Cardio-Oncology Resource Center

Cardio-Oncology Resource Center

Disclosures: Westin reports receiving grants from the Arvid Nilssons Fond, Frimodt-Heineke Fonden, Hjertecentrets Forskningsudvalg, Højmosegård-legatet, Erik og Susanna Olesens Almenvelgørende Fond, Pfizer and Rigshospitalet. Please see the study for all other authors’ relevant financial disclosures. Wechalekar reports no relevant financial disclosures.
November 09, 2021
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Incidence of cardiac amyloidosis on the rise, mortality improving

Disclosures: Westin reports receiving grants from the Arvid Nilssons Fond, Frimodt-Heineke Fonden, Hjertecentrets Forskningsudvalg, Højmosegård-legatet, Erik og Susanna Olesens Almenvelgørende Fond, Pfizer and Rigshospitalet. Please see the study for all other authors’ relevant financial disclosures. Wechalekar reports no relevant financial disclosures.
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The incidence of cardiac amyloidosis in the Danish population increased in the last 2 decades, translating to a potential increase in the global population, according to research published in JACC: CardioOncology.

Cardiac amyloidosis (CA) has been associated with a high 5-year mortality of 44% to 65% after diagnosis. Screening studies show higher prevalence of occult CA than previously expected, indicating that the burden of CA is underestimated,” Oscar Westin, MD, from the Heart Center at the University Hospital of Copenhagen, Denmark, and colleagues wrote. “Recent advances in diagnostic modalities, such as bone tracer scintigraphy, cardiac magnetic resonance imaging, and mass spectrometry, as well as state-of-the-art pharmacotherapy have brought increased attention to identifying patients with transthyretin amyloidosis ... However, no unselected national data exist. Our objective was therefore to describe the temporal trends in a Danish population of CA patients and to examine the changes in patient characteristics over the past 2 decades.”

For this analysis, researchers utilized Danish national registries to identify all patients with any form of amyloidosis 1998 and 2017 as well as their comorbidities and pharmacotherapy.

A total of 1,572 patients in Denmark were diagnosed with amyloidosis in the prior 2 decades, of which 619 met the criteria for cardiac amyloidosis.

According to the study, cardiac amyloidosis was defined as any amyloidosis diagnosis with a concomitant diagnosis of HF, cardiomyopathy or atrial fibrillation or a procedural code for pacemaker implantation.

Cardiac amyloidosis in Danish population

The median age at initial cardiac amyloidosis diagnosis increased from 67.4 years in 1998-2002 to 72.3 years in 2013-2017.

Researchers among those receiving a diagnosis of cardiac amyloidosis, the percentage of men increased from 62.1% to 66.2% during the study period.

The overall incidence of cardiac amyloidosis in the Danish population aged 65 years of more also increased from 0.88 per 100,000 person-years to 3.56 per 100,000 person-years.

The researchers stated that because the rising incidence of cardiac amyloidosis is chiefly in elderly men, it is likely primarily driven by wild-type transthyretin amyloidosis.

Although 2-year mortality among patients with cardiac amyloidosis decreased from 82.6% in 1998-2002 to 50.2% in 2013-2017, mortality among patients with cardiac amyloidosis remained higher compared with the general population (P < .0001).

“CA is increasingly diagnosed in Denmark, and the increasing frequency of male patients and median age at diagnosis suggests that [wild-type transthyretin amyloidosis] is driving the increase. Greater early recognition and resultant less advanced cases might explain decreasing mortality, as the trends uncovered in this retrospective study are observed before national approval of disease-modifying drugs for [transthyretin amyloidosis],” the researchers wrote. “Future studies on clinical outcomes in CA are needed as novel disease-modifying drugs are approved for use in treatment of both [light chain amyloidosis] and [transthyretin amyloidosis], revolutionizing the therapeutic possibilities and further drawing important attention to the disease.”

“Amyloidosis is no longer rare”

In a related editorial Ashutosh D. Wechalekar, MBBS, DM, of the National Amyloidosis Centre at University College London, discussed the implications of these findings as well as future of amyloidosis care.

“With more than half a billion persons projected to be aged 80 years or older by 2050, a substantially larger population will be at risk of developing cardiac [transthyretin] amyloidosis and will also have higher risk of monoclonal gammopathies; hence, increasing the risk of developing [light chain] amyloidosis,” Wechalekar wrote. “The ever-increasing incidence and recognition make amyloidosis a new global health care problem waiting in the wings.

“Amyloidosis is no longer rare. Physicians must think of amyloidosis early as differential diagnosis to initiate specific confirmatory investigations or a tissue biopsy enabling early diagnosis to allow for more effective treatments and better outcomes,” Wechalekar wrote.

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