Diverse multi-organ histopathologic changes in a failed Fontan patient

Area of Science:

• Cardiology
• Gastroenterology
• Pathology

Background:

• Protein-losing enteropathy (PLE) is a known complication following the Fontan procedure, a palliative surgery for single-ventricle congenital heart defects.
• This report examines a unique case of a patient with PLE 12 years post-Fontan, focusing on multi-organ histopathological findings.

Observation:

• The patient, diagnosed with PLE at 28 months after a Fontan procedure for right isomerism and single ventricle, experienced recurrent intestinal bleeding and renal failure.
• Autopsy revealed intrapulmonary artery thickening consistent with pulmonary hypertension and significant liver cirrhosis, despite minimal elevation in liver enzymes.
• Notably, characteristic dilated lymphatics of PLE were absent in the intestinal mucosa, and renal histological changes were subtle despite clinical renal failure.

Findings:

• Histopathological examination revealed pulmonary hypertension and liver cirrhosis in a patient with a history of Fontan procedure and protein-losing enteropathy.
• The absence of typical PLE histological features, such as dilated lymphatics, and subtle renal changes highlight potential discrepancies between clinical presentation and organ-level pathology.
• Medial and intimal thickening in intrapulmonary arteries suggested pulmonary hypertension, a significant finding in the context of Fontan circulation.

Implications:

• The findings suggest that clinical manifestations in Fontan patients with PLE may not always correlate directly with the extent of histopathological changes in all organs.
• Understanding these discrepancies is crucial for accurate diagnosis, management, and prognostication in long-term survivors of the Fontan procedure.
• Further research is warranted to elucidate the mechanisms behind multi-organ changes and their clinical significance in failed Fontan circulation.