Transthyretin amyloid cardiomyopathy in patients with unexplained increased left ventricular wall thickness

Amyloid cardiomyopathy (CA) was previously considered a rare disease; however, rapid advancements in imaging modalities have led to an increased frequency of its diagnosis. The aim of this prospective study was to assess the prevalence and clinical phenotype of transthyretin amyloidosis (ATTR) cardi...

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Published in:The international journal of cardiovascular imaging 2024-08, Vol.40 (8), p.1693-1703
Main Authors: Holcman, Katarzyna, Kostkiewicz, Magdalena, Szot, Wojciech, Ćmiel, Bogdan, Mróz, Krystian, Stępień, Agnieszka, Graczyk, Katarzyna, Dziewięcka, Ewa, Karabinowska-Małocha, Aleksandra, Sachajko, Zuzanna, Podolec, Piotr, Rubiś, Paweł
Format: Article
Language:English
Subjects:
Amyloid
Amyloidosis
Arrhythmia
Atria
Cardiac Imaging
Cardiology
Cardiomyopathy
Carpal tunnel syndrome
Computed tomography
Diagnosis
Echocardiography
EKG
Electrocardiography
Heart
Imaging
Logit models
Low voltage
Medicine
Medicine & Public Health
Multivariate analysis
Myocardium
Original Paper
Phenotypes
Photon emission
Polyneuropathy
Prediction models
Radiology
Single photon emission computed tomography
Spine (lumbar)
Stenosis
Thickening
Thickness
Tomography
Transthyretin
Ventricle
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Summary:Amyloid cardiomyopathy (CA) was previously considered a rare disease; however, rapid advancements in imaging modalities have led to an increased frequency of its diagnosis. The aim of this prospective study was to assess the prevalence and clinical phenotype of transthyretin amyloidosis (ATTR) cardiomyopathy in patients exhibiting unexplained increased left ventricular (LV) wall thickness. From 2020 to 2022, we enrolled 100 consecutive adults with unexplained increased LV wall thickness in the study. The analysis included clinical data, electrocardiography, transthoracic echocardiography, single-photon emission computed tomography/computed tomography with 3,3-disphono-1,2-propanodicarboxylic acid, genetic testing. Overall, 18% of patients were diagnosed with CA, comprising 5% with light-chain amyloidosis, and 12% with ATTR. To evaluate associations with the ATTR diagnosis, a LOGIT model and multivariate analysis were applied. Notably, age, polyneuropathy, gastropathy, carpal tunnel syndrome, lumbar spine stenosis, low voltage, ventricular arrhythmia, LV mass, LV ejection fraction, global longitudinal strain (GLS), E/A, E/E′, right ventricle (RV) thickness, right atrium area, RV VTI, TAPSE, apical sparing, ground glass appearance of myocardium, thickening of interatrial septum, thickening of valves, and the “5–5–5” sign were found to be significantly associated with ATTR (p 
ISSN:1875-8312
1569-5794
1875-8312
1573-0743
DOI:10.1007/s10554-024-03158-z