Late outcome after the Fontan procedure

Area of Science:

• Cardiology
• Pediatric Cardiac Surgery
• Congenital Heart Disease

Background:

• Limited data exist on long-term outcomes following the Fontan procedure.
• The Fontan procedure is a palliative surgery for complex congenital heart defects.

Purpose of the Study:

• To evaluate the intermediate to late functional status and survival rates of patients who underwent the Fontan procedure.
• To identify potential complications and long-term sequelae in this patient population.

Main Methods:

• Retrospective review of the first 500 patients who underwent the Fontan procedure at Children's Hospital, Boston.
• Follow-up data collection on functional status, survival, and development of complications.

Main Results:

• A 10-year survival probability of 71.4% was observed among 410 contacted patients, with 31 late failures.
• The majority of patients were in functional class I or II, indicating good functional capacity.
• Protein-losing enteropathy was rare; atrial flutter developed in 16% of patients.

Conclusions:

• Patients undergoing the Fontan procedure demonstrate a considerable long-term survival rate with generally good functional status.
• Atrial flutter is a notable complication, and protein-losing enteropathy is infrequent.
• Indefinite, lifelong follow-up is crucial for patients who have had a Fontan procedure to manage potential long-term complications.