Psychological factors affecting Marfan syndrome patients with or without cardiac surgery.

BACKGROUND: Marfan syndrome (MFS) is a systemic connective tissue disorder belonging to a group of rare diseases. Several psychologically distressing factors can challenge life for MFS patients. The aim of the present study was, therefore, to assess the psychological and psychosocial aspects of MFS with the goal of identifying a means of improving disease management for patients. METHODS: A total of 66 adult patients with MFS were enrolled into the study prospectively and were divided into operated (OP) and non-operated (NOP) subgroups. Multiple questionnaire tests were used to determine the mental and physical state of our patients. Demographic and surgical data were collected. The results of the tests were also compared to the Hungarostudy (HS) population (representing the average Hungarian population) by using a propensity-matched control. RESULTS: OP group scores yielded more alcohol consumption (P<0.001), while NOP group showed more sleep disturbances. Scores on the MMSE, BECK, STAI and STAI-T tests showed no significant difference comparing the OP and NOP groups. MFS patients appear to have moderate pain-related disability and mild depressive symptoms and sleep disturbances (P<0.05) compared to the HS group. On 10-point scale, MFS patients were more satisfied with their lives (P<0.001) and considered themselves happier (P<0.001) than the HS population; however, they also spent more days on sick leave and in hospital over the past year. The HS group yielded a higher overall percentage of current smokers and pack-per-year consumption than the MFS patients overall (P=0.003 and P<0.001 respectively). CONCLUSIONS: Marfan patients' psychosocial life differs in many ways (including sleep disturbances, healthier lifestyle, pain-related suffering) from the average Hungarian population. Therefore, as part of a multidisciplinary approach during treatment, modern management of MFS should include psychosocial exploration and psychological support in addition to traditional medical options.

The effects of acute and elective cardiac surgery on the anxiety traits of patients with Marfan syndrome.

BACKGROUND: Marfan syndrome is a genetic disease, presenting with dysfunction of connective tissues leading to lesions in the cardiovascular and skeletal muscle system. Within these symptoms, the most typical is weakness of the connective tissue in the aorta, manifesting as aortic dilatation (aneurysm). This could, in turn, become annuloaortic ectasia, or life-threatening dissection. As a result, life-saving and preventative cardiac surgical interventions are frequent among Marfan syndrome patients. Aortic aneurysm could turn into annuloaortic ectasia or life-threatening dissection, thus life-saving and preventive cardiac surgical interventions are frequent among patients with Marfan syndrome. We hypothesized that patients with Marfan syndrome have different level of anxiety, depression and satisfaction with life compared to that of the non-clinical patient population. METHODS: Patients diagnosed with Marfan syndrome were divided into 3 groups: those scheduled for prophylactic surgery, those needing acute surgery, and those without need for surgery (n = 9, 19, 17, respectively). To examine the psychological features of the patients, Spielberger's anxiety (STAI) test, Beck's Depression questionnaire (BDI), the Berne Questionnaire of Subjective Well-being, and the Satisfaction with Life scale were applied. RESULTS: A significant difference was found in trait anxiety between healthy individuals and patients with Marfan syndrome after acute life-saving surgery (p < 0.01). The mean score of Marfan syndrome patients was 48.56 (standard deviation (SD): 5.8) as compared to the STAI population mean score of 43.72 (SD: 8.53). No difference was found between groups on the BDI (p > 0.1). Finally, a significant, medium size effect was found between patient groups on the Joy in Living scale (F (2.39) = 3.51, p = 0.040, η2 = 0.15). CONCLUSIONS: Involving psychiatric and mental-health care, in addition to existing surgical treatment interventions, is essential for more successful recovery of patients with Marfan syndrome.