Marfan SyndromeLIMA IINCT04776681
Living With Marfans and Your Aorta: Surgical Outcomes Study
An observational study of Marfan Syndrome and Marfan Syndrome Cardiovascular Manifestations, run by Barts & The London NHS Trust at 1 site in United Kingdom.
Trial 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. Read more about this study
- Who is it for?
- Ages 18 to 120 years
- Conditions the study is for
- Marfan Syndrome, Marfan Syndrome Cardiovascular Manifestations
- How many people
- 28 participants
- Where
- 1 siteUnited Kingdom
- Study dates
- 26 Jul 2021to 31 Dec 2025
- Phase
- Observational
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 to 120 years
- All sexes
- No healthy volunteers
- Adult, Older adult
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.
Who may be able to join
- >/= 18 years old at the time of surgery;
- have validated diagnosis of MFS (using revised Ghent criteria);
- having aorto-vascular surgery
Who cannot join
- <18years old at the time of surgery
- Unable or unwilling to give written informed consent.
- Inability to understand written and/or verbal English
Trial locations and contacts
1 site in 1 country.
About this 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)
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.
Read the 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.
- Medical subject headings
- Marfan Syndrome
What is being measured
The primary outcome is the main question the study is designed to answer.
Change from Baseline Health-related Quality of Life (HRQoL)
HRQoL will be measured using the Short Form Health Survey 36 (SF-36). SF-36 consists of 8 sub-scales. All scales do contribute in different proportions to the scoring of the physical and mental dimensions, represented by the Physical Component Summary (PCS) and Mental Component Summary (MCS). Using an algorithm, raw scores can be converted to a transformed scale scores ranging from 0-100, with higher score indicating a better health state.
Measured: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
Change from Baseline Health-related Quality of Life (HRQoL)
HRQoL will be measured using EQ-5D. EQ-5D is divided into 2 components: health care description and evaluation. In the description part, summation of score ranges from 1 to 3; with 1 indicates the best health state and 3 indicates more severe or frequent problem. The evaluation part utilises a visual analogue scale to indicate the general health status with 100 indicating the best health status and 0 indicating the worst health status.
Measured: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
Secondary outcomes (6)
Change from baseline psychosocial factor: depression
Depression will be measured using Centre for Epidemiological Studies Depression Scale (CES-D). The overall score ranges from 0 to 60, with higher scores indicating a more severe depressive symptoms. In general population, a cut-off score of 16 or above is used to identify those with significant clinical signs of depression.
Measured: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
Change from baseline psychosocial factor: fatigue
Fatigue will be determined using Fatigue Severity Scale (FSS). The minimum possible score is 9 and maximum possible score is 63. Higher score indicates greater fatigue severity.
Measured: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
Change from baseline psychosocial factor: stigma
Stigma will be measured using Perceived Stigma Questionnaire (PSQ). PSQ is composed of 4 sub-scales. Score on each sub-scale ranges from 1 to 6. Mean scores of >/= 2.0 on the devaluation and discrimination sub-scale indicates feeling of discriminated against or devalued. Mean scores of </= 3.5 on the withdrawal and secrecy sub-scales and </= 2.0 on the education sub-scale indicates the use of secrecy / withdrawal and education as coping styles, respectively.
Measured: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
Change from baseline psychosocial factor: self esteem
Self-esteem will be measured using Rosenberg Self-esteem Scale (RSS); score ranges from 10 (min) -40 (max); higher score indicates higher self-esteem.
Measured: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
Change from baseline psychosocial factors: pain and illness perception
Pain and Illness Perception will be determined using Illness Perception Questionnaire (IPQ). IPQ is divided into 5 components. The identity component is scored by summing the number of items identified at 'occasionally' or greater resulting to a total score ranging from 0 to 12 for the core list. The four other components are presented in mixed order and can be rated using a 5-point Likert scale ranging from 'strongly disagree' (1) to 'strongly agree (5).
Measured: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
Change from baseline post-operative morbidity outcomes
In-hospital post-operative morbidity outcomes will be identified and quantified using the Cardiac Post-Operative Morbidity Score (C-POMS) tool. C-POMS is a simple, validated tool assessing in-hospital total morbidity burden (score 0-13) derived by noting the presence of 13 morbidity domains on days three (D3), five (D5), eight (D8) and fifteen (D15) after surgery. Higher scores indicate more in-hospital post-operative morbidity.
