History of extracardiac/cardiac events in cardiac amyloidosis: prevalence and time from initial onset to diagnosis.

Abstract

AIMS:

Cardiac amyloidosis (CA) has a poor prognosis which is aggravated by diagnostic delay. Amyloidosis extracardiac and cardiac events (AECE and ACE) may help improve CA diagnosis and typing. The aim of this study was to compare AECE and ACE between different CA types and assess their relationship with survival.

METHODS AND RESULTS:

This retrospective cohort study conducted in France from June 2008 to May 2019, at the Henry Mondor Hospital. This cohort included 983 patients with CA. Mean age at inclusion was 73.1 ± 11.4 years, 726 (75.1%) were male and the mean body mass index was 24.5 ± 4.1 kg/m2 . Among them, 321 had immunoglobulin light chain (AL) amyloidosis, 434 had wild-type transthyretin (ATTRwt), and 212 had hereditary transthyretin (ATTRv). The first AECE and/or ACE occurred at a mean age of 63 ± 11 years for AL and ATTRv, and 70 ± 12 years for ATTRwt (P < 0.01). The median (Q1-Q3) delay between declaration of the first events and diagnosis varied from 11.1 (5.9; 34.8) months for AL to 92.2 (39.0; 174.7) months for ATTRwt (P < 0.01). The nature of the onset of AECE or ACE varied based on amyloidosis type, heart failure symptoms for AL (26%) and integumentary symptoms for ATTRv with cardiologic or mixed phenotype (39%) and ATTRwt (42%). In AL and ATTRwt, a short delay between the onset of the first AECE or ACE and diagnosis was associated with reduced survival rate (log-rank test P-value <0.01).

CONCLUSIONS:

This study highlights the impact of amyloidosis type and evolution on diagnostic delay and on prognosis. Physicians must be aware and vigilant in front of extracardiac and cardiac events to considerably improve early diagnosis of amyloidosis.

• Authors: Mounira Kharoubi, Mélanie Bézard, Arnault Galat, Fabien Le Bras, Elsa Poullot, Valérie Molinier-Frenkel, Pascale Fanen, Benoit Funalot, Anissa Moktefi, Jean-Pascal Lefaucheur, Mukedaisi Abulizi, Jean-François Deux, François Lemonnier, Soulef Guendouz, Coraline Chalard, Amira Zaroui, Vincent Audard, Emilie Bequignon, Diane Bodez, Emmanuel Itti, Luc Hittinger, Etienne Audureau, Emmanuel Teiger, Silvia Oghina, Thibaud Damy
• Journal: ESC heart failure (ESC Heart Fail) Vol. 8 Issue 6 Pg. 5501-5512 (12 2021) ISSN: 2055-5822 [Electronic] England
• PMID: 34714605 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.

Topics

• Amyloidosis (diagnosis, epidemiology)
• Delayed Diagnosis
• Humans
• Immunoglobulin Light-chain Amyloidosis (complications, diagnosis, epidemiology)
• Male
• Prevalence
• Retrospective Studies