Marfan Syndrome and Quality of Life in the GenTAC Registry. Academic Article uri icon

Overview

abstract

  • BACKGROUND: Previous small studies suggested reduced quality of life (QOL) for people with Marfan syndrome (MFS) compared with those without MFS. The national registry of GenTAC (Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions) is a longitudinal observational cohort study of patients with conditions that predispose to thoracic aortic aneurysms and dissections, including MFS. At the time of registry enrollment, GenTAC study participants are asked to complete questionnaires about demographics, medical history, health habits, and QOL. OBJECTIVES: This study assessed QOL in GenTAC participants with MFS and identify associated factors using self-reported data. METHODS: QOL was assessed using the 4 subscales of the Physical Component Summary (PCS) of the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36): physical functioning; role limitations due to physical health; bodily pain; and general health. We studied the association of QOL with self-reported demographics, health behaviors, physical impairments, surgeries, comorbid medical conditions, medications, and MFS severity. RESULTS: In the GenTAC registry, 389 adults with MFS completed the SF-36. Mean age was 41 years, 51% were men, 92% were white, and 65% were college graduates. The mean PCS composite score was 42.3. In bivariate analysis, predictors of better QOL included college education, marital status, higher household income, private health insurance, full-time employment, moderate alcohol use, fewer prior surgeries, fewer comorbid conditions, absence of depression, and less severe MFS manifestations. In a multivariable analysis, insurance status and employment remained significant predictors of QOL. CONCLUSIONS: In a large cohort of patients with MFS in the GenTAC registry, health-related QOL was below the population norm. Better QOL was independently associated with socioeconomic factors, not factors related to general health or MFS severity.

publication date

  • June 13, 2017

Research

keywords

  • Aortic Aneurysm, Thoracic
  • Aortic Dissection
  • Marfan Syndrome
  • Quality of Life
  • Registries

Identity

PubMed Central ID

  • PMC5519341

Scopus Document Identifier

  • 85020296116

Digital Object Identifier (DOI)

  • 10.1016/j.jacc.2017.04.026

PubMed ID

  • 28595698

Additional Document Info

volume

  • 69

issue

  • 23