Outcomes After Fontan Procedure in Patients with Cyanotic Congenital Heart Disease Comparing Reduced vs Increased Pulmonary Blood Flow in Univentricular Heart

What this Trial Is About

This research aims to compare clinical outcomes in patients with cyanotic congenital heart disease CHD and univentricular heart physiology who undergo staged surgical palliation culminating in the Fontan procedure. It focuses on differences between those with pulmonary oligemia, meaning reduced pulmonary blood flow, and pulmonary plethora, meaning increased pulmonary blood flow. The study seeks to understand how these pulmonary blood flow conditions affect survival, lung development, and overall health after the Fontan procedure. Participants will receive standard clinical care involving staged surgeries including the Bidirectional Glenn and Fontan procedures. They will be grouped based on their pulmonary blood flow typeeither oligemia or plethoraand followed for about 1.5 years after the Fontan procedure. Assessments will include clinical exams, laboratory tests, imaging, and hemodynamic studies, with no experimental treatments administered. Throughout the study, researchers will monitor mortality and morbidity, pulmonary artery pressures and resistance, exercise capacity through a six-minute walk test, neurocognitive function using standardized tests, and quality of life questionnaires. Biomarker levels and lung tissue samples will also be analyzed during surgeries. Follow-up evaluations will occur up to 12 months post-Fontan to gather comprehensive data for improving care in this patient group.

Fontan Outcomes in Oligemia vs Plethora in Univentricular CHD