OBJECTIVES

: 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.

METHODS

: 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.

RESULTS

: 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.

CONCLUSIONS

: Hospital resource utilization is high in MFS patients, especially those <18 years old. Disease-related complications and patient comorbidities drive resource use.