Inpatient rehabilitation for adult patients with Marfan syndrome: an observational pilot study

Abstract

BackgroundAdvances 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.ResultsPatients 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.ConclusionsIn 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.

Systematic review of quality of life in persons with hereditary thoracic aortic aneurysm and dissection diagnoses

“…Five studies reported only on patients with MFS according to Ghent 1 nosology. Five studies included only patients assessed according to the revised Ghent nosology (Ghent 2). One study included patients verified Ghents 1 and 2 criteria, and another study included both patients with verified Ghent 1 (n = 13) and patients without verified diagnosis (n = 11).…”

“…All papers used validated, multi‐dimensional scales to measure QoL. Short Form Medical Survey (SF‐36) was used in 10 of the 17 papers dealing with adults and two studies used Short Form Medical survey (SF‐12). Three studies used the Satisfaction with Life Scale (SWLS), one the Ferrans and Power Quality of life Index, cardiac version (QLI, cardiac III), and one used the Ferrans QLI .…”

“…Five studies 9,37,39,46,51 reported only on patients with MFS according to Ghent 1 nosology. Five studies 34,[43][44][45]52 included only patients assessed according to the revised Ghent nosology (Ghent 2).…”

“…Most papers did not describe the geographical catchment of their respondents. In 11 studies 34,[36][37][38][39][40]43,45,46,48,52 the participants were recruited from medical clinics where the researchers worked; in three studies 44,49,53 from the Marfan Foundation. Three studies 9,35,51 recruited although both clinic and the Marfan Foundation, one study recruited by the GenTac register, 47 one announced for volunteers on a web-site 41 and one study 50 did not describe the recruiting process.…”

“…Five papers 9,37,43,48,53 compared their results of QoL with healthy controls matched for age and gender, 11 studies [34][35][36][39][40][41][44][45][46][47]51 with norm data and/or other diagnoses, one study 50 only with other diseases. Three studies 38,49,52 did not describe any comparison groups (Table 3 and Table S3A).…”