Living with Marfans and your aorta: Surgical outcomes study

  • Research type

    Research Study

  • Full title

    Living with Marfan syndrome II: the psychosocial and health-related quality of life effects of surgical interventions for aorto-vascular manifestations (LIMA II Study)

  • IRAS ID

    293099

  • Contact name

    Julie Sanders

  • Contact email

    j.sanders@qmul.ac.uk

  • Sponsor organisation

    Queen Mary University of London

  • Clinicaltrials.gov Identifier

    NCT04776681, ClinicalTrials.gov ID

  • Duration of Study in the UK

    3 years, 11 months, 31 days

  • Research summary

    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 gaps in our knowledge and will provide the information needed to design interventions for MFS patients to help improve their mental health, well-being and quality of life after heart surgery. It 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, we 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.

  • REC name

    East Midlands - Leicester Central Research Ethics Committee

  • REC reference

    21/EM/0107

  • Date of REC Opinion

    17 May 2021

  • REC opinion

    Further Information Favourable Opinion