title: Outcome and incidence of appropriate implantable cardioverter-defibrillator therapy in patients with cardiac amyloidosis.
abstract: BACKGROUND
  Cardiac amyloidosis (CA) is associated with a poor prognosis with the proposed mechanism of sudden cardiac death in the majority of patients being pulseless electrical activity. However, the incidence of ventricular arrhythmias (VA) and implantable cardioverter-defibrillator (ICD) indications in CA patients are unclear. We performed a detailed evaluation of our CA population undergoing ICD implantation and assessed appropriate ICD therapy and survival predictors.
  
  
  METHODS
  We included consecutive patients from June 2008 to November 2014 in five centers. ICDs were systematically interrogated and clinical data recorded during follow-up.
  
  
  RESULTS
  Forty-five patients (35 males, mean age 66±12years) with CA who underwent ICD implantation (84.4% primary prevention) were included. CA types were hereditary transthyretin in 27 patients (60%), light chain (AL) in 12 (27%) and senile in 6 (13%). After a mean follow-up of 17±14months, 12 patients (27%) had at least 1 appropriate ICD therapy occurring after 4.7±6.6months. Patients with or without ICD therapy had no significant differences in baseline characteristics, amyloidosis type, LVEF, and type of prevention although there was a trend towards a better 2D global longitudinal strain in patients with ICD therapy (P=0.08). Over the follow-up, 12 patients died (27%) and 6 underwent cardiac transplantation (13%). From multivariate analysis a worse prognosis was associated with higher NT-proBNP level (>6800pg/mL, HR=5.5[1.7-17.8]) and AL type (HR=4.9[1.5-16.3]).
  
  
  CONCLUSIONS
  Appropriate ICD therapies are common (27%) in CA patients. No specific strong VA predictor could be identified. However, patients with advanced heart disease, especially with AL-CA, display a poorer outcome.
authors: AP-HP, University Hospital Henri Mondor, Department of Cardiology, Creteil, France. Electronic address: david.a.hamon@gmail.com.
authors: David
authors: D
authors: Hamon
authors: AP-HP, University Hospital Antoine-Béclère, Cardiology Unit, French Reference Center for Familial Amyloid Polyneuropathy, Clamart, France.
authors: Vincent
authors: V
authors: Algalarrondo
authors: AP-HP, University Hospital Pitié-Salpetrière, Cardiology Unit, Paris, France.
authors: Estelle
authors: E
authors: Gandjbakhch
authors: AP-HP, University Hospital Bichat, Cardiology Unit, Paris, France.
authors: Fabrice
authors: F
authors: Extramiana
authors: AP-HP, European Hospital Georges Pompidou, Cardiology Unit, Paris, France.
authors: Eloi
authors: E
authors: Marijon
authors: AP-HP, University Hospital Henri Mondor, Department of Cardiology, Creteil, France.
authors: Nathalie
authors: N
authors: Elbaz
authors: AP-HP, University Hospital Antoine-Béclère, Cardiology Unit, French Reference Center for Familial Amyloid Polyneuropathy, Clamart, France.
authors: Dounia
authors: D
authors: Selhane
authors: AP-HP, University Hospital Henri Mondor, Department of Cardiology, Creteil, France.
authors: Jean-Luc
authors: JL
authors: Dubois-Rande
authors: AP-HP, University Hospital Henri Mondor, Department of Cardiology, Creteil, France.
authors: Emmanuel
authors: E
authors: Teiger
authors: AP-HP, University Hospital Henri Mondor, Department of Neurology, Creteil, France; UPEC, AP-HP Henri-Mondor Teaching Hospital, Inserm U955, IMRB GRC Mondor Amyloidosis Network, Inserm Clinical Investigation Center 1430, Créteil F-94000, France.
authors: Violaine
authors: V
authors: Plante-Bordeneuve
authors: AP-HP, University Hospital Henri Mondor, Department of Cardiology, Creteil, France; UPEC, AP-HP Henri-Mondor Teaching Hospital, Inserm U955, IMRB GRC Mondor Amyloidosis Network, Inserm Clinical Investigation Center 1430, Créteil F-94000, France.
authors: Thibaud
authors: T
authors: Damy
authors: AP-HP, University Hospital Henri Mondor, Department of Cardiology, Creteil, France; UPEC, AP-HP Henri-Mondor Teaching Hospital, Inserm U955, IMRB GRC Mondor Amyloidosis Network, Inserm Clinical Investigation Center 1430, Créteil F-94000, France.
authors: Nicolas
authors: N
authors: Lellouche
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D017147:Defibrillators, Implantable / Q000639:trends*; D004562:Electrocardiography / Q000401:mortality / Q000639:trends; D005260:Female; D005500:Follow-Up Studies; D006331:Heart Diseases / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome
keywords: Cardiac amyloidosis; Hereditary transthyretin; Implantable cardioverter defibrillator; Sudden cardiac death; Ventricular arrhythmia