PATIENT CHARACTERISTICS AND HEALTH OUTCOMES IN ADULTS DIAGNOSED WITH MARFAN SYNDROME IN THE UNITED STATES

Author(s)

Noone JM1, Gordon BD2, Zacherle E2, Whitmire S2, Howden R2, Blanchette CM2, Clark LA2
1Precision Health Economics, Davidson, NC, USA, 2University of North Carolina at Charlotte, Charlotte, NC, USA

OBJECTIVES: Marfan syndrome is a rare systemic connective tissue disorder caused by mutations in the gene encoding fibrillin-1 (FBN1). Marfan Syndrome has an estimated prevalence of 1 in 5,000-10,000. Disease manifestations include, but are not limited to, cardiovascular (CV) and pulmonary disorders. Among Marfan patients > 26 years we compared health outcomes, costs and clinical factors. Comparisons were made between CV and pulmonary-related hospital discharges and those not primarily associated with CV or pulmonary manifestations. METHODS: We used data from the 2012 Nationwide Inpatient Sample (NIS) to complete the study. Marfan Syndrome patients >26 years were identified using ICD-9 code 759.82. Patients were stratified by primary diagnosis; CV (CCS:096,097,100,101,103-113,115-118) or pulmonary-related (CCS:128-131,133,134) conditions. Descriptive and inferential statistics were used to compare demographics [race, Charlson Comorbidity Index (CCI), region, and payer information] and healthcare outcomes and charges [length of stay (LOS), mortality, and total charges]. Chi-square and t-tests were used for statistical analysis. The reference group for all analyses included hospital discharges that were not CV or pulmonary-related. RESULTS:

Conference/Value in Health Info

2016-10, ISPOR Europe 2016, Vienna, Austria

Value in Health, Vol. 19, No. 7 (November 2016)

Code

PSY6

Topic

Epidemiology & Public Health

Topic Subcategory

Disease Classification & Coding

Disease

Cardiovascular Disorders, Rare and Orphan Diseases, Respiratory-Related Disorders

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