The Cardiac Amyloidosis Registry Study (CARS): Rationale, Design and Methodology

LILY K. STERN; JUSTIN L. GRODIN; MATHEW S. MAURER; FREDERICK L. RUBERG; AYAN R. PATEL; MICHEL G. KHOURI; LORI R. ROTH; MANDAR A. ARAS; A. N.J.U. BHARDWAJ; PRIYANKA BHATTACHARYA; YEVGENIY BRAILOVSKY; BRIAN M. DRACHMAN; IMO A. EBONG; NOWELL M. FINE; HANNA GAGGIN; DEEPA GOPAL; J. A.N. GRIFFIN; DANIEL JUDGE; P. A.U.L. KIM; JOSHUA MITCHELL; SUMEET S. MITTER; RAJEEV C. MOHAN; HANNIA RAMOS; A. L.E.X. REYENTOVICH; FAROOQ H. SHEIKH; BRETT SPERRY; SPENCER CARTER; MARCUS UREY; JOBAN VAISHNAV; AMANDA R. VEST; MICHELLE M. KITTLESON; JIGNESH K. PATEL

doi:10.1016/j.cardfail.2023.09.016

The Cardiac Amyloidosis Registry Study (CARS): Rationale, Design and Methodology

LILY K. STERN, JUSTIN L. GRODIN, MATHEW S. MAURER, FREDERICK L. RUBERG, AYAN R. PATEL, MICHEL G. KHOURI, LORI R. ROTH, MANDAR A. ARAS, A. N.J.U. BHARDWAJ, PRIYANKA BHATTACHARYA, YEVGENIY BRAILOVSKY, BRIAN M. DRACHMAN, IMO A. EBONG, NOWELL M. FINE, HANNA GAGGIN, DEEPA GOPAL, J. A.N. GRIFFIN, DANIEL JUDGE, P. A.U.L. KIM, JOSHUA MITCHELLSUMEET S. MITTER, RAJEEV C. MOHAN, HANNIA RAMOS, A. L.E.X. REYENTOVICH, FAROOQ H. SHEIKH, BRETT SPERRY, SPENCER CARTER, MARCUS UREY, JOBAN VAISHNAV, AMANDA R. VEST, MICHELLE M. KITTLESON, JIGNESH K. PATEL

Research output: Contribution to journal › Article › peer-review

Abstract

Background: CARS (Cardiac Amyloidosis Registry Study) is a multicenter registry established in 2019 that includes patients with transthyretin (ATTR, wild-type and variant) and light chain (AL) cardiac amyloidosis (CA) evaluated at major amyloidosis centers between 1997 and 2025. CARS aims to describe the natural history of CA with attention to clinical and diagnostic variables at the time of diagnosis, real-world treatment patterns, and associated outcomes of patients in a diverse cohort that is more representative of the at-risk population than that described in CA clinical trials. Methods and Results: This article describes the design and methodology of CARS, including procedures for data collection and preliminary results. As of February 2023, 20 centers in the United States enrolled 1415 patients, including 1155 (82%) with ATTR and 260 (18%) with AL CA. Among those with ATTR, wild-type is the most common ATTR (71%), and most of the 305 patients with variant ATTR have the p.V142I mutation (68%). A quarter of the total population identifies as Black. More individuals with AL are female (39%) compared to those with ATTR (13%). Conclusions: CARS will answer crucial clinical questions about CA natural history and permit comparison of different therapeutics not possible through current clinical trials. Future international collaboration will further strengthen the validity of observations of this increasingly recognized condition.

Original language	English (US)
Journal	Journal of Cardiac Failure
DOIs	https://doi.org/10.1016/j.cardfail.2023.09.016
State	Accepted/In press - 2023
Externally published	Yes

Keywords

Cardiac amyloidosis
light chain amyloidosis
registry
transthyretin amyloidosis

ASJC Scopus subject areas

Cardiology and Cardiovascular Medicine

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10.1016/j.cardfail.2023.09.016

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STERN, LILY. K., GRODIN, JUSTIN. L., MAURER, MATHEW. S., RUBERG, FREDERICK. L., PATEL, AYAN. R., KHOURI, MICHEL. G., ROTH, LORI. R., ARAS, MANDAR. A., BHARDWAJ, A. N. J. U., BHATTACHARYA, PRIYANKA., BRAILOVSKY, YEVGENIY., DRACHMAN, BRIAN. M., EBONG, IMO. A., FINE, NOWELL. M., GAGGIN, HANNA., GOPAL, DEEPA., GRIFFIN, J. A. N., JUDGE, DANIEL., KIM, P. A. U. L., ... PATEL, JIGNESH. K. (Accepted/In press). The Cardiac Amyloidosis Registry Study (CARS): Rationale, Design and Methodology. Journal of Cardiac Failure. https://doi.org/10.1016/j.cardfail.2023.09.016

