Fontan procedure for tricuspid atresia

Area of Science:

• Pediatric Cardiology
• Congenital Heart Surgery
• Cardiovascular Physiology

Background:

• Tricuspid atresia is a severe congenital heart defect requiring complex surgical palliation.
• Previous surgical approaches like shunts and pulmonary artery banding have limitations.
• The Fontan procedure offers a theoretical advantage in managing single-ventricle physiology.

Purpose of the Study:

• To evaluate the outcomes of the Fontan procedure in patients with tricuspid atresia.
• To assess the operative mortality and long-term functional status following the Fontan procedure.

Main Methods:

• Retrospective analysis of 29 patients who underwent the Fontan procedure for tricuspid atresia.
• Data collected included patient demographics, pre-operative hemodynamics (pulmonary vascular resistance, pulmonary arterial pressure), associated anomalies, and previous surgeries.
• Outcomes assessed included hospital mortality, need for pacemaker implantation, and exercise capacity during follow-up.

Main Results:

• Four hospital deaths (13.8%) occurred, primarily in patients with complicating features.
• The mortality rate decreased to 4.5% in the last 22 consecutive patients.
• No late deaths were observed; 17 out of 19 patients followed for ≥3 months had no or only mild exercise restriction.

Conclusions:

• The Fontan procedure is associated with low operative mortality and good functional outcomes in carefully selected patients with tricuspid atresia.
• This approach demonstrates significant advantages over historical palliative procedures.
• Careful patient selection based on anatomy and hemodynamics is crucial for successful Fontan palliation.