Benke Kálmán, Ágg Bence, Pólos Miklós, Sayour Alex Ali, Radovits Tamás, Bartha Elektra, Nagy Péter, Rákóczi Balázs, Koller Ákos, Szokolai Viola, Hedberg Julianna, Merkely Béla, Nagy Zsolt B, Szabolcs Zoltán
Heart and Vascular Center, Semmelweis University, Városmajor Street 68, Budapest, 1122, Hungary.
Hungarian Marfan Foundation, Városmajor Street 68, Budapest, 1122, Hungary.
BMC Psychiatry. 2017 Jul 17;17(1):253. doi: 10.1186/s12888-017-1417-9.
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.
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.
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, η = 0.15).
Involving psychiatric and mental-health care, in addition to existing surgical treatment interventions, is essential for more successful recovery of patients with Marfan syndrome.
马凡综合征是一种遗传性疾病，表现为结缔组织功能障碍，导致心血管和骨骼肌系统出现病变。在这些症状中，最典型的是主动脉结缔组织薄弱，表现为主动脉扩张（动脉瘤）。这进而可能发展为主动脉环扩张，或危及生命的主动脉夹层。因此，马凡综合征患者经常需要进行挽救生命和预防性的心脏外科手术。主动脉瘤可能会发展为主动脉环扩张或危及生命的主动脉夹层，因此马凡综合征患者经常需要进行挽救生命和预防性的心脏外科手术。我们假设，与非临床患者群体相比，马凡综合征患者的焦虑、抑郁程度以及生活满意度有所不同。
被诊断为马凡综合征的患者被分为三组：计划进行预防性手术的患者、需要进行急诊手术的患者以及无需手术的患者（分别为9例、19例、17例）。为了检查患者的心理特征，应用了斯皮尔伯格焦虑量表（STAI）、贝克抑郁问卷（BDI）、伯尔尼主观幸福感问卷以及生活满意度量表。
在进行挽救生命的急诊手术后，健康个体与马凡综合征患者在特质焦虑方面存在显著差异（p < 0.01）。马凡综合征患者的平均得分为48.56（标准差（SD）：5.8），而STAI总体平均得分为43.72（SD：8.53）。在BDI方面，各组之间未发现差异（p > 0.1）。最后，在患者组之间的生活乐趣量表上发现了显著的中等效应（F(2.39) = 3.51，p = 0.040，η = 0.15）。
除了现有的手术治疗干预措施外，让精神科和心理健康护理参与进来，对于马凡综合征患者更成功的康复至关重要。
