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Quality of life in patients with Marfan syndrome: a cross-sectional study of 102 adult patients

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posted on 2021-07-22, 13:37 authored by Caroline Andonian, Sebastian Freilinger, Stephan Achenbach, Peter Ewert, Ulrike Gundlach, Harald Kaemmerer, Nicole Nagdyman, Rhoia Clara Neidenbach, Lars Pieper, Jörg Schelling, Michael Weyand, Jürgen Beckmann
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.

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Publication

Cardiovascular Diagnosis and Therapy;11 (2), 602-611

Publisher

AME Publishing

Note

peer-reviewed

Language

English

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