Measured: 3, 5, 8 and 15 days after surgery
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted2 Mar 2021
- Study start26 Jul 2021
- Primary completion30 Dec 2025
- Study completion31 Dec 2025
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Marfan SyndromeStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 28 participants (actual)
Sponsor and investigators
- Lead sponsor
- Barts & The London NHS Trust
- Sponsor type
- University, hospital or other
- Collaborators
- Liverpool Heart and Chest Hospital NHS Foundation Trust, Aortic Dissection Awareness UK & Ireland.
- Responsible party
- Sponsor
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT04776681
- Sponsor's protocol ID
- 293099
- Acronym
- LIMA II
- Status last verified
- Feb 2026
- First submitted
- 18 Feb 2021
- Last updated
- 20 Feb 2026
- Results posted
- No
- Data monitoring committee
- Yes
- FDA-regulated drug
- No
- FDA-regulated device
- No
Study at a glance
From the registry record, last updated by the sponsor on 20 Feb 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- 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)
- ClinicalTrials.gov ID
- NCT04776681
- Acronym / sponsor ID
- LIMA II / 293099
- Study type
- Observational
- Recruitment status
- Completed
- Enrolment
- 28 participants (actual)
- Lead sponsor
- Barts & The London NHS TrustUniversity, hospital or other
- Collaborators
- Liverpool Heart and Chest Hospital NHS Foundation Trust, Aortic Dissection Awareness UK & Ireland.
- Study start
- 26 Jul 2021 (actual)
- Primary completion
- 30 Dec 2025 (actual)
- Study completion
- 31 Dec 2025 (actual)
- First posted
- 2 Mar 2021 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
Study design
- Observational model
- Cohort
- Time perspective
- Prospective
Who may qualify
- Ages
- 18 to 120 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- 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.
Key inclusion criteria (3 listed)
- >/= 18 years old at the time of surgery;
- have validated diagnosis of MFS (using revised Ghent criteria);
- having aorto-vascular surgery
Key exclusion criteria (3 listed)
- <18years old at the time of surgery
- Unable or unwilling to give written informed consent.
- Inability to understand written and/or verbal English
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
Change from Baseline Health-related Quality of Life (HRQoL)
Time frame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
HRQoL will be measured using the Short Form Health Survey 36 (SF-36). SF-36 consists of 8 sub-scales. All scales do contribute in different proportions to the scoring of the physical and mental dimensions, represented by the Physical Component Summary (PCS) and Mental Component Summary (MCS). Using an algorithm, raw scores can be converted to a transformed scale scores ranging from 0-100, with higher score indicating a better health state.
Change from Baseline Health-related Quality of Life (HRQoL)
Time frame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
HRQoL will be measured using EQ-5D. EQ-5D is divided into 2 components: health care description and evaluation. In the description part, summation of score ranges from 1 to 3; with 1 indicates the best health state and 3 indicates more severe or frequent problem. The evaluation part utilises a visual analogue scale to indicate the general health status with 100 indicating the best health status and 0 indicating the worst health status.
6 secondary outcomes
Change from baseline psychosocial factor: depression
Time frame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
Change from baseline psychosocial factor: fatigue
Time frame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
Change from baseline psychosocial factor: stigma
Time frame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
Change from baseline psychosocial factor: self esteem
Time frame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
Change from baseline psychosocial factors: pain and illness perception
Time frame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery
Change from baseline post-operative morbidity outcomes
Time frame: 3, 5, 8 and 15 days after surgery
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 20 Feb 2026.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
The record lists no contact. Use the official record to reach the sponsor.
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Marfan Syndrome and Marfan Syndrome Cardiovascular Manifestations, run by Barts & The London NHS Trust at 1 site in United Kingdom.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 3 inclusion and 3 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 20 Feb 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United Kingdom. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Change from Baseline Health-related Quality of Life (HRQoL) (time frame: before surgery, 6 weeks after hospital discharge, 6 months and 12 months after surgery). The study lists 2 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 20 Feb 2026.