Efficiency of energy-rich therapy for cellular energy metabolic disturbances in children with mitral valve prolapse

Objective: to determine impairments in cell energy metabolism (the levels of amino acids and carnitine) in children with connective tissue dysplasia, such as mitral valve prolapse, and to evaluate the efficiency of energy-rich therapy.Subjects and methods. High-performance liquid chromatography- mass spectrometry was used to determine the levels of carnitines and amino acids in dried blood spots from 57 children with mitral valve prolapse (1–2 variants without regurgitation) and in 38 children having Health Groups 1 and 2. The clinical efficiency of prolonged (12-month) combined use of energy-rich medications (L-carnitine, coenzyme Q10) was evaluated.Results. As compared with the controls, the children with mitral valve prolapse showed a significant change, although not beyond the normal range, in the values of free and bound carnitine and amino acids (primarily methionine). There was a significant increase of these indicators in the blood during 12-month combined energy-rich therapy.Conclusion. The findings suggest that that there are moderate, but significant tissue metabolic changes in children with mitral valve prolapse. The found changes are a rationale for the use of energy-rich therapy in this category of children. Analysis of dried blood spots by liquid chromatography tandem mass spectrometry is recommended for the early diagnosis of cellular energy metabolic disturbances in children with signs of connective tissue dysplasia, such as mitral valve prolapse