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Fabry Disease — EECC MCQ

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HardCardiomyopathyFabry DiseaseEECC

A patient with hereditary haemochromatosis develops exertional dyspnoea and ventricular ectopy. Echocardiography shows mild biventricular dysfunction but cannot quantify myocardial iron. Which CMR parameter most directly measures the iron burden that should guide chelation or venesection response?

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Correct answer: B — Myocardial T2-star relaxation time measured in the ventricular septum

Paramagnetic iron creates local magnetic-field inhomogeneity and shortens myocardial T2-star, making septal T2-star the established direct CMR measure used to quantify cardiac iron and monitor response. Native T1 may fall with iron and can provide complementary tissue information but is not the standard quantitative replacement for T2-star. Extracellular-volume fraction estimates interstitial expansion, LGE depicts focal fibrosis or scar, and strain measures myocardial mechanics; none quantifies iron burden as directly.

Reference: 2023 ESC Guidelines for cardiomyopathies. https://academic.oup.com/eurheartj/article/44/37/3503/7246608