Cardiac Complications of Propionic and Other Inherited Organic Acidemias.

ABSTRACT: Clinical observations and experimental studies have determined that systemic acid-base disturbances can profoundly affect the heart. A wealth of information is available on the effects of altered pH on cardiac function but, by comparison, much less is known about the actions of the organic anions that accumulate alongside H⁺ ions in acidosis. In the blood and other body fluids, these organic chemical species can collectively reach concentrations of several millimolar in severe metabolic acidoses, as in the case of inherited organic acidemias, and exert powerful biological actions on the heart that are not intuitive to predict. Indeed, cardiac pathologies, such as cardiomyopathy and arrhythmia, are frequently reported in organic acidemia patients, but the underlying pathophysiolo

SUBMITTER: Park KC 

PROVIDER: S-EPMC7782273 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature