Safe People
Lead applicant organisation name	University Hospital Birmingham NHS Foundation Trust

Safe Projects
Project title	Utility of exercise-stress echocardiography in arrhythmogenic right ventricular dysplasia / cardiomyopathy (ARVCD)
Lay summary	ARVC is an inherited condition that is one of the leading causes of ventricular arrhythmias (VA) and sudden cardiac death (SCD), particularly in young and athletic patients. The main feature on assessment of the heart after death is fatty replacement of heart muscle, leading to thinning of the wall of the heart, localised bulging called aneurysms, and areas of inflamed tissue. At the level of the cell, the main problem lies in something called the desmosome, loss of which results in widening and breakage of links between cells, separation of heart muscle cells from each other, and eventually muscle cell death. Exercise seems to accentuate these changes, with physical exercise accentuating the loss of linkage between heart muscle cells. Exercising patients with ARVC is used clinically to see if this can bring on abnormal heart rhythms. If this happens, patients are known to be at higher risk in future and can be started on different treatments. Some studies have also used echocardiography, which is ultrasound of the heart, to pick up changes in the structure, shape, and function mainly of the main pumping chamber of the right heart, called the right ventricle. Data are limited, although the test called stress echocardiography is increasingly used in clinical assessment of patients with suspected or known ARVC. Our hypothesis is that changes in RV structure and function will occur in non-definite, early and in definite ARVC patients that may help not only diagnose the condition but also improve prediction of those with the greatest risk of heart failure, abnormal heart rhythms and death. This is a retrospective single-centre data collection of adults aged over 18 years with arrhythmogenic right ventricular cardiomyopathy (ARVC) who have undergone investigations at University Hospitals Birmingham NHS Foundation Trust.
Public benefit statement	Our expectations are that we will identify markers that predict patients who will do better and do less well with their disease, so we can target monitoring and future therapies to improve outcome and develop additional strategies to deal with risk.
Latest approval date	31/10/2024

Safe Data
Dataset(s) name	PATHWAY Research Data Hub: PWY014 dataset

Safe Setting
Access type	Data released via Letter of Authorisation. All researchers have received training in the care, use and protection of personal data, enabling them to comply with their responsibilities under the Data Protection Act.

Safe Outputs
Link	Not yet Published