Marfan Syndrome and Quality of Life

Study Questions:

Is quality of life (QOL) among adults with Marfan syndrome (MFS) worse than general populations?


Data from GenTAC (Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions), a national registry, were used for the present analysis. GenTAC is a longitudinal observational cohort study of patients with conditions that predispose to thoracic aortic aneurysms and dissections, including MFS. For the present analysis, only participants with MFS were included. QOL was assessed using the four subscales of the Physical Component Summary of the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36): physical functioning, role limitations due to physical health, bodily pain, and general health. QOL was examined in relation to self-reported demographics, health behaviors, physical impairments, surgeries, comorbid medical conditions, medications, and MFS severity.


A total of 389 participants with MFS who completed the SF-36 were included. Mean age was 41 years; 51% of the participants were male, 92% were white, and 65% were college graduates. The mean Physical Component Summary score was 42.3. Predictors of better QOL included college education, marital status, higher household income, private health insurance, full-time employment, moderate alcohol use, fewer prior surgeries, fewer comorbid conditions, absence of depression, and less severe MFS manifestations. In a multivariable analysis, insurance status and employment remained significant predictors of QOL.


The authors concluded that in a large cohort of patients with MFS in the GenTAC registry, health-related QOL was below the population norm. Better QOL was independently associated with socioeconomic factors, not factors related to general health or MFS severity.


These data suggest that adults with MFS may have worse health-related QOL compared to those without MFS, but mildly so. Factors associated with worse health-related QOL were not related to health or MFS, but rather employment status.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Prevention, Vascular Medicine, Aortic Surgery, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement

Keywords: Aortic Aneurysm, Thoracic, Comorbidity, Depression, Employment, Health Behavior, Heart Defects, Congenital, Insurance Coverage, Insurance, Health, Marfan Syndrome, Marital Status, Pain, Primary Prevention, Quality of Life, Socioeconomic Factors, Vascular Diseases

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