Pediatric and Congenital Heart Disease Session 2: Frontiers in Single Ventricle Heart Disease (2020)-Extracardiac Imaging in the Failing Fontan

Extracardiac Imaging in the Failing Fontan

Video Transcription

Video Summary

The talk reviewed extracardiac complications of failing Fontan circulation: plastic bronchitis, protein-losing enteropathy (PLE), and liver disease. Plastic bronchitis is caused by thick bronchial casts and is linked to abnormal retrograde pulmonary lymphatic flow; CT and MRI can show lymphatic congestion and guide catheter embolization. PLE results from protein loss into the intestines, likely due to hepatoduodenal lymphatic collaterals; conventional imaging is nonspecific, but lymphatic MRI can identify leakage and potential interventions. Fontan liver disease is nearly universal, with fibrosis, congestion, nodules, and late cirrhosis; imaging biomarkers like liver stiffness and flow measurements may help assess severity and prognosis.

Keywords

Fontan circulation

plastic bronchitis

protein-losing enteropathy

Fontan liver disease

lymphatic imaging