1. Cardiac 'hypertrophy' phenotyping: differentiating aetiologies with increased left ventricular wall thickness on echocardiography
- Author
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Aaisha Ferkh, Catherina Tjahjadi, Luke Stefani, Paul Geenty, Karen Byth, Kasun De Silva, Anita C. Boyd, David Richards, Peter Mollee, Dariusz Korczyk, Mark S. Taylor, Fiona Kwok, Eddy Kizana, Arnold C. T. Ng, and Liza Thomas
- Subjects
cardiac amyloidosis ,Fabry disease ,cardiac hypertrophy ,infiltrative cardiomyopathies ,echocardiography ,strain imaging ,Diseases of the circulatory (Cardiovascular) system ,RC666-701 - Abstract
AimsDifferentiating phenotypes of cardiac “hypertrophy” characterised by increased wall thickness on echocardiography is essential for management and prognostication. Transthoracic echocardiography is the most commonly used screening test for this purpose. We sought to identify echocardiographic markers that distinguish infiltrative and storage disorders that present with increased left ventricular (LV) wall thickness, namely, cardiac amyloidosis (CA) and Anderson–Fabry disease (AFD), from hypertensive heart disease (HHT).MethodsPatients were retrospectively recruited from Westmead Hospital, Sydney, and Princess Alexandra Hospital, Brisbane. LV structural, systolic, and diastolic function parameters, as well as global (LVGLS) and segmental longitudinal strains, were assessed. Previously reported echocardiographic parameters including relative apical sparing ratio (RAS), LV ejection fraction-to-strain ratio (EFSR), mass-to-strain ratio (MSR) and amyloidosis index (AMYLI) score (relative wall thickness × E/e′) were evaluated.ResultsA total of 209 patients {120 CA [58 transthyretin amyloidosis (ATTR) and 62 light-chain (AL) amyloidosis], 31 AFD and 58 HHT patients; mean age 64.1 ± 13.7 years, 75% male} comprised the study cohort. Echocardiographic measurements differed across the three groups, The LV mass index was higher in both CA {median 126.6 [interquartile range (IQR) 106.4–157.9 g/m2]} and AFD [median 134 (IQR 108.8–152.2 g/m2)] vs. HHT [median 92.7 (IQR 79.6–102.3 g/m2), p
- Published
- 2023
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