Key result
Serial cardiac MRI in LCHAD deficiency reveals stable myocardial scar but evolving septal hypertrophy.
Why the study?
LCHAD deficiency is a rare fatty-acid oxidation disorder that can cause subclinical cardiac involvement, including fibrosis and arrhythmias.
Does serial cardiac MRI detect arrhythmogenic substrates in patients with LCHAD deficiency?
Case Report (n=1)
Does serial cardiac MRI detect arrhythmogenic substrates in patients with LCHAD deficiency?
Cardiac MRI is useful for early detection and longitudinal surveillance of arrhythmogenic substrates in LCHAD deficiency.
Hypothesis-generating for cardiac MRI surveillance in LCHAD deficiency; prospective trials needed before clinical adoption.
Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency is a rare fatty-acid oxidation disorder that can cause subclinical cardiac involvement, including fibrosis and arrhythmias. We present a 22-year-old woman with LCHAD deficiency who demonstrated stable myocardial scar burden but evolving septal hypertrophy on serial cardiac MRI despite preserved ventricular function. This case highlights MRI's role in early detection and longitudinal surveillance of arrhythmogenic substrates.
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Kantheti et al. (2026) conducted a case report in Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency (n=1). Serial cardiac MRI was evaluated. Serial cardiac MRI in a 22-year-old woman with LCHAD deficiency revealed stable myocardial scar burden but evolving septal hypertrophy despite preserved ventricular function.
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