(P066) CARDIAC MANIFESTATIONS IN CHILDREN AND ADULTS WITH LONG-CHAIN FATTY ACID OXIDATION DISORDERS (LC-FAOD): A UNITED STATES RETROSPECTIVE CLAIMS DATABASE ANALYSIS

Background: The goals of this study were to better understand cardiac manifestations and long-term outcomes in individuals with LC-FAOD.

METHODS AND RESULTS: This analysis was done using IQVIA PharMetrics® claims data. Individuals with confirmed LC-FAOD were identified and a non–LC-FAOD comparator group was created using 1:20 exact matching for age, gender, payer type, and enrollment start year. Cardiac-related complications were examined using ICD-10 diagnostic codes at any billing point during the study period. Cardiac diagnoses and assessments were evaluated using Current Procedural Terminology (CPT®) codes in a non-inpatient setting.

A total of 337 pediatric (peds) and 86 adult individuals with LC-FAOD, and 6,740 pediatric and 1,720 adult controls, were included in the study. Cardiomyopathy (peds: 8.0%, adults: 10.5%) and arrhythmias (peds: 8.9%, adults: 14.0%) were common amongst individuals with LC-FAOD and were significantly higher than in comparators. LC-FAOD adults had significantly more cardiac conditions compared with pediatric patients and comparators. The most common cardiac conditions in adults with LC-FAOD were chronic ischemic heart disease (18.6%), non-rheumatic mitral or tricuspid valve disorders (11.6% and 12.8%, respectively), heart failure (10.5%), atrioventricular block and left bundle branch block (7.0%), and cardiomegaly (5.1%). Individuals with LC-FAOD underwent significantly more cardiac-related procedures than comparators. Electrocardiograms (peds: 35.9%; adults: 77.9%) and echocardiograms (peds: 44.8%; adults: 53.5%) were commonly used in individuals with LC-FAOD.

Conclusion: Cardiac issues are common in adults and children with LC-FAOD. This analysis shows the importance of assessing the cardiac status of individuals with LC-FAOD. Additional and more detailed analyses as well as natural history studies are needed to inform healthcare providers of cardiac risks associated with LC-FAOD and reduce morbidity and mortality for these individuals.