-mixed]), differe

101 raphy was demonstrated to predict coexisting ATTR-CM, comparable data from four-dimensional (4D) card

102 performance in the detection of concomitant ATTR-CM by assessing LV and left atrial GLS, relative ap

103 rty-four patients with genetically confirmed ATTR were assessed; 37 were symptomatic; mean age at ons

104 015) in patients with suspected or confirmed ATTR-CM (global chi(2) = 6.892, P = 0.02) and an LVEF of

105 ion: In patients with suspected or confirmed ATTR-CM and preserved LVEF, representing an early diseas

106 Data of 2,356 consecutive ATTR-CM patients were analyzed: 260 (11%) received SGLT2

107 here is no available treatment that depletes ATTR from the heart for the amelioration of cardiac dysf

108 Across cohorts and modalities, AI-derived ATTR-CM probabilities from 7352 TTEs and 32 205 ECGs div

109 all thickness score, and Mayo Clinic derived ATTR score (transthyretin cardiac amyloidosis score) wer

110 The goal of this study was to describe ATTR in the United States by using data from the THAOS (

111 s for technetium-99m-pyrophosphate to detect ATTR-CM were analyzed, including longitudinal strain (LS

112 ensitivity and 92% specificity for detecting ATTR cardiac amyloidosis with an area under the curve of

113 m PYP) cardiac imaging noninvasively detects ATTR cardiac amyloidosis, but the accuracy of this techn

114 Deep learning models trained to discriminate ATTR-CM from age/sex-matched controls on TTE videos (AI-

115 clinical parameters accurately discriminated ATTR V122I amyloidosis from nonamyloid HF in a case-matc

116 Considered a rare disease, ATTR amyloidosis may be more prevalent than thought, par

117 A and no MG are strongly suggestive of early ATTR-type, but require urgent histologic corroboration.

118 A grade 0 radionuclide scan all but excludes ATTR-CM but occurs in most patients with AL-CA.

119 ECV was similar in carriers and extracardiac ATTR but rose from early-stage to ATTR-cardiomyopathy (C

120 thotopic liver transplantation for familial (ATTR) amyloidosis, was developed.

121 ielded excellent discriminatory capacity for ATTR V122I amyloidosis (AUC = 0.97; 95% CI, 0.93-1.00),

122 ) had the highest prediction performance for ATTR CA.

123 (11.4%) and 174 (21.6%) tested positive for ATTR-CM, respectively.

124 though noninvasive diagnosis is possible for ATTR cardiac amyloidosis, histological demonstration and

125 escribes these consensus recommendations for ATTR associated with cardiomyopathy as a resource to aid

126 r wall thickness among patients referred for ATTR-CM evaluation.

127 gand, could be used as a diagnostic test for ATTR V122I amyloidosis.

128 New therapies are in testing for ATTR, whereas those for AL have followed multiple myelom

129 ew and very effective specific therapies for ATTR-CM.

130 Because therapy for ATTR-CM may be most effective when administered before s

131 Acoramidis, an approved oral therapy for ATTR-CM, achieves early, near-complete (>=90%) TTR stabi

132 performed to identify optimal thresholds for ATTR V122I amyloidosis identification.

133 PYP cardiac imaging as a diagnostic tool for ATTR cardiac amyloidosis and its association with surviv

134 pone, a potent binder in clinical trials for ATTR, we combined rational design and molecular dynamics

135 Tc-PYP cardiac imaging distinguishes AL from ATTR cardiac amyloidosis and may be a simple, widely ava

136 omyopathy from amyloidosis, and then AL from ATTR.

137 hod was successfully tested using serum from ATTR patients with known variants (Val30-->Met and Val12

138 oximately half of V142I carriers with HF had ATTR-CA, while 55.8% of all ATTR-CA cases had normal TTR

139 (87%) had AL-CA; 60/61 (98%) without MG had ATTR-CM.

