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Determinants of Quality of Life in Marfan Syndrome

Received August 25, 2006; revised November 13, 2006; accepted November 20, 2006. From the DSSAeP, Sezione di Psichiatria, Università di Pavia and Servizio Psichiatrico di Diagnosi e Cura, IRCCS Policlinico San Matteo, Pavia, Italy; Servizio di Biometria ed Epidemiologia Clinica; and Centro per le Malattie Genetiche Cardiovascolari, Gruppo Interdisciplinare per la Sindrome di Marfan , IRCCS Policlinico San Matteo, Pavia, Italy. Send correspondence and reprint requests to Dr. Paolo Fusar-Poli, Department of Applied and Psychobehavioural Sciences, University of Pavia, via Bassi 21, 27100 Pavia, Italy. e-mail: p.fusar{at}libero.it
© 2008 The Academy of Psychosomatic Medicine

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.

Key Words: Marfan Syndrome • Quality of Life

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