title: Longitudinal systolic strain, cardiac function improvement, and survival following treatment of light-chain (AL) cardiac amyloidosis.
abstract: OBJECTIVE
  To determine whether echocardiographic longitudinal systolic strain (LS) parameters identify short-term improvement following chemotherapy for light-chain (AL) cardiac amyloidosis (CA). Among patients with CA, standard echocardiographic measures are commonly unchanged at 1 year following successful chemotherapy, despite observed reductions in cardiac biomarkers.
  
  
  RESULTS
  We retrospectively identified 61 patients with AL-CA treated with high-dose melphalan or bortezomib-based regimens. Patients were classified by hematologic response at 1 year into two groups: complete response (CR; n = 18, or 30%) or non-CR (non-CR; n = 43, or 70%), and followed for 20 months. Serum free light chains (FLC), B-type natriuretic peptide (BNP), troponin I (TnI), and echocardiography including LS, were acquired at baseline and 1 year. Seven patients died (11.5%), all in the non-CR group (P < 0.01). At 1 year, while reductions were observed in BNP (44% CR, 18% non-CR) and FLC (94% CR, 73% non-CR), both P < 0.05 from baseline, there were no differences in wall thickness, EF, or diastolic function in either group. LS improved only in the CR group with notable improvement in apical to basal strain ratio (P < 0.05). Strain improvement and BNP reduction were correlated (R = 0.6, P < 0.01). Baseline global LS < -10.2% was associated with survival and proved superior to BNP and TnI. The addition of global LS to biomarkers identified the patients at highest risk of mortality.
  
  
  CONCLUSIONS
  These data suggest that LS is a sensitive measure of pre-treatment cardiac functional impairment in AL-CA, can predict survival over and above that of cardiac biomarkers, and detect early cardiac functional improvement following chemotherapy.
authors.affiliation: Amyloidosis Center, Boston University School of Medicine, Boston Medical Center, Boston, MA, USA.|Department of Internal Medicine, Experimental Medicine PhD program, University of Pavia School of Medicine, Pavia, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Salinaro
authors.affiliation: Amyloidosis Center, Boston University School of Medicine, Boston Medical Center, Boston, MA, USA.|Section of Cardiovascular Medicine, Department of Medicine, Boston University School of Medicine Boston, Boston Medical Center, 88 E Newton St., Boston, MA 02118, USA.
authors.forename: Hans K
authors.initials: HK
authors.lastname: Meier-Ewert
authors.affiliation: Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT, USA.
authors.forename: Edward J
authors.initials: EJ
authors.lastname: Miller
authors.affiliation: Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT, USA.
authors.forename: Shivda
authors.initials: S
authors.lastname: Pandey
authors.affiliation: Amyloidosis Center, Boston University School of Medicine, Boston Medical Center, Boston, MA, USA.|Section of Hematology/Oncology, Department of Medicine, Boston University School of Medicine, Boston Medical Center, Boston, MA, USA.
authors.forename: Vaishali
authors.initials: V
authors.lastname: Sanchorawala
authors.affiliation: Amyloidosis Center, Boston University School of Medicine, Boston Medical Center, Boston, MA, USA.
authors.forename: John L
authors.initials: JL
authors.lastname: Berk
authors.affiliation: Amyloidosis Center, Boston University School of Medicine, Boston Medical Center, Boston, MA, USA.|Section of Hematology/Oncology, Department of Medicine, Boston University School of Medicine, Boston Medical Center, Boston, MA, USA.
authors.forename: David C
authors.initials: DC
authors.lastname: Seldin
authors.affiliation: Amyloidosis Center, Boston University School of Medicine, Boston Medical Center, Boston, MA, USA.|Section of Cardiovascular Medicine, Department of Medicine, Boston University School of Medicine Boston, Boston Medical Center, 88 E Newton St., Boston, MA 02118, USA.
authors.forename: Frederick L
authors.initials: FL
authors.lastname: Ruberg
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols; D015415:Biomarkers / Q000097:blood; D000069286:Bortezomib / Q000008:administration & dosage*; D001900:Boston; D009202:Cardiomyopathies / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D015331:Cohort Studies; D018618:Echocardiography, Doppler, Color / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006334:Heart Function Tests; D006785:Hospitals, University; D006801:Humans; D000075363:Immunoglobulin Light-chain Amyloidosis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D053208:Kaplan-Meier Estimate; D008297:Male; D008558:Melphalan / Q000008:administration & dosage*; D008875:Middle Aged; D011379:Prognosis; D012372:ROC Curve; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D016019:Survival Analysis; D016896:Treatment Outcome; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging* / Q000209:etiology / Q000401:mortality
chemical_list: D015415:Biomarkers; D000069286:Bortezomib; D008558:Melphalan
keywords: cardiac amyloidosis; cardiac functional improvement; chemotherapy; longitudinal strain