Ketogenic Diet: Rapid Onset of Selenium Deficiency-Induced Cardiac Decompensation

Abstract

Selenium-deficiency cardiomyopathy is a known secondary complication from long-term treatment with a ketogenic diet for medical refractory epilepsy. Our patient, a 5-year-old boy on a ketogenic diet for intractable seizures, had a normal selenium level before starting the diet, but he shortly thereafter developed acute reversible cardiomyopathy and ventricular tachycardia, which was unmasked during a hospitalization for an elective operative procedure. His cardiomyopathy was suspected to be secondary to a selenium-deficient state and was confirmed by way of a markedly low serum selenium level and supported by rapid improvement with the initiation of selenium supplementation and cessation of the ketogenic diet. For patients being initiated on a ketogenic diet, current screening guidelines call for baseline and follow-up selenium levels every 3 months during the first year along with RDA selenium supplementation, which is 30 mcg/day. Most of the new ketogenic diet formulas meet this requirement. Our patient underwent elective surgery before his planned 3-month selenium level check and had potentially preventable complications. Secondary to this experience, we suggest that all patients initiated on a ketogenic diet should have a preoperative electrocardiogram (EKG), an echocardiogram, and selenium level determined before any elective surgery. These steps would prevent unnecessary perioperative morbidity and mortality.

Pediatric Cardiomyopathies: causes, epidemiology, Clinical course, Preventive Strategies and Therapies

“…The long-term use of the ketogenic diet in children with refractory seizure disorders is a risk factor of selenium-associated cardiomyopathy. Selenium supplements and the cessation of a ketogenic diet can reverse this cardiomyopathy [215]. …”

Biochemical assessment of patients following ketogenic diets for epilepsy: Current practice in the UK and Ireland

“…Individuals following a KD have been shown to have reduced serum 25‐hydroxyvitamin D concentration (as were individuals solely on antiepileptic drugs) and reduced bone mass (to a greater extent than in individuals solely on drug therapy); another study found 25‐hydroxyvitamin D levels (which were mostly low at diet initiation) to improve initially on commencement of a KD, including supplementation, but to decline after three months . Selenium deficiency has been reported in 66 individuals on KD treatment, associated with cardiomyopathy in two of these patients and sudden cardiac death in another two . A trend of decreasing plasma selenium was noted in participants of the original randomized controlled trial at GOSH, with a significant decrease between baseline and six months in children on the classical KD, although mean plasma selenium was maintained within the GOSH reference ranges …”

Recomendaciones para el manejo clínico pediátrico de la dieta cetogénica en el tratamiento de la epilepsia refractaria

“…Aunque es infrecuente, por su gravedad, es indispensable la evaluación cardiológica periódica. [24][25][26] Con respecto al efecto que la dieta ejerce sobre el crecimiento, los últimos reportes indican que, con aporte calórico y proteico adecuados, se logra un ritmo de crecimiento normal. 27,28 En cada consulta, los datos de antropometría se evalúan y comparan con los patrones de crecimiento recomendados por la Sociedad Argentina de Pediatría.…”