Mortality and Morbidity Outcomes in Marfans

NCT04774172 | Completed DMC

• 1 other identifier: 294022
• Study Type: observational
• Target: 6,700
• Locations: 1 country
• Sites: 1

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. Improvement in diagnostics and medical and surgical interventions have increased life expectancy. However, the natural history and the influence of medical or surgical interventions in the UK population are not fully described. Further, the incidence of aortovascular surgery in this patient group is unknown as MFS is not routinely documented in the National Institute of Cardiovascular Outcome Research (NICOR) national cardiac surgery dataset and therefore, there is currently no mechanism for exploring the aortovascular outcomes for this patient group. The investigators aim to undertake a 10-year secondary analysis of linked national data (National Institute of Cardiovascular Outcome Research (NICOR), Office of National Statistics (ONS), Hospital Episode Statistics (HES)) to identify the UK incidence and outcome of aorto-vascular surgery in patients with Marfan syndrome (MFS). This includes associated hospital length of stay, mortality and morbidity rates. Understanding mortality alongside morbidity will allow the investigators to study further the burdens that the aortovascular manifestations may place on MFS population as well as to continuously evaluate the efficacy of either the health care system or an implemented intervention in place. Further, these metrics will be useful for the stakeholders to effectively prioritise which complications to tackle and to allocate resources toward as well as proactively manage the potential onset of a health event

Conditions

Marfan Syndrome; Marfan Syndrome Cardiovascular Manifestations

Outcome Measures

Primary Outcomes (1)

• Mortality mortality rate 1-year after surgery. Mortality rate 1 year after aorto-vascular surgery

  mortality rate 1-year after surgery

  1 year after surgery

Secondary Outcomes (5)

• Morbidity outcomes

  within 1 year after surgery

• Morbidity outcomes

  within 1 year after surgery

• Morbidity outcomes

  within 1 year after surgery

• Morbidity outcomes

  within 1 year after surgery

• Morbidity outcomes

  within 1 year after surgery

Eligibility Criteria

• Age: 18 Years - 120 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

The study will include all eligible patients who are identified as MFS using the ICD-10 code Q874 through the National Health Service Digital (NHSD) from January 2010 to December 2019.

You may qualify if:

• \>/= 18 years old at the time of surgery
• Identified MFS in the HES database using the ICD diagnosis code Q874 between January 2010- December 2019
• Had an aorto-vascular surgery in England and Wales, as identified in the NICOR Adult Cardiac Surgery database

You may not qualify if:

• \< 18 years old at the time of surgery

Sponsors & Collaborators

• Barts & The London NHS Trust: lead
• Liverpool Heart and Chest Hospital NHS Foundation Trust: collaborator
• Aortic Dissection Awareness UK & Ireland.: collaborator

Study Sites (1)

St Bartholomews Hospital

London, EC1A 7BE, United Kingdom

Location

MeSH Terms

Conditions

Marfan Syndrome

Condition Hierarchy (Ancestors)

Bone Diseases, Developmental; Bone Diseases; Musculoskeletal Diseases; Heart Defects, Congenital; Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Abnormalities, Multiple; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Genetic Diseases, Inborn; Connective Tissue Diseases; Skin and Connective Tissue Diseases

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: February 18, 2021
• First Posted: March 1, 2021
• Study Start: December 1, 2023
• Primary Completion: June 30, 2024
• Study Completion: June 30, 2024
• Last Updated: August 23, 2024

Record last verified: 2024-08

Locations

GB (1)