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, V5–V6 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.
clinicAL CARDIOLOGY
IMAGE IN CARDIOVASCULAR MEDICINE
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
Address for correspondence: Martyna Kuryła, MD, Department of Internal Medicine and Cardiology, Medical University of Warsaw, ul. Lindleya 4, 02–005 Warszawa, Poland, tel: +48 22 5021144, fax: +48 22 5022142, e-mail: martyna.kuryla@gmail.com
Received: 5.02.2022 Accepted: 26.08.2023
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