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

Abstract

Background: We 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.

Methods: The 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.

Results: The 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.

Conclusion: To improve QOL in patients with MFS, comprehensive interventions are necessary to assess and manage biobehavioral factors, social support, and disease-related factors.

Fig. 1

Research framework. FH of MFS = family history of Marfan syndrome; Ao = sinus of Valsalva of diameter Z (= Z score), indicating the presence of aortic root dilatation (when standardized with respect to age and body size); FEN 1 = fibrillin-1 mutation; EL = ectopia lentis; OP of CV = operation on the cardiovascular system

Fig. 2

Path diagram for the hypothetical model. ^* P < 0.05; ^** P < 0.01. x1 = Age; x2 = Education level; x3 = Economic status; x4 = Family support; x5 = Friend support; x6 = Special support; y1 = Dilatation of sinus of Valsalva; y2 = Fibrillin-1 mutation; y3 = Family history of Marfan syndrome; y4 = Frequency of operations on the cardiovascular system; y5 = Anxiety; y6 = Depression; y7 = Fatigue; y8 = Pain; y9 = Body image; y10 = Physical component score; y11 = Mental component score