UNDERSTANDING TRANSTHYRETIN AMYLOID CARDIOMYOPATHY (ATTR-CM)
認識轉甲狀腺素蛋白類澱粉沉著症 造成之
心肌病變 (ATTR-CM)
Amyloidosis is a multisystemic disease in which TTR proteins with unstable structures misfold and aggregate into amyloid fibrils, which deposit in the heart and other organs.1 The most common amyloid fibril proteins that can infiltrate the heart and lead to cardiac amyloidosis1 are immunoglobulin light chain amyloid fibril protein (AL) and transthyretin amyloid fibril protein (ATTR).2-5
ATTR-CM IS A LIFE-THREATENING, PROGRESSIVE, INFILTRATIVE, RARE DISEASE THAT CAN OFTEN BE OVERLOOKED AS A CAUSE OF HEART FAILURE6,7
ATTR-CM 是一種危及生命、漸進性、浸潤性且罕見的疾病，為心臟衰竭的病因但常被忽略6,7
Once diagnosed, untreated patients with ATTR-CM have a median survival of ~2 to 3.5 years
Illustrative representation.
EARLY DIAGNOSIS OF ATTR-CM IS CRITICAL, AS PROGNOSIS WORSENS RAPIDLY WITH CONTINUED AMYLOID DEPOSITION, SUBSEQUENT ADVANCING ORGAN DYSFUNCTION, AND SIGNIFICANT REDUCTION IN QUALITY OF LIFE.3,6
MAIN TYPES OF CARDIAC AMYLOIDOSIS
心臟類澱粉沉積症的主要類型
It is important to clinically differentiate between
Most Common Types of Cardiac Amyloidosis
AL amyloidosis5
Wild-type
Hereditary
WILD-TYPE vs HEREDITARY ATTR-CM
野生型ATTR-CM 和 遺傳型ATTR-CM
Wild-type ATTR-CM (wtATTR-CM)
Hereditary ATTR-CM (hATTR-CM)
MECHANISM OF DISEASE
疾病機轉
In
Illustrative representation.
ATTR AMYLOID DEPOSITION SITES*
ATTR 類澱粉蛋白沉積位置*
ATTR amyloidosis is a multisystemic disease in which amyloid deposits accumulate in various tissues and organs in the body.4,6,9-16
EARLY, ACCURATE DIAGNOSIS OF ATTR-CM MAY BENEFIT PATIENT CARE AND LEAD TO IMPROVED OUTCOMES.6
Know the clues to look for to avoid missing a diagnosis. Learn the clues
ENCOUNTERING ATTR-CM
面對 ATTR-CM 的對策
Meet Dr. Detective, a cardiologist who specializes in diagnosing tough cases. Watch as he suspects and then detects that his patient has
References: 1. Benson MD, Buxbaum JN, Eisenberg DS, et al. Amyloid nomenclature 2018: recommendations by the International Society of Amyloidosis (ISA) nomenclature committee. Amyloid. 2018;25(4):215-219. 2. Narotsky DL, Castaño A, Weinsaft JW, Bokhari S, Maurer MS. Wild-type transthyretin cardiac amyloidosis: novel insights from advanced imaging. Can J Cardiol. 2016;32(9):1166.e1-1166.e10. 3. Maurer MS, Elliott P, Comenzo R, Semigran M, Rapezzi C. Addressing common questions encountered in the diagnosis and management of cardiac amyloidosis. Circulation. 2017;135(14):1357-1377. 4. Nativi-Nicolau J, Maurer MS. Amyloidosis cardiomyopathy: update in the diagnosis and treatment of the most common types. Curr Opin Cardiol. 2018;33(5):571-579. 5. Rapezzi C, Merlini G, Quarta CC, et al. Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types. Circulation. 2009;120(13):1203-1212. 6. Witteles RM, Bokhari S, Damy T, et al. Screening for transthyretin amyloid cardiomyopathy in everyday practice. JACC Heart Fail. 2019;7(8):709-716. 7. Maurer MS, Hanna M, Grogan M, et al. Genotype and phenotype of transthyretin cardiac amyloidosis: THAOS (Transthyretin Amyloid Outcome Survey). J Am Coll Cardiol. 2016;68(2):161-172. 8. Donnelly JP, Hanna M. Cardiac amyloidosis: An update on diagnosis and treatment. Cleve Clin J Med. 2017;84(12)(suppl 3):12-26. 9. Connors LH, Sam F, Skinner M, et al. Heart failure resulting from age-related cardiac amyloid disease associated with wild-type transthyretin: a prospective, observational cohort study. Circulation. 2016;133(3):282-290. 10. Ruberg FL, Berk JL. Transthyretin (TTR) cardiac amyloidosis. Circulation. 2012;126(10):1286-1300. 11. Yanagisawa A, Ueda M, Sueyoshi T, et al. Amyloid deposits derived from transthyretin in the ligamentum flavum as related to lumbar spinal canal stenosis. Mod Pathol. 2015;28(2):201-207. 12. Giannini G, Nast CC. An organ system-based approach to differential diagnosis of amyloid type in surgical pathology. Arch Pathol Lab Med. 2020;144(3):379-387. doi:10.5858/arpa.2018-0509-RA 13. Freudenthaler S, Hegenbart U, Schönland S, Behrens HM, Krüger S, Röcken C. Amyloid in biopsies of the gastrointestinal tract—a retrospective observational study on 542 patients. Virchows Arch. 2016;468(5):569-577. doi:10.1007/s00428-016-1916-y 14. Shin SC, Robinson-Papp J. Amyloid neuropathies. Mt Sinai J Med. 2012;79(6):733-748. 15. Adams D, Ando Y, Beirão JM, et al. Expert consensus recommendations to improve diagnosis of ATTR amyloidosis with polyneuropathy [published online ahead of print January 6, 2020]. J Neurol. doi:10.1007/s00415-019-09688-0 16. Cho YJ, Chun YS, Rhyu KH, et al. Amyloid arthropathy of the hip joint associated with multiple myeloma: a case report. Hip Pelvis. 2016;28(2):127-131. doi:10.5371/hp.2016.28.2.127