Caroline Andonian1,2,3, Sebastian Freilinger1, Stefan Achenbach4, Peter Ewert1, Ulrike Gundlach4, Harald Kaemmerer1, Nicole Nagdyman1, Rhoia Clara Neidenbach1, Lars Pieper5, Jörg Schelling6, Michael Weyand4, Jürgen Beckmann2,7,8. 1. Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University Munich, Munich, Germany. 2. Department of Sport and Health Sciences, Chair of Sport Psychology, Technical University Munich, Munich, Germany. 3. Sigmund Freud University, Vienna, Austria. 4. Department of Cardiology, University of Erlangen, Erlangen, Germany. 5. Department of Behavioral Epidemiology, Technical University of Dresden, Dresden, Germany. 6. Faculty of Medicine, Ludwig Maximilians University (LMU) of Munich, Munich, Germany. 7. School of Human Movement and Nutrition Sciences, University of Queensland, Australia. 8. Health Research Institute, University of Limerick, Limerick, Ireland.
Abstract
BACKGROUND: Marfan syndrome (MFS) is a genetically determined multiorgan disease that leads to severe physiological and psychological impairments in adult life. Little consensus exists regarding quality of life (QOL) in individuals with MFS. The present study sought to investigate QOL in a representative cohort of adults with MFS. METHODS: Patient-reported outcome measures from a representative sample of 102 adults with MFS (39.3±13.1 years of age; 40.2% female) were retrospectively analyzed and compared with those from adults with different congenital heart defects (CHD), at the German Heart Center Munich. QOL was assessed using the updated five-level version of the EQ-5D. RESULTS: Differences between both populations were analyzed. Subjects affected by MFS reported an overall reduced QOL. Compared to CHD patients, individuals with MFS scored significantly lower in the dimensions of pain/discomfort, anxiety/depression, mobility and usual activities (P<0.05). CONCLUSIONS: Patients with MFS are at high risk for impaired QOL, especially in mental and physical domains. Psychosocial consequences of MFS cost resources for both, patients and professionals. Current findings highlight the great importance of additional psychological support to cope with disease-related challenges. Increased attention should be directed towards enhancing their subjective wellbeing to potentially improve their QOL and long-term health outcomes. 2021 Cardiovascular Diagnosis and Therapy. All rights reserved.
BACKGROUND: Marfan syndrome (MFS) is a genetically determined multiorgan disease that leads to severe physiological and psychological impairments in adult life. Little consensus exists regarding quality of life (QOL) in individuals with MFS. The present study sought to investigate QOL in a representative cohort of adults with MFS. METHODS: Patient-reported outcome measures from a representative sample of 102 adults with MFS (39.3±13.1 years of age; 40.2% female) were retrospectively analyzed and compared with those from adults with different congenital heart defects (CHD), at the German Heart Center Munich. QOL was assessed using the updated five-level version of the EQ-5D. RESULTS: Differences between both populations were analyzed. Subjects affected by MFS reported an overall reduced QOL. Compared to CHD patients, individuals with MFS scored significantly lower in the dimensions of pain/discomfort, anxiety/depression, mobility and usual activities (P<0.05). CONCLUSIONS: Patients with MFS are at high risk for impaired QOL, especially in mental and physical domains. Psychosocial consequences of MFS cost resources for both, patients and professionals. Current findings highlight the great importance of additional psychological support to cope with disease-related challenges. Increased attention should be directed towards enhancing their subjective wellbeing to potentially improve their QOL and long-term health outcomes. 2021 Cardiovascular Diagnosis and Therapy. All rights reserved.
Entities:
Keywords:
EQ-5D; Marfan syndrome (MFS); prevention; psychological situation; quality of life
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