Infected thrombus in a Fontan circulation.

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiovascular Surgery

Background:

• The Fontan operation is a palliative surgical procedure for univentricular heart physiology, established in 1968.
• Long-term outcomes following the Fontan operation are historically poor, with significant morbidity.
• A substantial proportion of patients develop complications within a decade of the procedure.

Purpose of the Study:

• To summarize the natural history and common complications associated with the Fontan operation.
• To highlight the diagnostic modalities used for evaluating Fontan-associated morbidities.
• To emphasize the importance of clinical and imaging follow-up in managing these patients.

Main Methods:

• Review of the natural history and outcomes of patients undergoing the Fontan operation.
• Identification of common complications, including tachyarrhythmias, liver disease, protein-losing enteropathy, heart failure, thrombosis, and infective endocarditis.
• Discussion of diagnostic tools such as echocardiography and magnetic resonance imaging (MRI).

Main Results:

• By 10 years post-Fontan operation, most patients experience at least one complication.
• Complications encompass a wide spectrum, including cardiac, hepatic, gastrointestinal, and thromboembolic events.
• Echocardiography and MRI are primary imaging techniques for complication detection.

Conclusions:

• The Fontan operation, while life-extending, is associated with significant long-term complications.
• Regular clinical assessment and advanced imaging are crucial for early detection and management.
• Ongoing surveillance is essential for improving the quality of life and outcomes for Fontan patients.