Benninghoven Dieter, Hamann Denise, von Kodolitsch Yskert, Rybczynski Meike, Lechinger Julia, Schroeder Friedrich, Vogler Marina, Hoberg Eike
Muehlenberg-Clinic for Rehabilitation, Bad Malente-Gremsmühlen, Germany.
Clinic of Psychosomatic Medicine and Psychotherapy, University of Luebeck, Lübeck, Germany.
Orphanet J Rare Dis. 2017 Jul 12;12(1):127. doi: 10.1186/s13023-017-0679-0.
Advances in medical, interventional and surgical treatment have increased average life expectancy of patients with congenital heart defects. As a result a new group of adult patients with congenital cardiac defects requires medical rehabilitation. Patients with Marfan syndrome (MFS) are a relevant group among these patients. So far, no reports on the effectiveness of specialized rehabilitation programmes for MFS patients exist. We implemented an inpatient 3-week rehabilitation program for MFS patients at the Muehlenberg-Clinic for rehabilitation and assessed the medical safety as well as the impact of the program on physical fitness and psychological wellbeing of participants by means of an observational pilot study. The comprehensive multidisciplinary program included medical, physiotherapeutic, psychological and social issues. Two groups including 8 and 10 individuals with verified MFS attended the programme. Medically adverse events that occurred during the rehabilitation were registered. Adverse events were defined as: any new cardiac arrhythmias such as atrial fibrillation, ventricular tachycardia, cardiac syncope or any complications located at the aorta. Psychological assessment was performed using Short Form-36 (SF-36), hospital anxiety and depression scale and other psychometric questionnaires. Medical examinations included assessment of maximum power in bicycle ergometry. All assessments were performed at the beginning and at the end of the rehabilitation. Psychometric assessments were repeated 1 year after the end of the programme for both groups, respectively.
Patients were highly satisfied with the programme and improved in almost all psychological and physical fitness assessments. The pre-post-comparison resulted in significant positive changes for mental health (p < .001 for SF-36 Mental Health), fatigue (p < .05 for Fatigue Severity Scale), nociception (p < .05 for SF-36 Pain) and vitality (p < .05 for SF-36 Vitality). Physical fitness improved from admission to discharge (p < .001 for maximum power in bicycle ergometry, p < .05 for maximum nordic walking distance). Considerable improvements persisted through 1 year follow-up. Medical assessments excluded medical problems or adverse events caused by participation in the programme.
In our study, inpatient rehabilitation was both safe and helpful for MFS patients. They benefited in terms of physical fitness, health related quality of life and in terms of psychological wellbeing. An evaluation of the efficacy of the programme in a controlled design as well as further conceptual improvements of our current program is desirable.
医学、介入治疗和外科治疗的进展提高了先天性心脏缺陷患者的平均预期寿命。因此，出现了一批需要医学康复的成年先天性心脏缺陷患者。马凡综合征（MFS）患者是这类患者中的一个相关群体。到目前为止，尚无关于针对MFS患者的专门康复计划有效性的报告。我们在穆伦贝格康复诊所为MFS患者实施了一项为期3周的住院康复计划，并通过一项观察性试点研究评估了该计划的医疗安全性以及对参与者身体健康和心理健康的影响。这个综合的多学科计划涵盖了医学、物理治疗、心理和社会问题。两组分别有8名和10名经确诊的MFS患者参加了该计划。记录了康复期间发生的医学不良事件。不良事件定义为：任何新的心律失常，如心房颤动、室性心动过速、心脏晕厥或位于主动脉的任何并发症。使用简短健康调查问卷（SF-36）、医院焦虑抑郁量表和其他心理测量问卷进行心理评估。医学检查包括评估自行车测力计的最大功率。所有评估均在康复开始时和结束时进行。两组在该计划结束1年后分别重复进行心理测量评估。
患者对该计划高度满意，几乎在所有心理和身体健康评估中都有改善。前后比较显示心理健康（SF-36心理健康量表，p <.001）、疲劳（疲劳严重程度量表，p <.05）、痛觉（SF-36疼痛量表，p <.05）和活力（SF-36活力量表，p <.05）有显著的正向变化。从入院到出院，身体健康状况有所改善（自行车测力计最大功率，p <.001；北欧式健走最大距离，p <.05）。在1年的随访中，相当大的改善持续存在。医学评估排除了因参与该计划导致的医学问题或不良事件。
在我们的研究中，住院康复对MFS患者既安全又有帮助。他们在身体健康、与健康相关的生活质量和心理健康方面都受益。需要在对照设计中评估该计划的疗效，并对我们目前的计划进行进一步的概念改进。
