Transcatheter closure of a fenestration in intracardiac Fontan circulation

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Interventional Cardiology

Background:

• Tricuspid atresia is a severe congenital heart defect requiring complex surgical palliation.
• The Fontan procedure creates an intra-atrial conduit for single-ventricle physiology but can lead to complications like shunts.
• Right-to-left shunts post-Fontan circulation cause significant desaturation and impact patient quality of life.

Observation:

• A 12-year-old patient with tricuspid atresia experienced significant desaturation due to a right-to-left shunt from intra-atrial conduit dehiscence.
• The shunt developed after initial intracardiac Fontan circulation correction at age five.
• Transcatheter closure of the Fontan fenestration was performed at age eight using a 10 mm Amplatzer device.

Findings:

• The transcatheter closure effectively eliminated the right-to-left shunt, resolving the patient's desaturation.
• The child is now asymptomatic with normal oxygen saturation and no residual shunts.
• Device placement was normal, and the child remains well at age 12.

Implications:

• Percutaneous closure of Fontan fenestrations with Amplatzer devices is a safe and effective therapeutic option.
• This minimally invasive approach offers good clinical results, including normal oxygen saturation and low morbidity.
• The technique is accessible, cost-effective, and technically suitable for most cardiac catheterization laboratories, improving outcomes for complex congenital heart disease patients.