The systemic right ventricle and the Fontan circulation: addressing late complications

Abstract

Thanks to advances in care, more patients with congenital heart disease (CHD) are reaching adulthood (ACHD). However, many face serious long-term complications such as heart failure. This thesis focuses on two complex CHD groups: patients with a systemic right ventricle (sRV) and those with a Fontan circulation. Treating these complications remains challenging, as these groups are often excluded from large clinical trials. For sRV patients, I studied the heart failure drug sacubitril/valsartan, one of the four pillars of standard heart failure treatment. Our findings suggest it is safe and may improve heart function, exercise capacity, and quality of life. I also examined SGLT2 inhibitors—another treatment pillar—which show promise for sRV-related heart failure. An eHealth-supported care pathway was developed for remote monitoring and support. I also investigated the role of valve surgery and chronic pacing. In Fontan patients, I explored long-term complications such as veno-venous collaterals and arrhythmias. Closing these collaterals can improve oxygen levels, while atrial arrhythmias are linked to impaired autonomic function. As the ACHD population grows, effective therapies are crucial. This research provides important insights and a foundation for future studies to improve long-term outcomes and quality of life.