STERN, LILYK, GRODIN, JUSTINL, MAURER, MATHEWS, RUBERG, FREDERICKL, PATEL, AYANR, KHOURI, MICHELG, ROTH, LORIR, ARAS, MANDARA, BHARDWAJ, ANJU, BHATTACHARYA, PRIYANKA, BRAILOVSKY, YEVGENIY, DRACHMAN, BRIANM, EBONG, IMOA, FINE, NOWELLM, GAGGIN, HANNA, GOPAL, DEEPA, GRIFFIN, JAN, JUDGE, DANIEL, KIM, PAUL, MITCHELL, JOSHUA, MITTER, SUMEETS, MOHAN, RAJEEVC, RAMOS, HANNIA, REYENTOVICH, ALEX, SHEIKH, FAROOQH, SPERRY, BRETT, CARTER, SPENCER, UREY, MARCUS, VAISHNAV, JOBAN, VEST, AMANDAR, KITTLESON, MICHELLEM & PATEL, JIGNESHK 2023, 'The Cardiac Amyloidosis Registry Study (CARS): Rationale, Design and Methodology', Journal of Cardiac Failure. https://doi.org/10.1016/j.cardfail.2023.09.016

@article{d55ae77a59414a50a8758004a1c845e4,

title = "The Cardiac Amyloidosis Registry Study (CARS): Rationale, Design and Methodology",

abstract = "Background: CARS (Cardiac Amyloidosis Registry Study) is a multicenter registry established in 2019 that includes patients with transthyretin (ATTR, wild-type and variant) and light chain (AL) cardiac amyloidosis (CA) evaluated at major amyloidosis centers between 1997 and 2025. CARS aims to describe the natural history of CA with attention to clinical and diagnostic variables at the time of diagnosis, real-world treatment patterns, and associated outcomes of patients in a diverse cohort that is more representative of the at-risk population than that described in CA clinical trials. Methods and Results: This article describes the design and methodology of CARS, including procedures for data collection and preliminary results. As of February 2023, 20 centers in the United States enrolled 1415 patients, including 1155 (82%) with ATTR and 260 (18%) with AL CA. Among those with ATTR, wild-type is the most common ATTR (71%), and most of the 305 patients with variant ATTR have the p.V142I mutation (68%). A quarter of the total population identifies as Black. More individuals with AL are female (39%) compared to those with ATTR (13%). Conclusions: CARS will answer crucial clinical questions about CA natural history and permit comparison of different therapeutics not possible through current clinical trials. Future international collaboration will further strengthen the validity of observations of this increasingly recognized condition.",

keywords = "Cardiac amyloidosis, light chain amyloidosis, registry, transthyretin amyloidosis",

author = "STERN, {LILY K.} and GRODIN, {JUSTIN L.} and MAURER, {MATHEW S.} and RUBERG, {FREDERICK L.} and PATEL, {AYAN R.} and KHOURI, {MICHEL G.} and ROTH, {LORI R.} and ARAS, {MANDAR A.} and BHARDWAJ, {A. N.J.U.} and PRIYANKA BHATTACHARYA and YEVGENIY BRAILOVSKY and DRACHMAN, {BRIAN M.} and EBONG, {IMO A.} and FINE, {NOWELL M.} and HANNA GAGGIN and DEEPA GOPAL and GRIFFIN, {J. A.N.} and DANIEL JUDGE and KIM, {P. A.U.L.} and JOSHUA MITCHELL and MITTER, {SUMEET S.} and MOHAN, {RAJEEV C.} and HANNIA RAMOS and REYENTOVICH, {A. L.E.X.} and SHEIKH, {FAROOQ H.} and BRETT SPERRY and SPENCER CARTER and MARCUS UREY and JOBAN VAISHNAV and VEST, {AMANDA R.} and KITTLESON, {MICHELLE M.} and PATEL, {JIGNESH K.}",

note = "Publisher Copyright: {\textcopyright} 2023",

year = "2023",

doi = "10.1016/j.cardfail.2023.09.016",

language = "English (US)",

journal = "Journal of Cardiac Failure",

issn = "1071-9164",

publisher = "Churchill Livingstone",

}

TY - JOUR

T1 - The Cardiac Amyloidosis Registry Study (CARS)

T2 - Rationale, Design and Methodology

AU - STERN, LILY K.

