Outcomes after contemporary Fontan operation

Aims and outline of this thesis

The aim of this thesis was to study medium to long term outcome in a large contemporary cohort of Fontan patients. These are patients that have been operated upon according to a modern technique (either ILT or ECC TCPC) in a staged manner. Furthermore, completion of the Fontan-circulation was performed before the age of 7. Special emphasis of this thesis will be the comparison of the ILT and ECC techniques.

Main objectives were:

• To compare and assess risk factors for mortality and morbidity for ECC and ILT patients

• To study ventricular function and reserve and identify predictors for impaired function using echocardiography as well as CMR, including dobutamine-stress CMR.

• To assess exercise capacity and identify predictors for deterioration of exercise capacity.

• To assess rhythm status and the incidence of arrhythmias.

• To determine the health related quality of life in modern Fontan patients and identify predictors for lower quality of life.

• To study flow dynamics and power loss inside the TCPC pathway.

• To study shear stress and growth of the pulmonary arteries after Fontan completion.