OUS award for outstanding research article

Oslo University Hospital awards six outstanding research articles twice per year. One of this year's winners was the article "Highly malignant disease in childhood-onset arrhythmogenic right ventricular cardiomyopathy", which was published in the European Heart Journal in August.

Published 7/8/2024
A group of people posing for a photo
The co-authors Marianne Forså, Martin Prøven Bogsrud, Isotta Castrini, Kristina Haugaa and Marit Kristine Smedsrud flanked by research director Erlend Smeland, who was responsible for the award ceremony. Private photo.

The article is the result of a collaborative project between the ProCardio Center for Innovation at the Division of Cardiovascular and Pulmonary Diseases (HLK) and the Department of Pediatric Cardiology. The article was nominated by both sites. First author Marit Kristine Smedsrud received the award on behalf of the co-authors. Professor at the Department of Cardiology and head of ProCardio, Kristina Haugaa, was the study's final author.

The current study was the first in the world to investigate the occurrence of serious events in the heart muscle in children with ARVC, as well as the usefulness of family investigation in early childhood for ARVC-related mutations. The study showed a high incidence of serious cardiac events in children with ARVC. Children who developed ARVC before the age of 12 years had a particularly malignant phenotype. In addition, a full 18% of the children included through family screening fulfilled the criteria for a definitive ARVC diagnosis before the age of 18. The results of the study showed that the prevalence of ARVC in childhood is underestimated and that the children who develop ARVC often have a highly malignant phenotype.

In an editorial in the European Heart Journal, it is advocated that the study in question should be used as a documentation basis for the revision of the current international guidelines for ARVC.

Highly malignant disease in childhood-onset arrhythmogenic right ventricular cardiomyopathy. Smedsrud MK, Chivulescu M, Forså MI, Castrini I, Aabel EW, Rootwelt-Norberg C, Bogsrud MP, Edvardsen T, Hasselberg NE, Früh A, Haugaa KH. Eur Heart J. 2022 Dec 1;43(45):4694-4703. PMID: 36036653

Arrhythmogenic cardiomyopathies in children: seek and you shall find. Kaski JP. Eur Heart J. 2022 Dec 1;43(45):4704-4706. PMID: 36263790 ​