Determinants of quality of life in Marfan syndrome.

BACKGROUND

Marfan syndrome (MFS) is a rare, heritable disorder that affects connective tissue. Men and women are equally affected. Clinical manifestations involve multiple sites, especially bones and ligaments and heart and blood vessels.

OBJECTIVE

Authors sought to investigate quality of life (QoL) in MFS patients, assessing positive and negative sociodemographic factors and self-perceived well-being and functional status.

METHOD

Thirty-six patients affected by MFS were interviewed and were administered the SF-36 psychometric questionnaire.

RESULTS

Subjects affected by MFS reported an impaired quality of life in the psychological domain but not in the physical domain, as compared with a healthy population. Being male and older was significantly associated with a poorer perceived mental QoL.

CONCLUSION

The authors found that MFS negatively influences QoL, increases psychological distress, and may be a possible risk for some psychiatric disorders.