Due to copyright issues, an electronic copy of the thesis must be ordered from the faculty. For the faculty to have time to process the order, the order must be received by the faculty at the latest 2 days before the public defence. Orders received later than 2 days before the defence will not be processed. After the public defence, please address any inquiries regarding the thesis to the candidate.
Trial Lecture – time and place
See Trial Lecture.
Adjudication committee
- First opponent: Professor David O. Arnar, University of Iceland
- Second opponent: Professor Charlotte B. Ingul, NTNU - Norwegian University of Science and Technology
- Third member and chair of the evaluation committee: Associate Professor Lars Fjellbirkeland, University of Oslo
Chair of the Defence
Professor II Jonny Hisdal, University of Oslo
Principal Supervisor
Professor Kristina H. Haugaa, University of Oslo
Summary
Arrhythmogenic cardiomyopathy is a genetic cardiac disease associated with high risk of life-threatening arrhythmias and sudden cardiac death. There is high variability between patients in regards to the severity of disease manifestations, with some patients going through life with no or mild symptoms, while others die at young age.
In the thesis Disease manifestations and predictors of arrhythmia in patients with arrhythmogenic cardiomyopathy, Christine Rootwelt-Norberg and co-authors demonstrated that in a Norwegian cohort of arrhythmogenic cardiomyopathy patients, almost half had experienced life-threatening arrhythmia by last follow-up. Risk of presenting with life-threatening arrhythmia as first disease manifestation was reduced after implementation of genetic testing in Norway in 2007, but was still considerable with close to half of families identified due to arrhythmic events in the proband.
Many arrhythmogenic cardiomyopathy patients end up with an implantable cardioverter defibrillator to prevent sudden cardiac death. However, the selection of patients to receive a defibrillator is highly challenging. During 4 years follow-up in patients without previous life-threatening events, precise risk prediction was achieved by applying a risk prediction model including exercise history, T-wave inversions on ECG and cardiac dysfunction by echocardiographic strain imaging.
Previous studies have reported worse outcome in male than in female arrhythmogenic cardiomyopathy patients. Rootwelt-Norberg and co-authors demonstrated more penetrant disease and worse phenotype in males, but this difference was confounded by sex differences in exercise habits. After implementation of exercise restrictions, disease progression was similar between the sexes.
The studies of this thesis improve the understanding of disease manifestations of arrhythmogenic cardiomyopathy and provide new data relevant to risk stratification in clinical practice.
Additional information
Contact the research support staff.