Survival of Double Inlet Left Ventricle Patients Without Fontan Circulation

Patients with univentricular hearts are currently palliated with the Fontan procedures. This results in an unphysiologic circulation with poor long-term survival. On the other hand there is a small, selected subgroup of patients with univentricular hearts of the double inlet left ventricle (DILV) type that survives up to old age without ever having undergone any Fontan procedures. Considering the relatively bleak data on long-term survival of DILV patients palliated with the Fontan procedure, it seems highly relevant to investigate and determine factors that allow a selection of unoperated patients to live to a comparatively high age without apparent major complaints. This might have an impact on how (and if, at all) certain univentricular patients are selected for operation in the future. For some, a different surgical procedure, or even therapeutic nihilism might be a more viable alternative. The investigators hypothesise that favourable intracardiac streaming plays a role in making these patients viable: certain intracardiac anatomical characteristics allow for favourable flow patterns. The primary objective of this study is to describe the intracardiac anatomy and hemodynamics of DILV patients without Fontan circulation using 4D MRI. Furthermore, the functional status and quality of life of these patients will be assessed.