000008416 001__ 8416 000008416 005__ 20240416125319.0 000008416 0247_ $$2DOI$$a10.6083/cj82k793z 000008416 037__ $$aIR 000008416 041__ $$aeng 000008416 245__ $$aSudden cardiac death in young adults with long-chain 3-hydroxyacyl coa dehydrogenase deficiency (LCHADD) 000008416 260__ $$bOregon Health and Science University 000008416 269__ $$a2020 000008416 336__ $$aAbstract 000008416 520__ $$aLong-chain 3-Hydroxyacyl CoA Dehydrogenase Deficiency (LCHADD) is an autosomal recessive defect in fatty acid oxidation that presents with hypoketotic hypoglycemia and/or hypertrophic cardiomyopathy in infancy, and recurrent rhabdomyolysis in adolescence, however, sudden cardiac death has not been a previously reported complication of LCHADD. We have conducted a case review study comparing young adult LCHADD patients who have experienced sudden cardiac arrest events (n=5) to similar patients who have not (n=5) for the purpose of evaluating associated cardiac risk factors. 000008416 540__ $$fCC BY 000008416 542__ $$fIn copyright - joint owners 000008416 650__ $$aDeath, Sudden, Cardiac$$029385 000008416 650__ $$aStem Cell Research$$039025 000008416 650__ $$aCardiology$$016143 000008416 650__ $$aFatty Acids$$018911 000008416 650__ $$aMyocardial Infarction$$022601 000008416 650__ $$aRare Diseases$$034662 000008416 650__ $$aAdolescent$$014285 000008416 650__ $$aYoung Adult$$038480 000008416 650__ $$aCardiomyopathy, Hypertrophic$$016146 000008416 650__ $$aRisk Factors$$025522 000008416 6531_ $$afatty acid oxidation disorders 000008416 6531_ $$aLCHAD deficiency 000008416 6531_ $$aheart attack 000008416 6531_ $$agenetic diseases 000008416 691__ $$aOHSU-PSU School of Public Health$$041366 000008416 692__ $$aDepartment of Molecular and Medical Genetics$$041428 000008416 7001_ $$aSaini, Ajesh$$uOHSU/PSU 000008416 7001_ $$aGonzales-De Alba, Cesar$$uOregon Health and Science University$$041354 000008416 711__ $$aResearch Week$$uOregon Health and Science University$$d2020 000008416 7201_ $$aDevine, Tiffany$$uOregon Health and Science University$$041354$$eCollaborator$$7Personal 000008416 8564_ $$960a14927-bc87-407a-adbc-1176d4d06abe$$s137299$$uhttps://digitalcollections.ohsu.edu/record/8416/files/ResearchWeek.2020.Saini.Ajesh.pdf 000008416 905__ $$a/rest/prod/cj/82/k7/93/cj82k793z 000008416 980__ $$aResearch Week