Abdominal Imaging of Children and Young Adults With Fontan Circulation: Pathophysiology and Surveillance

Area of Science:

• Cardiology
• Radiology
• Pediatric Medicine

Background:

• The Fontan procedure enhances survival for children with functional single ventricle.
• This procedure, however, leads to chronically elevated systemic venous pressure.
• Elevated venous pressure results in multisystemic complications affecting various organs.

Purpose of the Study:

• To discuss the pathophysiology of Fontan-associated complications.
• To review imaging modalities used for assessment.
• To outline current surveillance recommendations for these patients.

Main Methods:

• Review of pathophysiology of Fontan circulation.
• Discussion of various imaging techniques (e.g., MRI, CT, Ultrasound).
• Illustration of common and uncommon imaging findings.

Main Results:

• Fontan circulation is linked to cardiac and extracardiac comorbidities.
• Liver and intestines are particularly vulnerable to damage.
• Increased risk of malignancies like hepatocellular carcinoma and neuroendocrine tumors is noted.

Conclusions:

• Improved survival in Fontan patients is accompanied by significant comorbidities.
• Radiologists must be familiar with imaging findings of Fontan-associated liver disease and abdominal complications.
• Expertise in imaging is essential for managing these complex patients in general practice.