ProCardio Center for Innovation

ProCardio annual report 2022

Nordic multicentre study on arrhythmogenic cardiomyopathy

Arrhythmogenic cardiomyopathy is a genetic disease that causes damage to the cardiac muscle cells, which in turn results in high risk of experiencing life-threatening arrhythmias. Implantation of a cardiac defibrillator (ICD) is an important, but challenging, part of patient management.

In this multicentre Scandinavian research project, patient data from more than 700 patients from Norway, Sweden, Denmark and Finland, have been made available through the “Nordic ARVC registry”. OUS Rikshospitalet is the largest contributor to the registry. Several papers describing disease manifestations, arrhythmic risk and ICD complications in arrhythmogenic cardiomyopathy patients have already been published, and further publications are planned.

Project leader: K.H. Haugaa

Risk prediction in lamin A/C cardiomyopathy

Lamin A/C cardiomyopathy is a disease causing heart failure and cardiac arrhythmias, often at a young age. The most challenging part of patient management is selection of patients to receive implantation of a cardiac defibrillator (ICD), to prevent sudden cardiac death.

We are investigating predictors of life-threatening arrhythmias in patients with lamin A/C cardiomyopathy, by the use of electrocardiogram (ECG) and echocardiography. The aim is to improve the diagnostic criteria for selection of patients to receive an ICD.

Project leader: K.H. Haugaa

Atrial myopathy in lamin A/C cardiomyopathy

Lamin A/C cardiomyopathy is a disease causing heart failure and cardiac arrhythmias. Atrial arrhythmias are often one of the first symptoms of the disease and may result in severe complications such as stroke.

We are investigating the relationship between atrial cardiomyocyte dysfunction and development of atrial arrhythmias, and exploring echocardiographic predictors of future atrial arrhythmias in these patients.

Project leader: K.H. Haugaa

Lamin A/C cardiomyopathy in children

Lamin A/C (LMNA) mutations cause familial dilated cardiomyopathy (DCM) with variable expressivity of symptoms such as early-onset atrioventricular block, ventricular arrhythmia and progressive DCM. Sudden cardiac death due to VA occurs frequently, often before the development of DCM. However, data on children with LMNA mutations are missing, and the prevalence of LMNA cardiomyopathy in childhood and the optimal age for starting genetic and clinical follow-up on relatives are not defined.
We aim to explore the prevalence of cardiac events in LMNA genotype-positive children and to investigate the penetrance of LMNA cardiac phenotype in genotype positive relatives during childhood.

Project leader: Marit Kristine Smedsrud

Ventricular arrhythmias in patients with arrhythmic mitral valve syndrome

Patients with arrhythmic mitral valve syndrome (AMVS) have frequent ventricular arrhythmias that are potentially life threatening and there is no established non-invasive treatment. There is a lack of data on the effect of antiarrhythmic therapy in patients with AMVS, along with prognostics risk markers. 

We aim to improve general knowledge about ventricular arrhythmias in patients with AMVS and to explore possible treatment options for decreasing ventricular arrhythmia burden in these patients.

Project leader: K.H. Haugaa

Main supervisor: Eivind Aabel

PhD student: Cecilie Bugge

Randomized controlled trial on flecainide and metoprolol in arrhythmic mitral valve prolapse

Patients with arrhythmic mitral valve prolapse are at risk of potentially life-threatening arrhythmias, and there is no established drug therapy with evidence from randomized controlled trials. Flecainide, a drug primarily used to suppress benign arrhythmias, has been shown to suppress arrhythmias in other cardiomyopathies, but its effect in patients with arrhythmic mitral valve prolapse has not been studied. 

FLECAPRO (NCT05631730) is a prospective, open-label, blinded-endpoint, randomized, crossover trial. The main objective of FLECAPRO is to assess whether the combination of flecainide and metoprolol can reduce the number of ventricular tachyarrhythmias without imposing worse short-term arrhythmic risk, compared to standard beta blocker therapy. The results from this trial will potentially change clinical guidelines and improve the outcome and quality of life for patients with arrhythmic mitral valve prolapse.

Principal investigator: E.W. Aabel

Exercise in patients with lamin A/C genotype related cardiomyopathy.

Lamin A/C dilated cardiomyopathy is an autosomal dominant inheritable disease with a high lifetime penetrance. There are indications that competitive sports are related to adverse events in patients with lamin A/C genotype, but the evidence is sparse and the effect of recreational exercise is unknown.

We investigated the relationship between lifetime exercise hours, cardiac function, and arrhythmias in patients with lamin A/C genotype.

Project leader: K.H. Haugaa

Late adverse cardiac effects of cancer treatments

Cancer survival has been increasing for decades, but the treatments are often cardiotoxic. We investigate the late adverse effects of cancer drugs and treatments, like radiation treatment, on cardiac morbidity and health.

Current projects include the study of Cisplatin-induced cardiotoxicity in survivors of testicular cancer, and the multi-center study on the cardiotoxicity of modern treatment regimes for Hodgkins lymphoma.

