title: Clinical and echocardiographic characteristics for differentiating between transthyretin-related and light-chain cardiac amyloidoses.
abstract: Differential diagnosis between transthyretin (TTR) and immunoglobulin light-chain (AL) cardiac amyloidoses is essential due to significantly different prognoses and therapeutic options. Therefore, clinical characteristics of patients with biopsy-proven cardiac amyloidosis were investigated to differentiate TTR from AL amyloidosis. From September 2006 to May 2014, 46 patients were confirmed to have cardiac amyloidosis (TTR, n = 28; AL, n = 18) in our institute. The median age of patients with TTR amyloidosis was 78 years (range 61-90) with 27 (96 %) males, while that of patients with AL amyloidosis was 66 (range 52-76) with 12 (67 %) males. There were no statistically significant differences in echocardiographic findings regarding left ventricular (LV) systolic function or diastolic dysfunction between the two groups. Interestingly, serum brain natriuretic peptide (BNP) levels in patients with AL amyloidosis were significantly higher than those in TTR amyloidosis patients. In contrast, the LV wall was significantly thicker in patients with TTR amyloidosis than in those with AL amyloidosis. Therefore, the ratio of BNP to LV mass index (LVMI) at presentation in AL amyloidosis patients was significantly higher than that in TTR patients (6.7 vs 2.9, p = 0.0006). A BNP-LVMI ratio of less than 3.5 had a diagnostic sensitivity and specificity for TTR amyloidosis of 71 and 83 %, respectively. One-year overall survival was 88.7 % in the patients with TTR amyloidosis and 23.7 % in the patients with AL amyloidosis. Our analysis indicates that the BNP-LVMI ratio, as well as age and sex, may be useful parameters for distinguishing TTR from AL cardiac amyloidosis.
authors.affiliation: Department of Hematology, Kokura Memorial Hospital, 3-2-1 Asano, Kokurakita-Ku, Kitakyushu, Fukuoka, 802-8555, Japan.
authors.forename: Minako
authors.initials: M
authors.lastname: Mori
authors.affiliation: Department Cardiology, Kokura Memorial Hospital, Kitakyushu, Japan.
authors.forename: Yoshimori
authors.initials: Y
authors.lastname: An
authors.affiliation: Department of Hematology, Kokura Memorial Hospital, 3-2-1 Asano, Kokurakita-Ku, Kitakyushu, Fukuoka, 802-8555, Japan.
authors.forename: Oju
authors.initials: O
authors.lastname: Katayama
authors.affiliation: Department of Hematology, Kokura Memorial Hospital, 3-2-1 Asano, Kokurakita-Ku, Kitakyushu, Fukuoka, 802-8555, Japan.
authors.forename: Tomoya
authors.initials: T
authors.lastname: Kitagawa
authors.affiliation: Department of Hematology, Kokura Memorial Hospital, 3-2-1 Asano, Kokurakita-Ku, Kitakyushu, Fukuoka, 802-8555, Japan.
authors.forename: Yuya
authors.initials: Y
authors.lastname: Sasaki
authors.affiliation: Department of Hematology, Kokura Memorial Hospital, 3-2-1 Asano, Kokurakita-Ku, Kitakyushu, Fukuoka, 802-8555, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Onaka
authors.affiliation: Department of Hematology, Kokura Memorial Hospital, 3-2-1 Asano, Kokurakita-Ku, Kitakyushu, Fukuoka, 802-8555, Japan.
authors.forename: Akihito
authors.initials: A
authors.lastname: Yonezawa
authors.affiliation: Diagnostic Pathology, Kokura Memorial Hospital, Kitakyushu, Japan.
authors.forename: Kenichiro
authors.initials: K
authors.lastname: Murata
authors.affiliation: Diagnostic Pathology, Kokura Memorial Hospital, Kitakyushu, Japan.
authors.forename: Tadaaki
authors.initials: T
authors.lastname: Yokota
authors.affiliation: Department Cardiology, Kokura Memorial Hospital, Kitakyushu, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Ando
authors.affiliation: Department of Hematology, Kokura Memorial Hospital, 3-2-1 Asano, Kokurakita-Ku, Kitakyushu, Fukuoka, 802-8555, Japan. imadak@orchid.plala.or.jp.
authors.forename: Kazunori
authors.initials: K
authors.lastname: Imada
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D028227:Amyloid Neuropathies, Familial / Q000000981:diagnostic imaging* / Q000473:pathology*; D000686:Amyloidosis / Q000000981:diagnostic imaging* / Q000473:pathology*; D009202:Cardiomyopathies / Q000000981:diagnostic imaging* / Q000473:pathology*; D003937:Diagnosis, Differential; D004452:Echocardiography; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000009:adverse effects; D000075363:Immunoglobulin Light-chain Amyloidosis; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies
chemical_list: D007147:Immunoglobulin Light Chains
keywords: Cardiac amyloidosis; Left ventricular mass index; Light-chain amyloidosis; Plasma cell neoplasm; Transthyretin-related amyloidosis