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Clinical profile and outcome of cardiac amyloidosis in a Spanish referral center

Authors :
Juan Francisco Oteo
Pablo García-Pavía
Jesus G. Mirelis
Vanessa Moñivas
Esther Gonzalez-Lopez
Ana Briceño
Javier Segovia
Clara Salas
Isabel Krsnik
Belén Bornstein
Ángela López-Sainz
Manuel Gómez-Bueno
Aitor Hernandez-Hernandez
Francisco José Hernández-Pérez
Juan Antonio López
Maria Alejandra Restrepo-Cordoba
Fernando Domínguez
Jesús Vázquez
Marta Cobo-Marcos
Miguel A. Cavero
Luis Alonso-Pulpón
Susana Mingo Santos
F. Javier de Haro-del Moral
Source :
Revista Española de Cardiología (English Edition). 74:149-158
Publication Year :
2021
Publisher :
Elsevier BV, 2021.

Abstract

Cardiac amyloidosis (CA) is produced by amyloid fiber deposition in the myocardium. The most frequent forms are those caused by light chains (AL) and transthyretin (ATTR). Our objective was to describe the diagnosis, treatment and outcomes of CA in a specialized Spanish center.We included all patients diagnosed with CA in Hospital Universitario Puerta de Hierro Majadahonda from May 2008 to September 2018. We analyzed their clinical characteristics, outcomes, and survival.We included 180 patients with CA, of whom 64 (36%) had AL (50% men; mean age, 65±11 years) and 116 had ATTR (72% men; mean age 79±11 years; 18 with hereditary ATTR). The most common presentation was heart failure in both groups (81% in AL and 45% in ATTR, P.01). Other forms of presentation in ATTR patients were atrial arrhythmias (16%), conduction disorders (6%), and incidental finding (6%); 70 patients (40%), had a previous alternative cardiac diagnosis. Diagnosis was noninvasive in 75% of ATTR patients. Diagnostic delay was higher in ATTR (2.8±4.3 vs 0.6±0.7 years, P.001), but mortality was greater in AL patients (48% vs 32%, P=.028). Independent predictors of mortality were AL subtype (HR, 6.16; 95%CI, 1.56-24.30; P=.01), female sex (HR, 2.35; 95%CI, 1.24-4.46; P=.01), and NYHA functional class III-IV (HR, 2.07; 95%CI, 1.11-3.89; P=.02).CA is a clinical challenge, with wide variability in its presentation depending on the subtype, leading to diagnostic delay and high mortality. Improvements are needed in the early diagnosis and treatment of these patients.

Details

ISSN :
18855857
Volume :
74
Database :
OpenAIRE
Journal :
Revista Española de Cardiología (English Edition)
Accession number :
edsair.doi.dedup.....0035388ad75825ae2856c0d8d70f6df8
Full Text :
https://doi.org/10.1016/j.rec.2019.12.020