Clinical profile and outcome of cardiac amyloidosis in a Spanish referral center
| Autor: | 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 |
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| Rok vydání: | 2021 |
| Předmět: |
Adult
Male medicine.medical_specialty Delayed Diagnosis 030204 cardiovascular system & hematology 03 medical and health sciences 0302 clinical medicine Internal medicine medicine Humans Prealbumin Referral and Consultation Aged Aged 80 and over Heart Failure Amyloid Neuropathies Familial biology business.industry Myocardium High mortality Mean age Amyloidosis General Medicine Middle Aged medicine.disease Transthyretin Cardiac amyloidosis Heart failure biology.protein Referral center Female Cardiomyopathies business |
| Zdroj: | Revista Española de Cardiología (English Edition). 74:149-158 |
| ISSN: | 1885-5857 |
| DOI: | 10.1016/j.rec.2019.12.020 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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