AU - GRODIN, JUSTIN L.

AU - MAURER, MATHEW S.

AU - RUBERG, FREDERICK L.

AU - PATEL, AYAN R.

AU - KHOURI, MICHEL G.

AU - ROTH, LORI R.

AU - ARAS, MANDAR A.

AU - BHARDWAJ, A. N.J.U.

AU - BHATTACHARYA, PRIYANKA

AU - BRAILOVSKY, YEVGENIY

AU - DRACHMAN, BRIAN M.

AU - EBONG, IMO A.

AU - FINE, NOWELL M.

AU - GAGGIN, HANNA

AU - GOPAL, DEEPA

AU - GRIFFIN, J. A.N.

AU - JUDGE, DANIEL

AU - KIM, P. A.U.L.

AU - MITCHELL, JOSHUA

AU - MITTER, SUMEET S.

AU - MOHAN, RAJEEV C.

AU - RAMOS, HANNIA

AU - REYENTOVICH, A. L.E.X.

AU - SHEIKH, FAROOQ H.

AU - SPERRY, BRETT

AU - CARTER, SPENCER

AU - UREY, MARCUS

AU - VAISHNAV, JOBAN

AU - VEST, AMANDA R.

AU - KITTLESON, MICHELLE M.

AU - PATEL, JIGNESH K.

PY - 2023

Y1 - 2023

N2 - Background: CARS (Cardiac Amyloidosis Registry Study) is a multicenter registry established in 2019 that includes patients with transthyretin (ATTR, wild-type and variant) and light chain (AL) cardiac amyloidosis (CA) evaluated at major amyloidosis centers between 1997 and 2025. CARS aims to describe the natural history of CA with attention to clinical and diagnostic variables at the time of diagnosis, real-world treatment patterns, and associated outcomes of patients in a diverse cohort that is more representative of the at-risk population than that described in CA clinical trials. Methods and Results: This article describes the design and methodology of CARS, including procedures for data collection and preliminary results. As of February 2023, 20 centers in the United States enrolled 1415 patients, including 1155 (82%) with ATTR and 260 (18%) with AL CA. Among those with ATTR, wild-type is the most common ATTR (71%), and most of the 305 patients with variant ATTR have the p.V142I mutation (68%). A quarter of the total population identifies as Black. More individuals with AL are female (39%) compared to those with ATTR (13%). Conclusions: CARS will answer crucial clinical questions about CA natural history and permit comparison of different therapeutics not possible through current clinical trials. Future international collaboration will further strengthen the validity of observations of this increasingly recognized condition.

AB - Background: CARS (Cardiac Amyloidosis Registry Study) is a multicenter registry established in 2019 that includes patients with transthyretin (ATTR, wild-type and variant) and light chain (AL) cardiac amyloidosis (CA) evaluated at major amyloidosis centers between 1997 and 2025. CARS aims to describe the natural history of CA with attention to clinical and diagnostic variables at the time of diagnosis, real-world treatment patterns, and associated outcomes of patients in a diverse cohort that is more representative of the at-risk population than that described in CA clinical trials. Methods and Results: This article describes the design and methodology of CARS, including procedures for data collection and preliminary results. As of February 2023, 20 centers in the United States enrolled 1415 patients, including 1155 (82%) with ATTR and 260 (18%) with AL CA. Among those with ATTR, wild-type is the most common ATTR (71%), and most of the 305 patients with variant ATTR have the p.V142I mutation (68%). A quarter of the total population identifies as Black. More individuals with AL are female (39%) compared to those with ATTR (13%). Conclusions: CARS will answer crucial clinical questions about CA natural history and permit comparison of different therapeutics not possible through current clinical trials. Future international collaboration will further strengthen the validity of observations of this increasingly recognized condition.

KW - Cardiac amyloidosis

KW - light chain amyloidosis

KW - registry

KW - transthyretin amyloidosis

UR - http://www.scopus.com/inward/record.url?scp=85180559870&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85180559870&partnerID=8YFLogxK

U2 - 10.1016/j.cardfail.2023.09.016

DO - 10.1016/j.cardfail.2023.09.016

M3 - Article

C2 - 37907148

AN - SCOPUS:85180559870

SN - 1071-9164

JO - Journal of Cardiac Failure

JF - Journal of Cardiac Failure

ER -

The Cardiac Amyloidosis Registry Study (CARS): Rationale, Design and Methodology

Abstract

Keywords

ASJC Scopus subject areas

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