Vol 30, No 5 (2023)
Image in Cardiovascular Medicine
Published online: 2023-10-27

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Amyloidosis with phenotype of hypertrophic cardiomyopathy

Martyna Kuryła1, Martyna Skrzyńska-Kowalczyk1, Katarzyna Kurnicka1, Katarzyna Perzanowska-Brzeszkiewicz1, Piotr Pruszczyk1, Jacek Grzybowski2, Michał Ciurzyński1
Pubmed: 37933406
Cardiol J 2023;30(5):854-855.


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Cardiology Journal

2023, Vol. 30, No. 5, 854–855

DOI: 10.5603/cj.94041

Copyright © 2023 Via Medica

ISSN 1897–5593

eISSN 1898–018X

Amyloidosis with phenotype of hypertrophic cardiomyopathy

Martyna Kuryła1Martyna Skrzyńska-Kowalczyk1Katarzyna Kurnicka1Katarzyna Perzanowska-Brzeszkiewicz1Piotr Pruszczyk1Jacek Grzybowski2Michał Ciurzyński1
1Department of Internal Medicine and Cardiology, Medical University of Warsaw, Poland
2Department of Cardiomyopathy National Institute of Cardiology, Warsaw, Poland

Address for correspondence: Martyna Kuryła, MD, Department of Internal Medicine and Cardiology, Medical University of Warsaw, ul. Lindleya 4, 02005 Warszawa, Poland, tel: +48 22 5021144, fax: +48 22 5022142, e-mail: martyna.kuryla@gmail.com

Received: 5.02.2022 Accepted: 26.08.2023

This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially.

A 57-year-old woman with a hypertrophic cardiomyopathy (HCM) and end stage renal disease treated with hemodialysis was admitted with symptoms of decompensated heart failure in New York Heart Association class III. The serum levels of N-terminal pro-B-type natriuretic peptide and troponin were elevated (> 35000 pg/mL and 0.161 ng/mL). The electrocardiogram (ECG) revealed sinus rhythm, first degree atrioventricular block (PQ 215 ms) and increased QRS voltages (Sokolow-Lyon criteria of left ventricular [LV] hypertrophy [LVH] 4.5 mV and Cornell index 3.8 mV), repolarization abnormalities with deep T-wave inversion in I, aVL, V5V6 leads reflecting LVH (Fig. 1A). Transthoracic echocardiography showed marked concentric LVH (interventricular septum 23 mm, posterior wall 17 mm) with heterogeneous, speckled pattern of the myocardium (Fig. 1B), evident systolic anterior motion (SAM; Fig. 1C) resulting in moderate telesystolic mitral regurgitation and LV outflow tract obstruction (LVOTO) with peak systolic gradient up to 100 mmHg (Fig. 1D, E). Tissue Doppler assessment confirmed significantly impaired LV relaxation (Fig. 1F). Signs of elevated LV filling pressure with E/e’ ratio 22.5 were observed. LV ejection fraction was not decreased. Intensified hemodialysis resulted in improvement of the patient’s clinical condition. A bone marrow and heart biopsy were performed and a diagnosis of AL amyloidosis with heart and kidney involvement was made. Echocardiography and ECG suggested obstructive HCM (sarcomeric disease), while hematologic evaluation confirmed a diagnosis of HCM caused by AL. Electrocardiographic features of LVH are not typically found in amyloidosis. Echocardiographic findings generally can be present in both HCM and amyloidosis, but LVOTO and SAM occur rarely in amyloidosis. Cardiac amyloidosis is usually diagnosed late because of its non-specific symptoms.

Figure 1. Electrocardiogram and transthoracic echocardiography (TTE); A. Electrocardiogram features of the left ventricular hypertrophy; B. Significant hypertrophy of the interventricular septum and posterior wall, with speckled pattern of the myocardium (arrows); TTE, long axis view; C. Systolic anterior motion (SAM) — displacement of the distal portion of the anterior mitral leaflet obstructing the left ventricular outflow tract (arrow); TTE, long axis view; D. Moderate mitral regurgitation (arrow) as a result of SAM; TTE, color Doppler, four chamber view; E. Spectral Doppler across the LVOT; TTE, continuous wave Doppler; F. Decreased myocardial velocity of the lateral mitral annulus (e’) indicating significantly impaired LV relaxation; AML — anterior mitral leaflet; Ao — aorta; LA — left atrium; LV — left ventricle; LVOT — left ventricular outflow tract.
Conflict of interest: None declared