TY - JOUR
T1 - Temporal Trends of Wild-Type Transthyretin Amyloid Cardiomyopathy in the Transthyretin Amyloidosis Outcomes Survey
AU - THAOS Investigators
AU - Nativi-Nicolau, Jose
AU - Siu, Alfonso
AU - Dispenzieri, Angela
AU - Maurer, Mathew S.
AU - Rapezzi, Claudio
AU - Kristen, Arnt V.
AU - Garcia-Pavia, Pablo
AU - LoRusso, Samantha
AU - Waddington-Cruz, Márcia
AU - Lairez, Olivier
AU - Witteles, Ronald
AU - Chapman, Doug
AU - Amass, Leslie
AU - Grogan, Martha
AU - Barroso, Fabio Adrian
AU - Van Cleemput, Johan
AU - Fine, Nowell
AU - Schmidt, Hartmut
AU - Gess, Burkhard
AU - Moelgaard, Henning
AU - Planté-Bordeneuve, Violaine
AU - Adams, David
AU - Inamo, Jocelyn
AU - Vita, Giuseppe
AU - Cirami, Calogero Lino
AU - Luigetti, Marco
AU - Emdin, Michele
AU - Sekijima, Yoshiki
AU - Yamashita, Taro
AU - Jeon, Eun Seok
AU - Gonzalez Duarte Briseno, Maria Alejandra
AU - Nienhuis, Hans
AU - Azevedo, Olga
AU - Campistol Plana, Josep Maria
AU - Moreno, Juan Gonzalez
AU - Costello, Jose Gonzalez
AU - Wixner, Jonas
AU - Parman, Yesim
AU - Shah, Sanjiv
AU - Quan, Dianna
AU - Marburger, Tessa
AU - Polydefkis, Michael
AU - Gottlieb, Stephen
AU - Ralph, Jeffrey
AU - Sarswat, Nitasha
AU - Luo, Jin
AU - Murali, Srinivas
AU - Cotts, William
AU - Drachman, Brian
AU - Steidley, David
N1 - Publisher Copyright:
© 2021 The Authors
PY - 2021/10
Y1 - 2021/10
N2 - Background: Transthyretin amyloid cardiomyopathy results from the accumulation of wild-type (ATTRwt) or variant (ATTRv) transthyretin amyloid fibrils in the myocardium. THAOS (Transthyretin Amyloidosis Outcomes Survey) is a global, longitudinal, observational survey of patients with ATTRv and ATTRwt amyloidosis and asymptomatic patients with transthyretin mutations. Objectives: This study explored temporal trends in ATTRwt amyloidosis diagnoses using data from THAOS. Methods: Using THAOS data from December 2007 to January 2020, the following comparisons were made according to year: ATTRwt amyloidosis diagnoses in the United States versus rest of the world, ATTRwt versus ATTRv amyloidosis with cardiac-associated mutations diagnoses, and ATTRwt amyloidosis diagnoses by tissue biopsy versus bone scintigraphy. Results: There were 1,069 patients with ATTRwt amyloidosis and 525 with ATTRv amyloidosis with cardiac mutations enrolled in THAOS. The median time from symptom onset to ATTRwt amyloidosis diagnosis did not change over the past 5 years (>60 months from 2015–2019). ATTRwt amyloidosis diagnoses increased from 2 in 2005 to >100 per year from 2016, with a more pronounced increase in the United States compared with the rest of the world. Diagnoses of ATTRwt amyloidosis by tissue biopsy increased yearly and peaked in 2014 before declining, whereas diagnoses by bone scintigraphy increased markedly since 2011. ATTRv amyloidosis with cardiac mutation diagnoses increased from 3 in 2005 to 37 in 2011, then plateaued. The proportion of patients with ATTRwt amyloidosis diagnosed with New York Heart Association functional class III/IV heart failure decreased from 2012 (46.4%) to 2019 (16.0%). Conclusions: In the past decade, ATTRwt amyloidosis diagnoses increased worldwide. Despite the growing utilization of bone scintigraphy, patients are diagnosed several years after symptom onset.
AB - Background: Transthyretin amyloid cardiomyopathy results from the accumulation of wild-type (ATTRwt) or variant (ATTRv) transthyretin amyloid fibrils in the myocardium. THAOS (Transthyretin Amyloidosis Outcomes Survey) is a global, longitudinal, observational survey of patients with ATTRv and ATTRwt amyloidosis and asymptomatic patients with transthyretin mutations. Objectives: This study explored temporal trends in ATTRwt amyloidosis diagnoses using data from THAOS. Methods: Using THAOS data from December 2007 to January 2020, the following comparisons were made according to year: ATTRwt amyloidosis diagnoses in the United States versus rest of the world, ATTRwt versus ATTRv amyloidosis with cardiac-associated mutations diagnoses, and ATTRwt amyloidosis diagnoses by tissue biopsy versus bone scintigraphy. Results: There were 1,069 patients with ATTRwt amyloidosis and 525 with ATTRv amyloidosis with cardiac mutations enrolled in THAOS. The median time from symptom onset to ATTRwt amyloidosis diagnosis did not change over the past 5 years (>60 months from 2015–2019). ATTRwt amyloidosis diagnoses increased from 2 in 2005 to >100 per year from 2016, with a more pronounced increase in the United States compared with the rest of the world. Diagnoses of ATTRwt amyloidosis by tissue biopsy increased yearly and peaked in 2014 before declining, whereas diagnoses by bone scintigraphy increased markedly since 2011. ATTRv amyloidosis with cardiac mutation diagnoses increased from 3 in 2005 to 37 in 2011, then plateaued. The proportion of patients with ATTRwt amyloidosis diagnosed with New York Heart Association functional class III/IV heart failure decreased from 2012 (46.4%) to 2019 (16.0%). Conclusions: In the past decade, ATTRwt amyloidosis diagnoses increased worldwide. Despite the growing utilization of bone scintigraphy, patients are diagnosed several years after symptom onset.
KW - bone scintigraphy
KW - registry
KW - wild-type transthyretin amyloidosis
UR - http://www.scopus.com/inward/record.url?scp=85122704357&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85122704357&partnerID=8YFLogxK
U2 - 10.1016/j.jaccao.2021.08.009
DO - 10.1016/j.jaccao.2021.08.009
M3 - Article
AN - SCOPUS:85122704357
SN - 2666-0873
VL - 3
SP - 537
EP - 546
JO - JACC: CardioOncology
JF - JACC: CardioOncology
IS - 4
ER -