Journal article
Describing mode of death in three major cardiac amyloidosis subtypes to improve management and survival
Amyloid, Vol.29(2), pp.79-91
04/03/2022
DOI: 10.1080/13506129.2021.2013193
PMID: 35114877
Abstract
The three main cardiac amyloidosis (CA) types have different progression and prognosis. Little is known about the mode of death (MOD) which is commonly attributed to cardiovascular causes in CA. Improving MOD's knowledge could allow to adapt patient care.
This retrospective study describes the MOD that occurred during long-term follow-up in CA patients in light-chain (AL), transthyretin hereditary (ATTRv) or wild-type (ATTRwt).
Patients referred to and cared for, at the French referral centre for CA, Henri Mondor Hospital, Créteil between 2010 and 2016 were included. Clinical information surrounding patient deaths were investigated and centrally evaluated by two blinded clinical committees which classified MOD as cardiovascular, non-cardiovascular or unknown and sub-classified it depending on its subtype.
From the 566 patients included, 187 had AL, 206 ATTRv and 173 ATTRwt. During the 864 patient-year follow-up, 160 (28%) deaths occurred, with median survival time of 17.3 months (interquartile range 5.1-35.4). The most frequent MOD was cardiovascular (64%) of which worsening heart failure occurred most frequently and for which, 69% were of AL subtype, 79% ATTRv and 76% ATTRwt. Sudden death also occurred more frequently in AL subtype accounting for 29% of AL deaths. Non-cardiovascular MOD occurred in 26% of patients overall. Among these, infection was the most common non-cardiovascular MOD in any type of CA (80%).
Mortality is high during natural course of CA and differs between subtypes. The main MOD were worsening heart failure, sudden death and infection, opening room to optimise management.
Details
- Title: Subtitle
- Describing mode of death in three major cardiac amyloidosis subtypes to improve management and survival
- Creators
- Mounira Kharoubi - Assistance Publique – Hôpitaux de ParisDiane Bodez - InsermMélanie Bézard - Assistance Publique – Hôpitaux de ParisAmira Zaroui - Assistance Publique – Hôpitaux de ParisArnault Galat - Assistance Publique – Hôpitaux de ParisSoulef Guendouz - Assistance Publique – Hôpitaux de ParisThierry Gendre - InsermLuc Hittinger - InsermDavid Attias - Centre Cardiologique du NordDania Mohty - Hôpital DupuytrenEric Bergoend - Assistance Publique – Hôpitaux de ParisEmmanuel Itti - InsermFabien Lebras - InsermDavid Hamon - InsermElsa Poullot - InsermValérie Molinier-Frenkel - AP-HP, Department of Immunobiology, Henri Mondor University HospitalNicolas Lellouche - Hôpitaux Universitaires Henri-MondorJean-François Deux - AP-HP, Department of Radiology, Henri Mondor University HospitalBenoit Funalot - InsermPascale Fannen - InsermSilvia Oghina - Assistance Publique – Hôpitaux de ParisRaphael Arrouasse - InsermPhilippe Lecorvoisier - Inserm, Clinical Investigations Center 1430, AP-HP, DMU Saphire, Henri Mondor University HospitalSarah Souvannanorath - InsermAurelien Amiot - Université Paris-Est CréteilEmmanuel Teiger - Hôpitaux Universitaires Henri-MondorWulfran Bougouin - Paris Cardiovascular Research CenterThibaud Damy - Inserm
- Resource Type
- Journal article
- Publication Details
- Amyloid, Vol.29(2), pp.79-91
- DOI
- 10.1080/13506129.2021.2013193
- PMID
- 35114877
- NLM abbreviation
- Amyloid
- ISSN
- 1350-6129
- eISSN
- 1744-2818
- Publisher
- Taylor & Francis
- Language
- English
- Date published
- 04/03/2022
- Academic Unit
- Internal Medicine
- Record Identifier
- 9984691513402771
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