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Metabolic cardiomyopathy from propionic acidemia precipitating cardiac arrest in a 25-year-old man
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Metabolic cardiomyopathy from propionic acidemia precipitating cardiac arrest in a 25-year-old man
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Journal Article
Metabolic cardiomyopathy from propionic acidemia precipitating cardiac arrest in a 25-year-old man
2018
Overview
A 25-year-old man with a medical history of propionic acidemia was brought to hospital after he collapsed while jogging. He was unresponsive, received immediate bystander cardiopulmonary resuscitation and was defibrillated by paramedics from an initial rhythm of ventricular fibrillation with successful return of spontaneous circulation. He had been diagnosed with propionic acidemia at two years of age when he presented with developmental delay, and metabolic work-up showed the classic profile of 3-OH-propionic and methylcitric acids on analysis of organic acids in urine, low carnitine with increased propionylcarnitine on acylcarnitine profile and increased glycine on quantitative amino acid analysis of plasma. At the time of the patient's witnessed cardiac arrest, initial laboratory investigations showed an anion gap metabolic acidosis and lactic acidemia (lactate level 7.9 mM). Although the patient's ventricular arrhythmia may have been due to a reversible cause, we decided to proceed with insertion of an implantable cardioverter-defibrillator. We based this on
