Two Decades of Cardiac Amyloidosis

Abstract

Background Cardiac amyloidosis (CA) has been associated with poor outcomes. Screening studies suggest that CA is overlooked—especially in the elderly. Recent advances in treatment have brought attention to the disease, but data on temporal changes in CA epidemiology are sparse. Objectives The aim of this work was to describe all patients with CA in Denmark, examining changes in patient characteristics from 1998 to 2017. Methods All patients with any form of amyloidosis diagnosed from 1998 to 2017, as well as their comorbidities and pharmacotherapy, were identified in Danish nationwide registries. CA was defined as any diagnosis code for amyloidosis combined with a diagnosis code for heart failure, cardiomyopathy, or atrial fibrillation or a procedural code for pacemaker implantation, regardless of the order. The index date was defined as the date of meeting those criteria. Patients were divided into 5-year periods by index date. For comparison, we also included control subjects (1:4 ratio) from the general population. Results CA criteria were met by 619 patients. Comparing 1998-2002 vs 2013-2017, the median age at baseline increased from 67.4 years (interquartile range [IQR]: 53.9-75.2 years) to 72.3 years (IQR: 66.0-79.3 years). The frequency of male patients increased from 62.1% to 66.2%. The incidence of CA rose from 0.88 to 3.56 per 100,000 person-years in the Danish population aged ≥65 years, and the 2-year mortality decreased from 82.6% (IQR: 69.9%-90.5%) to 50.2% (IQR: 43.1%-56.9%). Compared with control subjects, the mortality among CA patients was significantly higher (log-rank test: P < 0.0001). Conclusions CA, as defined in this study, was increasingly diagnosed on a national scale. The increasing frequency of male patients and median age suggest that wild-type transthyretin amyloidosis is driving this increase. Greater recognition of earlier, less advanced cases might explain decreasing mortality.

Prevalence, characteristics, and mortality of patients with transthyretin amyloid cardiomyopathy in the Nordic countries

“…1 ATTR-CM is associated with poor quality of life 2 and half of all ATTR-CM patients die within 4 years of diagnosis. [3][4][5][6] Despite the severity of the disease, diagnosis is often delayed or ATTR-CM remains undiagnosed due to the diffuse symptoms of the disease 2 and lack of awareness among healthcare professionals. 1,7 One reason for insufficient diagnostic attention to ATTR-CM in the medical community is lack of reliable knowledge on epidemiology of the disease as most studies have focused on smaller series of patients referred to tertiary care centres.…”

“…ATTR‐CM is associated with poor quality of life 2 and half of all ATTR‐CM patients die within 4 years of diagnosis 3–6 . Despite the severity of the disease, diagnosis is often delayed or ATTR‐CM remains undiagnosed due to the diffuse symptoms of the disease 2 and lack of awareness among healthcare professionals 1,7 .…”

Healthcare resource use of patients with transthyretin amyloid cardiomyopathy

“…9 Therefore, early diagnosis of ATTR-CM is essential to ensure that patients can benefit from disease-modifying treatment in order to maximize quality of life and reduce the need for healthcare interactions. 9,10 Although the epidemiology of ATTR-CM is beginning to emerge in published research using register data, 8,[11][12][13] treatment patterns for ATTR-CM patients and their healthcare resource use are not well documented. There is a large body of work on the humanistic and economic burden of heart failure from around the world, [14][15][16] but only few studies on the healthcare resource use of ATTR-CM.…”

“…Although the epidemiology of ATTR‐CM is beginning to emerge in published research using register data, 8,11–13 treatment patterns for ATTR‐CM patients and their healthcare resource use are not well documented. There is a large body of work on the humanistic and economic burden of heart failure from around the world, 14–16 but only few studies on the healthcare resource use of ATTR‐CM 17–19 .…”

A 65-Year-Old Woman with an Enlarged Tongue Due to Amyloidosis

“…For instance, the reported incidence in the UK was around 500 new cases annually, with an estimated 0.5–1.0 deaths/100 patients [ 3 ]. The reported incidence in the Danish population was 3.56 per 100 000 person-years [ 4 ]. Amyloidosis can be hereditary in origin or acquired as a complication of a primary disease with unclear patho-physiology [ 2 ].…”