The failing Fontan: what's NEXT...?

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiac Surgery

Background:

• The Fontan procedure is a palliative surgery for complex single ventricle congenital heart defects.
• Survivors face risks including ventricular dysfunction, plastic bronchitis, protein-losing enteropathy, and late cardiac failure.
• Late Fontan failure presents a significant clinical challenge, with cardiac transplantation as the only definitive treatment.

Observation:

• This report details an adolescent female with severe late cardiac failure post-Fontan conversion.
• The patient initially required extracorporeal life support (ECLS) for resuscitation.
• The patient was subsequently transitioned to a Berlin Heart EXCOR® ventricular assist device (VAD).

Findings:

• Successful bridging to cardiac transplantation was achieved using mechanical circulatory support.
• The combination of ECLS and VAD provided crucial hemodynamic support.
• The patient ultimately underwent successful heart transplantation.

Implications:

• Mechanical circulatory support offers a viable short-term solution for patients with late Fontan failure awaiting transplantation.
• This case highlights the importance of advanced support strategies in managing complex pediatric cardiac conditions.
• Successful transplantation offers a durable option for end-stage Fontan-associated cardiac dysfunction.