Patients with congenital heart defects, such as Marfan syndrome (MFS), are experiencing longer life expectancies due to new advances in medicine-resulting in a greater need for medical rehabilitation.
Patients with congenital heart defects, such as Marfan syndrome (MFS), are experiencing longer life expectancies due to new advances in medicine—resulting in a greater need for medical rehabilitation.
The life expectancy of patients with MFS, an autosomal dominant genetic disorder of the connective tissue, can often be improved through vascular and cardiac surgery. Rehabilitation has become more necessary for patients after surgery, as well as for those living with cardiovascular problems or MFS.
An observational pilot study, from the Orphanet Journal of Rare Diseases, evaluates a newly designed rehabilitation program for MFS patients in Germany, in order to confirm the efficacy and safety of the program, while analyzing its impact on the physical fitness and psychological wellness of the patients.
“A comprehensive and integrated approach to rehabilitation is pivotal,” the authors wrote. “So far, rehabilitation programs specific for MFS appear to exist only in Norway and scientifically evaluated programs do not exist. However sparse studies and case reports emphasize the need for psychosocial support of affected persons.”
The study of the new German program consisted of a 3 weeks of inpatient rehabilitation for MFS patients at the Muehlenberg-Clinic.
Two groups, of 8 and 10 individuals, attended the program. The patients’ somatic, educative, psychological, and social rehabilitation goals were recorded and used to formulate a rehabilitation plan, which incorporated various activities to encourage patients to overcome their hesitancy to their own physical abilities. Group therapy and counseling for job issues and for diet were also included in the program.
At the end of the program, no adverse medical event was reported for any of the patients, physical fitness was improved, and psychological distress decreased. Furthermore, 16 of the 18 MFS patients completed a 1-year follow-up questionnaire and significant positive differences in psychological distress, somatization, and physical role functioning were reported.
“It might be considered unrealistic to expect participating in a 3 weeks rehabilitation program to influence quality of life for patients with MFS because quality of life is a broad and comprehensive psychological construct,” the study declared. “Interestingly, our rather short 3- week program was, in fact, sufficient to increase quality of life in MFS patients.”
Due to the substantial improvements in the quality life of MFS patients, the researchers encourage the development of similar programs for patients with other congenital heart defects in the future.