Echocardiographic markers of left ventricular hypertrophy and concentric remodeling – limitations in diagnostics of cardiac amyloidosis, Fabry disease and hypertrophic cardiomyopathy

Autor: Judyta Znamirowska, Mateusz Tometczak, Justyna Topa, Patrycja Pastor, Damian Waksmundzki, Adam Grzebinoga, Katarzyna Mizia-Stec
Jazyk: English<br />Polish
Rok vydání: 2024
Předmět:
Zdroj: Annales Academiae Medicae Silesiensis, Vol 78, Pp 219-225 (2024)
Druh dokumentu: article
ISSN: 1734-025X
DOI: 10.18794/aams/189128
Popis: Introduction: Left ventricular hypertrophy (LVH) is a relevant sign associated with an increased risk of sudden death. The causes of LVH including cardiac amyloidosis (CA), Fabry disease (FD), hypertrophic cardiomyopathy (HCM) are associated with an inauspicious prognosis. Transthoracic echocardiography (TTE) remains the first-step baseline diagnostic method. Material and methods: A retrospective one-center analysis of 86 patients (pts) with increased left ventricular (LV) wall thickness in TTE was performed. The inclusion criteria were interventricular septum (IVS) above 10 mm in males, 9 mm in females and the final diagnosis of CA, FD or HCM. The study population was divided into three subgroups: CA (13 pts), FD (7 pts), HCM (66 pts). The LV mass index (LVMI), relative wall thickness (RWT) and type of remodeling were analyzed. Results: Increased LVMI occurred in 90.9% pts with CA, all with FD, 89.5% with HCM.RWT exceeded the normal range among 92.3% pts with CA, 57.1% with FD, 92.4% with HCM. Concentric hypertrophy was diagnosed in 75% pts with CA, 57.1% with FD, 84.2% with HCM and eccentric in 8.3% pts with CA, 42.9% with FD, 5.3% with HCM (p = 0.01). An abnormal IVS/PWT index was observed in 23.1% pts with CA, 28.6% with FD, 79.7% with HCM (p = 0.00001). Conclusions: Although cardiac hypertrophy is a typical sign, it does not occur in all subjects with CA, FD, HCM. More detailed analysis including the form of hypertrophy as well as left atrium remodeling are required to be characterized for specific diseases: CA, FD, HCM. Asymmetrical hypertrophy is more specific for HCM.
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