Peder Sörensson

Background: The population of adult congenital heart disease (ACHD), defined as patients, above the age of 16 years, is rapidly growing. The most complex disease is the Fontan circulation in which the venous blood passively flows into the pulmonary circulation and an uni-ventricle heart (UVH) physiology drives the systemic circulation. Surgical therapy is mandatory for survival. Long term follow-up reveals a high mortality and morbidity driven by late complications such as arrhythmias, heart failure and thrombotic events. There are approximately 500 patients with TCPC in Sweden of whom 260 are above the age of 16. A limiting factor for this group of patients is the lack of solid epidemiological data, precise quality of life (QoL) estimations and accurate non-invasive diagnostic methods. Most of contemporary treatment recommendations are based on consensus opinions/documents and small studies from local, or national registries. Currently only few studies have been performed in the adult population and long-term follow-up are scarce. Improved knowledge is needed in all these areas to facilitate clinical decisions regarding treatment, monitoring and follow-up. Aims: This proposal seeks to answer following questions: What is the long-term survival risk in patients with UVH compared with controls? To determine incidence and prevalence regarding thromboembolic and bleeding events? Can QoL (including cardiac fatigue, depression, physical activity) be improved by psychological cognitive behavior therapy (CBT) intervention? Can early detection of deterioration in cardiac function and microvascular dysfunction identify patients before the symptoms progresses? Workplan: We will conduct a register-based retrospective study using the Swedish national patient registers to identify patients with UVH and estimate all-cause mortality compared to matched controls without congenital heart disease. Study two is an open, randomized clinical quality-of-life (QoL) trial to either internet-delivered CBT for 6 weeks or control group. The last study is explorative and will provide improved pathophysiological understanding in order to early detect cardiac disease using magnetic resonance imaging. Significance: This project seeks to improve the epidemiology knowledge, psychological well-being and the pathophysiological understanding in order to improve the long-term prognosis and QoL in a young adult patient population.

Impaired cardiac function often results in heart failure which represents a major burden of morbidity and mortality. Cardiac magnetic resonance imaging (MRI) is considered to be the reference method for assessment of cardiac function. However, the relatively long scan times makes MRI sensitive to motion. Most current cardiac MRI techniques require multiple breath holds, which many patients with heart failure are unable to perform., resulting in non-diagnostic image quality. The aim of this project is to develop a novel framework for adaptive image acquisition, where the pulse sequence is controlled by online motion estimators. This will enable acquisition that is robust to respiratory motion and arrhythmia, as well as fetal motion during examination of fetal hearts. The methods will be evaluated both in healthy volunteers, heart failure patients, and in pregnant women. The pulse sequence and corresponding image reconstruction will be implemented directly on the clinical scanner platform, making it immediately available for clinical use. The research will take place immersed in a clinical environment at the Karolinska University Hospital, with full access to dedicated cardiac MRI scanners and relevant patient populations. The proposed approach is highly original, fills an important unmet clinical need in medicine, and involves novel approaches that push the technical limits of image acquisition and reconstruction beyond state-of-the-art.