Epidemiology of Cardiac Amyloidosis–Associated Heart Failure Hospitalizations Among Fee-for-Service Medicare Beneficiaries in the United States

Abstract

Background: Cardiac amyloidosis is a substantially underdiagnosed disease, and contemporary estimates of the epidemiology of amyloidosis are lacking. This study aims to determine the incidence and prevalence of cardiac amyloidosis among Medicare beneficiaries from 2000 to 2012. Methods and Results: Medicare beneficiaries were counted in the prevalence cohort in each year they had (1) ≥1 principal or secondary International Classification of Diseases , Ninth Revision code for amyloidosis and (2) ≥1 principal or secondary International Classification of Diseases, Ninth Revision code for heart failure or cardiomyopathy within 2 years after the systemic amyloidosis code. A beneficiary was counted in the incidence cohort only during the first year in which they met criteria. Primary outcomes included the prevalence and incidence of hospitalizations for cardiac amyloidosis. There were 4746 incident cases of cardiac amyloidosis in 2012 and 15 737 prevalent cases in 2012. There was also a significant increase in the prevalence rate (8 to 17 per 100 000 person-years) and incidence rate (18 to 55 per 100 000 person-years) from 2000 to 2012, most notable after 2006. Incidence and prevalence increased substantially more among men, the elderly, and in blacks. Conclusions: The incidence and prevalence rates of cardiac amyloidosis are higher than previously thought. The incidence and prevalence rates of cardiac amyloidosis among hospitalized patients have increased since 2000, particularly among specific patient subgroups and after 2006, suggesting improved amyloidosis awareness and higher diagnostic rates with noninvasive imaging. In light of these trends, cardiac amyloidosis should be considered during the initial work up of patients ≥65 years old hospitalized with heart failure.

Transthyretin cardiac amyloidosis: an update on diagnosis and treatment

“…81 For example, early screening for ATTR-CA might be useful for initial evaluation of elderly patients with newly diagnosed HFpEF with LVH or elderly patients hospitalized for HF. 2,99 With the emergence of new efficient agents including tafamidis, tailor-made medicine will be needed according to the individual disease stage and clinical phenotype. However, there are several problems to be solved: what should we do with treatment strategies for non-responders?…”

“…ATTR-CA has been increasingly recognized as a cause of HFpEF. 2,99 With the emergence of new efficient agents including tafamidis, tailor-made medicine will be needed according to the individual disease stage and clinical phenotype. 98 Also, with the rise of nuclear imaging techniques, the early noninvasive diagnosis has become possible with high diagnostic accuracy.…”

Hemodynamic Profiles and Their Prognostic Relevance in Cardiac Amyloidosis

“…The present study assessed the prognostic relevance of intracardiac and PAPs in a well-characterized, prospectively followed cohort of CA patients. The major findings of our study were that (1) ATTRwt and AL patients do not differ with respect to hemodynamics, but (2) hemodynamic parameters are of greater prognostic importance for ATTRwt patients compared to AL.…”

“…Once deemed an ultra-rare disease, cardiac amyloidosis (CA) is now considered an important etiology in the diagnostic work-up of heart failure (HF) and an increasing number of cardiologists are confronted with the difficult task of treating these patients [1,2]. In HF, irrespective of left ventricular ejection fraction (LVEF), intracardiac filling pressures and pulmonary arterial pressures (PAPs) play a central pathophysiological role and are among the strongest predictors of adverse outcomes [3,4].…”

Editorial to •Trends, burden, and impact of arrhythmia on cardiac amyloid patients: A 16‐year nationwide study from 1999 to 2014•

“…Cardiac amyloidosis (CA), commonly resulting from extracellular protein fibril deposition throughout the body's organ systems, is an important cause of heart failure 1 . In recent years, the contemporary estimates reporting on CA have shown a significant increase in the prevalence rate (18‐55 per 100 000 person‐years) 2 . The two major forms of CA accounting for approximately 95% of CA are the transthyretin amyloidosis (ATTR) and immunoglobulin light‐chain (AL) types.…”