Mortality and Morbidity Outcomes in Marfans
Mortality and Morbidity Outcomes in Marfans
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
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.
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