Mortality and Morbidity Outcomes in Marfans

Mortality and Morbidity Outcomes After Aortovascular Surgery in Patients With Marfan Syndrome: A UK Experience

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

Study Overview

• Status: Not yet recruiting
• Conditions: Marfan Syndrome; Marfan Syndrome Cardiovascular Manifestations

Detailed Description

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.

• Study Type: Observational
• Enrollment (Estimated): 10000

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 120 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No

• 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.

Description

Inclusion Criteria:

• >/= 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

Exclusion Criteria:

• < 18 years old at the time of surgery

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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 Outcome Measures

Outcome Measure	Measure Description	Time Frame
Morbidity outcomes	(i) Underlying cause of death and multiple causes of death	within 1 year after surgery
Morbidity outcomes	Date of each Finished Consultant Episode	within 1 year after surgery
Morbidity outcomes	Primary and secondary diagnostic codes	within 1 year after surgery
Morbidity outcomes	Primary procedural codes	within 1 year after surgery
Morbidity outcomes	Hospital episode length	within 1 year after surgery

Collaborators and Investigators

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

Study record dates

Study Major Dates

• Study Start (Estimated): December 1, 2023
• Primary Completion (Estimated): March 30, 2024
• Study Completion (Estimated): March 30, 2024

Study Registration Dates

• First Submitted: February 18, 2021
• First Submitted That Met QC Criteria: February 25, 2021
• First Posted (Actual): March 1, 2021

Study Record Updates

• Last Update Posted (Actual): June 22, 2023
• Last Update Submitted That Met QC Criteria: June 19, 2023
• Last Verified: June 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Pathologic Processes; Heart Diseases; Cardiovascular Diseases; Disease; Congenital Abnormalities; Genetic Diseases, Inborn; Musculoskeletal Diseases; Connective Tissue Diseases; Bone Diseases; Heart Defects, Congenital; Cardiovascular Abnormalities; Musculoskeletal Abnormalities; Abnormalities, Multiple; Bone Diseases, Developmental; Limb Deformities, Congenital; Syndrome; Marfan Syndrome; Arachnodactyly
• Other Study ID Numbers: 294022

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No