140 , pharmaceutical therapy that slows or halts ATTR-CM progression and favorably affects clinical outco

141 f 137 women (2.2%; 95% CI, 0.4%-6.3%) having ATTR-CM (P = .002).

142 d after liver transplantation for hereditary ATTR amyloidosis, although gastrointestinal symptom scor

143 from a destabilizing mutation in hereditary ATTR amyloidosis (hATTR) or from an aging-linked process

144 inal manifestations are common in hereditary ATTR amyloidosis and are important for the patients' mor

145 Swedish patients with hereditary ATTR amyloidosis transplanted between 1990 and 2012 were

146 Twenty-four patients with hereditary ATTR amyloidosis who had undergone LTx and have had thei

147 of NTLA-2001 in six patients with hereditary ATTR amyloidosis with polyneuropathy, three in each of t

148 Excluding patients with hereditary ATTR amyloidosis, 1515 patients with a clinical diagnosi

149 with wild-type ATTR-CM, 205 with hereditary ATTR-CM associated with the V1221 variant (V122I-hATTR-C

150 ts with ATTR than in those with AL; however, ATTR is associated with higher cell volume, which sugges

151 er the curve 0.992, P<0.0001 for identifying ATTR cardiac amyloidosis.

152 nal protein, scintigraphy, or biopsy and, if ATTR associated with cardiomyopathy is identified, TTR g

153 AI-augmented screening may improve ATTR-CM detection and identify patients who are missed b

154 In ATTR amyloidosis, survival correlated with NYHA function

155 acokinetics, and pharmacodynamics of AG10 in ATTR-CM patients with symptomatic, chronic heart failure

156 II symptoms at baseline who were enrolled in ATTR-ACT, a placebo-controlled study of tafamidis held a

157 tium recently convened a group of experts in ATTR amyloidosis who, through an iterative process, agre

158 ulse, with ventilatory efficiency highest in ATTR-CM (mean [SD] ventilatory efficiency/volume of carb

159 Functional impairment in ATTR-CM and ATTR-mixed phenotypes (peak mean [SD] oxygen

160 t needs and areas of active investigation in ATTR-CM.

161 LA reservoir remained significantly lower in ATTR-CA compared to AL-CA (p = 0.03), but not LA booster

162 of contemporary heart failure medication in ATTR-CM.

163 patients have the T60A missense mutation in ATTR where tyrosine is replaced by adenine at position 6

164 iovascular hospitalization rates observed in ATTR-ACT; future projections used a parametric survival

165 ardiac involvement in relation to outcome in ATTR.

166 uced the cumulative burden of CV outcomes in ATTR-CM over 30 months.

167 work for staging and therapeutic planning in ATTR amyloidosis.

168 tions are currently not widely prescribed in ATTR-CA, and those that received medication had more sev

169 le model that detects disease progression in ATTR-CA.

170 A total of 441 patients were randomized in ATTR-ACT, and 436 patients had available echocardiograph

171 mmonest pattern of ventricular remodeling in ATTR.

172 iac amyloid burden and treatment response in ATTR-CM, with changes in ECV being independently associa

173 independent predictor of patient survival in ATTR amyloidosis.

174 I-ECG) were deployed to generate study-level ATTR-CM probabilities (0%-100%).

175 AS treatment, AS-ATTR transfers into a lone ATTR cardiomyopathy phenotype.

176 t of cardiomyopathy and heart failure in LTx ATTR amyloid patients is related to amyloid fibril compo

177 utcomes for a relatively large series of LTx ATTR patients with the Val30Met (mutation are available,

178 US Food and Drug Administration for managing ATTR amyloidosis, depending on clinical phenotype.

179 L (mean age, 62 years +/- 10), 44 had mutant ATTR (mean age, 68 years +/- 10), and 66 had wild-type A

180 ECV, mean AL was 0.54 +/- 0.07, mean mutant ATTR was 0.60 +/- 0.07, and mean wild-type ATTR was 0.57

181 , mean AL was 107 g/m(2) +/- 30; mean mutant ATTR was 137 g/m(2) +/- 29; and mean wild-type ATTR was

