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Gepubliceerd in: Netherlands Heart Journal 11/2020

Open Access 05-02-2020 | Heart Beat

A hypertrophic senile heart

Auteurs: I. Arts-Birstonaite, J. Jaspers Focks, C. Camaro, R. Nijveldt

Gepubliceerd in: Netherlands Heart Journal | Uitgave 11/2020

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In this case report we describe a 77-year-old man with a history of bilateral carpal tunnel syndrome who presented with dyspnoea on exertion. The electrocardiogram showed low voltage QRS complexes, while echocardiography demonstrated prominent concentric hypertrophy of the left ventricle with speckled appearance of the myocardium (Fig. 1a). Coronary angiography revealed severe proximal left anterior descending artery stenosis for which intervention was performed. Since symptoms persisted, cardiac magnetic resonance imaging was performed for further evaluation (Fig. 1b, c). The proper late gadolinium enhancement (LGE) images were difficult to obtain due to the inability to suppress the signal of ‘normal’ myocardium—a pathognomonic phenomenon for diffuse amyloid fibrils deposition in the whole myocardium (Fig. 1d, e). Endomyocardial biopsy verified the diagnosis of transthyretin cardiac amyloidosis (ATTR-CA) with Congo red staining of transthyretin fibrils (Fig. 1f).
ATTR-CA exists in mutated and wild-type (ATTRwt) amyloidosis. This type is underestimated among the elderly, especially in patients with aortic stenosis and secondary left ventricular hypertrophy [1]. Studies show its incidence of 25% in octogenarians [1]. In this case, the concomitant presence of carpal tunnel syndrome could have been a clue.
Besides supportive therapy with diuretics, tafamidis has recently been introduced. This benzoxazole derivate binds to transthyretin fibrils, preventing further dissociation to monomers and depositions in the myocardium. It is particularly effective in the early stage of disease (NYHA class I–II) and in ATTRwt [2].
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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 ...

Literatuur
1.
go back to reference Ruberg RL, Grogan M, Hanna M, et al. Transthyretin amyloid cardiomyopathy: JACC state-of-the-art review. J Am Coll Cardiol. 2019;73(22):2872–91.CrossRefPubMed Ruberg RL, Grogan M, Hanna M, et al. Transthyretin amyloid cardiomyopathy: JACC state-of-the-art review. J Am Coll Cardiol. 2019;73(22):2872–91.CrossRefPubMed
2.
go back to reference Maurer MS, Schwartz JH, Gundapaneni B, et al. Tafamidis treatment for patients with transthyretin amyloid cardiomyopathy. N Engl J Med. 2018;379(11):1007–16.CrossRef Maurer MS, Schwartz JH, Gundapaneni B, et al. Tafamidis treatment for patients with transthyretin amyloid cardiomyopathy. N Engl J Med. 2018;379(11):1007–16.CrossRef
Metagegevens
Titel
A hypertrophic senile heart
Auteurs
I. Arts-Birstonaite
J. Jaspers Focks
C. Camaro
R. Nijveldt
Publicatiedatum
05-02-2020
Uitgeverij
Bohn Stafleu van Loghum
Gepubliceerd in
Netherlands Heart Journal / Uitgave 11/2020
Print ISSN: 1568-5888
Elektronisch ISSN: 1876-6250
DOI
https://doi.org/10.1007/s12471-020-01370-9

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