Key result
Adults with long-chain fatty acid β-oxidation disorders had higher left ventricular myocardial mass (P=0.011) and mass-to-volume ratio (P=0.008) than matched controls.
Why the study?
Cardiomyopathy is a severe complication in long-chain fatty acid beta-oxidation disorders (LCFAOD), but it was unknown whether latent cardiac abnormalities exist in adult patients.
Does long-chain fatty acid β-oxidation deficiency alter heart function, myocardial tissue characteristics, and myocardial lipid content in adult patients?
Comparison
Adult LCFAOD patients vs gender-, age-, and BMI-matched control subjects
Design
Case-control study
Authors
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Alerts clinicians to possible subclinical cardiac remodeling in adult LCFAOD; hypothesis-generating and requires prospective confirmation before practice change.
Case-Control (n=28)
Does long-chain fatty acid β-oxidation deficiency alter heart function, myocardial tissue characteristics, and myocardial lipid content in adult patients?
p-value: p=0.011
Adult patients with LCFAOD exhibit subclinical hypertrophic left ventricular remodeling and altered LV torsion despite normal ejection fraction, suggesting a chronic cardiac disease phenotype driven by energy deficiency.
Knottnerus et al. (2020) conducted a case-control in Long-chain fatty acid β-oxidation disorders (LCFAOD) (n=28). Long-chain fatty acid β-oxidation disorders (LCFAOD) vs. Matched control subjects was evaluated on Left ventricular (LV) myocardial mass (p=0.011). Adults with long-chain fatty acid β-oxidation disorders had higher left ventricular myocardial mass (P=0.011) and mass-to-volume ratio (P=0.008) than matched controls.
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