182 or carrying the p.V142I TTR variant, and NAC ATTR Stage I biomarkers at the time of diagnosis who did

183 Estimated survival among UK NAC ATTR Stage Ia patients was comparable to UK general popu

184 ATTR-CM and non-ATTR-CM cohorts were compared.

185 , with minimal washout, through 150 min; non-ATTR-CM participants showed consistently lower myocardia

186 ith ATTR cardiac amyloidosis and 50 with non-ATTR cardiac amyloidosis [34 with AL amyloidosis and 16

187 Although increased awareness of ATTR-CM and the advent of effective therapy have led to

188 ata from the largest international cohort of ATTR V122I patients, followed up at the UK National Amyl

189 asia was present, histologic confirmation of ATTR amyloid was required.

190 ion should be reconsidered in the context of ATTR-CM.

191 Tc 99m PYP cardiac imaging for detection of ATTR cardiac amyloidosis.

192 sitivity and specificity in the detection of ATTR-CM were 96.3% (95% CI: 81.0, 99.9) and 58.9% (95% C

193 d others in the recognition and diagnosis of ATTR associated with cardiomyopathy.

194 Diagnosis of ATTR CA was determined using standard criteria.

195 A definitive diagnosis of ATTR depends on the detection and identification of TTR

196 A definitive diagnosis of ATTR relies on the detection and identification of TTR v

197 used to noninvasively make the diagnosis of ATTR-CA, based on ventricular myocardial uptake.

198 ional studies indicate that the diagnosis of ATTR-CM may be underrecognized in a significant proporti

199 ac scintigraphy can confirm the diagnosis of ATTR-CM only when combined with blood and urine testing

200 uidelines support the nonbiopsy diagnosis of ATTR-CM using cardiac scintigraphy, yet emphasize its us

201 ques allow accurate noninvasive diagnosis of ATTR-CM without the need for confirmatory endomyocardial

202 ers that best associated with a diagnosis of ATTR-CM.

203 osis in this population, but the efficacy of ATTR-specific medication remains unclear.

204 ought to characterize the natural history of ATTR-CM and compare outcomes and quality of life among p

205 lgorithm may be useful for identification of ATTR V122I amyloidosis in elderly African American patie

206 identified RBP4 as a sensitive identifier of ATTR V122I amyloidosis (area under the curve [AUC] = 0.7

207 nd basal LS were the strongest predictors of ATTR-CM, AUC of 0.87 (95% CI: 0.83, 0.90), superior to t

208 udinal strain each predicted the presence of ATTR-CM with an area under the curve (AUC) of at least 0

209 ticipants older than 75 years, prevalence of ATTR-CA was 17.17% (95% CI, 9.74%-24.60%).

210 y may enable scalable risk stratification of ATTR-CM during its preclinical course.

211 and Drug Administration for the treatment of ATTR-CM.

212 therapies are now approved for treatment of ATTR-CM.

213 patients with ATTR variant polyneuropathy or ATTR cardiomyopathy.

214 In Afro-Caribbean patients, ATTR V122I is an underappreciated cause of heart failure

215 f CPET parameters across disease phenotypes (ATTR with cardiomyopathy [ATTR-CM], polyneuropathy, or b

216 Exclusions included prior ATTR-CM testing, hypertrophic cardiomyopathy, expected l

217 tor cohort of 25 patients with biopsy-proven ATTR V122I amyloidosis recruited from September 1, 2009,

218 In the THAOS registry, ATTR in the United States is overwhelmingly a disorder o

219 Andersen-Gill models adjusted for age, sex, ATTR disease type, and National Amyloidosis Centre stage

220 s, and the hereditary and "senile systemic" (ATTR) variants from mutant and wild-type transthyretin (

221 frequent and causes more organ disease than ATTR.

