The objective of analysis was to examine age-related hospital utilization in patients diagnosed with Marfan syndrome (MFS), a rare connective tissue disorder affecting multiple organ systems. This non-interventional, retrospective study analyzed MFS-diagnosed patients with hospital visits between October 2015 and September 2017 in the de-identified Vizient health system database. Study cohorts were defined by age (<18, 18-35, 36-50, 51-64, >65). Univariate comparisons were analyzed using Chi-square tests for categorical and ANOVA for continuous variables. Multivariate general linear regression models were used to identify drivers of length of stay (LOS) and cost. The study population included 9,279 visits across 365 facilities. Comorbidity rates increased with age. Hypertension (1.1% vs 52.1%, p<.0001), PVD (11.9% vs 45.3% p<.0001), and CHF (3.0% vs 26.6%, p<.0001) had the largest increases from the <18 to the 65+ cohort. Some disease-related complications such as arrhythmias (1.2% vs 31.6%, p<.0001) and aortic aneurysm (1.6% vs 16.8%, p<.0001) increased between the <18 and 65+ cohorts while others such as aortic ectasia (11.0% vs 2.3%, p<.0001) and scoliosis/kyphosis (11.8% vs 3.5%, p<.0001) decreased. Older patients were more likely to be inpatients (28.6% 65+ vs 5.9% <18, p<.0001) and have higher mortality (1.6% 65+ vs 0.2% <18, p<.0001). Admission type, LOS and cost varied by cohort. The highest LOS (11.2 days) and cost ($49,590) occurred in the <18 cohort after excluding newborns. Room and board, surgery, and diagnostic testing were the largest sources of cost in the <18 cohort. Age was significantly correlated with 14 of 18 disease-related complications and 15 of 16 comorbidities. In multivariate analyses age was not an independent risk factor for increased utilization, however patient comorbidities and disease-related complications did increase utilization. Hospital resource utilization is high in MFS patients, especially those <18 years old. Disease-related complications and patient comorbidities drive resource use.
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