title: 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.
  
  
  CONCLUSIONS
  To improve QOL in patients with MFS, comprehensive interventions are necessary to assess and manage biobehavioral factors, social support, and disease-related factors.
authors.affiliation: Department of nursing, Grown-Up Congenital Heart Clinic, Heart Vascular and Stroke Institute, Samsung Medical Center, Seoul, Korea.
authors.forename: Ju Ryoung
authors.initials: JR
authors.lastname: Moon
authors.affiliation: Redcloss College of Nursing, Chung-Ang University, Seoul, Korea.
authors.forename: Yong Ae
authors.initials: YA
authors.lastname: Cho
authors.affiliation: Department of Pediatrics, Grown-Up Congenital Heart Clinic, Heart Vascular and Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 135-710, Korea. herzhuh@gmail.com.
authors.forename: June
authors.initials: J
authors.lastname: Huh
authors.affiliation: Department of Pediatrics, Grown-Up Congenital Heart Clinic, Heart Vascular and Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 135-710, Korea.
authors.forename: I-Seok
authors.initials: IS
authors.lastname: Kang
authors.affiliation: Department of Medicine, Division of Cardiology, Heart Vascular and Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Duk-Kyung
authors.initials: DK
authors.lastname: Kim
mesh_terms: D000328:Adult; D000368:Aged; D003710:Demography; D005260:Female; D015438:Health Behavior; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000523:psychology*; D008875:Middle Aged; D008962:Models, Theoretical; D011788:Quality of Life; D056910:Republic of Korea; D012307:Risk Factors; D012944:Social Support