222 tion fraction at enrollment, suggesting that ATTR-CM should be considered as a possible diagnosis in

223 The ATTR-ACT study will provide important insight into the e

224 (mean age, 74.5 years) using inputs from the ATTR-ACT trial (Transthyretin Amyloidosis Cardiomyopathy

225 The description of the ATTR neuropathy phenotype, especially in the T60A patien

226 This study was independent of the ATTR-ACT trial sponsor.

227 ); P<0.001), and in the AL compared with the ATTR samples (2.48+/-0.40 versus 1.52+/-0.22 DPM/mm(2);

228 0 people in the US have heart failure due to ATTR amyloidosis.

229 on of patients were waitlisted for LT due to ATTR/PH in the post-FDA approval era, and these patients

230 tracardiac ATTR but rose from early-stage to ATTR-cardiomyopathy (CM).

231 25% of patients with amyloid transthyretin (ATTR) amyloidosis die within 24 months of diagnosis.

232 globulin light chain (AL) and transthyretin (ATTR) amyloidosis, are substantially influenced by cardi

233 n light chain amyloidosis and transthyretin (ATTR) amyloidosis.

234 Cardiac transthyretin (ATTR) amyloidosis is a progressive and fatal cardiomyopa

235 oglobulin light-chain (AL) or transthyretin (ATTR) type-and healthy volunteers (n = 5) were investiga

236 are lacking in patients with transthyretin (ATTR) amyloidosis.

237 luated data for patients with transthyretin (ATTR) cardiac amyloidosis and NYHA class I-III symptoms

238 Transthyretin Cardiomyopathy Clinical Trial (ATTR-ACT), a multicenter, international, double-blind, p

239 ereditary (ATTRm) amyloidosis; 125 wild-type ATTR (ATTRwt) amyloidosis].

240 age, 68 years +/- 10), and 66 had wild-type ATTR (mean age, 75 years +/- 7).

241 ition 122 (Val122Ile) (n = 91) and wild-type ATTR (n = 189).

242 or from an aging-linked process in wild-type ATTR amyloidosis (wtATTR).

243 Wild-type ATTR is estimated to have a prevalence of 155 to 191 cas

244 t ATTR was 0.60 +/- 0.07, and mean wild-type ATTR was 0.57 +/- 0.06 versus 0.27 +/- 0.03 in healthy s

245 TR was 137 g/m(2) +/- 29; and mean wild-type ATTR was 133 g/m(2) +/- 27 versus 65 g/m(2) +/- 15 in he

246 with normal TTR genetic sequence (wild-type ATTR) or with abnormal TTR genetic sequence (variant ATT

247 ity of life was poor; diagnosis of wild-type ATTR-CM was delayed >4 years after presentation with car

248 We studied 711 patients with wild-type ATTR-CM, 205 with hereditary ATTR-CM associated with the

249 deposition of misfolded proteins, typically ATTR (transthyretin) or AL (light chains).

250 er aortic valve replacement (TAVR) underwent ATTR screening by blinded 99mTc-DPD bone scintigraphy (P

251 Here, we present outcomes for non-Val30Met ATTR patients after LTx, as reported to the Familial Amy

252 The incidence of variant ATTR amyloidosis is estimated to be 0.3 cases per year p

253 with abnormal TTR genetic sequence (variant ATTR).

254 hate ((99m)Tc-PYP) scintigraphy in AL versus ATTR.

255 9m PYP imaging of 171 participants (121 with ATTR cardiac amyloidosis and 50 with non-ATTR cardiac am

256 164 patients (28%) had scans consistent with ATTR-CA while 117 patients (20%) had AU.

