Global Longitudinal Strain Alteration of the Left Ventricle in Children with Organic Aciduria: Cardiac Disease in Organic Aciduria.

Introduction: Cardiac complications are well-documented in propionic acidemia (PA), and there are a few reported cases of cardiomyopathies in methylmalonic acidemia (MMA). Left-ventricular global longitudinal strain (LV GLS) measurement is known to be able to detect early ventricular dysfunction, leading potentially to cardiomyopathy. The aim of our study was to evaluate left-ventricular global longitudinal strain (LV GLS) in MMA and PA patients and compare it with the pediatric general population. Methods: In this monocentric retrospective study, 26 patients with organic aciduria (OA) were included. Demographic, clinical, electrocardiographic and echocardiographic data were collected. The mean LV GLS in MMA and PA patients was compared with the GLS in the pediatric general population. Results: The left-ventricular ejection fraction (LVEF) was similar between MMA and PA patients and in the normal range (66.27 ± 6.24% vs. 61.41 ± 11.02%; p = 0.182). LV GLS was significantly lower in PA patients than in MMA patients (-15.8 ± 5.67% vs. -20.6 ± 3.19%; p = 0.011). LV GLS was significantly lower in PA patients when compared with the general pediatric population (p = 0.029). Conclusions: Patients with propionic acidemia may have impaired global longitudinal strain even in the presence of normal LVEF. LV GLS might be a useful tool for cardiac follow-up in pediatric patients with OA.