Health-related quality of life in Marfan syndrome: a cross-sectional study of Short Form 36 in 84 adults with a verified diagnosis.
OBJECTIVE
To explore health-related quality of life as measured with Short Form 36 in adults with verified Marfan syndrome and to compare with the general population, other groups with chronic problems and studies on Marfan syndrome. Furthermore, to study potential correlations between the scores on the subscales of Short Form 36 and the presence of biomedical criteria and symptoms of Marfan syndrome.


METHODS
Cross-sectional study. Short Form 36 was investigated in 84 adults with verified Marfan syndrome.


RESULTS
The study group had reduced scores on all eight subscales of Short Form 36 compared with the general population, comparable with other groups with chronic diseases. Compared with earlier Short Form 36 results in Marfan syndrome, we found lower scores for social function, vitality, general health, bodily pain, and role physical. No correlations of substantial explanatory values were found between the Short Form 36 subscales and gender, body mass index, ascending aortic surgery, use of beta-blockers, visual acuity, joint hypermobility, fulfillment of the five major Ghent criteria, and number of major criteria fulfilled. Potential explanations are discussed.


CONCLUSIONS
Persons with Marfan syndrome have reduced scores for health-related quality of life as measured with Short Form 36, comparable with those in other chronic disorders and disabilities. The reduction does not seem to be related to biomedical criteria or symptoms of Marfan syndrome.
Rand-Hendriksen
Svend
S
TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital, Nesoddtangen, Norway. svend.rand-hendriksen@sunnaas.no
Johansen
Heidi
H
Semb
Svein Ove
SO
Geiran
Odd
O
Stanghelle
Johan K
JK
Finset
Arnstein
A
D000328:Adult; D001824:Body Constitution / Q000502:physiology; D015992:Body Mass Index; D003430:Cross-Sectional Studies; D006801:Humans; D016014:Linear Models; D008382:Marfan Syndrome / Q000473:pathology* / Q000523:psychology*; D008603:Mental Health; D009664:Norway; D011788:Quality of Life / Q000523:psychology*; D018709:Statistics, Nonparametric; D011795:Surveys and Questionnaires