Structural equation modeling of the quality of life for patients with marfan syndrome

Abstract

BackgroundWe used structural equation modeling to evaluate the quality of life (QOL) for patients with Marfan syndrome (MFS). The goal was to provide guidelines to facilitate the development of interventions and strategies to improve the QOL for patients with MFS.MethodsThe participants fulfilled the Ghent 2 criteria for MFS and they comprised patients who visited the cardiology outpatient department of a tertiary hospital in Seoul, Korea, between August 17, 2013 and April 17, 2014. Demographic, social support, disease-related factors, biobehavioral factors, and QOL data were collected in one-on-one interviews.ResultsThe final analyses included 218 patients. Anxious and depressed patients comprised 63.8 and 71.5 % of the sample, respectively. For the hypothetical model, the goodness-of-fit index = 0.91, normal fit index = 0.93, and comparative fit index = 0.90. The outcome was suitable for the recommended level, so the hypothetical model appeared to fit the data. In patients with MFS, the QOL was affected significantly by social support, disease-related factors, and biobehavioral factors. These variables explained 72.4 % of the QOL in patients with MFS. Biobehavioral factors had the strongest and most direct effects on QOL.ConclusionTo improve QOL in patients with MFS, comprehensive interventions are necessary to assess and manage biobehavioral factors, social support, and disease-related factors.

Systematic review of quality of life in persons with hereditary thoracic aortic aneurysm and dissection diagnoses

“…Five studies reported only on patients with MFS according to Ghent 1 nosology. Five studies included only patients assessed according to the revised Ghent nosology (Ghent 2). One study included patients verified Ghents 1 and 2 criteria, and another study included both patients with verified Ghent 1 (n = 13) and patients without verified diagnosis (n = 11).…”

“…Three studies recruited although both clinic and the Marfan Foundation, one study recruited by the GenTac register,one announced for volunteers on a web‐site and one study did not describe the recruiting process. Six studies had more than 200 participants but the respondents probably represent only a small percentage of the estimated national Marfan population. In most studies, the response rate was low.…”

“…All papers used validated, multi‐dimensional scales to measure QoL. Short Form Medical Survey (SF‐36) was used in 10 of the 17 papers dealing with adults and two studies used Short Form Medical survey (SF‐12). Three studies used the Satisfaction with Life Scale (SWLS), one the Ferrans and Power Quality of life Index, cardiac version (QLI, cardiac III), and one used the Ferrans QLI .…”

“…Twelve studies found decreased QoL in MFS compared to general population (GP), but the results were divergent and conflicting. Figure shows a comparison of the results from the 12 papers using SF‐36 or SF‐12 of the 17 papers of adults with MFS.…”

“…Some studies have found significant associations between sleep complaints; dural ectasia; executive dysfunctions (focus, memory and reasoning, and inhibition), severe scoliosis, painand fatigue to decreased QoL in patients with MFS. Only two studies have found that cardiovascular symptoms were significantly associated to decreased QoL. In contrast to this, several studies found no association between QoL and the biomedical symptoms of MFS.…”