Clinical trial NCT04776681: Living With Marfans and Your Aorta: Surgical Outcomes Study
Official 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)
Study type: OBSERVATIONAL
Overall status: RECRUITING
Conditions: Marfan Syndrome
Marfan Syndrome Cardiovascular Manifestations
Lead sponsor: Barts & The London NHS Trust
Sponsor class: OTHER
Collaborators: name: Liverpool Heart and Chest Hospital NHS Foundation Trust
name: Aortic Dissection Awareness UK & Ireland.
First posted: 2021-03-02
Enrollment: 28
Sex: ALL
Minimum age (years): 18
Maximum age (years): 120
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: All eligible consecutive patients with a diagnosis of MFS at three large UK cardiac centres will be identified prior to surgery, either through pre-operative outpatient clinic or via the clinical team.

Brief 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 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.

Detailed description: 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.

Eligibility criteria: 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

Primary outcomes:
measure: Change from Baseline Health-related Quality of Life (HRQoL)
timeFrame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
measure: Change from Baseline Health-related Quality of Life (HRQoL)
timeFrame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
Secondary outcomes:
measure: Change from baseline psychosocial factor: depression
timeFrame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
measure: Change from baseline psychosocial factor: fatigue
timeFrame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
measure: Change from baseline psychosocial factor: stigma
timeFrame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
measure: Change from baseline psychosocial factor: self esteem
timeFrame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
measure: Change from baseline psychosocial factors: pain and illness perception
timeFrame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
measure: Change from baseline post-operative morbidity outcomes
timeFrame: 3, 5, 8 and 15 days after surgery
Other outcomes:
Locations:
city: London
country: United Kingdom
facility: St Bartholomews Hospital
Study officials:
References: