Fontan Patients: Comprehensive Evaluation of Pulmonary Circulation and Ventricular Function
Status:
Completed
Trial end date:
2014-03-01
Target enrollment:
Participant gender:
Summary
In patients with one anatomical or functional ventricular chamber, which encompasses a
spectrum of rare and complex congenital cardiac malformations, a staged surgical approach in
view of an ultimate Fontan operation has become the procedure of choice. Especially in the
earlier era, perioperative mortality was the leading cause of death. However, many patients
have a long and high-quality life, continuously improved by a better understanding of Fontan
hemodynamics and the refinement of the surgical procedures. Nevertheless, the prospect of
eventual failure of the Fontan circulation remains a major concern. More specifically,
evaluation of the pulmonary circulation becomes particularly important as the failing Fontan
circulation has become a common indication for cardiac transplantation. Although essential,
especially in the preoperative setting, a comprehensive evaluation of the pulmonary
circulation remains difficult in this patient population
Our global hypothesis is that the absence of pulsatile pulmonary flow may lead to the
development of pulmonary vascular lesions after the Fontan operation and that - together the
absence of a subpulmonary ventricle for pressure generation - this increasing afterload will
result in systemic ventricular underfilling and will eventually lead to a failing Fontan
circulation.