Long-Term Renal Involvement in Association with Fontan Circulation

Area of Science:

• Cardiology
• Nephrology
• Pediatric Medicine

Background:

• Fontan-associated multiorgan dysfunction is a significant clinical concern.
• Understanding the pathophysiology of Fontan nephropathy is crucial for long-term patient management.
• Renal disease progression requires detailed characterization in adult Fontan survivors.

Purpose of the Study:

• To investigate the incidence and clinical associations of nephropathy in long-term Fontan patients.
• To clarify the pathophysiology of Fontan nephropathy through a long-term observational study.
• To identify risk factors and clinical variables associated with renal dysfunction in Fontan survivors.

Main Methods:

• Retrospective review of medical records for 128 consecutive Fontan patients (age 15-37).
• Data collection included demographics, cardiac parameters, liver function (Forns index), and renal function (proteinuria, eGFR).
• Statistical analysis, including multivariable analysis, was used to assess associations between variables.

Main Results:

• Twenty-nine percent of patients exhibited proteinuria or reduced eGFR.
• Proteinuria was associated with older age at Fontan procedure, higher cardiac index, and elevated central venous pressure.
• Reduced eGFR correlated with lower mean renal perfusion pressure; proteinuria linked to increased cardiac index and lower oxygen saturation in severe cases.

Conclusions:

• Approximately 30% of Fontan patients develop proteinuria or reduced eGFR.
• Suboptimal Fontan circulation may contribute to the development of nephropathy.
• Further research is needed to elucidate the mechanisms and optimize management of Fontan nephropathy.