Why the study?
Cardiomyopathies and arrhythmias are common in organic acidemia, but the underlying pathophysiological processes and the role of accumulating organic anions such as propionate remain unclear.
This review highlights the pathophysiological role of organic anions, particularly propionate, in the development of cardiomyopathies and arrhythmias in patients with propionic acidemia.
May warrant cardiac surveillance in propionic acidemia; leaves open whether anion-targeted therapies alter outcomes.
Systemic acid-base imbalances significantly influence the heart, both clinically and experimentally. Extensive knowledge exists regarding the impact of pH on cardiac function, yet the role of organic anions accumulating in acidosis is less explored. In severe metabolic acidosis cases, such as hereditary organic acidemias, these organic compounds can reach millimolar concentrations in blood and other body fluids, exerting significant physiological effects on the heart. Cardiomyopathies and arrhythmias are common in patients with organic acidemia, although the underlying pathophysiological processes remain unclear. Research into organic anion physiology, particularly concerning propionate - which accumulates in propionic acidemia (PA), a form of organic acidemia commonly associated with cardiac illness - has increased substantially in recent years. The purpose of this review is to provide an overview of the cardiac sequelae observed in PA patients who suffer from cardiac diseases.
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Lütfü Aşkın (2024) studied this question.
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