Cardiac MRI as surveillance and risk stratification of arrhythmogenic foci in long-chain hydroxyacyl-CoA dehydrogenase deficiency
Abstract
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.
Fields of Study & Subjects
Metabolism and Genetic Disorders
Peroxisome Proliferator-Activated Receptors
Cardiovascular Function and Risk Factors
Publication details
- Journal / source
- Baylor University Medical Center Proceedings
- Publisher
- Informa UK Limited
- Pages
- 1–3
- Publication date
- 15 July 2026
- Language
- EN