Amyloid cardiomyopathy in a large integrated health care system

Light Chain (AL) and transthyretin (ATTR) amyloidosis are the most common forms of amyloid cardiomyopathy. Population based studies describing the epidemiology and clinical features of amyloid cardiomyopathy are often based in tertiary medical centers and thus may be limited by referral bias. We per...

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Bibliographic Details
Published in:The American heart journal 2019-10, Vol.216, p.42-52
Main Authors: Czobor, Peter, Hung, Yun-Yi, Baer, David, McGlothlin, Dana, Weisshaar, Dana, Zaroff, Jonathan
Format: Article
Language:English
Subjects:
Age Factors
Aged
Aged, 80 and over
Amyloid Neuropathies, Familial - blood
Amyloid Neuropathies, Familial - mortality
Amyloid Neuropathies, Familial - physiopathology
Amyloidosis
Anticoagulants
Biomarkers
Biopsy
Brain natriuretic peptide
California
Cardiomyopathies - blood
Cardiomyopathies - mortality
Cardiomyopathies - physiopathology
Cardiomyopathy
Cardiovascular disease
Cause of Death
Cohort Studies
Comorbidity
Congestive heart failure
Coronary artery
Coronary artery disease
Coronary Artery Disease - mortality
Delivery of Health Care, Integrated
Demographics
Diabetes mellitus
Diabetes Mellitus - mortality
Diagnosis
Echocardiography
Epidemiology
Female
Health care
Health care facilities
Heart failure
Heart Failure - etiology
Heart Failure - mortality
Hospitalization
Humans
Immunoglobulin Light-chain Amyloidosis - blood
Immunoglobulin Light-chain Amyloidosis - mortality
Immunoglobulin Light-chain Amyloidosis - physiopathology
Kaplan-Meier Estimate
Laboratories
Male
Medical prognosis
Medical records
Middle Aged
Mortality
Natriuretic Peptide, Brain - blood
Patients
Peptides
Population studies
Proportional Hazards Models
Statistical models
Stroke Volume
Transthyretin
Treatment Outcome
Ventricle
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Summary:Light Chain (AL) and transthyretin (ATTR) amyloidosis are the most common forms of amyloid cardiomyopathy. Population based studies describing the epidemiology and clinical features of amyloid cardiomyopathy are often based in tertiary medical centers and thus may be limited by referral bias. We performed a cohort study of 198 patients diagnosed and treated in the Kaiser Permanente Northern California health care system who had a confirmed diagnosis of cardiac amyloidosis between 2001 and 2016. Associations between demographic, clinical, laboratory and imaging data and patient outcomes were quantified using multivariable Cox proportional hazard models for both the AL and ATTR groups. The average length of follow up was 2.8 years (SD 2.9 years) and overall survival was 69.1 percent at one year and 35.4 percent at five years. In the AL group, lower left ventricular ejection fraction (HR 1.33 per 5-point decrease, P 
ISSN:0002-8703
1097-6744
DOI:10.1016/j.ahj.2019.06.008