title: Aortic stenosis and transthyretin cardiac amyloidosis: the chicken or the egg?
abstract: Aortic stenosis (AS) and transthyretin cardiac amyloidosis (TTR-CA) are both frequent in elderly. The combination of these two diseases has never been investigated.
  
  
  
  To describe patients with concomitant AS and TTR-CA.
  
  
  
  Six cardiologic French centres identified retrospectively cases of patients with severe or moderate AS associated with TTR-CA hospitalized during the last 6 years.
  
  
  
  Sixteen patients were included. Mean ± SD age was 79 ± 6 years, 81% were men. Sixty per cent were NYHA III-IV, 31% had carpal tunnel syndrome, and 56% had atrial fibrillation. Median (Q1;Q4) NT-proBNP was 4382 (2425;4730) pg/mL and 91% had elevated cardiac troponin level. Eighty-eight per cent had severe AS (n = 14/16), of whom 86% (n = 12) had low-gradient AS. Mean ± SD interventricular septum thickness was 18 ± 4 mm. Mean left ventricular ejection fraction and global LS were 50 ± 13% and -7 ± 4%, respectively. Diagnosis of TTR-CA was histologically proven in 38%, and was based on strong cardiac uptake of the tracer at biphosphonate scintigraphy in the rest. Eighty-one per cent had wild-type TTR-CA (n = 13), one had mutated Val122I and 19% did not had genetic test (n = 3). Valve replacement was surgical in 63% and via transcatheter in 13%. Median follow-up in survivors was 33 (16;65) months. Mortality was of 44% (n = 7) during the whole follow-up period.
  
  
  
  Combination of AS and TTR-CA may occur in elderly patients particularly those with a low-flow low-gradient AS pattern and carries bad prognosis. Diagnosis of TTR-CA in AS is relevant to discuss specific treatment and management.
authors.affiliation: UPEC, Créteil F-94000, France.|MondorAmyloidosis Network, Créteil F-94000, France.|Department of Cardiology, AP-HP, Henri-Mondor Teaching Hospital, Créteil F-94000, France.|INSERM U955, Créteil F-94000, France.|DHU A-TVB, Créteil F-94000, France.
authors.forename: Arnault
authors.initials: A
authors.lastname: Galat
authors.affiliation: UPEC, Créteil F-94000, France.|MondorAmyloidosis Network, Créteil F-94000, France.|Department of Cardiology, AP-HP, Henri-Mondor Teaching Hospital, Créteil F-94000, France.|INSERM U955, Créteil F-94000, France.|DHU A-TVB, Créteil F-94000, France.
authors.forename: Aziz
authors.initials: A
authors.lastname: Guellich
authors.affiliation: UPEC, Créteil F-94000, France.|MondorAmyloidosis Network, Créteil F-94000, France.|Department of Cardiology, AP-HP, Henri-Mondor Teaching Hospital, Créteil F-94000, France.|INSERM U955, Créteil F-94000, France.|DHU A-TVB, Créteil F-94000, France.
authors.forename: Diane
authors.initials: D
authors.lastname: Bodez
authors.affiliation: Department of Cardiology, AP-HP, Antoine Béclère Teaching Hospital, Clamart F-92140, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Slama
authors.affiliation: Department of Cardiology, Bordeaux Teaching Hospital, Pessac F-33604, France.
authors.forename: Marina
authors.initials: M
authors.lastname: Dijos
authors.affiliation: Department of Cardiology, AP-HP, Bichat Teaching Hospital, Paris F-75018, France.
authors.forename: David Messika
authors.initials: DM
authors.lastname: Zeitoun
authors.affiliation: Department of Cardiology, AP-HP, Bichat Teaching Hospital, Paris F-75018, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Milleron
authors.affiliation: Department of Cardiology, Centre Cardiologique du Nord, Saint-Denis F-93200, France.
authors.forename: David
authors.initials: D
authors.lastname: Attias
authors.affiliation: UPEC, Créteil F-94000, France.|MondorAmyloidosis Network, Créteil F-94000, France.|Department of Cardiology, AP-HP, Henri-Mondor Teaching Hospital, Créteil F-94000, France.|INSERM U955, Créteil F-94000, France.|DHU A-TVB, Créteil F-94000, France.
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Dubois-Randé
authors.affiliation: Department of Cardiology, Dupuytren Teaching Hospital, Limoges F-87042, France.
authors.forename: Dania
authors.initials: D
authors.lastname: Mohty
authors.affiliation: UPEC, Créteil F-94000, France.|MondorAmyloidosis Network, Créteil F-94000, France.|INSERM U955, Créteil F-94000, France.|DHU A-TVB, Créteil F-94000, France.|Department of Public Health, Henri-Mondor Teaching Hospital, Créteil F-94000, France.|CEpiA (Clinical Epidemiology and Ageing), EA4393, Université Paris Est (UPE), UPEC, F-94000, Créteil, France.
authors.forename: Etienne
authors.initials: E
authors.lastname: Audureau
authors.affiliation: UPEC, Créteil F-94000, France.|MondorAmyloidosis Network, Créteil F-94000, France.|Department of Cardiology, AP-HP, Henri-Mondor Teaching Hospital, Créteil F-94000, France.|INSERM U955, Créteil F-94000, France.|DHU A-TVB, Créteil F-94000, France.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Teiger
authors.affiliation: UPEC, Créteil F-94000, France.|MondorAmyloidosis Network, Créteil F-94000, France.|Department of Nuclear Medicine, AP-HP, Henri-Mondor Teaching Hospital, Créteil F-94000, France.
authors.forename: Jean
authors.initials: J
authors.lastname: Rosso
authors.affiliation: UPEC, Créteil F-94000, France.|MondorAmyloidosis Network, Créteil F-94000, France.|Department of Cardiology, AP-HP, Henri-Mondor Teaching Hospital, Créteil F-94000, France.|INSERM U955, Créteil F-94000, France.|DHU A-TVB, Créteil F-94000, France.
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Monin
authors.affiliation: UPEC, Créteil F-94000, France thibaud.damy@hmn.aphp.fr.|MondorAmyloidosis Network, Créteil F-94000, France.|Department of Cardiology, AP-HP, Henri-Mondor Teaching Hospital, Créteil F-94000, France.|INSERM U955, Créteil F-94000, France.|DHU A-TVB, Créteil F-94000, France.
authors.forename: Thibaud
authors.initials: T
authors.lastname: Damy
mesh_terms: D000368:Aged; D028227:Amyloid Neuropathies, Familial; D001024:Aortic Valve Stenosis; D005260:Female; D006801:Humans; D008297:Male; D020097:Natriuretic Peptide, Brain; D010446:Peptide Fragments; D011228:Prealbumin; D013318:Stroke Volume; D016896:Treatment Outcome
chemical_list: D010446:Peptide Fragments; D011228:Prealbumin; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain
keywords: Aortic stenosis; Cardiac amyloidosis; Low-flow low-gradient; Transthyretin