Skip to main content
main-content
Top

Tip

Swipe om te navigeren naar een ander artikel

29-07-2019 | Review Article | Uitgave 11/2019 Open Access

Netherlands Heart Journal 11/2019

Cardiac amyloidosis: the need for early diagnosis

Tijdschrift:
Netherlands Heart Journal > Uitgave 11/2019
Auteurs:
M. I. F. J. Oerlemans, K. H. G. Rutten, M. C. Minnema, R. A. P. Raymakers, F. W. Asselbergs, N. de Jonge
Belangrijke opmerkingen

Electronic supplementary material

The online version of this article (https://​doi.​org/​10.​1007/​s12471-019-1299-1) contains supplementary material, which is available to authorized users.

Abstract

Amyloidosis is a collection of systemic diseases characterised by misfolding of previously soluble precursor proteins that become infiltrative depositions, thereby disrupting normal organ structure and function. In the heart, accumulating amyloid fibrils lead to progressive ventricular wall thickening and stiffness, resulting in diastolic dysfunction gradually progressing to a restrictive cardiomyopathy. The main types of cardiac amyloidosis are amyloid light chain (AL) amyloidosis caused by an underlying plasma cell dyscrasia, amyloid transthyretin (TTR) amyloidosis of wild-type (normal) TTR at older age (ATTRwt) and hereditary or mutant amyloid TTR (ATTRm) in which a genetic mutation leads to an unstable TTR protein. Overall survival is poor once heart failure develops, underlining the need for early referral and diagnosis. Treatment for AL amyloidosis has improved markedly over the last decades, and TTR amyloidosis gene silencers and orally available transthyretin stabilisers are ready to enter the clinical arena after recent positive outcome trials. Novel therapies aiming at fibril degradation with monoclonal antibodies are under investigation. In this review, we focus on ‘red flag’ signs and symptoms, diagnosis and management of cardiac amyloidosis which differs considerably from the general management of heart failure. Only by increasing awareness, prognosis for patients with this devastating disease can be improved.

Onze productaanbevelingen

Netherlands Heart Journal

Het Netherlands Heart Journal wordt uitgegeven in samenwerking met de Nederlandse Vereniging voor Cardiologie en de Nederlandse Hartstichting. Het tijdschrift is Engelstalig en wordt gratis beschikbaa ...

Extra materiaal
Literatuur
Over dit artikel

Andere artikelen Uitgave 11/2019

Netherlands Heart Journal 11/2019 Naar de uitgave