257 nter study evaluated patients diagnosed with ATTR amyloidosis from May 2019 to September 2022 who und

258 s of all consecutive patients diagnosed with ATTR-CA at the National Amyloidosis Centre between 2000

259 spective analysis of patients diagnosed with ATTR-CA at the National Amyloidosis Centre who underwent

260 hort study evaluated patients diagnosed with ATTR-CM at the National Amyloidosis Centre (NAC) in the

261 regression analyses among participants with ATTR cardiac amyloidosis showed that an H/CL ratio of 1.

262 Conclusion: In participants with ATTR-CM, myocardial uptake peaked by 10 min after inject

263 hy (LVH) was present in 79% of patients with ATTR (70% sigmoid septum and 30% reverse septal contour)

264 ntly higher percentage of U.S. patients with ATTR amyloid seen at cardiology sites had wild-type dise

265 tively recruited subjects, 122 patients with ATTR amyloid, 9 asymptomatic mutation carriers, and 119

266 Among the 506 patients with ATTR amyloidosis included in this study, the mean (SD) a

267 ed for a substantial number of patients with ATTR and in all patients with light chain amyloidosis an

268 observational cohort study of patients with ATTR attending the National Hospital Inherited Neuropath

269 ificity for differentiation of patients with ATTR cardiac amyloidosis (irrespective of genotype) from

270 About one-half of patients with ATTR cardiac amyloidosis have a history of carpal tunnel

271 ated with worse survival among patients with ATTR cardiac amyloidosis.

272 Patients with ATTR had a higher total cell volume than did healthy sub

273 eposition is more extensive in patients with ATTR than in those with AL; however, ATTR is associated

274 ns were similar between the 25 patients with ATTR V122I amyloidosis (mean [SD] age, 72.2 [7.4] years;

275 ejection fraction was lower in patients with ATTR V122I amyloidosis (mean [SD], 40% [14%] vs 57% [14%

276 BP4 concentration was lower in patients with ATTR V122I amyloidosis compared with nonamyloid controls

277 frican American patients and 9 patients with ATTR V122I amyloidosis comprised the validation cohort.

278 Patients with ATTR V122I had the worst prognosis compared with other c

279 O formulation, eplontersen, in patients with ATTR variant polyneuropathy or ATTR cardiomyopathy.

280 tracellular volume, whereas in patients with ATTR, the increase is extracellular, with an additional

281 A total of 103 patients with ATTR-CA (100 wild type; 3 variant) were enrolled.

282 observational study including patients with ATTR-CA diagnosed by endomyocardial biopsy was conducted

283 2000 and 2022 identified 2371 patients with ATTR-CA.

284 sociated with poor outcomes in patients with ATTR-CM and is an independent prognostic marker at diagn

285 HELIOS-B randomized 655 patients with ATTR-CM to vutrisiran (25 mg subcutaneously every 12 wee

286 Consecutive patients with ATTR-CM who underwent diagnostic bone tracer scintigraph

287 HELIOS-B randomized patients with ATTR-CM with NYHA functional class I-III (functional cla

288 tre observational study of 879 patients with ATTR-CM, either wild-type TTR genotype or carrying the p

289 Among 1422 patients with ATTR-CM, RV uptake accompanying left ventricular uptake

290 Among patients with ATTR-CM, treatment with vutrisiran led to a lower risk o

291 fe and effective treatment for patients with ATTR-CM.

292 lic function over 30 months in patients with ATTR-CM.

293 diac structure and function in patients with ATTR-CM.

294 urgent HF visits vs placebo in patients with ATTR-CM.

295 al Amyloidosis Centre stage in patients with ATTR-CM.

296 Subjects with ATTR cardiac amyloid had a significantly higher semiquan

297 ailable method for identifying subjects with ATTR cardiac amyloidosis, which should be studied in a l

298 In addition, eight healthy subjects with ATTR mutations (mean age, 47 years +/- 6) and 47 healthy

299 s with AU, both in patients with and without ATTR-CA respectively (HR, 2.27 [95% CI, 1.37-3.78]; P<0.

300 Black participants aged 75 years or younger, ATTR-CA was observed in 3.42% of participants (95% CI, 1