Living With Marfans and Your Aorta: Surgical Outcomes Study
Living With Marfans and Your Aorta: Surgical Outcomes Study
Marfan Syndrome (MFS) is a genetic disease affecting the eyes, skeleton, heart and arteries.
 Despite MFS affecting multiple organ systems, cardiovascular manifestations are the most
 serious and life threatening. Approximately 80% of adult MFS patients will have a dilated
 aortic root by age 40 years with aortic aneurysm and dissection the leading causes of
 morbidity and mortality. Living with a diagnosis of Marfan Syndrome, including undergoing and
 recovering from heart surgery, affects patients' mental health, well-being and quality of
 life in ways that are not well understood.

 This study will address the current knowledge gaps in this area and will provide the
 information needed to design interventions to help improve the MFS patients' mental health,
 well-being and quality of life after heart surgery. The study will include adult MFS patients
 who are undergoing aorto-vascular surgery.

 The overall aim of the study is to explore the psychosocial and health-related quality of
 life (HRQoL) effects of the surgical interventions for aorto-vascular manifestations of MFS
 in 3 large UK cardiac centres. To achieve this, the researchers will ask the potential
 participants, after obtaining informed consent, to complete a series of accepted / validated
 questionnaires to measure the health-related quality of life (SF-36 and EQ5D questionnaire)
 and psychosocial factors such as depression (CES-D questionnaire), fatigue (Fatigue Severity
 Scale), stigma (Perceived Stigma Questionnaire), self-esteem (Rosenberg Self-esteem Scale),
 pain and illness perception (Illness Perception Questionnaire). Participants will be asked to
 complete the questionnaires before surgery and at various time points after surgery (at 6
 weeks after hospital discharge and at 6 and 12 months after surgery). The research team will
 also collect in-hospital post-operative morbidity burden following aorto-vascular surgery
 using cardiac post-operative morbidity score (C-POMS) tool from the patients and clinical
 records. The association of C-POMS with psychosocial and HRQoL outcomes will also be
 examined.
Marfan Syndrome (MFS) is a rare autosomal-dominant connective tissue disorder with
 pleiotropic manifestations primarily affecting the skeletal, ocular, and cardiovascular
 systems. Despite MFS affecting multiple organ systems, cardiovascular manifestations are the
 most serious and life threatening. Approximately 80% of adult MFS patients will have a
 dilated aortic root by age 40 years with aortic aneurysm and dissection the leading causes of
 morbidity and mortality. However, there is limited evidence worldwide on health-related
 quality of life (HRQoL) and psychosocial aspects of the recovery of MFS patients undergoing
 surgical intervention. Undergoing a heart operation can be a stressful and life-threatening
 experience involving a lot of uncertainties, long periods of absence from work and social
 activities as well as limitation on physical activities which can potentially lead to
 psychosocial problems and poor health-related quality of life. Therefore, there is a specific
 need for high quality descriptive and exploratory research in this area.

 The overarching aim of this prospective observational cohort study is to explore the
 psychosocial and HRQoL effects of surgical interventions for aorto-vascular manifestations of
 MFS in three large UK cardiac centres. Potential participants will be recruited from the
 outpatient pre-admission clinic. HRQoL and psychosocial factors will be determined and
 quantified using validated questionnaires before surgery and at various time points after
 surgery (at 6 weeks after hospital discharge and at 6 and 12 months after surgery). The
 questionnaires will be delivered either in paper or electronic format, depending on
 participant's preference. In-hospital post-operative morbidity burden following
 aorto-vascular surgery using cardiac post-operative morbidity score (C-POMS) tool will also
 be determined from the patients and clinical records and its association with psychosocial
 and HRQoL outcomes will be examined.

 The outcome of the study will significantly contribute to the UK and international
 evidence-base on the HRQoL and psychosocial impact of the aorto-vascular surgical
 interventions on MFS patients and will provide clinicians with the foundation that will serve
 as basis for appropriate interventions. The result will also be useful to better understand
 the condition and potentially guide the researchers in devising a patient care pathway that
 includes this aspect of care as well as develop a holistic and comprehensive service for this
 patient group.
Inclusion Criteria:

 1. >/= 18 years old at the time of surgery;

 2. have validated diagnosis of MFS (using revised Ghent criteria);

 3. having aorto-vascular surgery

 Exclusion Criteria:

 1. <18years old at the time of surgery

 2. Unable or unwilling to give written informed consent.

 3. Inability to understand written and/or verbal English