Plain English Summary
Background and study aims
Arrhythmogenic cardiomyopathy (AC) is an inherited disease that can cause sudden cardiac death (SCD). Cardiac involvement is characterised by heart muscle cell loss. This leads to an enlarged heart, predominantly in the right ventricle (RV), which can then progress to heart failure. Patchy cell loss in the heart wall can cause electrical instability, which manifests as ventricular arrhythmia, which increases the risk of sudden death. The treatment is based on anti-arrhythmic medication and insertion of an implantable cardioverter defibrillator (ICD) , which protects from SCD in case of ventricular arrhythmia. The treatment for heart failure depends on the progress of the disease. In some cases a heart transplant is needed.
Cardiac MRI, the gold standard for RV analysis is not suitable for patients with ICD and echocardiography is often inadequate for this task. Regardless, disease progression still needs to be analysed.
Recent developments in CT imaging and image analysis have changed the options. CT scanning of the heart can now be performed with very low irradiation to the patient (less than 3 mSv). Our research team has developed a new model to measure heart volume and wall deformation using cardiac CT. The aim of this study is to assess ventricular volume and heart function using cardiac CT in AC patients with ICD to follow-up the progress of the disease.
Who can participate?
Adult patients from the tertiary care centre in Linköping University Hospital, Sweden, with a definite diagnosis of AC and implanted ICD.
What does the study involve?
Participants will undergo an examination of their heart using a CT scan, along with additional standard clinical follow-up echocardiography. For patients older than 60, blood samples will be taken to check kidney function.
What are the possible benefits and risks of participating?
The benefits are an increased ability to detect disease progression more accurately, in order to optimise the treatment to prevent the development of heart failure.
During the CT scan examination, the patient receives intravenous contrast. This could harm kidney function, particularly if an individual already has marginal kidney disease. A blood test is done prior to CT scan to safeguard. Any time a venipuncture is performed, there is a risk of a haematoma.
Where is the study run from?
University Hospital of Linköping (Sweden)
When is the study starting and how long is it expected to run for?
December 2017 to December 2019]
Who is funding the study?
Who is the main contact?
Meriam Åström Aneq
ARVC och plötslig hjärtdöd
Evaluation of ventricular function in patients with arrhythmogenic cardiomyopathy using multimodality imaging
Arrhythmogenic cardiomyopathy (AC) is an hereditary heart disease characterised by myocardial cell loss and fibrosis resulting in electrophysiological abnormalities and potentially fatal arrhythmias, as well as contractile dysfunction of the right (RV) and left ventricles (LV). The treatment of AC is based on antiarrythmic therapy and the insertion of an implantable cardioverter defibrillator (ICD), which means that the assessment of the heart by cardiac magnetic resonance (CMR) not suitable. We use a new software on cardiac CT to track and quantify global and segmental changes in the ventricular function in AC patients with an ICD. We hypothesise that this new software on cardiac CT could help following the progress of disease to adjust the treatment.
Regional Ethical board in Linköping (EPN), 21/03/2018, 2018/35-31
Observational prospective single-centre case series
Primary study design
Secondary study design
Patient information sheet
Not available in web format, please use contact details to request a participant information sheet
Patients fulfilling definite Task Force Criteria for AC carrying an ICD undergo cardiac CT for heart function analysis. The study examination includes CT-scanning of the heart. A radiology technician performs the CT scan. During the test, the patient is lying on a table that is moved through the opening of the CT scanner. The examination takes 8-10 minutes. For patients older than 60 years old, renal function is checked via a blood sample to safeguard the use of iodine contrast.
Primary outcome measure
Assessment of the right and left ventricular volume and function, assessed using time-resolved CT scanning of the heart at the time of the scan
Secondary outcome measures
Software ability to detect regional, focal ventricular abnormalities in AC patients with ICD, assessed using a software that detects myocardial “pixels” and calculates the changes during the heart cycle at the time of the scan
Overall trial start date
Overall trial end date
Reason abandoned (if study stopped)
Participant inclusion criteria
1. Referred for diagnostic cardiac CT
2. Fulfil definite Task Force Criteria for arrhythmogenic cardiomyopathy
3. Implantable cardioverter defibrillator
4. Aged 18 years or older
Target number of participants
All patients at Linköping hospital with definite AC diagnosis and an ICD are included.
Participant exclusion criteria
1. Renal insufficiency
2. Pregnant women
Recruitment start date
Recruitment end date
Countries of recruitment
Trial participating centre
University hospital of Linköping
Funding Body Type
Funding Body Subtype
Results and Publications
Publication and dissemination plan
Planned publication in a high-impact peer-reviewed journal
IPD sharing statement:
The datasets generated during and/or analysed during the current study will be available upon request from Dr M. Åström Aneq. firstname.lastname@example.org. Measurements of heart volume and wall strain will be available by the end of the recruitment of patients and analysis of data, planned on 31/05/2019. Patients sign a consent that the CT measurements will be used for publication. All measurements are anonymised.
Intention to publish date
Participant level data
Available on request
Basic results (scientific)