OBJECTIVES

: Marfan syndrome (MFS) is a rare disorder affecting connective tissue and impacting multiple organ systems. Limited data exists describing hospital utilization in MFS patients. The objective of analysis was to describe MFS patient hospital utilization and examine acute care services in the MFS population.

METHODS

: This non-interventional, retrospective study analyzed a cross-section of MFS-diagnosed patients with hospital visits between October 2015 and September 2017 in the de-identified Vizient health system database, which consists of over 400 hospitals across the United States.

RESULTS

: The study population included 9,279 visits across 4,748 unique patients. Males represented 52.0% of the population. Average age was 35.5 with 22.2% of the population under the age of 18. Nearly 70% of visits were in 300+ bed, teaching facilities and 96% of visits were in urban hospitals Payer type was 39% commercial, 17% Medicaid, 20% Medicare, and 24% other/unknown.

The average Charlson Comorbidity Index was 1.2. The most prevalent comorbidities included peripheral vascular disease (PVD, 30.0%), hypertension (22.3%), chronic pulmonary disease (CPD, 18.8%), and congestive heart failure (CHF, 11.7%). Although nicotine dependence was high (13.5%), alcohol dependence was low (1.1%). The most common disease-related complications included aortic aneurysm (11.6%), eye disorders (6.6%), mitral valve disorders (6.6%), scoliosis/kyphosis (6.2%), asthma (6.1%), and aortic ectasia (5.9%).

Utilization was primarily outpatient (83.6%) with 31.3% of all visits classified as urgent or emergency. Inpatient length of stay was 7.7 days with an average cost of $22,472 per visit. Average cost of outpatient visits was $1,412. Average inpatient payment was $24,580 while average outpatient payment was $1,471. In-hospital mortality rate was low with 0.5% overall mortality and 2.4% mortality for inpatients.

CONCLUSIONS

: MFS patients incur substantial health care utilization and costs.