Project leader: S.I. Sarvari

Risk stratification in patients corrected for tetralogy of Fallot: a comprehensive cardiac imaging study

Tetralogy of Fallot is one of the most common congenital heart diseases. The main cause of death in operated patients is sudden cardiac death, which in many cases represents the first presentation of an arrhythmia.

We aim to contribute to the improvement of risk stratification in Tetralogy of Fallot patients, by identifying the subpopulation of patients who are at higher risk for sudden cardiac death, arrhythmias and heart failure.

Project leader: M.E. Estensen

Disease progression in probands and mutation positive family members with arrhythmogenic cardiomyopathy

Arrhythmogenic cardiomyopathy (AC) is an inheritable cardiomyopathy in which probands are generally known to have more severe disease than family members when first evaluated.

We aim to describe disease penetrance in family members at genetic diagnosis, compare disease progression between probands and family members and also to assess the impact of structural disease progression on arrhythmic outcome in AC patients and mutation positive family members.

Project leader: K.H. Haugaa

Cardiac imaging in Brugada syndrome

Brugada syndrome is a genetic primary arrhythmia disorder with a high risk for sudden death. Although previously described as a purely electrical disorder, subtle structural and functional abnormalities appear to be present. We therefore investigate the role of echocardiography as a tool for risk stratification in Brugada syndrome.

Project leader: K.H. Haugaa

Cardiac resynchronization therapy - Acute response parameters

Patients with systolic heart failure and prolonged QRS width on the electrocardiogram are eligible for Cardiac resynchronization therapy (CRT). Clinical response to this treatment is, however, highly variable and one third of the patients do not benefit at all.

The aim of this study was to assess novel electro-mechanical markers of acute hemodynamic response under CRT implantation to increase CRT response prediction.

Project leader: E. Kongsgård

Risk of life-threatening ventricular arrhythmias in high-performance athletes

Endurance athletes can tolerate extreme doses of cardiac stress, and are considered among the most healthy individuals in our society. Therefore, it is tragically counter intuitive when an athlete suffers unexpected sudden cardiac death. Advanced cardiac imaging and electrocardiography may identify individuals at risk of such events.

The aim in this project is to describe the phenotypes of athletes with complex ventricular arrhythmias, compare them to healthy athletes, and to assess the candidate predictors in a separate sample to gain knowledge of risk stratification of high-performance athletes.

Project leader: K.H. Haugaa

Prevalence of systolic and diastolic dysfunction in patients with acute myocardial infarction and implications for prognosis

Diastolic dysfunction is a result of reduced relaxation of the left ventricle, often combined with increased stiffness and/or reduced restoring forces. Patients with signs of diastolic dysfunction have an elevated risk of major adverse cardiovascular events after acute myocardial infarction (AMI).

We will evaluate the presence of systolic dysfunction and diastolic dysfunction after AMI, and investigate whether this has prognostic implications.

Project leader: T. Edvardsen

Effect of pregnancy on disease manifestation in patients with Lamin A/C cardiomyopathy

Lamin AC (LMNA) cardiomyopathy is a cardiac genetic disease characterized by ventricular dilatation with reduced cardiac function and early onset of malignant ventricular arrhythmias in young age.

We investigate the relationship between pregnancy and cardiac morphology, function and occurrence of ventricular arrhythmias in LMNA patients.

Project leader: K.H. Haugaa

Electromechanical Presages of Sudden Cardiac Death in the Young: integrating imaging, modelling and genetics for patient stratification

In the young (<40 years), sudden cardiac death (SCD) is often the first manifestation of a genetic cardiac disease causing lethal arrhythmias.

The EMPATHY project aims to unravel the complex pro-arrhythmic electro-mechanical interactions in the apparently healthy yet vulnerable hosts of genetic cardiac diseases by combining three different but highly complementary scientific fields, being clinical cardiac imaging (OSLO), genetics and cellular electrophysiology (MILAN), and multi-scale computational modelling (MAASTRICHT).

Project leader: K.H. Haugaa

Improving echocardiographic risk prediction in arrhythmogenic cardiomyopathy

Arrhythmogenic cardiomyopathy is a genetic disease, affecting the heart and causing potentially lethal arrhythmias. In order to improve echocardiographic risk prediction in patients with this relatively rare disease, we are combining data and expertise from two of the largest patient cohorts in Europe, the Oslo cohort and the Utrecht cohort from the Netherlands.

Project leader: K.H.Haugaa

Development of new ultrasound technologies and methods

Pål Brekke's research is focused on the development of new ultrasound technologies and methods, with focus on high frame rate ultrasound and machine learning/artificial intelligence applications. He is also employed part time by the Norwegian Research Council funded BigMed project, in which big data analytics are being employed to improve sudden cardiac death prediction and prevention.

Brekke is supervising a medical student researcher, Magnus Rogstadkjernet in his project, which will employ machine learning tools in automating aspects of echo analysis.

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