None listed
Conditions
Brief summary
Systemic Sclerosis (SSc) is an autoimmune disorder in which normal tissue is replaced with scar tissue. Normally, the immune system helps to defend the body against various infections and diseases. In SSc, the immune system stimulates the cells to produce excess collagen (protein). The excess collagen is deposited in skin and other organs (heart muscle, lungs, blood vessels, skeletal muscles and joints) causing hardening and thickening of normal tissues (similar to scar formation). The prevalence of SSc is 443 per million population, and women are more commonly affected. One of the major causes of SSc-related death is right heart (right ventricle, RV) failure, and thus assessment of its function is important in determining survival. The RV is affected through primary heart muscle involvement and/or due to involvement of blood vessels supplying the heart. RV dysfunction is silent until advanced and routine investigations do not detect it at an early stage. Hence, there is a need for a safe and sensitive non-invasive technique for early detection of RV dysfunction. Advanced imaging techniques like cardiac magnetic resonance imaging show potential in detecting early abnormalities in the RV, before symptoms present. We propose to utilize this technique to detect early RV abnormalities in patients with SSc. This will identify those at an increased risk and allow initiation of disease modifying therapy, thereby improving outcomes.
Interventions
All participants will undergo BOLD and Parametric T1/T2 mapping Cardiac Magnetic Resonance (CMR) of their RV and Speckle tracking Echocardiography (Echo).
Both patient population and normal volunteers will have CMR and echo assessments. Echo assessment will apart of the patient populations annual routine follow up with their rheumatologist. CMR assessment will be done within 3 months of this echo.
CMR will be carried out in the presence of radiographer and cardiologist. Echo will be perform
All participants will undergo BOLD and Parametric T1/T2 mapping Cardiac Magnetic Resonance (CMR) of their RV and Speckle tracking Echocardiography (Echo). Both patient population and normal volunteers will have CMR and echo assessments. Echo assessment will apart of the patient populations annual routine follow up with their rheumatologist. CMR assessment will be done within 3 months of this echo. CMR will be carried out in the presence of radiographer and cardiologist. Echo will be performed by sonographer. During CMR scan, participants will be administered with adenosine and gadolinium. Adenosine is a drug that mimics the effect of exercise on your heart. Cardiac images will be taken both at rest and at adenosine stress. There will be no designated time between rest and stress periods however if the patient's conditions requires this, adequate time will be given.
Sponsors
Flinders Medical Centre
Study design
Allocation
Non-randomised trial
Intervention model
Other
Primary purpose
Prevention
Masking
Open (masking not used)
Eligibility
Sex/Gender
All
Age
18 Years to No maximum
Healthy volunteers
Yes
Inclusion criteria
Patient population: -Male and female aged greater than/ equal to 18 years -Confirmed diagnosis of systemic sclerosis -Screen negative for PAH based on Australian Scleroderma Interest Group (ASIG) algorithm or normal mean PAP (8-20 mmHg; assessed by right heart catheterisation). (Screen negative implies NT-proBNP < 210 pg/ml and pulmonary function test demonstrating DLCO > 70% predicted with FVC/DLCO <1.8) -Normal echocardiography. -No history of cardiac disease (myocardial infarction, angina, cardiomyopathy/heart failure, pulmonary thromboembolism and significant valvular heart disease). Normal healthy controls: -Male and female aged greater than/ equal to 18 years -No history of cardiac disease -Normal screening echocardiogram (post consent) which includes assessment of left and right ventricular function, diastolic function and pulmonary artery pressures
Exclusion criteria
-Inability to provide informed consent -Prior documented LV/RV systolic dysfunction. -Presence of significant coronary artery disease (>50% stenosis). -Contraindications of CMR, including: o Extreme claustrophobia o Implantable cardiac devices and other contra-indications to CMR (metal in eyes, intracranial clips) o Inability to lie flat for one hour -Contraindication to gadolinium, including: o Estimated Glomerular Filtration Rate (eGFR) of <45mL/min/1.73m2 -Contraindications to adenosine, including: o second or third degree atrioventricular block o obstructive pulmonary disease o dipyridamole use
Outcome results
None listed